Cleft Maxillary Reconstruction 3031246357, 9783031246357

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Cleft Maxillary Reconstruction Pat Ricalde Editor

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Cleft Maxillary Reconstruction

Pat Ricalde Editor

Cleft Maxillary Reconstruction

Editor Pat Ricalde Craniomaxillofacial Surgery Florida Craniofacial Institute Tampa, FL, USA

ISBN 978-3-031-24635-7    ISBN 978-3-031-24636-4 (eBook) https://doi.org/10.1007/978-3-031-24636-4 © Springer Nature Switzerland AG 2023 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Foreword

Dr. Ricalde has assembled a masterful contribution to cleft care and a complement to the advances of craniomaxillofacial surgery. Enlisting the support of eleven other skillful contributors who are well-seasoned cleft care providers, she has beautifully presented the importance of skeletal construction in dealing with the most horrific human malformation, cleft lip, maxilla, and palate. Unlike many contemporary textbooks on cleft surgery which focus on soft tissue outcomes, the author and contributors come from a background of understanding the importance of function, oral and nasal health, and its ultimate role in facial esthetics. Functional and esthetic rehabilitation is emphasized throughout the book. MacIntosh’s contribution is the most detailed chronicle on the history of repair of the cleft maxilla. Told by a detail-oriented, multi-lingual surgeon, who investigated the topic thoroughly and has a legacy as a skilled maxillofacial surgeon and historian, the chapter and references are priceless. The chapter by Yatabe and Kelly is a succinct summary of the orthodontist’s perspective from birth to complete maturation of the cleft patient including infant orthopedics, pre- and post-surgical orthodontic management during the perioperative bone graft phase, the preparation for maxillary advancement, etc. The considerations of dental development and its impact on the timing of surgery are clearly explained and emphasize the importance of an “individual” rather than “everybody the same” approach. Another important point made in this chapter is the consideration of “burn out” and “burden of care” which many of these patients and their families experience. The chapter by Kinard and Posnick on Outcome of Construction on the Cleft Maxilla reviews the literature on the subject. Importantly, it points to the major limitations of assessing the topic—no standard way of measuring the outcomes. Dr. Ricalde’s former mentor, Dr. Jeffrey Posnick, has contributed a thorough and succinct summary of his approach to managing the cleft maxilla at the time of orthognathic surgery. The remaining chapters are filled with wonderful information on dealing with the unilateral and bilateral cleft maxilla and palate. Bone donor sources and bone substitutes are thoroughly reviewed by Dr. Caccamese while Drs. Drew and Edwards discuss unusual circumstances including a variety of soft tissue flaps to cover cleft defects. Dr. Ricalde’s contributions include four chapters, two of which she enlisted the support of former fellows, Dr. Wilson and Dr. Ahson, to discuss the anatomy of unilateral and bilateral cleft defects.

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Foreword

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The other contributions are her techniques for bone grafting the unilateral and bilateral cleft maxilla. Finally, Dr. Padwa discusses the surgeon’s role in establishing the final occlusion, whether it be by prosthetic management, surgical closure, orthodontic closure, etc. Overall, this production is a “must” for any surgeon involved with skeletal construction of the cleft maxilla and palate. Those fortunate enough to read this text will benefit and learn. Timothy A. Turvey Professor of Craniofacial and Surgical Services University of North Carolina Chapel Hill, USA University of North Carolina Hospitals Chapel Hill, USA

Contents

1 Alveolar  Cleft Grafting: Origins, Advances, Prospects����������������   1 Robert Bruce MacIntosh 2 Clinical and Diagnostic Anatomy ��������������������������������������������������  25 David Wilson and Pat Ricalde 3 Clinical  and Diagnostic Findings During Mixed Dentition����������  41 Imran Ahson and Pat Ricalde 4 Orthodontic  Considerations for Patients with Cleft Lip and Palate ������������������������������������������������������������������������  59 Marilia Yatabe Ioshida and Katherine Kelly 5 Donor Site Options��������������������������������������������������������������������������  77 John F. Caccamese 6 Surgical  Repair of Maxillary Unilateral Cleft Defect������������������  89 Pat Ricalde 7 Surgical  Repair of Maxillary Bilateral Cleft Defect �������������������� 105 Pat Ricalde 8 Special Situations ���������������������������������������������������������������������������� 113 Stephanie J. Drew and Sean P. Edwards 9 Modified  Orthognathic Surgery to Manage the Residual Alveolar Cleft(s) �������������������������������������������������������� 127 Jeffrey C. Posnick 10 Establishing  the Final Occlusion���������������������������������������������������� 155 Bonnie Padwa 11 Patient  Outcomes for Maxillary Cleft Management: Literature Review and Guiding Principles������������������������������������ 167 Brian Kinard and Jeffrey C. Posnick

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Alveolar Cleft Grafting: Origins, Advances, Prospects Robert Bruce MacIntosh

1.1 Introduction Rehabilitation of the alveolar cleft is very often the unwanted child in the family of cleft reparative efforts. There is no glamour in its successful result, no dramatically evident improvement in speech (in most cases), no liberating sense of satisfaction for either patient or family, and, particularly when done as a repeated procedure in a scarred environment, can be difficult to accomplish surgically. Even its existence avoids recognition; patients are readily recognized as suffering from “cleft lip and palate” imperfections, but rarely from “cleft lip, alveolus, and palate” even though its rehabilitation generally calls for independent maneuvers. It frequently remains inadequately addressed after even repeated procedures for lip and palate rehabilitation. Even in the instance of fully satisfactory lip reconstruction and palatal cleft obliteration from bicuspid through uvula, the residual tract from mouth to nose, antrum, and throat along the course of the alveolar cleft has significant functional and esthetic consequences. Accumulation of bulk food debris—despite even aggressive hygienic measures—contamination of the airway with oral flora, nuisance leakage of antral/nasal contents into the mouth, lamentable disruption of R. B. MacIntosh (*) Department of Oral and Maxillofacial Surgery, University of Detroit Mercy School of Dentistry, Detroit, MI, USA e-mail: [email protected]

the alveolar arches and dental occlusion, absent or non-functional canine or incisor teeth, compromise to lip and lateral nasal esthetics, and, in some cases, speech, all cry for resolution of the residual alveolar defect. The Western literature suggests that initial attempts at palatal cleft closure, probably including the alveolus, were limited to soft tissue maneuvers, though as early as the first decades of the nineteenth century. A secondary reference cites the first of such procedures being undertaken in Germany in 1824; incorporation of bone into the defect awaited isolated, variably successful, efforts 80–90 years later. Special recognition of the alveolar cleft per se, however, and emphasis on its bony reconstruction have found light in the literature only over the past 60  years, with increased awareness and purpose in establishing normal bulk, contour, and growth to the cleft alveolus. Over that time, literally hundreds of contributions on the topic have flooded the literature, most of them enlarging our knowledge base but many confounding our understanding of what is scientifically sound and clinically proven in addressing the challenge (Fig. 1.1). Chief among the obstacles in differentiating well-founded, repeatable, pertinent knowledge from that less accurate or useful are variations in patient age at operation, type of soft tissue bed, surgical technique, donor site, radiographic evaluation, role of orthodontic care, and accuracy of reported data. These variables have caused Witsenberg [1] to

© Springer Nature Switzerland AG 2023 P. Ricalde (ed.), Cleft Maxillary Reconstruction, https://doi.org/10.1007/978-3-031-24636-4_1

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early eradication of the defect and the problems encountered with primary osteoplasty. Late secondary osteoplasty allows time for complementary orthodontic integration, better ensures retention of permanent canine and lateral incisor teeth, and, in most reports, offers less impediment to midfacial development. By the 1970s, most cleft surgery centers had recognized the advantages of grafting bone into the alveolar defect. By general consensus, these were deemed to be:

Fig. 1.1  Fundamentals of alveolar cleft grafting: Nasal closure with elevated palatal or septal flaps, occlusion of intra-osseous defect with grafted bone, labial closure with any of a myriad of gingival, sulcular, or labial flaps. (With permission, from Coots BK: Alveolar bone grafting: past, present, and new horizons. Semin Plast Surg, 2012; 26:178–183; originally, from Craven C, et  al., Ensuring success in alveolar bone grafting: 3-dimensional approach. J Cranio-Fac Surgery 2007; 18:855–859)

concede that gathering cohesive information on an international basis is essentially impossible. One of the fundaments in alveolar cleft grafting is recognition of the terminology relating to time of surgery. Primary osteoplasty generally denotes bone grafting in infancy, before any tooth eruption, and often in combination with primary lip closure; early secondary osteoplasty, grafting done after eruption of the deciduous dentition, often into a soft tissue bed prepared at earlier lip or soft tissue alveolus closure; late secondary osteoplasty, grafting done after eruption of the anterior and most posterior permanent teeth. (Variations in definitions are discussed below.) The literature describes an early enthusiasm for primary osteoplasty, reflecting a desire to establish normal maxillary anatomy as early as possible in the developing infant. Those hopes became frustrated within decades, as too-frequent graft resorption, failure to grow, disruption of the dentition, and, most significantly, the inhibition of midfacial growth became manifest. Early secondary osteoplasty was promoted as a compromise between aspirations for

Development of continuity and therewith stability of the maxillary arch Resistance to lip scar contraction of the alveolar arch Enhancement of orthopedic or later orthodontic care Provision of bone into which the permanent canine and lateral incisor could erupt Improvement of esthetics at the lateral nasal rim and ala Normalization, and even augmentation, of maxillary growth Most surgical groups had also begun to understand the importance of coordinated orthodontic management in cleft patients. A factor relative to data interpretation is the questionable value of reports from multiple surgeons treating too few patients, a reality that periodically encourages a call for centralization of all cleft care, not only in terms of alveolar reconstruction. Encouragingly, however, increasing recognition of the significance of the alveolar cleft and the biologic and esthetic challenges it poses have become better understood in recent decades. Debates over surgical timing, types of bone tissue, selection of donor sites, bone metabolism and repair, sophisticated radiographic tracking, and precision in orthodontic care have brought new discipline to the grafting of the alveolar cleft patient. All these factors, supported by international discussion, are helping to elevate alveolar bone grafting from a second level of attention to a prominence comparable to that of the repair of other cleft entities.

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

1.2 Therapeutic Maneuvers Accepting definitions of primary grafting and secondary grafting is essential in designing and communicating surgical technique. Several interpretations arose early and more have been contributed over the past five decades [2–13]. Originally, primary osteoplasty seems to have defined bone grafting carried out at the time of initial cleft surgery of the lip, often in combination with whole palatal reconstruction; by other definition, primary could refer to the first alveolar osteoplasty regardless of what other cleft surgery had gone before. In still another connotation, primary would refer only to surgery done at the time of the primary dentition, and any surgery in an older patient would become automatically secondary. Koberg [2] in 1973 suggested that primary osteoplasty referred to bone grafting done in infancy concomitantly with primary closure of the lip and alveolus, early secondary osteoplasty to bone grafting carried out during the deciduous dentition years, and late secondary osteoplasty to grafting done after the eruption of the permanent teeth. Other authors have referred to this latter stage as tertiary osteoplasty. Epstein and Davis [4] related alveolar bone grafting to palatal repair, making early osteoplasty any surgery prior to palate repair, immediate osteoplasty subsequent to palatal repair, and delayed osteoplasty any surgery done following permanent tooth eruption. Van der Maij and Baart [5] determined that “late secondary” osteoplasty would relate to any patient over 12 years old, and Jeyaraj and Sahoo [6] added “very late secondary” to relate to patients 16 years of age or older. This confusion in basic definitions is one of the elements making evaluation of and research on alveolar cleft surgery so difficult. Currently, a reference to help interpret the literature and offer a baseline for new contributions might read: Primary alveolar osteoplasty: grafting done at the time of first closure of the lip and soft tissue alveolus, regardless of patient age; Early secondary alveolar osteoplasty: grafting done at any time subsequent to primary lip and soft tissue alveolar closure, until the anticipated

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time of eruption of the lateral incisor or canine teeth; Late secondary alveolar osteoplasty: Grafting done at any time after the eruption of the lateral incisor or cuspid teeth, regardless of the patient’s age. Another issue of consequence is the role of periosteal grafts. This has been a major topic since Skoog’s breakthrough information in 1965 [14]. Skoog at that time described his experience with eight patients who underwent soft tissue closure of their alveolar clefts concomitantly with their primary cheiloplasties at ages 3–6 months. The essence of the alveolar cleft surgery was the periosteum-to-periosteum apposition of the palatal/vomer and labial mucoperiosteal flaps at wound closure, without an interposed bone graft. All wounds gave evidence of new bone formation across the voids, and gave rise to the term “boneless bone grafting” (Fig. 1.2). This seeming phenomenon evoked enthusiasm for the new technique of gingivo-periosteoplasty which has continued to the present day. Robinson and Wood in 1969 [15] also noted in their series of alveolar cleft closures that several patients developed bone at the sites into which no bone had been grafted. Three years later, Riitsila [16] described his use of free tibial periosteal grafts in the closure of alveolar clefts concomitant with primary cheiloplasty in 11 8–10-week-old infants; he observed callus formation at 2 weeks postoperatively, and bone at the alveolar sites within 6  weeks. In 2008, Cutting and Grayson [17] and Matic and Power [18] reported their 20-year histories with employment of gingivo-­ periosteoplasty. Both groups had employed the Latham appliance (see below) with GPP in their study groups; the Cutting group reflected more satisfaction with the technique than did the Matic group. In that same year, Sato and Grayson [19], in a radiographic evaluation, reported better bone formation with gingivo-periosteoplasty in conjunction with bone grafting than with bone grafting alone. In recent years, the surgical community in general seems to have adopted a generally ambivalent stance in regard to gingivo-periosteoplasty. At least moderate support has come from several

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Fig. 1.2 Skoog’s sketch of periosteum-to-periosteum grafting: Obliteration of alveolar cleft by rotation of anteriorly based mucoperiosteal flap off lateral maxilla onto periosteal surfaces of palatal muco-periosteal flaps rotated

to close the nasal defect; no bone placed into defect. (With permission, from Skoog T: The use of periosteal flaps in the repair of clefts of the primary palate. Cleft Palate Journal, 1965; 2:332–339)

authors [20–24]. Berkowitz [25, 26], however, in light of a 40-year experience, has expressed his dissatisfaction with the procedure. Further, in considerations of lip and nasal esthetics, radiographic site evaluation, clinical bulk of bone, and maxillary growth, other authors have recorded lack of support for the technique [23, 24, 27–29]. Hopper and Al-Mufarrej [30] authored an updated overall view of gingivo-periosteoplasty in 2014. In the early 1980s, interest in gingivo-­ periosteoplasty was enhanced by the introduction of a novel orthopedic device for use in cleft patients. The Latham appliance, anchored in the bone of the infant palate with an arrangement of

screw-driven pressure arms and traction elastics, allowed manipulation of the cleft alveolar elements into more natural positions. The basic idea in unilateral clefts was to move the weak segment forward into closer apposition with the pre-­ maxilla, and in bilateral cases to widen the posterior alveolar elements to accommodate a retracted pre-maxilla. The presumption was that decreasing the widths of the alveolar clefts would simplify soft tissue closure of the residual defects, the execution of gingivo-periosteoplasty, and the insertion of smaller grafts if desired. This was a premise, however, in which many surgeons have not concurred. Smaller is not necessarily better,

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

some argue, and narrowing the cleft makes manipulation of the soft tissues more difficult, potentially compromising the chances for a thorough gingivo-periosteoplasty. Millard [31], however, in 1999 reported his 15-year experience with the Latham appliance in early infancy, followed by gingivo-­ periosteoplasty and lip adhesion at 3–4  months and definitive lip repair 3–5  months later. He compared the results of this sequence with his former routine of lip adhesion alone at 3 months and definitive lip closure at 6–8  months, and found that only 3% of the Latham group required subsequent alveolar cleft grafting as opposed to 66% treated with his earlier sequence. Cutting and Grayson [17], strong proponents of gingivoperiosteoplasty, preferred to narrow the cleft with the Latham appliance to no more than 1 mm, and reported excellent bone proliferation and bony union. These authors also noted retrusion in midface growth, but attributed that not to the gingivo-periosteoplasty, but rather to the detrimental effect of the Latham appliance. At that same time, Matic and Power [18] found much less significant bone development than Cutting and Grayson, and also determined a deficiency in maxillary growth cephalomerically. These authors, too, attributed this as much to the Latham appliance as to the gingivo-­ periosteoplasty. Conclusions in these two studies, however, suffer somewhat from a confounding of variables. In any event, the Latham appliance has not been widely accepted internationally, and finds fewer proponents of its utility in recent literature. Berkowitz [25, 26] has offered experienced expansive commentaries, and is not supportive of its use. A newer approach, naso-alveolar molding, has gained support as an alternative to achieving certain of the gains to which the Latham appliance was dedicated. Naso-alveolar molding entails insertion of a stabilized maxillary acrylic tray into the palate of the newborn which, through weekly or bi-weekly adjustments, brings the alveolar segments together, lessening cleft width. Rounded stents of the device project into the cleft-side nostril or nostrils, and the distorted ala or alae are drawn around the projections to provide normal contour to the immature malleable

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lateral crura of the lower nasal cartilages. The anticipated effects are lessening of the width of the cleft to be subsequently bone grafted, non-­ surgical lengthening of the columella, lessening of the tension on the lip and sulcus soft tissues and thus less scarring at the time of subsequent lip repair, and more normal ala contouring. After its introduction in the 1980s [32], the procedure found strong support in some quarters. Grayson [33] described his positive experiences with the technique in several contributions, as have others [34, 35]. Other authors have been less enthusiastic with its long-term esthetic stability, and have recognized the need for supplemental rhinoplasty [36]. Broder et  al. [35] suggested multicenter evaluation of the procedure to produce more “robust” scientific evaluation. The evaluations of NAM have been chiefly esthetic in regard to columella development and nasal contours, with little discussion in regard to its relationship with alveolar bone grafting. NAM has become, however, more commonly employed in recent years, and such considerations may be yet forthcoming. Though Dieffenbach [37] in 1826 and Krimer [38] in 1828 recorded attempts at soft tissue closure of the palate, Schmid [39] credits Wutzer in 1834 with an attempt to build a hard tissue bridge across the palate by linearly fracturing the palatal shelves and drawing them toward the midline (original reference currently unrecoverable). Von Eiselsberg [40] writing in 1901 described direct pedicle grafting of the three little finger phalanges to a cleft of the palate and alveolus in a 19-year old girl, to obliterate the defect and give support to the columella. The first stage entailed surgical retrusion of the pre-maxilla to lessen the size of the defect, and development of the palatal/septal bed; the second stage, 10 days later, included flaying of the volar surface of the left little finger to expose the bone, removal of the fingernail, and apposition of the finger skin flaps to the prepared flaps of the cleft defect with secure suturing. The patient’s hand and elbow were supported in a fixed position with a preoperatively prepared plaster splint. Three weeks later, the finger was amputated at the metacarpal joint. Von Eiselsberg reported that a splinter of bone sequestrated, but that the wound otherwise granulated and healed

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uneventfully (Fig.  1.3). Koberg [2] later termed this procedure “macabre”. Drachter [41] in his expansive 1914 treatise on clefts credits Helbing with closing the palatal cleft by simply applying pressure to the lateral maxilla after first making a horizontal lateral wall osteotomy on the cleft side above the level of the molars, forcing the hemi-maxillary segment toward the midline, and suturing the elevated soft tissues across the palate. Drachter’s inspiration for repairing palates came as much from his interest in pathology-induced perforations as from cleft disharmonies. He also described defect closure with a tibial bone/periosteal graft, which he termed a “partial success”. He emphasized the importance of preparing a good wound base, and described nasal closure with turbinate and vomer flaps. Drachter also ridiculed von Eiselsberg: “….one could just as soon use the big toe for that surgery”. It is probably appropriate to credit Schmid [42–43] with the first sole focus on alveolar cleft bone grafting. In his earliest writing, he alluded to the use of intraoral bone grafts for reconstructive surgery in general even in the pre-antibiotic era in the latter years of World War II.  In 1953, he discussed the use of iliac crest grafts for primary alveolar cleft reconstruction in a 17-year old female with a bilateral deformity. In his 1964 report [44], he reported

Fig. 1.3  von Eiselsberg’s sketch of direct graft of little finger phalanges to cleft of palate and alveolus, at 3-weeks post-operatively (see text). (From: von Eiselsberg F: Zur Technik der Uranoplastik. Archiv Klin Chir, Bd 64, Heft 3, pages 509–529, 1901

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having treated his first cleft patient with alveolar bone grafts in June of 1951. His experience by the early 1960s had shown him that reflection of the mucoperiosteum at the alveolar cleft site very frequently led to inhibition of mid-face development, and that the earlier the operation was carried out the greater the inhibition seemed to be. He also commented that part of the retrusion might have been due to scar contraction at the site of lip repair. He reported indirectly, as well, that Drachter had also noted a disturbance in maxillary development in his experiences of 40 years earlier. Between 1955 and 1961, the Scandinavians made similar contributions to the literature on alveolar cleft grafting in both primary and secondary procedures. All the writing from this group at that time emphasizes integrated interplay between facial orthopedics/orthodontics and surgery. The writing is occasionally difficult to follow, again because of the confusing use of primary and secondary. In general, primary refers to the initial surgical intervention including the definitive placement of a bone graft; secondary, in general, seems to refer to bone grafting after earlier surgical or orthopedic procedures have been completed. Nordin and Johansson [45] in 1955 reported their first results with alveolar cleft grafting following orthopedic preparation of the alveolar arches. Nordin [46] in 1957 commented on the surgery of Johansson noted two years earlier, and reported anew on the use of secondary grafting procedures in patients 11–12  years old with previously unoperated alveolar clefts. In 1960, Johansson and Ohlsson [47] described the use of alveolar cleft bone grafts in children 7 years of age and older who had undergone earlier soft tissue maneuvers. A year later, the same authors expanded their discussion to cover both primary and secondary procedures [7]. In the primary procedures undertaken some 6–7 months following initial soft tissue maneuvers including temporary closure of the lip, cancellous bone from the proximal tibia was used as a graft, and definitive lip repair was accomplished at the same time; for their later

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

grafting procedures, iliac crest bone was preferred. Barkdáhl and Nordin [48] in 1961 reported their experiences with tibial grafts to alveolar defects dating from 1954, in a technique that entailed resection of the inferior turbinate and use of its mucosa sutured to that of the septum and vomer to afford a generous nasal floor closure of the defect. They also discussed their experiences using banked bone as graft material, 5 of 12 of such cases having failed. Contributions increasingly arose from other sources. Stellmach [49] in 1959 reported his experiences with 37 primary alveolar grafts in infants. He innovated the mobilization and rotation of a vomer flap to entirely cover a chipped rib graft on the labial and palatal surfaces, protected nasally by a mucoperiosteal flap reflected off the nasal cleft margins (Fig. 1.4). Stellmach credited Schrudde for his contribution to the procedure, and Schrudde later commented on his activity in cleft grafting dating from 1955 [9]. An American experience was recorded by Brauer and Cronin [50] in 1964, in which they described their policy of bone grafting the infant alveolus at 8–12  months of age, following lip closure and pre-surgical palatal orthopedics to reduce cleft size and produce improved segment alignment. These authors offered a strong endorsement of incorporating maxillary orthopedics into cleft palate management. Earlier authors had noted the semantic importance of referring to these skeletal maneuvers as “orthopedic” rather than “orthodontic”, since in many contexts they were employed before tooth eruption. Rosenstein [51, 52], working with Kernahan beginning in 1965, was a strong proponent for coordinated early arch orthopedics and cleft grafting, in a protocol that called for lip repair at 6–8 weeks, a molding prosthesis until 4–8 months of age, rib graft to the alveolar defect at that termination, and palatal closure at approximately 1 year. Writing at the turn of the twenty-first century, Rosenstein [53] reported his satisfaction with some 600 patients so treated, in terms of maxillary growth and high percentage of bone support for both lateral incisors and canines.

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Pruzansky [8], on the other hand, was an early severe critic of primary osteoplasty in infancy, arguing that it was not necessary for good arch development at that age, was costly, and even “barbaric”. Widmaier [54] in 1967 wrote that he and Schuchardt had been the first to carry out cleft alveolar grafting simultaneously with primary lip repair, and noted that Schmid, in his innovative procedure of 1951, had put the graft over the cleft as a bridge, while he, Widmaier, preferred to fill the whole defect with the iliac crest particles and cover it with a lateral cortical bridge. He carried out simultaneous definitive lip closure. He later reported a 17-year observation period during which he had noted no growth disturbance as a result of reflecting a septal mucosal flap for nasal floor construction [55]. Both Schuchardt et al. [10] and Robinson and Wood [15] advocated primary alveolar osteoplasty at the time of definitive lip repair. Schuchardt had used full thickness rib grafts in some 400 cases at the time of his 1969 report; Robinson and Wood had used split rib and chips in their 5-year experience to that date. The latter authors had adopted a policy of delaying cleft grafting until 3–4 months following lip repair in especially wide unilateral instances, and for some 3–4 months after initial soft tissue cleft alveolus closure in bilateral cases. They had noted spontaneous tooth eruption in some of their patients, and occasional spontaneous bone formation subsequent to soft tissue cleft closure, evidence of Skoog’s “boneless bone grafting”. Holmann [56] generally favored primary alveolar grafting but felt it should be delayed until development of the deciduous dentition to avoid interference with pre-surgical orthopedic efforts. Though by the 1970s the anticipated benefits of primary alveolar bone grafting had become well recognized, a myriad of techniques and variations of techniques promulgated, and dissatisfactions with the principle were also developing. Chief among these was interference with growth of the maxilla, particularly in the antero-posterior projection. Evaluation of tooth eruption at the graft site, volume of bone actually surviving the

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a

b

c

d

Fig. 1.4  Stellmach’s sketch of circumferentially elevated nasal flap closure (a, b) combined with rotated vomer flap (c, d) for oral closure around alveolar cleft bone graft. (With permission, from Stellmach R: Primäre

Knochenplastik bei Lippen-Kiefer-Gaumenspalten am Säugling unter besonderer Berücksichtigung der Transplantatdeckung. Arch Klin Chir, 1959; 292:865–869)

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

grafting, and degree of paranasal esthetic gain were other targets of skepticism. As early as 1964, Rehrmann [57] had questioned the validity of primary grafting, and in 1971 reported his evaluation of 50 children he had treated with primary grafts and 50 he had treated without grafts. The patients had been operated at a mean age of 8 months, and evaluated 9–10 years postoperatively. Rehrmann used rib as the donor bone, and found a significantly greater degree of vertical, horizontal, and sagittal malocclusion in the grafted group; he thereafter abandoned primary and early secondary alveolar grafting. Jolleys and Robertson [11] reported their first 8 years’ experience in primary grafting, using the term to define the initial involvement of alveolar repair, in most cases following earlier definitive lip repair. The alveolar rib grafting took place at 12–15 months of age, and by 2½–3½ years of age demonstrated definite limitation in maxillary growth. They, too, discontinued use of primary osteoplasty. Schmid and Widmaier [58] referred to the technical difficulty in primary grafting, and suggested its use only in wide clefts, and, if the segments were “compressed”, they be expanded orthopedically prior to grafting. This view would stand in contrast to the protagonists of the Latham appliance. In that same year, Friede and Johansson [59] recapitulated the Johansson scheme of temporary lip closure, tibial alveolar bone grafting with definitive lip repair, and subsequent repair of the soft palate, and then focused on 53 such patients all of whom demonstrated impeded maxillary growth. They emphasized that most reports on the subject to that date were inadequate because of varying techniques, different definitions of surgical timing, short postoperative observation periods, and subjectivity. Hathaway et  al. [60], compiling data from a five-center American study, found that the institution with primary alveolar bone grafting as a variable accrued the lowest scores in maxillary development. Ross [61] argued that primary bone grafts are not placed at the site of intrinsic maxillary growth and thus cannot enhance such growth, and, in fact, might attenuate it. He also supported the question as to whether attenuation was due to

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the graft itself or the scarring resulted from the soft tissue manipulation. He suggested delaying bone grafting until at least the mixed dentition. In contrast, Steinhäuser [62] described 42 children operated with primary alveolar bone grafting at approximately 6 months of age and evaluated a minimum of 13 years post-surgically; those with unilateral clefts demonstrated less sagittal maxillary growth when compared to non-grafted controls, but those with bilateral defects actually demonstrated greater sagittal development when compared to their non-grafted pairs. By 15 years of age, there was little difference between the groups, as evaluated with cephalometric and panoramic films, and dental casts. Additionally, Semb [63] in a report emphasizing the relationship between patient age at the time of alveolar bone grafting and inhibition of maxillary growth found no significant detrimental influence if patients were operated at 8 years of age or older. The conclusion was based on cephalometric comparisons with non-operated control patients at 9 and 16 years of age. Keese and Schmelzle [12] were among the first to offer long-term investigations of graft bone growth around the teeth at the cleft site. They presented information on 177 patients having undergone primary bone grafting at 4–11 months of age whose graft sites they examined radiographically 23–30  years postoperatively, using the Bergland Index as a scale (Fig.  1.5). The Bergland Index measures bone height of interceptal bone from the crest of the alveolar ridge to the apices of the adjacent teeth, in increments of 25%; Indices I-IV represent bone height from 100% (normal) to 25%. Of their patients studied in the long term, only 15% had Bergland Indices of I and 42% had Indices of IV. Their evidence suggested the ultimate inadequacy of primary alveolar bone grafting in providing long-term bone support at cleft sites. Matthews and Broomhead [64] reported their experiences with 94 primary bone grafts supported with Stellmach flaps, 88% of which survived but in which only 22 of 70 involved canine teeth erupted through the grafts. The debate concerning primary alveolar cleft bone grafting continued into later commentary.

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MARGINAL BONE LEVEL SCORE 4

0% 25%

3 2 1

50% 75% 100%

Fig. 1.5  Bergland index for radiographically evaluating vertical height of alveolar bone graft cleft in relation to crestal bone height of adjacent teeth. (With permission, from Enemark H, Sindet-Peterson S, Bundgaard M: Long-term results after secondary bone grafting of alveolar clefts. J Oral Maxillofac Surgery, 1987; 45 (11):913–918)

Torikai et al. [65] proposed a single eight-phase surgical technique, to include alveolar grafting with palatal and turbinate bone and closure of the lip, alveolus, and palate, in a single session at 3–9 months of age, leaving only final lip and nose revision to later ages. Coots [13] in a review article in 2012 referred to van Alst’s opinion that if pre-surgical orthopedics cannot close segments to a 1  mm approximation, primary grafting is not worthwhile. He also opened a discussion of membranous versus endochondral graft bone sources (see below). In another overall discussion, Seifeldin [66] suggested that if alveolar bone grafting done prior to grafting of the palate stabilizes the arch, it also lessens the need for subsequent palatal expansion. Chang et al. [67] in 2016 represented a new way of imaging graft status in situ sonographically, thus avoiding X-ray exposure in young patients. Their calcification scores recorded a transverse measurement rather than the Bergland vertical values.

The smoldering skepticism and uncertainty surrounding primary osteoplasty dating from the 1960s, and the documentation of difficulties over subsequent decades, brought primary alveolar bone grafting to infrequent status in the new century, emphasized in relatively few cleft centers [11, 27, 57, 59, 60, 62, 68]. Chief among the problems were the impedance of midfacial growth and the influence of the procedure on the cleft-site dentition. The abiding basic question, again, was whether it was the bone graft itself or the soft tissue maneuvers to accommodate the graft that were reflected in the undesirable clinical results. Secondary alveolar grafting had been long established by the middle 1960s. Some of Schmid’s [42, 44] earliest iliac crest grafts were accomplished after lip closure, and Johansson and Ohlsson’s [47] and Nordin’s [46] patients were operated at 11–12  years of age and adolescence. Stenström and Thilander’s [69] patient group included adults. From the beginning, the Scandinavian patients all underwent coordinated orthopedic/orthognathic care. Pfeiffer [70] utilized rib grafts in his management of 153 previously operated bilateral cleft patients. He placed great emphasis on soft tissue bed preparation, and opened the lip for access in edentulous pre-­maxilla cases so as to carry out bilateral repair in one operation; with incisor teeth present in the pre-maxilla, and more limited access, he proceeded in two sessions with the lip intact, using the teeth for splint stabilization. Perko [71] reported his experiences with bilateral clefts, choosing to close both sides simultaneously, appropriately mobilizing and repositioning the central element but avoiding manipulation of the lateral segments because of concerns with vascularity. Perko preferred iliac cortex and marrow over rib grafts. Georgiade and Pickrell noted these European advances in their review of 1964 [72]. Epstein and Davis [4], reporting in 1990, offered another classification for secondary grafting: early secondary, prior to palatal closure; intermediate secondary, after palatal closure (2–9 years of age); delayed secondary, after permanent dentition eruption. Patients were o­ perated

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

at an average age of 11½ years and demonstrated 81% functional and 61% esthetic successes. Epstein and Davis preferred rib grafts. Cronin and Penoff [73] added an American contribution to the bilateral cleft grafting literature in 1971 in describing their “stabilization” of the pre-maxilla with grafts in 31 patients at a preferred age of 4–5 years, following prior orthodontic care. The 10-year experience of Hogeman and Jacobsson [74] revealed a 34% “success” rate when rib grafts were used in their secondary procedures, and 58–98% success with their subsequent use of iliac bone; they chose to operate patients only 12 years of age or older because earlier grafts did not keep pace with adjacent alveolar bone growth. Though certainly not the first, one of the most often-quoted secondary graft sources is that of Boyne and Sands [75, 76]. The popularity is probably due to three factors: publication of the material in probably the most widely read English language journal of the time; recognition of the confusion in the terms primary and secondary and a simplification of the distinction; a focus chiefly on the salvation of the cleft-site teeth. The authors commented with emphasis on their preference for iliac crest cortex/marrow as graft material over rib sources, arguing that the former provided a plastic osseous system, responsive to eruption forces in orthodontic manipulation, whereas the latter provide chiefly a relatively unresponsive supportive strut. They advised grafting at 7–8  years of age to support eruption of the lateral incisors and canines. This age range has been endorsed by other American writers [75, 77–80]. In conventional radiographic studies, Ames et al. [81] and Turvey et al. [80] found very good bone support and canine eruption percentages, Ames and Ryan stressing that iliac crest was superior to rib bone. Abeyholm and Bergland’s [79] radiographic review, however, displayed “normal” grafted alveolar height in only 38% of 89 clefts, and “less than normal” or worse in the remainder. They also stressed the importance of adequate alar base grafting for esthetics, and both they and Turvey et  al. [80] noted that palatal growth, particularly anteriorly, was normally complete by 9 or even 6–7 years of age, so that

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alveolar grafting at or near the time of canine eruption should provide only minimal interference with its development. Enemark et al. [82], radiographically studying three groups in their 224 patients—mean ages of 10  years (prior to canine eruption), 13 years (after canine eruption), and older than 16  years—found the best bone preservation in their youngest group. The Enemark group was also early in describing the importance of including attached gingiva in the buccal flap covering the graft to avoid postoperative periodontal difficulties. Simonsen [83] also stressed this point. Milstein et al. [84], applying some six monitors (gingival blood flow, oxygen saturation, tissue hemoglobin, etc.), measured the overall vascular status of gingival tissues at previously operated alveolar cleft sites prior to alveolar cleft bone grafting, and compared the findings to those of previously unoperated gingival tissues on the opposite side of the mouth; certain parameters indicated compromised vascularity at the previously operated sites, suggesting the potential for inhibited wound healing following anticipated bone grafting. LeCrepeau [85] reviewed the accuracy of dental films in evaluating grafts in 101 patients; 17% of the films suggested “success” in clinically non-functional grafts. LeCrepeau considered 3-D CT imaging to provide better analysis. Opitz et al. [86] also employed dental/panoramic films and found the best evidence of success in their 6–9  year olds, and the least substantial bone in patients operated after 14 years; interestingly, the best bone growth was centered about the lateral incisors in this group. Van der Maij et  al. [5] employed CT immediately postoperatively and 1 year postoperatively to evaluate bone mass in their group of 50 clefts, emphasizing the importance of buccal-palatal graft dimension, not only vertical height; at 1 year, 70% of the grafted bone remained in unilateral cases, but only 45% in bilateral clefts. This group joined previous others in suggesting that dental age might be a better index than calendar age for determining the time of secondary grafting, prior even to eruption of the lateral incisor. This was the position strongly favored by Precious [87], who endorsed grafting at 5–6 years of age, prior to orthodontic efforts, to

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better ensure lateral incisor retention and eruption. The Van der Maij group also commented on the inconsistency in radiographic review analysis, and suggested computerized tomography as the standard reference. In 2003, Hogan et al. [88] studied the eruption of canines into graft sites, and found that 95% of their patients operated at 8–12 years demonstrated continued root development and eruption almost similar to the normal pattern. In the same year, Hynes, et al. [89] reported that 76% of their group of 58 patients operated when their canines displayed 50% of root development demonstrated Bergland Indices of I or II, at a mean of 4½ years post-surgery. They found the Bergland scale not wholly representative of the graft status, but at least compatible with 3-D imaging results. Horswell [90] at that time endorsed state of dental development being more important than patient age in determining graft timing, but suggested 5–8 years as the preferable range for surgery. He emphasized stress on the graft, either through orthodontic maneuvers or through dental implant function, being strongly influential on its health. Radiographic studies in the new century became increasingly sophisticated. Iino and Ishii [91] compared periapical and occlusal film evaluation of late-term graft sites with CT imaging, and found that 40% of surgical outcomes could be overestimated by dental films because of inadequate evaluation of graft thickness. Jia and Fu [92], using occlusal films and the Bergland Index to determine height of the reconstructed dental septum, found that 80% of these grafts were healed by 6  months post-operation. Trindade et  al. [93], however, stressed that the Bergland Index mandates full eruption of the canine for assessment, and does not record graft resorption at the apical level; they suggested that the Chelsea Scale, which radiographically evaluates the amount of bone along the roots of cleft-site teeth, was a better monitor of graft success. Ojawa and Omura [94], doing graft volume comparisons with cone-beam CT 3-dimensional reconstructions immediately postoperatively and 6  months postoperatively, determined a 64% reduction in bone mass over that interim if the graft was placed prior to incisor migration, probably

R. B. MacIntosh

due to disuse atrophy. This group recommended grafting at age 5–7 years, prior to canine eruption, to take advantage of erupting lateral incisor bone stimulation and eruption into the graft. In measuring graft volume with cone beam CT at 1 year postoperatively, Fouzet-­ Roumazeille [95] found a value of 62% of the original. Wang et  al. [24] in a sophisticated cone beam CT and Bergland Index analysis compared the postoperative status of patients treated with gingivo-periosteoplasty in infancy to those treated with secondary alveolar bone grafting at approximately 10  years of age. Residual defects in both groups were greater apically than near the erupted canine crowns, and the gingivo-­periosteoplasty group had larger residual defects, 28% of them requiring additional grafting. Jabarri et al. [96] also determined significantly better Bergland scores in their secondary alveolar bone graft patients compared to those treated with gingivo-­periosteoplasty in infancy, but also found little difference between the groups and maxillary position by 18 years of age. Disseaux et al. [97] found that most graft resorption was reflected in thickness rather than height, so that 2-­dimensional cone beam CT scan overestimates graft success. Offert et al. [98] in a controlled but subjective study reported that paranasal esthetics in infants grafted between 2–4 years of age were clinically better than those in patients not undergoing grafting, but that this difference became less obvious with age. By the end of its first 60 years of application, bone grafting rehabilitation of the cleft alveolus had moved internationally from its European beginnings, to great degree had satisfied the advantages anticipated at its beginnings, and had demonstrated several shortcomings, most prominently its inhibition of maxillary growth. The advantages of coordinated orthodontic/orthopedic care had become universally recognized. Though definitions of primary and secondary osteoplasty remained varied and not universally accepted, a distinction between surgery at the time of initial lip closure and any time thereafter had become recognized. Bulk bone grafting had become preferable to gingivo-periosteoplasty alone, and the iliac crest was recognized as the most popular donor site. Within the limits

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

imposed by radiation exposure, 3-dimensional cone-beam CT had become recognized as the preferred imaging modality. The advantages and disadvantages of various bone donor sites, the various harvesting techniques, and elements of basic bone biology had become the targets of many investigators and clinicians.

1.3 Bone: Physiology and Donor Sites Lexer in 1908 [52, 99], in his long contribution on bone grafting, both autogenous and homologous (cadaveric), emphasized the importance of periosteum in success, admittedly without knowing why. Dick [100], writing shortly after the end of World War II, credited Macewen with the first use of homologous tibia, in 1912 [101]. Dick addressed the dilemma of whether grafted bone in fact survived or was simply physiologically replaced, and concluded that survival reflected a combination of cortical stability and cancellous growth. Mowlem echoed the same interpretation in 1963 [102], noting that by the 1940s the graft was regarded as a scaffold only, with new bone growing through it from the host, even if the two were not in contact; the process was a matter of “creeping substitution”. Mowlem also described his use of a rib graft to replace a hemimandible, but made no mention of cleft surgery. By the 1960s, banked homologous bone had become available and Burwell [103] expressed the view that autogenous grafting success was a matter of degeneration and proliferation; homografts healed somehow differently but could induce osteogenesis, as could also non-osseous connective tissue, an echo of Skoog’s “boneless bone grafting”. Other basic investigation at that time reflected the debate of differences in the physiology of endochondral and membranous bone. Smith et al. in a rabbit study [104] noted an increase in bone volume at membranous (skull) graft sites, and up to 64% resorption of endochondral grafts at 1 year post-implantation. The conclusion was that the endochondral grafts lost their normal stress lines when transplanted and thus atrophied, while

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the skull grafts, not normally under stress, realized no change in environment and maintained dimension. Koole [105] reviewed the genesis of osteoblasts from embryonic neuroectoderm, and emphasized the role of stress in bone architecture allowing membranous bone to accept the loads of cleft grafting. In a rabbit and monkey study, Zins et al. [106] also determined that transplanted membranous bone maintained better volume dimension than endochondral bone, because the former revascularized more rapidly. Wolfe [107] reviewed his use of cranial cortico-­cancellous grafts for cleft alveolus reconstruction, finding them to be equally as serviceable as iliac crest grafts, and expressed the opinion that the donor site was unimportant as long as the graft material was cortico-cancellous. Osaki et al. [108, 109], on the basis of intensive rabbit studies, voiced essentially the same conclusion. Using fluorochrome markers and CT scanners, this group found that cortical grafts, regardless of endochondral or membranous origin, maintained good volume, and cancellous bone, from either mandible or ilium, demonstrated the greatest resorption. Rahwashdeh and Telfah [110] stressed these findings in their review article of 2008. Dragool et al. [111] as early as 1973 demonstrated root resorption in teeth coming in contact with fresh iliac crest grafts in periodontal reconstructions. The authors offered no explanation but observed that such resorption could occur as early as 3  weeks post-grafting. Some of the involved teeth healed with new cementum. Ellegard et al. [112], working with monkeys, and Schallhorn et al. [113], in the clinical patient setting, demonstrated root resorption in teeth against which periodontal iliac crest grafts had been placed. Ellegard did not observe this resorption when maxillary bone grafts (membranous) or frozen homologous iliac crest grafts were positioned against the teeth of the test animals. Enemark et al. [82] were among the first to report canine root resorption clinically at sites of cleft graft contact. Newlands [114] subsequently reported the same phenomenon occurring around the lateral incisors. Sindet-Pedersen et  al. [115] found no root resorption in 20 clefts treated with

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mandibular symphyseal grafts, nor any in 20 patients undergoing iliac crest grafts, and noted no significant difference in grafted alveolar bone height between the two groups. Kapis [116], without referring to clefts particularly, offered a commentary on the use of rib grafts in the reconstruction of World War I cranial war wounds. He preferred harvesting the 12th rib from the lateral approach to better avoid pneumothorax, and included attached perirenal fat for soft tissue augmentation. Clementschitsch [117] reported his success with intraoral bony reconstruction dating from World War II before the antibiotic era, emphasizing the need for haste between harvest and implantation, maintenance of the graft in warm blood over that interim, and good stabilization of the graft in situ. As noted earlier, Mowlem wrote from the same period, reporting both grafting principles and his experience with the rib in particular. Witsenburg and Freihofer [118] in 1990 described the use of stacked rib graft segments covered labially with cortical shields in a series of 11-year old alveolar cleft patients. Cortical segments were also layered paranasally. At 60–91 months postoperatively, clinical and radiographic evaluation demonstrated good periodontal results, and all preoperatively unerupted canines had erupted through the grafts. The rib as a source for alveolar grafting has lost emphasis in later decades. Ames and Ryan [81] spoke of their preference for iliac crest cortico-­cancellous grafts after earlier disappointment with rib tissue. Unquestionably, the iliac crest became the predominant donor source by the end of the twentieth century, one major stimulus being the reports of Boyne and Sands [75, 76]. A plethora of reports on this donor site have been offered since, as illustrated in the reviews of Witsenburg [119] and latterly Rahwashedeh et al. [110]. Rudman [119] studied the morbidities of iliac crest grafting and expressed the opinion that the benefits justified using it as a source. Kessler et al. [120] compared their experiences with the anterior and posterior approaches to the iliac crest, and reported more generous harvest and less morbidity with posterior access, and greater

R. B. MacIntosh

pain and gait impairment with anterior. Kokkinos et  al. [121] included a rim of crestal cartilage with their cortico-cancellous iliac grafts to better enhance the lateral pyriform rim and alveolar crest depression. Sharma et  al. [122] compared trephine iliac crest harvest with the more conventional open approach, and found it offering shorter operating time, less patient discomfort, shorter hospital confinement, but less cortico-­ cancellous material. Tessier [123] provided a discussion of cranial grafts in 1982, in which he described his experience with 234 grafts in 103 patients. He acknowledged potential hazards, but reported the donor site regenerating totally in less than a month in young patients. Tessier did not describe the use of these grafts in cleft patients in this report. Wolfe [107] and Wolfe and Berkowitz [124] did report cranial graft use in cleft alveolus patients, emphasizing the convenience of proximity to the host site, minimal discomfort, and shorter hospitalization, as compared to iliac crest donor patients. Wolfe also reported, however, that 10% of his patients demonstrated poor diploic space. Denny et  al. [125] in 1999 voiced strong support for milled cranial grafts; convenience of the site was an argued advantage, and their panoramic and 3-D cone beam CT imaging of bone volume at 1  year postoperatively demonstrated “good” or “acceptable” results of 66–88%, with better scores in patients operated before age 12. Jackson et  al. [126] noted Múller’s [127] early use of a cranial bone flap, and then reviewed their then-6-­ year experience with 307 such grafts. By that time, however, Jackson had abandoned cranial grafts for alveolar reconstruction because of their firmness and relative lack of cancellous tissue. Turvey [128] offered a critique and rebuttal of Jackson’s paper, describing his satisfaction with the cranium as a source. Eichorn et al. [129] utilized cranial grafts at 4–56  months of age in a series of 31 patients in what they termed primary osteoplasty, and determined a Bergland success rate of 76% at 12–114  months postoperatively, attributing the success to the lesser resorption rate of membranous bone. Though Lexer [52], Macewen [101], and Drachter [41] contributed early endorsement of

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

the tibia as a donor site, little had been written of its role in alveolar cleft grafting until recently. Drachter’s report probably records the first use of a tibial graft in cleft repair. Much has been written since 2000 regarding the tibia as a source of cortico-cancellous bone, chiefly for application to orthognathic or, more extensively, sinus lift pre-prosthetic, surgery. Hereford et al. [130] in a cadaver study described the medial and lateral surgical approaches to the proximal tibia, were able to extract some 25  ml of bone from either site, and anticipated no particular surgical morbidity in clinical practice. Hughes and Revington [131], however, encountered two tibial fractures in their clinical series of 75 alveolar cleft patients, having extracted only 5–10 ml of bone substance from the lateral approach. Savarajingam et  al. [132] conducted comparative radiographic densitometry studies on a series of alveolar cleft iliac crest and tibial grafts from 6  days to 3  months postoperatively, and observed an initial decrease in bone density in both groups, but no significant differences between the two at final evaluation. Sindet-Pedersen and Enemark [115] compared mandibular symphyseal cortico-cancellous material to that of the iliac crest, and at 12 months postoperatively could determine no significant clinical or radiographic differences between the two groups of 20 patients each. There was less bone available from the symphyseal site, however.

1.4 Adjunctive Elements Since the beginning of alveolar cleft reconstruction, and particularly in recent decades, various agents have been introduced to enhance grafted bone’s preservation, accelerate its healing, or augment its growth. Early attempts included saturation in whole blood and introduction of topical antibiotics. Skoog [133], building on his experience with periosteoplasty, introduced the insertion of oxidized cellulose hemostatic agent between layers of periosteum in an effort to stabilize and maintain the coagulum and increase the bulk of regenerated bone. In a rabbit experiment, he substantiated resorption of the cellulose, thickening of the peri-

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osteum, and production of bony islands within a period of 28 days. The material was then included in clinical practice. Ames and Ryan [81] included oxidized cellulose in their series of 41 patients treated with cortico-­ cancellous iliac grafts, but could determine no significant benefit from the additions. The general interest in the application of platelet-rich plasma to maxillofacial reconstruction found a focus in several alveolar cleft group studies. Lee et  al. [134] studied the degree of resorption and bone density of cleft grafts in two groups of patients operated at 8–9 years of age. Using dental films and an aluminum equivalency scale, no significant defense against bone resorption was found in the group having undergone iliac crest grafting with additive PRP, in comparison with the children having been grafted with iliac cortico-cancellous bone alone. Marukawa et al. [135], however, using occlusal, panoramic, and CT imaging, compared similar groups operated at 9–23  years of age and evaluated 1  year postoperatively, and reported a significant reduction in graft resorption in the patients receiving PRP at their graft sites. Hegab et al. [136] determined slightly higher graft preservation in their iliac cortico-cancellous group treated with PRP than in the group without, a finding of some clinical but not statistical significance. Gupta et  al. [137] used cone-beam CT to evaluate bone density in their 8–30 year old cadres of ten patients each; the group treated with PRP demonstrated statistically significant denser bone, but the maximum observation period was only 6  months. In an experimental study of dog mandibles, Huh et  al. [138] determined that artificial defects in bone healed better radiographically and histologically when grafted with autogenous particulate bone plus platelet-enriched fibrin adhesive than with the particulate bone alone; the test grafts were denser at 6 weeks, and the bone-only grafts demonstrated elements of non-vital (lacking osteocytes) tissue. In a clinical study of 27 patients 7–16 years of age undergoing secondary cleft osteoplasty, Sigura et  al. [139] evaluated graft volume, bone density, and bone quality, in a group receiving autogenous particulate grafts enhanced with fibrin adhesive, and compared

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those values to those of a group grafted without the fibrin adhesive; the fibrin adhesive test group demonstrated decidedly less resorption and loss of volume than did the graft-only cadre. The authors emphasized the superiority of CT evaluation over conventional 2-D evaluation of graft sites, but evaluated their subjects only at “a minimum of three months”. In a fundamental study of human mesenchymal stem cell (hMSC) behavior under the influence of fibrin adhesive, Catelas et  al. [140] determined that, although the stem cells could be guided into osteogenic differentiation, no mature bone-forming cells developed. The physiology and efficacy of bone morphogenic protein found a focus in maxillofacial surgery by the end of the twentieth century, with a pertinence to cleft grafting. Wouter et  al. [141] carried out an extensive review of literature relating BMP to alveolar reconstruction in 2001, but found only a handful of reports satisfying criteria for meaningful information. Nonetheless, the necessities for engineering bone growth were established, that is, osteogenic cells, osteo-­ conductive scaffold, and osteo-inductive hormones. Autogenous bone combined with BMP-2 was reported as the preferable in vivo vehicle for alveolar clefting, more productive than autogenous bone alone. In another survey article, Freitas et al. [3] supported this conclusion, and in their own cone beam analysis found better interdental septum evaluations in their grafts supplemented with human recombinant rhBMB-2. Morgan et al. [142] described favorable findings in bone fill and tooth eruption in a preliminary study using rhBMP-2 impregnated into absorbable collagen sponges as lone grafts, that is, without bone. Fallucco et  al. [143] also performed 17 primary alveolar reconstructions utilizing solely BMP-2 on absorbable collagen sponges; SPECT (single photon emission computerized tomography) analysis, using the Hounsfield bone density scale at 6 months postoperatively, described highly effective union of the dental arches. Goss and Hunter [144] investigated postoperative nasal stenosis in a group of alveolar cleft patients operated at an average age of 3.5 years,

R. B. MacIntosh

and found approximately twice as high an incidence in those children receiving BMP-­ augmented grafts as those not receiving BMP, and felt the degree of stenosis was dose-­ dependent. In a study emphasizing the superiority of 3-D CBCT analysis over occlusal films, Liang et  al. [145] observed essentially equal operative site bone filling at 6 and 9 months postoperatively in two groups, one treated with iliac crest bone alone and the other with BMP-­ augmented demineralized bone matrix without autogenous bone. The BMP group also demonstrated greater postoperative edema. Earlier writing had recorded an awakening of the potentials for DBM (demineralized bone matrix) in cleft surgery. In 1987, Kraut [146] described his experience with five secondary alveolar cleft reconstructions utilizing solely DBM (freeze-dried bank bone) blocks in patients 10–17 years old, in four of whom the malposed canines erupted. Merckx et al. [147] studied isolated xenographic DBM in an animal investigation, comparing its effectiveness in reconstruction of primary tooth extraction sites to wounds repaired with iliac crest (endochondral) or mandibular (intramembranous) grafts, and to unreconstructed naturally healing wounds; permanent teeth erupted into all wounds, but the DBM neither integrated nor resorbed, but was displaced by the erupting teeth. Multiple authors have commented on combined autogenous/derived DBM grafts. Goudy and Lott [148], in a comparative radiographic review of 103 patients, found no advantage in cleft-site reconstruction in the group treated with autogenous bone plus DBM over the group managed with autogenous bone alone. Sivak et  al. [149] reconstructed previously failed autogenous bone grafted alveolar cleft sites with autogenous iliac crest grafts, autogenous bone/DBM allograft grafts, or DBM alone, and found the DBM-alone sites as radiographically successful as the combination grafts; the cost of the alloplast was considered a potential disadvantage. Susarla ­ et al. [150] compared rapidity of canine eruption through iliac crest grafts harvested in customary open fashion with eruption through similar iliac crest grafts combined with DBM, and found

1  Alveolar Cleft Grafting: Origins, Advances, Prospects

equally adequate eruption in the two groups. MacIsaac et al. [151], in a similar study, observed earlier canine eruption in the full iliac crest donor site group, but overall higher rates of engraftment and bone graft survival in the graft-DBM group. Elfahmawary et  al. [152], in a CBCT analysis, determined that smaller mandibular symphyseal grafts augmented with DBM were equal to bulkier iliac crest grafts in terms of bone fill, graft resorption, and bone density at 6  months postoperatively. In a single case report, Corre et  al. [153] reported the use of calcium phosphate to augment autologous bone and PRP in repairing an alveolar cleft, with resulting complete occlusion of the cleft and spontaneous canine eruption at 8  years postoperatively. Lazarou et  al. [154] described a larger primary alveolar cleft series reconstructed with calcium sulfate paste or crystals; evaluated clinically and with panoramic and occlusal films at 3–7  years postoperatively, the sites demonstrated normal canine eruption and no clinically significant resorption. Literature entries concerning the reasons for alveolar grafting failure and the need for reoperation are few. Shirani et  al. [155], however, reviewed 54 patients grafted at an average of 15  years of age and evaluated with panoramic films and the Bergland scale at 33 months postoperatively. Approximately 41% of the patients were judged to objectively require reoperation, with the major influencing variable being orthodontic management for those in the group for whom the need for revision surgery was notably less. The presumed benefits of orthodontic care were widening of the cleft facilitating surgical manipulation, and orthodontic physical stress on the graft encouraging greater bone density. Borba et al. [156] studied 71 patients at 1 year postoperatively and found some 41% had experienced surgical complications; some 28% of patients overall required reoperation, and, of those, 85% had experienced post-surgical complications of wound dehiscence, infection, resorption (perhaps aggravated by over-compression of the grafts), or periodontal compromise. Distraction osteogenesis has gained recognition as a therapeutic option for many craniofacial

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imbalances. Its use in alveolar cleft management potentially offers the advantages of lessening the width of the defect and thus the bulk of the necessary graft (in some cases obviating the need for bone grafting entirely), manipulating the segments into better arch form prior to grafting, and transporting undisturbed attached gingiva with the dento-alveolar bony segment. Disadvantages include awkwardness of some appliances, compromised hygiene, patient intolerance, excessive unbalanced stress on supportive teeth, and imprecision in movement. To avoid or minimize these difficulties, most attempts at segmental movement in cleft patients have been accomplished with precision tooth-­ borne devices. Liou et al. [157] described a regimen of preoperative orthodontic palatal expansion, distraction-narrowing of the residual cleft, GPP at the reduced cleft site, and ultimate orthodontic alignment to bring the unerupted canine through the newly generated bone into the dental arch. The reported patients were 4–12 years old; distraction was active over a 3-week period, and the unerupted teeth were drawn into the mouth within 1–3  months of beginning efforts. Dolamazet et al. [158] similarly endorsed distraction in their experience with eight patients, but noted the possibilities of imperfect positioning of the transport segment and incomplete bony closure on the nasal sides of the clefts. Veja et  al. [159] moved both anterior and buccal segments in their group of adolescent alveolar cleft patients, with new bone formation at all sites of union. Hegab [160], however, though finding success in his group of patients aged 16–24  years, noted slight displacement of the transported segment at the completion of movement, and residual triangular bone defects that required either GPP or bone grafting for solid union. Tissue engineering, the controlled cultivation of biologic material in the laboratory or in living organisms, has excited the clinical reconstruction community in recent decades. The prospect of obliterating an alveolar cleft with living tissue without donor site morbidity, and with morphological precision and shorter surgical time, would be universally celebrated. Efforts at cultivating new bone inspire laboratories around the world.

R. B. MacIntosh

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Multiple matrices are being employed (polymers, inorganic bone, ceramics), as are autogenous and allogenic cells, marrow stem cells, and biologic regulators such as the BMPs. In vitro studies offer the best control of measurement, but in  vivo experimentation provides the more realistic physiologic and mechanical environment. Maxillofacial surgery became enamored with HA (hydroxyapatite) in the 1980s and 1990s in various contexts, and it became an engineering matrix candidate. But HA is not the same as natural bone matrix, and so far natural bone mineral has not been duplicated. Tricalcium phosphate (TCP), a ceramic, has been employed but can dissolve prematurely and can invoke inflammation. Seeding of natural bone matrices with bone cells grown in  vitro has been studied. Various efforts to incorporate BMP into carriers of collagen, TCP, or other ceramics are ongoing, as are investigations of the possibility that implanted natural bone mineral by itself might serve as a matrix for new bone formation. Ultimately, it may prove more efficacious to regenerate tissue in vivo than in vitro. Spector [161] has offered a broad overall perspective of such efforts.

1.5 Summary Now, in the early years of the twenty-first century, alveolar cleft bone grafting, for its recognized role in development of a normal alveolar arch and functional dentition, shares equal emphasis with both cleft lip and palate restoration. The necessity of integrating timely bony reconstruction with coordinated orthodontic/ orthopedic maneuvers is well recognized; expansion of the maxillary arch, accommodation to the scarring of lip repair, and protection of the cleft-­ site canine and lateral incisor are paramount in these combined maneuvers. Nasal alveolar molding, when feasible, is a benefit in early care. Secondary osteoplasty, by whatever definition beyond grafting as an initial reconstructive maneuver, is the preferable policy, and autogenous cortico-cancellous bone is the overwhelming bone choice. The iliac crest serves as the

preferable donor site, though cranial, tibial, and rib grafts still have their advocates. Grafting preferably takes place between the fifth and ninth years to benefit the health and eruption of the canine and lateral incisor teeth. To date, no fully acceptable substitute for autogenous bone exists. Homologous bone, alloplastic matrices, and autogenous blood products have been investigated, and it may be that human mesenchymal stem cell grafts and/or osteo-­ inductive hormones may ultimately provide in vitro or in vivo relief from the donor site and technical rigors of autogenous reconstruction.

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23 151. MacIsaac ZM, Rottgers SA, et  al. Alveolar reconstruction in cleft patients: decreased morbidity and improved outcomes with supplemental demineralized bone matrix and cancellous allograft. Plast Reconstr Surg. 2012;130:625–32. 152. Elfaramawi TI, Faramawey WI, et al. (Oral abstract) Deproteinized bovine bone graft in maxillary alveolar cleft reconstruction, vol. 73. AAOMS; 2015. p. e14. 153. Corre P, Khonsari RH, et  al. Synthetic calcium phosphate ceramics in secondary alveoloplasty. (les céramiques phosphocalciques synthétiques dans l’alvéoplastie secondaire.). Rev Stomatol Chir Maxillofac. 2012;113:131–5. 154. Lazarou SA, Contodimos GB, Gkegkes ID. Correction of alveolar cleft with calcium-based bone substitutes. J Craniofac Surg. 2011;22:854–7. 155. Shirani G, Abbasi AJ, Mohebbi SZ. Need for revision surgery after alveolar cleft repair. J Craniofac Surg. 2012;23:378–81. 156. Borba AM, Borges AH, et  al. Predictors of complication for alveolar cleft bone graft. Br J Oral Maxillofac Surg. 2014;52:174–8. 157. Liou EJW, Chen PKT, et  al. Interdental distraction osteogenesis and rapid orthodontic tooth movement: a novel approach to approximate a wide alveolar cleft or bony defect. Plast Reconstr Surg. 2000;105:1262–72. 158. Dolanmaz D, Karaman AI, et  al. Management of alveolar clefts using dento-osseous transport distraction osteogenesis. Angle Orthod. 2003;73:723–9. 159. Vega O, Pérez D, et  al. A new device for alveolar bone transportation. Craniomaxillofac Trauma Reconstr. 2011;4:91–105. 160. Hegab A.  Closure of the alveolar cleft by bone segment transport using an intraoral tooth-borne custom-made distraction device. J Oral Maxillofac Surg. 2012;70:337–48. 161. Spector M.  Basic principles of tissue engineering. In: Tissue engineering: applications in maxillofacial surgery and periodontics. Lynch SE, Genco RJ, Marx RE.  Chicago: Quintessence Publishing Company; 1999. p. 3–16.

2

Clinical and Diagnostic Anatomy David Wilson and Pat Ricalde

Cleft and craniofacial surgeons are required to possess an extensive knowledge in embryology and anatomy of the face to properly treat children with facial differences. Cleft lip and palate occurs in about 1/600 births and is the most common facial dysmorphology [1]. It is an immediately recognizable disruption of normal facial structure. This abnormal facial structure ultimately results in problems with feeding, speaking, hearing, and social integration. To understand craniofacial development and to treat children with facial differences successfully, basic knowledge of embryology, cell and molecular biology, and anatomy is crucial [2]. It is during embryological folding and neurulation that components of the face appear. The skeleton of the head and pharynx arises from neurocranium and viscerocranium. The neuroD. Wilson (*) Private Practice, Kalamazoo Oral and Maxillofacial Surgery, Kalamazoo, MI, USA Western Michigan University Cleft and Craniofacial Clinic, Kalamazoo, MI, USA Western Michigan University School of Medicine, Kalamazoo, MI, USA e-mail: [email protected] P. Ricalde Craniomaxillofacial Surgery, Florida Craniofacial Institute, Tampa, FL, USA Tampa Bay Cleft and Craniofacial Center, Tampa, FL, USA University of South Florida, Tampa, FL, USA e-mail: [email protected]

cranium is composed of bones that protect the brain. The viscerocranium forms the bones of the face and pharyngeal arches. The frontonasal prominence and the pharyngeal arches start development on day 22 [3]. The pharyngeal arches are bilateral endodermal outpockets from the foregut (pharynx). The pharyngeal arches are unique in that they are covered by ectoderm and filled with ectomesenchyme (mesoderm and neural crest cells, NCCs) to give rise to the numerous facial structures [4]. There are four pairs of pharyngeal arches. The first pair gives rise to the lower jaw and upper jaw. Maxillary and mandibular prominences contain a central cartilage which contributes to structures of the face. The maxillary cartilage includes the incus and alisphenoid (located on lateral orbital wall), which eventually becomes encapsulated by the maxilla, zygoma, and squamous portion of the temporal bone. The mandibular cartilage is known as Meckel’s cartilage, which forms the malleus, sphenomandibular ligament, and the anterior ligament of the malleus. This cartilage disappears from the developing mandible [5]. The originating muscles and nerve supply to each corresponding pharyngeal arch are maintained during embryological development [6]. The first pharyngeal arch mesoderm adjacent to mescencephalon and rhombomeres 1 and 2 give rise to the muscles of mastication (temporalis, masseter, medial and lateral pterygoids), mylohyoid, anterior belly of digastric, tensor tympani, and tensor veli palatini muscles. These muscles

© Springer Nature Switzerland AG 2023 P. Ricalde (ed.), Cleft Maxillary Reconstruction, https://doi.org/10.1007/978-3-031-24636-4_2

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will all be innervated by cranial nerve V (Trigeminal) [7, 8]. The development of the face starts during the fourth week, and is complete during the tenth week. The face results from the joining of five prominences: the frontonasal prominence and two maxillary and mandibular prominences associated with the first pharyngeal arches [9]. During the fifth week, the five prominences begin to surround the stomodeum (primitive oral cavity). The maxillary prominences continue to enlarge medially and ventrally. During the same time, epithelial thickenings called nasal placodes enlarge from the frontonasal prominence. In the sixth week the nasal placodes center component invaginates to form a nasal pit, which will subdivide the prominence into medial and lateral nasal processes. The medial nasal processes will continue to enlarge and fuse to form the beginnings of the nasal septum and bridge of the nose in a region called the intermaxillary process. At the same time the maxillary prominences continue to grow and fuse with the intermaxillary process to form the upper lip, philtrum, and primary palate [10]. It is from the intermaxillary process that the philtrum and primary palate originate. The primary palate will also house the lateral and central incisors, teeth numbers 7–10 [11]. The mandibular prominences are fused by the fifth week and grow in continuity with each other. There is a small fissure at this time between the prominences, but during the fourth and fifth weeks, this depression will be filled by continued mesenchyme proliferation. The beginning of the primitive mouth occurs during the third week. Two faint depressions form in the ectoderm along the prechordal plate during gastrulation. However, these two depressions are devoid of mesoderm. The cranial depression is called the oropharyngeal membrane, which is the primitive mouth and the beginning of the gut tube. During the fifth week, this membrane breaks down due to lack of mesenchymal tissue to aid in proliferation and the oral cavity is formed. The maxillary and mandibular prominences grow laterally and fuse to form the cheeks. The finalized growth of these prominences ultimately determines the size of the mouth (Fig. 2.1a–e).

D. Wilson and P. Ricalde

Development of the nasal passage starts around the sixth week by the invagination of the nasal placodes forming the nasal pits. There is continued ventral growth of the medial and lateral nasal prominence with deepening of the nasal pit resulting in a nasal sac. Dorsal growth forms the primary palate and continued posterior growth forms the nasal sac. At the seventh week, the posterior portion of the sac forms an area called the nasal fin, which develops vacuoles to eventually form a thin layer called the oronasal membrane. This membrane ruptures to form the primitive choana. Failure of the rupture results in choanal atresia. The oral and nasal cavities are continuous with one another during the seventh week (Fig. 2.2a–e). In the seventh week, medial extensions from the maxillary prominences develop to form palatal shelves. This ultimately will form the secondary palate. The shelves begin with a vertical and downward projection parallel with the lateral surface of the tongue. By the end of the seventh week the palatal shelves rotate horizontally and fuse with each other and the primary palate. The fusion begins in the middle of the palatal shelves and continues in an anterior and posterior direction [12]. This explains why there can be a cleft of the alveolus and cleft of the soft palate but the hard palate can be intact. Cleft palates can form in all shapes and sizes. There are intramembranous condensations within the anterior portions of the shelves that form the bony hard palate and myogenic mesenchyme condensation of the posterior shelves to form the musculature of the soft palate. It is these condensations where specific gene expression occurs to allow for direct ossification of the hard palate and muscle union of the soft palate. Palatal shelf elevation is determined not only by mesenchymal development of the maxillary prominences but also on the growth and lowering of the mandibular prominences. Initially, the tongue fills the oral cavity, and if mandibular development is inhibited, the palate can also be affected by failure of fusion [13, 14]. An example of this is Pierre Robin Sequence, where deformation results in micrognathia, which in turn allows for glossoptosis, or posterior superior tongue positioning. This forms a barrier to the developing palatal shelves, and

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a

c

b

d

e

Fig. 2.1 (a–e) Development of the face. (a, b) During the sixth week the frontonasal prominence differentiates to medial and lateral nasal process. The frontonasal prominence invaginates to form the nasal pits. (c, d) During the seventh week the medial nasal prominence fuse to form intermaxillary process and fuse with the maxillary promi-

nence. E, By the tenth week, the intermaxillary process becomes the philtrum and upper lip. (From Schoenwolf, Bleyl, Brauer, Francis-West. Larsen’s Human Embryology. Elsevier, fifth ed. Fig.  17.18. Chap. 17, p 451)

they fail to fuse, resulting in a cleft palate [15]. Final formation of the definitive palate and choana is around the tenth week. The frontonasal and medial nasal prominence will continue to proliferate downward from the roof of the nasal cavity to fuse with the primary and secondary palate and form the nasal septum [16] (Fig. 2.3a–d). It is the failure of fusion between these facial prominences described above that results in the dysmorphology known as cleft lip and palate. This disruption of embryology is multifactorial, likely a result of genetic mutation and/or environmental insult in a susceptible host. The understanding of genetic mutation requires a basic understanding of cellular and molecular biology. Human embryological development requires cell-cell interaction to form intercellular communication through a cascade of signals that ultimately determines the fate of a cell [17]. Probably, the most important cell type to craniofacial mor-

phogenesis is neural crest cells (NCCs) [18]. Biological markers have aided our understanding of neural crest cells and how they affect embryological development. Neural crest cell subpopulations migrate through the frontonasal prominence and first pharyngeal arch. Neural crest cells establish the pattern of gene expression through signal transduction pathways that will determine the ectodermal- and mesenchymal-­ derived tissue for each pharyngeal arch [19]. It is theorized that inadequate migration or proliferation of NCC ectomesenchyme, excessive cell death, or disruption in the loss of cellto-­ cell adhesiveness plays a role in the establishment of cleft lip and palate [20]. Neural crest cells begin their life at the formation of the neural tube. Along the lateral aspects of the neural tube the ectoderm induces the development of neural crest cells [21]. This is mediated by bone morphogenetic proteins through Tgfß sig-

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a

b

c

d

e

Fig. 2.2 (a–e) Formation of nasal cavity and primitive choana. The sixth and seventh weeks show the invagination from the nasal pits to form the nasal cavity, break-­ down of the oronasal membrane, and the formation of the

primitive choana. The intermaxillary process moves posteriorly to form the primary palate (From Schoenwolf, Bleyl, Brauer, Francis-West. Larsen’s Human Embryology. Elsevier, 5th ed. Fig. 17.21. Chap. 17, p 454)

naling pathway. Bone morphogenic protein (BMP) signaling has been studied in detail [22]. BMP binds to a transmembrane receptor forming a receptor complex causing phosphorylation of proteins. This ultimately activates a transcriptional complex to turn on specific genes. It is theorized that this activation aids in migration of mesenchymal cells from the neural tube location. Furthermore, there is a conserved group of transcription factors (OTX2) [23] that aid in expression of Hox genes that play a role in neural crest cell migration through the pharyngeal

arches [24]. Neural crest cells from the segmented rhombomeres R1 and R2 at the hindbrain only migrate to the first pharyngeal arch. The continued expression from the Hox genes maintains their arch-specific migration [4, 10, 25]. This allows for neural crest cells from the first pharyngeal arch to differentiate to form connective, skeletal, dentin, and muscle tissue specific for the face [26]. Morphogenesis of the face additionally requires ectoderm-mesenchymal ­ communication. Fibroblast growth factor receptors through a tyrosine kinase signaling pathway

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a

b

c

d

Fig. 2.3 (a–d) Formation of secondary palate and nasal septum. The palatal shelves from the maxillary prominence grow vertical and will rotate horizontal during the eighth week to fuse with growing nasal septum. Fusion happens first at the midline, then fuse anterior and poste-

rior. It is the growth of the nasal septum that separates the nasal passages. Fusion is completed by the tenth week. (From Schoenwolf, Bleyl, Brauer, Francis-West. Larsen’s Human Embryology. Elsevier, 5th ed. Fig.  17.22. Chap. 17, p 455) [27]

turn on specific genes that work to interact with the underlying mesenchymal cells. Fibroblastic growth factors play a large role in facial morphogenesis. This mechanism is best seen in the development of craniosynostosis [28]. Expression of these receptors results in osteoblastic differentiation. This expression is important at the bone plates for intramembranous bony growth of the head; however, overexpression of these factors near the suture results in synostosis [29]. Finally, adhesion molecules, known as integrins, are proteins that must be expressed to aid in attachment and migration of neural crest cells. Integrins give cells information about their surroundings to allow the cell to know if it should attach, move, die or differentiate. If this pathway is impeded, final migration of neural crest cells may lead to craniofacial malformations [30, 31].

Cleft lip and palate malformations generally do not have other associated anomalies. However, isolated cleft palate and midline facial deformities can be a part of multiple primary malformations, otherwise known as a syndrome. And 13–50% of children with isolated cleft palate may have an underlying syndrome [32]. The two most common syndromes associated with isolated cleft palate are Stickler and 22q11.2 deletion [33]. The most common developmental midline defect is holoprosencephaly. Syndromic cleft palate is differentiated from non-syndromic cleft lip and palate due to their distinct etiologies. Syndromic cleft palate patients are more likely to have other associated anomalies and poorer speech outcomes [34]. Secondary velopharyngeal insufficency (VPI) surgeries, including palatal lengthening, pharyngeal and sphincter pharyngoplasty, and fat injections, are needed in 11%

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of the general cleft lip and palate population, but in as high as 36% in patients with 22q11.2 deletion anomalies [35]. Syndromic cleft patients can have poor oral-motor coordination, muscular hypotonia, connective tissue, nervous system, genetic/developmental disorders, and differing velopharyngeal anatomy that can affect velopharyngeal closure [36]. Anatomic evaluation of the velopharyngeal complex in patients with 22q11.2 syndrome has shown a shorter velum, a thinner levator veli palatini muscle, more obtuse anterior cranial base angle (Nasion-Sella-Basion), wider velopharyngeal width, and a larger pharyngeal airway volume [37]. 22q11.2 deletion syndrome is the most common chromosomal deletion, occurring in 1/4000 births. There are multiple genes within the deletion resulting in craniofacial and pharyngeal arch derivative abnormalities. The gene is crucial for the transcription and translation of certain transcription factors necessary for formation and development in the endoderm and mesoderm of the pharyngeal arches. One such transcription factor is T-box (Tbx1). Animal studies showing deletion of this transcription factor recapitulate the cardiac and craniofacial features of 22q11.2 syndrome [38]. Tbx1 loss of expression results in increased expression of BMP and retinoic acid [39, 40]. Retinoic acid is a known teratogen and overexpression causes hypoplasia of the pharyngeal arches. This is similar to the adverse effects of the medication Accutane (13-cis-retinoic acid) if taken during pregnancy [41]. Stickler syndrome is a group of conditions characterized by a distinctive facial appearance, eye abnormalities, hearing loss, and joint problems, most commonly resulting from mutations in collagens II and XI.  It is the most common syndrome seen in patients with Pierre Robin Sequence. There have been six genes linked to Stickler (COL2A1, COL11A1, COL11A2, COL9A1, COL9A2, COL9A3), but there are also patients with features of Stickler that do not express these gene mutations. Therefore, the diagnosis is primarily clinical. It is thought that the affected mutations in collagens that are expressed by chondrocytes lead to abnormal development of Meckel’s cartilage, resulting in decreased growth of the mandibular promi-

nences. Lack of mandibular growth does not allow the tongue to lower, the palatal shelves remain vertical, and the palate does not fuse resulting in a cleft palate [42]. Holoprosencephaly is the most common defect of the forebrain, affecting 1  in 16,000 births [43]. In holoprosencephaly there is only a single cerebral lobe with failure to form paired hemispheres [44]. This can be accompanied with facial midline defects including flat nose, ocular hypotelorism, and deficiencies arising from the frontonasal prominence. This results in a deficient or absent philtrum, premaxillary process, columella, nasal bones, and nasal septum, phenotypically appearing as midline cleft lip and palate and a single nostril [45]. The facial midline is also dependent on migration of neural crest cells. It is the regulation of genes through signaling pathways that affects this migration. The Sonic hedgehog (Shh) signaling pathway plays a large role in holoprosencephaly [46]. Shh is a secreted protein that interacts with transmembrane receptors. The presence or absence of Shh determines the transcriptional activity of targeted genes. Shh activation is required in midline facial development. Shh is expressed in the forebrain, subsequently activating Shh in the ectoderm of the frontonasal prominence [47]. The activation of Shh regulated genes aids in the migration of neural crest cells into the facial prominence resulting in development of the facial midline structures.

2.1 Anatomy 2.1.1 Unilateral Cleft Lip and Palate (UCLP) It is important to grasp the three-dimensionality of the cleft lip and palate defect. It affects the maxilla, nasal cartilages, mucosa, skin, muscle, and teeth. The cleft maxilla does not have the usual curve due to an under developed premaxilla and absent nasal floor. The lesser alveolar s­ egment of the cleft maxilla is displaced lateral, inferior, and posteriorly. The cleft maxilla is discontinuous at the location between adult lateral incisor and

2  Clinical and Diagnostic Anatomy

canine and extends through the hard and soft palate. This opening results in communication with the oral and nasal cavity. The cleft lip has loss of continuity of skin, vermillion, and mucosa. The mucosa and vermillion around the cleft is not attached to the underlying muscle of the lip, and is chronically exposed to the extra-­ oral environment, resulting in mucosa that is dry and thin. Often times it is recommended to use petroleum jelly to moisten this region until the surgical repair can be accomplished. Essential repair of the lip requires proportional width of dry vermillion lip to the non-cleft side to restore form. The nasal septum is composed of the perpendicular palate of the ethmoid bone, vomer, and septal cartilage. The nasal septum is deviated to the non-affected cleft side due to the incorrect insertion of the transverse muscles of the nose and the orbicularis (Fig.  2.4a, b). Upper and lower lateral cartilages aid in tip support and the nasal aperture. The base of the nose on the cleft side is positioned inferiorly resulting in flattening, widening, and depression of the lower lateral cartilage. Medial crural footplates attach to the nasal septum and anterior nasal spine which is deviated to the non-cleft side. This deformity is due to abnormal muscle attachment, not cartilaginous hypoplasia. The unilateral cleft lip and palate deformity represents disorientated facial musculature that contributes to facial asymmetry and functional deficits. This muscle dysmorphogenesis results in global facial deformity due to hypoplasia, hypofunction, and hypodevelopment of the muscle. The orbicularis oris muscle forms a sphincter around the oral cavity. The muscle is further subdivided into pars peripheralis and pars marginalis. The pars peripheralis decussation and dermal insertion contributes to the philtral anatomy and gives the appearance of lateral ridges [48]. The philtral dimple is due to a lack of dermal insertion [49]. The pars marginalis forms the white roll due to the curling of the muscle in the shape of a hook. Clefting results in failure to form this sphincter and hypoplasia of muscle near the cleft margin. Muscles that contribute to the nasal sill and proper nares symmetry include the nasalis, transverse nasalis, depressor septi nasi, levator

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labii superioris alaquae nasi, levator labii superioris, and minor zygomatic muscles. Nasalis and transverse nasalis action is to compress the nasal aperture and bring the alae and posterior part of the columella downward and laterally, respectively. The depressor septi action pulls the columella and nasal septum downward. Levator labii superioris alaeque nasi action raises and everts the upper lip and increases the curvature of the nasolabial fold. It also works to pull the lateral crus superiorly. The levator labii superioris acts to elevate and evert the upper lip. The minor zygomatic aids in the forming of the nasolabial fold. Facial clefting results in the disinsertion of these muscles and improper facial animation during function, which causes further distortion and asymmetry [50–52]. Disharmony of the face due to unilateral cleft lip and palate deserves comprehensive management. This includes primary cheilorhinoplasty, palatoplasty, and secondary bone grafting to cleft maxilla. During the mixed dentition it is common to see a cleft dental gap, residual oronasal fistula, residual bony defect, and nasal obstruction due to septal deviation. Scarring from previous surgeries can also result in maxillary hypoplasia in 16–75% of unilateral cleft lip and palate patients [53, 54]. In patients with cleft lip and palate the lateral incisor is normal in only about 7% of patients [55, 56]. When looking at correcting the dentofacial deformity in later adolescence, a combined orthodontic-orthognathic surgery must be considered. A modified LeFort I osteotomy may be done to advance the lesser segment one adult tooth. This will replace the lateral incisor with a canine. A modified LeFort I osteotomy will allow the surgeon to correct maxillary hypoplasia, close the cleft dental gap and oronasal fistula, and abolish the need for additional bone grafting in the cleft maxilla. An understanding of anatomy and blood supply to the maxilla to reduce surgical complications is necessary. If all cleft defects have previously been closed, a standard vestibular incision for proper visualization of maxilla and adequate downfracture is possible, and does not compromise the circulation of lesser and greater maxillary segments. A modification of incision design will be necessary if alveolar

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a

b

Fig. 2.4 (a) Diagram of abnormal muscle orientation in unilateral cleft lip deformity. (From Millard DR: The Unilateral Deformity. Cleft Craft 1976, p 27. http://calder.med.miami.

edu/Ralph_Millard/ebooks.html). (b) Intraoperative view demonstrating the abnormal muscle orientation of the orbicularis oris

clefting or fistulas are present. Downfracture of the maxilla will allow for exposure to repair the nasal septum, recontour pyriform rims, floor of the nose, and anterior nasal spine. Advancing the lesser segment to close the dental gap will allow for close approximation of the mucosa flaps. Closure of the oronasal fistula can be obtained without extensive subperiosteal undermining to the palatal flaps to avoid devascularization of the maxillary segment and under tension-free flaps. More can be read on this topic in Chap. 9.

of the medial crura. The alar cartilages are wide and flattened, due to the lateral pull from the distorted muscle attachments. The nasal septum is not subject to abnormal muscle pull and is symmetrical. The reconstruction of bilateral cleft lip, palate, and alveolus repair can be challenging (Fig. 2.5) [57]. The adolescent patient with bilateral cleft lip and palate suffers from similar issues of maxillary hypoplasia, bilateral cleft dental gap, residual oronasal fistulas, and residual bony defect of the cleft maxilla. The percentage of maxillary hypoplasia can be as high as 75% in this population and often can benefit from orthognathic surgery to correct the dentofacial deformity. Unfortunately, only about 15% of cleft teenagers will actually receive orthognathic surgery [58]. The three maxillary segments can be osteotomized, mobilized, and relocated to achieve functional and esthetic results. The premaxilla can be repositioned via a vomer osteotomy, but great care to maintain the blood supply to this segment is necessary to avoid necrosis. The blood supply ultimately comes to a small degree from the internal carotid, with branches to ophthalmic, anterior ethmoid artery, with terminal branches to the prolabium. The maxillary artery supplies the premaxilla through the posterior septal branch off the sphenopalatine artery. Due to the bilateral deformity, there is no anastomosis from facial and maxillary arteries. These small branches are likely to be injured during surgical reconstruc-

2.1.2 Bilateral Cleft Lip and Palate (BCLP) The facial anatomy of complete bilateral cleft lip is often more symmetric when compared to the unilateral deformity. The premaxilla can be rotated or displaced to the left or right and appear anteriorly protruded. Additional transverse development of the premaxilla results from excessive growth in the premaxillary-vomerine suture, lack of muscle stimulation, collapse of the lateral segments, and feeding techniques. The prolabium skin lacks a true vermillion, philtral ridges, dimple, and white roll due to the lack of orbicularis oris muscle. The orbicularis oris muscle is retained in the lateral labial elements and may have disinsertion into the alar base. The columella length is deficient and incorporated into the prolabium from inferior displacement

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Fig. 2.5 Abnormal muscle orientation in a bilateral cleft lip deformity can leave the premaxilla with unrestrained accentuated anterior growth from the nasal septum

tion, so the premaxilla becomes a dento-osseous-­ musculo-mucosal flap that maintains its blood supply via the labial pedicle. Attention to detail is necessary for good surgical outcomes [59].

2.1.3 Cleft Palate Anatomy of the cleft palate can vary greatly. As described above the palate first fuses in the middle and continues in an anterior and posterior direction. Therefore, the malformation can involve cleft of soft palate alone, hard and soft palate, involvement of the alveolus, and bilateral alveolus with hard/soft palate. The palatal mucosa has three distinct anatomical zones that contribute to the transverse growth of the maxilla. The three zones are the palatal, maxillary, and gingival fibro-mucosa. The palatal is toward the midline with the maxillary in the middle and gingival fibro-mucosa near the teeth. The palatal fibro-mucosa aids in the downward growth of the palatal shelves via bone deposition on the oral

side and resorption on the nasal side. This mucosa is reduced in the cleft palate patient; therefore, the growth of the nasal aperture and transverse growth of the palate are affected. Transverse growth is active up to the age of four, which is why some surgeons may argue delaying closure of the hard palate. This allows for establishing a functional velum without disrupting the palatal fibro-mucosa. The hard palate can be closed at 14–15 months and may require less palatal dissection due to narrowing of the palate under the influence of the lip, soft palate, and tongue. Cleft of the soft palate results in improper orientation of musculature sling necessary for proper speech [60]. The muscles attach to posterior aspect of the hard palate in a more anterior-­ posterior position versus the proper mediolateral orientation. The muscles that contribute to this sling include the levator and tensor veli palatini, palatoglossus, palatopharyngeus, and uvular muscles. In the normal palatal velum, the posterior aspect is formed by the levator palatine and palatopharyngeus muscles. When these muscles

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a

b

c

d

Fig. 2.6  Diagram showing a superior-based pharyngeal flap for treatment of velopharyngeal insufficiency. (a) Blue catheters are shown in lateral ports, and donor site is harvested (b) Recipient site is prepared and flap is inset (c) Flap is secured with sutures (d) Donor site is closed

and lateral ports verified. (From Sloan, Gerald. Posterior Pharyngeal Flap and Sphincter Pharyngoplasty: The State of the Art. Cleft Palate-Craniofacial Journal, March 200, Vol. 37 No. 2. Figure 2.1 , p 114)

contract, they raise and lengthen the soft palate to form velopharyngeal closure. The palatoglossus muscle goes from the posterior aspect of the palate to the lateral parts of the tongue and aids in extending the levator muscles. Closure of the soft palate requires re-orientation of these muscles to a transverse and posterior direction for proper velopharyngeal competence [61]. Despite adequate palatoplasty 25% of children with a repaired cleft palate may have some degree of velopharyngeal dysfunction [62]. Dysfunction can be due to either insufficiency with inadequate tissue or incompetence with inadequate function. Closure of the soft palate to the posterior pharynx requires the velum to move superior and posterior with the lateral walls of the pharynx moving medial. During speech the levator, palatopharyngeus, and superior constrictor of the pharynx must work in synchrony to permit the increase in intra-oral pressure to produce fricative and plosive consonants. If there is insufficient closure of the velopharyngeal port, surgery may be indicated.

The two most common secondary velopharyngeal insufficiency surgeries include superiorly based pharyngeal flap and dynamic sphincter pharyngoplasty. The pharyngeal flap is a superior pharyngeal constrictor myomucosal flap. The flap is inset into the soft palate to allow for competent velopharyngeal closure. The base of the flap is about the level of C2-C3 and thickness of the flap is to the prevertebral fascia. The width of the flap is based on the severity of deficient tissue in velopharyngeal port. The width of the flap must be thick enough to maintain blood supply, but not too thick to obliterate the lateral ports (Fig.  2.6). The innervation to the superior pharyngeal constrictor muscle is the pharyngeal branch of the vagus nerve. Dynamic sphincter pharyngoplasty is a superiorly based flap arising from palatopharyngeus muscle which forms the posterior tonsillar pillar. This is a bilateral myomucosal flap that is inset at the level of C1 down to prevertebral fascia, creating a single midline port which can open and close (Fig. 2.7). The flap is also innervated by the pharyngeal branch of the

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b

a

c

Fig. 2.7  Diagram showing dynamic sphincter pharyngoplasty for treatment of velopharyngeal insufficiency. (a) Blue catheters are shown in lateral ports as soft palate is retracted anteriorly (b) Bilateral myomucosal flaps are elevated and rotated (c) Flaps show sutured in place.

(From Sloan, Gerald. Posterior Pharyngeal Flap and Sphincter Pharyngoplasty: The State of the Art. Cleft Palate-Craniofacial Journal, March 200, Vol. 37 No. 2. Figure 2, p 116)

vagus nerve. The blood supply to both flaps comes from three branches off the external carotid: the pharyngeal and tonsillar branch off the ascending pharyngeal artery, tonsillar branch of lesser palatine artery which is from the maxillary artery, and ascending palatine from facial artery [63].

off the facial artery is the superior labial artery and is the blood supply to the upper lip. It is larger than the inferior labial artery and travels along the same course between the muscle and mucous membrane of the upper lip. It gives off an alar and septal branch that anastomosis with the blood supply of the nasal septum. These branches also help to form the philtral vascular arcade. They are located along the philtral columns and create a specific compartment of the upper lip helping its overall contour. The last branch of the facial artery is called the lateral nasal artery and travels along the side of the nose. It supplies blood to the alae and dorsum of the nose. The branch off the superficial temporal artery that contributes to the nose and upper lip is called the transverse facial artery. This artery transverses the parotid gland, crossing the masseter muscle between the parotid duct and zygomatic arch. It supplies blood to masseter, skin, and anastomoses with the facial artery. The maxillary artery is divided into three parts, the third from which

2.2 Vasculature Blood supply to the face is supplied from branches of the external carotid artery: facial, superficial temporal, and maxillary arteries (Fig.  2.8). Additional blood supply comes from the internal carotid artery via the ophthalmic arteries. The lower and upper lips are supplied by the facial artery. The second branch of the facial artery, which arises near the angle of the mouth, is called the inferior labial artery and supplies the lower lip. It runs between the orbicularis oris muscle and mucous membranes. The third branch

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Superior labial a.

Dorsal nasal a Terminal br. of ant. ethmoidal a.

Inferior labial a.

Angular a. Lateral nasal a.

Ascending septal br. of superior labial a. Facial a.

Inferior labial a.

Superior labial a.

Fig. 2.8  Diagram showing normal vasculature of the nose and upper lip. (From Millard DR: The Unilateral Deformity. Cleft Craft 1976, p 32. http://calder.med.miami.edu/Ralph_Millard/ebooks.html)

arises the infraorbital artery. This artery passes through infraorbital foramen to give blood to lateral aspect of the nose and upper lip. This artery anastomoses with the transverse facial, ophthalmic, and facial arteries. The maxillary artery also supplies vasculature to the hard and soft palate via the descending palatine artery which travels through the lesser and greater palatine foramen. The vessels at this point change name to lesser and greater palatine arteries and give blood supply to soft and secondary palate, respectively. The blood supply to the primary palate passes through the incisive canal at the incisive papilla. Once through the foramen the artery is called nasopalatine. This artery is from the sphenopalatine artery which is a branch off the maxillary artery. This artery also anastomosis with the greater palatine artery. The hard and soft palates also receive blood supply from ascending pharyngeal, palatine, and tonsillar arteries. The last contributing artery is the ophthalmic artery. The terminal branch of the artery is called the external nasal artery. This is a branch off the anterior eth-

moidal artery. It gives blood supply to the skin of the external nose. It also anastomoses with a septal branch off the superior labial artery. Understanding the vasculature is important when treating patients with cleft lip and palate, especially those with a bilateral deformity. It is well known that there is disruption to normal circulatory patterns, but it is not well described how the new vascular patterns offer blood supply to the premaxilla and prolabium. The bilateral complete cleft lip and palate deformity results in discontinuity of the superior labial artery from the philtrum and greater palatine artery with the premaxilla. The blood supply to the lateral segments of the upper lip is similar to that of the unilateral deformity. The superior labial artery runs along the labial margin and cleft edge and ends at the base of the ala. The prolabium and the premaxilla no longer receive blood supply from terminal branches of the superior labial artery. The prolabium receives blood supply from anterior ethmoid, nasal alar, and lateral nasal artery branches. The arteries traveling through the columella and

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Dorsal nasal a. Terminal br. of ant. athmoidal a. Angular a. Lateral nasal a. Ascending septal br. of superior labial a.

Superior labial a.

Facial a. Inferior labial a.

Fig. 2.9  Diagram of vasculature in the unilateral cleft lip anomaly. (From Millard DR: The Unilateral Deformity. Cleft Craft 1976, p 33. http://calder.med.miami.edu/Ralph_Millard/ebooks.html)

Fig. 2.10  Illustration of vasculature in the bilateral cleft lip anomaly, coronal view. Credits to Victoria Mañon for the illustration

dermal plexus are the sole blood supply to the prolabium and cannot be compromised during surgical repair. The premaxilla likely receives blood supply from the posterior septal artery off the sphenopalatine. Understanding the blood supply to the premaxilla and prolabium is important when performing any surgery on lip, palate, or alveolus in the bilateral cleft maxilla [64]. Surgeries performed during primary repair procedures directly influence vascular patterns and will contribute to healing, or lack thereof, of the secondary bone graft. Incision design during both primary and secondary surgeries is critical to

Fig. 2.11  Illustration of proposed vasculature to the premaxilla in the bilateral cleft lip anomaly, sagittal view. Credits to Victoria Mañon for the illustration

ensure blood supply is maintained, as hypoperfusion may be a much higher issue to bone graft failure and re-absorption than has been reported [65] (Figs. 2.9, 2.10, and 2.11). The blood supply to the complete cleft palate varies from the normal palate. The normal palate receives blood supply by six pairs of arteries: the greater palatine, lesser palatine, nasopalatine, ascending palatine, ascending pharyngeal, and tonsillar arteries. The blood supply of the complete cleft palate differs in such that the anterior branch of the ascending palatine artery becomes

38

smaller and the posterior branch has shifted forward and runs along the cleft edge. The palatine branch of the ascending pharyngeal does not run along the palato-pharyngeal muscle, but descends from the base of skull along the levator palatine muscle to the soft palate. Surgical dissection of the palatal muscles and posterior repositioning of the muscles are accomplished during repair in order to maximize speech outcomes. Vascular knowledge of the cleft palate is critical to prevent damage to palatal musculature which may lead to atrophy or scarring causing worsening speech outcomes.

2.3 Conclusion The most common facial malformation is cleft lip and palate. Understanding the embryology and intricate anatomy of the face is critical for the craniofacial surgeon who strives to improve surgical outcomes. Large advances have been ­ made in the understanding of dysmorphogenesis of the face, but the exact molecular mechanisms that lead to differentiation and proliferation of neural crest cells are still evolving. As laboratory scientists continue to increase their knowledge of molecular and gene biology, and as we continue to transition our knowledge from the laboratory to humans, there is hope in the future for prevention of these anomalies. Acknowledgements  Much appreciation for the illustrations by Victoria Mañon.

References 1. Mai CT, et al. National population-based estimates for major birth defects, 2010–2014. Birth Defects Res. 2019;111(18):1420–35. 2. Fogh-Anderson P.  Epidemiology and etiology of clefts. Birth Defects Orig Artic Ser. 1971;7(7):50–3. 3. Grevellec A, Tucker AS. The pharyngeal pouches and clefts: development, evolution, structure and derivatives. Semin Cell Dev Biol. 2010;21:325–32. 4. Kameda Y, et  al. Hes1 is required for the development of pharyngeal organs and survival of neural crest-­ derived mesenchymal cells in pharyngeal arches. Cell Tissue Res. 2013;353(1):9–25. https://

D. Wilson and P. Ricalde doi.org/10.1007/s00441-­013-­1649-­z. Epub 2013 May 19 5. Hickey SA, Scott GA, Traub P.  Defects of the first brachial cleft. J Laryng Otol. 1994;108:240–3. 6. Sperber GH, Sperber SM.  Pharyngogenesis. J Dent Assoc S Afr. 1996;51:777–82. 7. Graham A, Okabe M, Quinlan R.  The role of the endoderm in the development and evolution of the pharyngeal arches. J Anat. 2005;207:479–87. 8. Graham A, Smith A. Patterning the pharyngeal arches. BioEssays. 2001;23:54–61. 9. Kruchinskii GV. Classification of the syndromes of brachial arches 1 and 2. Acta Chir Plast. 1990;32:178–90. 10. Rizzoti K, et  al. SOX3 activity during pharyngeal segmentation is required for craniofacial morphogenesis. Development. 2007;134(19):3437–48. Epub 2007 Aug 29 11. Lee SK, Kim YS, Lim CY, Chi JG.  Prenatal growth patterns of the human maxilla. Acta Anat. 1992;145:1–10. 12. Bush JO, Jiang R. Palatogenesis: morphogenetic and molecular mechanism of secondary palate development. Development. 2012;139:231–43. 13. Burg ML, et al. Epidemiology, Etiology, and treatment of isolated cleft palate. Front Physiol. 2016;1(7):67. https://doi.org/10.3389/fphys.2016.00067. eCollection 2016 14. Greene RM, Linask KK, Pisano MM, et  al. Transmembrane and intracellular signal transduction during palatal ontogeny. J Craniofac Genet Dev Biol. 1991;11:262–76. 15. Kjaer I, Bach-Petersen S, Graem N, Kjaer T. Changes in human palatine bone location and tongue positioning during prenatal palatal closure. J Craniofac Genet Dev Biol. 1993;13:18–23. 16. Ferguson CA, Tuker AS, Sharpe PT. Temporospatial cell interactions regulating mandibular and maxillary arch pattering. Development. 2000;127:403–41. 17. Alberts B, Johnson A, Lewis J, Raff M, Roberts K, Walter P.  Molecular biology of THE CELL. 4th ed. New  York: Garland Science/Taylor&Francis Group; 2002. 18. Trainor PA.  Craniofacial birth defects: the role of neural crest cells in the etiology and pathogenesis of Treacher Collins syndrome and the potential for prevention. Am J Med Genet A. 2010;152A:2984–94. 19. Trainor PA, Tam PP. Cranial paraxial mesoderm and neural crest cells of the mouse embryo: co-­distribution in the craniofacial mesenchyme but distinct segregation in the branchial arches. Development. 1995;121:2569–82. 20. Minoux M, Antonarakis GS, Kmita M, et al. Rostral and caudal pharyngeal arches share a common neural crest ground pattern. Development. 2009;136:637–45. 21. Minoux M, Rijli F. Molecular mechanisms of cranial neural crest cell migration and patterning in craniofacial development. Developmental. 2010;137:2605–21. 22. Vielle-Grosjean I, Hunt P, Gulisano M, et al. Branchial HOX gene expression and human craniofacial development. Dev Biol. 1997;183:49–60.

2  Clinical and Diagnostic Anatomy 23. Zielinski D, et al. OTX2 duplication is implicated in hemifacial microsomia. PLoS One. 2014;9(5) 24. Fossat N, et  al. Temporal and spatial delineation of mouse Otx2 functions by conditional self-knockout. EMBO Rep. 2006;7:824–30. 25. Billmyre KK, et al. Sonic hedgehog from pharyngeal arch 1 epithelium is necessary for early mandibular arch cell survival and later cartilage condensation differentiation. Dev Dyn. 2015;244(4):564–76. https:// doi.org/10.1002/dvdy.24256. Epub 2015 Mar 13 26. Simoes-Costa M, Bronner M.  Establishing neural crest identity: a gene regulatory recipe. Development. 2015;142:242–57. 27. Schoenwolf GC, Bleyl SB, Brauer PR, Francis- West PH. Larsen’s human embryology. 5th ed. Philadelphia: Churchill Livingstone/ Elsevier; 2009. 28. Melville H, Wang Y, et al. Genetic basis of potential therapeutic strategies for craniosynostosis. Am J Med Genet A. 2010;(152A, 12):3007–15. 29. Wilkie AO. Craniosynostosis: genes and mechanisms. Hum Mol Genet. 1997;6(10):1647–56. 30. Cohen MM Jr. Malformations of the cranio- facial region: evolutionary, embryonic, genetic, and clinical perspectives. Am J Med Genet. 2002;115:245. 31. Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nat Rev Genet. 2011;12:167–78. 32. Impellizzeri A, et  al. Epidemiological characteristics of orofacial clefts and its associated congenital anomalies: retrospective study. BMC Oral Health. 2019;19(1):290. 33. Basta M, et al. A 35-year experience with syndromic cleft palate repair. Ann Plast Surg. 2014:1–4. 34. Sergouniotis PI, et  al. Agnathia-otocephaly complex and asymmetric velopharyngeal insufficiency due to an in-frame duplication in OTX2. J Hum Genet. 2015;60(4):199–202. 35. Kollar L, Baylis AL, Kirschner RE, et  al. Velopharyngeal structure and muscle variations in children with 22q11.2 deletion syndrome: an unsedated MRI study. Cleft Palate Craniofac J. 2019;56(9):1139–48. 36. Yagi H, et  al. Role of TBX1  in human del22q11.2 syndrome. Lancet. 2003;362(9393):1366–73. 37. Filip C, et al. Adults with 22q11.2 deletion syndrome have a different velopharyngeal anatomy with predisposition to velopharyngeal insufficiency. J Plastic Reconstr Anesthet Surg. 2017; 38. Passos-Bueno MR, Ornelas CC, Fanganiello RD.  Syndromes of the first and second pharyngeal arches: a review. Am J Med Genet A. 2009;149A:1853–9. 39. Zhang L, et al. TBX-1, a DiGeorge syndrome candidate gene, is inhibited by retinoic acid. Int J Dev Biol. 2006;50(1):55–61. 40. Haddad RA, et al. A case report of T-box 1 mutation causing phenotypic features of chromosome 22q11.2 deletion syndrome. Clin Diabetes Endocrinol. 2019;5:13.

39 41. Coberly S, et  al. Retinoic acid embryopathy: case report and review of literature. Pediatr Pathol Lab Med. 1996;16(5):823–36. 42. Robin N, et al. Stickler Syndrome. In: GeneReviews. Seattle: University of Washington; 2021. p. 1993–2017. 43. Honey EM, et al. Holoprosencephaly with clefts: data of 85 patients, treatment and outcome: part 1: history, subdivisions, and data on 85 Holoprosencephalic cleft patients. Ann Maxillofac Surg. 2019;9(1):140–5. 44. Sagai T, et al. SHH signaling mediated by a prechordal and brain enhancer controls forebrain organization. Proc Natl Acad Sci U S A. 2019;116(47):23636–42. 45. Kjaer I, Keeling J, Russell B, et  al. Palatal structure in human holoprosencephaly correlates with the facial malformation and demonstrates a new palatal developmental field. Am J Med Genet. 1997;73:387–92. 46. Hu D, Helms JA.  The role of sonic hedgehog in normal and abnormal craniofacial morphogenesis. Development. 1999;126(21):4873–84. 47. Roessler E, et  al. Mutations in the human sonic hedgehog gene cause holoprosencephaly. Nat Genet. 1996;14(3):357–60. 48. Rogers C, et  al. The philtrum in cleft lip: review of anatomy and techniques of construction. J Craniofac Surg. 2014;25:9–13. 49. Mitchell CA, et  al. The philtrum: anatomical observations from a new perspective. Plast Reconstr Surg. 2008;122:1756–60. 50. Fara M.  The anatomy of cleft lip. Clin Plast Surg. 1975;2(2):205–14. 51. Proff P, et  al. Morphofunctional changes of orofacial muscles in patients with unilateral or bilateral cleft lip, alveolus and palate. Ann Anat. 2007;189: 203–7. 52. Sambasivan R, Kuratani S, Tajbakhsh S.  An eye on the head: the development and evolution of craniofacial muscle. Development. 2011;138:2401–15. 53. Kornbluth M, et  al. Active presurgical infant Orthopedics for unilateral cleft lip and palate: inter-­ Center outcome comparison of Latham, modified McNeil, and Nasoalveolar Molding. Cleft Palate Craniofac J. 2018;55(5):639–48. 54. Michelle Kornbluth DMD, et  al. Active presurgical infant Orthopedics for unilateral cleft lip and palate: inter-Center outcome comparison of Latham, modified McNeil, and Nasoalveolar Molding. Cleft Palate Craniofac J. 2018;55(5):639–48. 55. Celikoglu M, et  al. Maxillary dental anomalies in patients with cleft lip and palate: a cone beam computer tomography study. J Clin Pediatr Dent. 2015;39(2):183–6. 56. De Stefani A, et al. Prevalence of hypodontia in unilateral and bilateral cleft lip and palate patients inside and outside the cleft area: a case-control study. J Clin Pediatr Dent. 2019;43(2):126–30. 57. Zhang JX, Arneja JS.  Evidence-based medicine: the bilateral cleft lip repair. Plast Reconstr Surg. 2017;140(1):152–65.

40 58. Ricalde P, Posnick JC.  Cleft-orthognathic surgery. Clin Plast Surg. 2004;31(2):315–30. 59. Posnick J.  Craniofacial and maxillofacial surgery in children and young adults (2-volume set). London: Elsevier; 2000. 60. Markus AF, et al. Primary closure of the cleft palate: a functional approach. Br J Oral Maxillofac Surg. 1993;31:71–7. 61. Cohen SR, Chen L, Trotman CA, Burdi AR.  Soft palate myogenesis: a developmental field paradigm. Cleft Palate Craniofac J. 1993;30:441–6. 62. Inman DS, et  al. Oro-nasal fistula development and velopharyngeal insufficiency following primary cleft

D. Wilson and P. Ricalde palate surgery--an audit of 148 children born between 1985 and 1997. Br J Plast Surg. 2005;58(8):1051–4. Epub 2005 Aug 8 63. Sloan G. Posterior pharyngeal flap and sphincter pharyngoplasty: the state of the art. Cleft Palate Craniofac J. 2000;37(2) 64. Zhang KQ. Artery supply of the lip and palate in normal and cleft patients. Zhonghua Kou Qiang Yi Xue Za Zhi. 1994;29(1):30–3–63. 65. Schultze U.  The blood supply of a unilateral and a bilateral cleft lip-maxilla-palate. Anat Anz. 1969;124(2):133–41.

3

Clinical and Diagnostic Findings During Mixed Dentition Imran Ahson and Pat Ricalde

Patients who are born with cleft lip and palate have often been through at least two surgical interventions by the time they reach the mixed dentition years. Therefore, whether due to the original deformity, nonsurgical interventions, or surgical influences, there will be differences in their clinical and radiographic findings compared to normal anatomy. These differences will vary based on the timing, technique, and number of previous interventions. It is important to perform a comprehensive history and physical examination to understand each patient and his/her respective differences. Observations from this presurgical evaluation can be divided into two categories: clinical findings and diagnostic findings. Taken altogether, they create an overall understanding of the patient. Once we understand our patient, we can give Supplementary Information The online version contains supplementary material available at https://doi. org/10.1007/978-­3-­031-­24636-­4_3.

I. Ahson Private Practice, New Hampshire Oral and Maxillofacial Surgery, Nashua, NH, USA Tufts University Medical Center, Boston, MA, USA P. Ricalde (*) Craniomaxillofacial Surgery, Florida Craniofacial Institute, Tampa, FL, USA Tampabay Cleft and Craniofacial Center, Tampa, FL, USA University of South Florida, Tampa, FL, USA e-mail: [email protected]

effective and predictable treatment recommendations. In this chapter we outline the clinical and diagnostic findings using case examples that will aid the surgeon in synthesizing this holistic view of the patient. The case examples for this chapter were selected because of their controversial management and unique challenges.

3.1 Clinical Findings For those patients who have not undergone surgical or nonsurgical intervention, it is helpful to see the congenital (unoperated) anomaly in order to make initial impressions about musculature and blood supply and to carefully consider each subsequent intervention. As the patient grows, the treatment that has been rendered will distort the clinical presentation. The physical exam should not be limited to clinical findings that affect surgical intervention but must also include issues that affect quality of life for the patient. The evaluation of a cleft-affected child during the mixed dentition is an opportunity to discuss the patient’s overall well-being including speech, mastication, and psychologic aspects of care before delving into dental and surgical issues.

3.1.1 Nonsurgical Interventions Nonsurgical procedures, including presurgical appliances and orthodontic devices, can alter the

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Fig. 3.1  Use of a Latham appliance in infancy led to unusual soft tissue changes on the dorsum of the tongue in this child. She had no previous surgery on the tongue

facial anatomy and create scar tissue in unsuspecting areas (Fig. 3.1). Equally important, previous nonsurgical interventions may have altered the patient and family’s expectations, trust in medical procedures, self-esteem, or their financial situation. Questions regarding previous orthodontic maneuvers should include how long the patient wore appliances. A patient who has already endured many medical, dental, or therapy visits will present very differently than one with little exposure to the medical arena [1].

3.1.2 Primary Cleft Lip Surgery There are hundreds of techniques that allow for the repair of a cleft lip, all of which will alter the vascular anatomy of the region and will change the native architecture of the tissues. An important factor to consider when planning technical aspects of surgery during the mixed dentition is the blood supply, and how it may have been altered due to

I. Ahson and P. Ricalde

the primary lip repair. Understanding the original deformity as well as subsequent cleft lip and palate repairs can give much information, especially in bilateral cases. For patients with unilateral cleft lip, most surgeons perform a variation of the Millard cleft lip repair, a straight-line repair, or an anatomic subunit approach [2] (Fig.  3.2). These procedures may disrupt the blood supply to the alveolus and teeth, especially if paired with a gingivoperiosteoplasty (GPP). The scars typically mature with grace but have variable effects to the aesthetics of the lip and nose, and to the development of the alveolus, maxilla, and dentition. The oral vestibule and cleft maxilla will need to be definitively managed later in life. Gingivoperiosteoplasty may close soft tissue fistulas but may not completely close the cleft bone defect. The procedure can create soft tissue/vascular/bone changes in the anterior maxilla that clinical exam alone is not adequate to assess. Exam may reveal intercanine collapse, partial ossification of alveolus, or scar tissue within the alveolus. This may present as a periodontal defect or with complex dental impactions later in life [3–5]. It is thought that the elevation of periosteum during a gingivoperiosteoplasty takes advantage of the osteoinductive nature of these tissues and possibly allows for bone formation to occur to avoid later bone grafting [6]. However, the lifting of periosteum may also disrupt the fine plexus of blood vessels and the nourishment that is provided to the alveolus from the periosteum and can create injury to teeth and growth issues to the anterior maxilla [7]. For this reason, many surgeons prefer to use a supraperiosteal dissection across the maxilla to advance the lip in wide clefts, and during nasal repairs, and minimize doing any surgery on the tooth bearing portion of the alveolus at this delicate early stage in life [8]. There is a contrary belief system that maintaining blood supply to the periosteum is of paramount importance, and perhaps a subperiosteal approach is better [9]. These technical details have not been scientifically validated, but it is likely that both create some degree of growth restriction to the area. Primary rhinoplasty procedures also can be disruptive. Whether due to direct injury or due

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Fig. 3.2  Illustration of Millard rotation advancement technique for repair of unilateral cleft lip

to alteration of the microvasculature, the nasal elements can undergo significant variation after primary repair [9]. This may be greater in bilateral cases, since the blood supply is more vulnerable and does not anastomose across the facial artery. Tissues must be meticulously managed during surgery for bilateral cleft lip surgery. Even in incomplete bilateral clefts, the blood supply may be altered, and soft tissue flaps must be considered carefully to avoid iatrogenic injury. Further detail can be obtained in Chap. 1.

Case Example Nine-year-old referred by his treating cleft team for second opinion regarding failed alveolar bone graft. It was thought the graft had failed due to infection. He was born with bilateral cleft lip and palate and had previously undergone bilateral cleft lip repair, palatoplasty, revision palatoplasty, as well as bilateral alveolar bone grafting from posterior iliac crest donor site. On presentation he was noted to have dehiscence of the orbicularis oris muscle, a large palatal fistula, and near

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a

b

c

d

Fig. 3.3 (a–d) Facial and oral views of patient with near total atrophy of the premaxilla due to hypoperfusion induced by previous surgical repairs

total atrophy of the premaxilla. The bone graft failure and premaxillary atrophy was not likely due to infectious origin; rather, a result of hypoperfusion due to lack of blood supply from previous overzealous surgical procedures (Fig. 3.3a–d).

3.1.3 Primary Cleft Palate Surgery There are four most-performed cleft palate repair techniques, which are often modified by surgeons based on training and experience [10] (Fig. 3.4a– d). A Bardach repair allows for the medialization of nasal and oral palatal flaps after mobilization and primary rotation and repair of the musculature. Typically, all wounds are primarily closed at

the conclusion of the procedure. A Von Langenbeck palatoplasty utilizes tunneling of the tissues through side ports, whereby the anterior mucosa is not released. A Furlow repair creates a double opposing z-plasty of the soft palate tissues and is combined with hard palate closure techniques per the individual surgeon. There is often a region that does not have primary closure that is left for granulation. A Veau-Wardill-Kilner procedure (VWK) allows for the posterior pushback of palatal tissues to try and elongate the soft palate and leaves a large area of denuded bone anteriorly. Although there are conflicting reports regarding best outcomes, the VWK has the distinction of highest complication rates regarding fistulas and growth restriction [11, 12].

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4

d

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4

Fig. 3.4 (a–d) Illustration of the four most common techniques for palatoplasty. (a) Bardach. (b) Von Langenbeck. (c) Furlow. (d) Veau-Wardill-Kilner

3.1.4 Oral Soft Tissue Normal oral mucosa is composed of various types of tissues with a distinct epithelium designed for the different areas of the mouth in which it covers. Attached gingiva is composed of keratinized tissue that firmly adheres to the surrounding teeth and bone, enabling it to withstand the forces of mastication. Alveolar mucosa is nonkeratinized, loose, and mobile, enabling it to shift and adapt with the contracting musculature during speech and mastication. This layer of mucosa is continuous with the rest of the oral mucosa and lip. The attached gingiva becomes the sulcular epithelium, which is nonkeratinized and lines the periodontal pocket. Just apical to the sulcular epithelium is junctional epithelium, a nonkeratinized stratified squamous epithelium that attaches to enamel, dentin, or cementum by way of epithelial attachment with hemidesmosomes. It is 0.97 mm in height and is an important

Fig. 3.5  Illustration of biologic width

part of the biologic width. Biologic width is the natural distance between the base of the gingival sulcus and the height of the alveolar bone, and usually measures 2.04 mm in height (Fig. 3.5). Tissues of the oral mucosa can be significantly altered after surgery. Ideally, attached gingiva will be found around the alveolus, connecting to

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the alveolar mucosa into the vestibule, with plenty of laxity to allow for saliva to distribute and moisten and protect the dentition. Shortening of the vestibule due to scar tissue or finger flaps can directly lead to compromised dentition. The vermillion is composed of wet and dry mucosae, which behave differently. If wet vermillion mucosa is used to reconstruct dry vermillion mucosal surfaces, there is often chronic crusting/ irritation of the area. Or, if skin is utilized to reconstruct mucosal surfaces, hair may grow into the mouth. Tissue characteristics should be carefully noted so that tissue types are used appropriately, and simultaneous correction of mismatched tissues can be addressed during bone grafting if needed. With surgery, natural characteristics are altered and scar tissue forms. Scar tissue is not a surgeon’s friend. With every operation scar tissue becomes more devilish and can expand, thicken, and disrupt blood supply and healthy maxillary growth. Even aggressive orthodontic and dental treatments can encourage this beast to grow. The best results are often those with fewest interventions, thoughtful planning, and meticulous technique, to minimize scar formation rather than attempts to debulk or remove it later. At times, letting scars mature naturally offer the best results. Other times, a hypertrophic scar can be managed with steroid injections. Case Example Nine-month-old presented for evaluation of his hypertrophic scar. He was born with left unilateral cleft lip and palate and underwent cleft lip/nasal repair at 3 months of age by another surgeon. The a

b

Fig. 3.6 (a–c) Nine-month-old with hypertrophic scar after cleft lip/ nasal surgery by another surgeon (at age 3 months) resulting in left nares obstruction. (a) Facial view on presentation. (b) Facial view 2 months after first steroid injection. Nostril is mostly patent and snoring has decreased significantly. (c) Facial view almost 4  years

resultant deformity led to obstructive nasal breathing and sleep apnea, due to his obligate nasal breathing. Two rounds of steroids were injected into the scar, one at the age of 12 months (during his palatoplasty) and again at 14 months of age (simultaneous with a sedated hearing test); therefore, surgical revision was avoided at this time, as was an additional general anesthetic. A formal lip/nasal revision at the time of his bone graft is planned (Fig. 3.6a–c).

3.1.5 Fistulas A palatal fistula is a known complication after palatoplasty for correction of a cleft palate, with an incidence at our institution of less than 0.5%, but documented in the literature up to 35% [13, 14]. Depending on the location, fistulas can increase the complexity of the alveolar cleft repair. Fistulas occur due to a variety of causes, but with an incidence that is so variable it must be considered that technical errors may have a much larger role than is currently described in the literature. In the U.S. there are no national standards for education and training of cleft surgeons, and no mandatory reporting of complications. It is therefore up to cleft teams and their surgeons to assess their own outcomes and encourage themselves to make improvements. Fistulas are particularly difficult to diagnose and challenging to manage [15, 16]. Children may squirm, normal mucosal contours and ridges may mask slit like fistulas, and erupting teeth can make perfect hiding spots for a fistula. With the emergence of gingivoperiosteoplasty (GPP), “hidden fistulas” are c

later. He received a total of two rounds of steroid injections, the first at age 12  months, the second at age 14 months. No surgery has been performed. Surgical revision has been delayed so as to coincide with bone grafting

3  Clinical and Diagnostic Findings During Mixed Dentition

even more common. This term is used by the senior author to describe an alveolar cleft palatal fistula that has been covered by gingiva, but a bone defect remains. They can also be described as complex periodontal defects that are a result of inadequate three-dimensional cleft maxillary repair. A hidden fistula is just as important to diagnose and manage as is a true (trans-mucosal) fistula. These fistula require a radiographic imaging study for diagnosis. Although the gold standard evaluation of a cleft maxillary fistula is with a maxillary occlusal X-ray, hidden fistula tend to be complex and often benefit from a cone beam computerized tomography scan (CBCT) to threedimensionally analyze the bone adjacent to impacted teeth [17]. Occasionally, a radiopaque identifying locator (such as gutta percha) can be utilized. It can assist in locating fistula in the hard palate, where even X-rays may miss detail, and to better detail the path of a fistula (Fig.  3.7, Video 3.1).

Fig. 3.7  Gutta percha points that curl back indicate there is no fistula present

a

b

Fig. 3.8 (a–c) Ten-year-old patient with complex impacted teeth #10, 11 due to previous gingivoperiosteoplasty. The cleft defect was elusive due to lack of soft tissue assist with feeding or with. (a) Panorex on initial

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Case Example A 10-year-old presents for second opinion after being followed by her cleft team for years. She was born with left unilateral cleft lip and palate. In infancy she underwent a primary cheilorhinoplasty, followed by a palatoplasty with GPP. On presentation there was no soft tissue fistulas. However, radiographic examination reveals a “hidden fistula”, or an incomplete alveolar cleft. CT scan demonstrates the lateral and canines have erupted into the defect. These defects can easily be missed on plain films; so patients must be inspected with a high index of suspicion. This patient eventually lost teeth #10, 11 (Fig. 3.8a–c).

3.1.6 Teeth in Cleft It is important to carefully inspect teeth in the cleft site. There may be missing teeth, supernumerary teeth, or malformed teeth, all of which can alter treatment plans [18]. Teeth need to be analyzed in respect to the overall size and shape of the maxilla and mandible, which may be too small to accommodate them. Typically, the final occlusal scheme is decided early in development, preferably during early mixed dentition. This includes whether the patient will be treated with orthodontics alone or will be treated with combined orthognathic surgery. Management of cleft dental gaps should also be carefully considered and will be discussed further in Chaps. 9 and 10. c

presentation. (b) Computer tomography axial slice demonstrating the cleft alveolus with teeth erupted into defect. (c) Intraoperative view demonstrating tissue in the cleft defect rather than bone

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a

b

c

Fig. 3.9 (a–c) Nine-year-old born with bilateral cleft lip and palate, underwent 12 procedures related to cleft lip and palate by another cleft team. Patient presented for a second opinion, and exam revealed ectopic eruption of #6

into the cleft defect and through the nasal floor. (a) Facial view demonstrating thick scars due to multiple revisions. (b) Occlusal view demonstrating tight vestibule, ectopic eruption of teeth, unrepaired alveolar cleft. (c)

Case Example Nine-year-old presented for second opinion after being followed by another cleft team since birth. He was born with bilateral cleft lip and palate and had undergone general anesthesia 12 times for procedures related to his cleft lip and palate. These included lip repair, palate repair, lip revisions, palate revisions, rhinoplasties, but was never bone grafted. On presentation he wore orthodontic appliances, but communication with the orthodontist confirmed that a clear treatment plan regarding his final occlusal scheme was not in place. Exam revealed tooth #6 had erupted horizontally into the cleft and through nasal floor with a poor prognosis. The patient had been seen several times by his cleft team and was offered lip and nasal revisions. The orthodontist and surgeon both thought the other person was addressing the impacted canine. Clear and deliberate conversations during the early mixed dentition may have improved the patient’s oral health, decreased unnecessary general anesthetics, improved patient self-esteem, and led to a better outcome (Fig. 3.9a–c).

the result of iatrogenic consequence. The final occlusal scheme must be considered at this mixed dentition stage so that an appropriate plan is set in motion to minimize unnecessary time in orthodontics and to eliminate fruitless procedures. The size of the greater and lesser segments should be considered while teeth are erupting, consideration made for serial extractions of teeth if needed in order to facilitate eruption and minimize abnormal tipping and flaring of teeth, which can result in instability, unhealthy periodontium, and relapse.

3.1.7 Malocclusion Patients with cleft lip and palate can suffer with a malocclusion that is genetically predisposed, or

Case Example Male born with Treacher Collins Syndrome and unilateral cleft lip and palate. After successful alveolar bone grafting and orthodontic expansion, his left maxillary canine remained impacted. In addition, he had skeletal bimaxillary retrusion with a dental class III relationship. The cleft team and family agreed he would undergo orthognathic surgery during the teenage years. It was decided to sacrifice #11 to avoid prolonged orthodontics during the mixed dentition, and to delay the orthodontic appliances until the teenage years. Once his permanent teeth erupted and he reached skeletal maturity, he underwent orthognathic surgery (Fig. 3.10a–e).

3  Clinical and Diagnostic Findings During Mixed Dentition

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a

b

Fig. 3.10 (a–e) Male born with Treacher Collins Syndrome and unilateral cleft lip and palate. Upon completion of alveolar bone grafting and orthodontic expansion, the left maxillary canine was impacted. After much deliberation, the cleft team elected to extract #11 rather than to prolong his time in orthodontics. At age 15 he

c

began stage 2 orthodontics and underwent orthognathic surgery at the age of 16. (a) Age 10, Facial and oral views. (b) Age 10, Panoramic X-ray. (c) Age 16, Panoramic X-ray. (d) Age 16, facial and oral views before orthognathic surgery. (e) Facial and oral views after orthognathic surgery

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d

e

Fig. 3.10 (continued)

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3  Clinical and Diagnostic Findings During Mixed Dentition

3.1.8 Speech This is an ideal time to do a final speech assessment. If there are any concerns about velopharyngeal incompetence, further diagnostic work-up may be prudent so that surgical intervention can be combined with alveolar cleft bone grafting.

3.1.9 Nasal Deformity As with all patients, the best time to perform rhinoplasty procedures is after growth cessation. The cleft nasal deformity at times is quite disabling, causing nasal obstructive breathing issues or significant psychosocial stigmata, and can benefit from revision in childhood. However, many surgeons are quick to recommend scar and nasal revisions, probably due to the discomfort it causes them, rather than due to complaints from the patient. This increases revision rates and the burden of care to patients and families [1, 19, 20]. The mixed dentition is a time to pause, reflect on our biases as cleft providers, and allow the child and parent to guide the conversation. If a decision is made to do a nasal revision, it should be coordinated along with or after the bone grafting procedure if possible. This allows for decreased general anesthetic exposure and time for recovery. Often nasal revisions will benefit from the stable foundation of a well-executed alveolar bone graft done (Fig. 3.6a–c).

3.1.10 Sleep Disorders Patients with cleft lip and palate are at risk for sleeprelated breathing disorders (SDB) [21]. They should

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be carefully screened for these issues and managed appropriately. If a patient is known to have obstructive sleep apnea, that may alter treatment planning significantly. Children with SDB may be poor candidates for certain orthodontic/orthopedic treatment plans that create clockwise rotation of the occlusal plane. Although there is certainly room for ongoing research, there is concern about the effects on airway space due to these mechanics. One must also be mindful of dental extractions and how they will alter the final treatment plan in these situations. For example, it is often unwise to remove mandibular bicuspids and retract incisors in a class III case in a cleft patient. Perhaps a maxillary advancement or even maxillomandibular advancement will be favorable [22]. If children are found to have obstructive nasal breathing issues contributing to SDB, a simultaneous conservative septoplasty, reduction of bilateral inferior turbinates, or even a rhinoplasty may be considered [23]. Conservative management with continuous positive airway pressure while sleeping can be effective in management of the sleep disorder, but can have further deleterious effects on maxillomandibular growth in children. This can accelerate the cycle of worsening SDB [24–27]. Case Example Fourteen-year-old born with unilateral cleft lip and palate, maxillary hypoplasia, and class III malocclusion. She was treatment planned for lower bicuspid extractions and compensatory orthodontics by a different cleft team to avoid orthognathic surgery. She presented for a second opinion and was diagnosed with sleep-disordered breathing. She instead underwent decompensation orthodontics followed by Lefort I maxillary advancement (Fig. 3.11a–c).

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a

b

c

Fig. 3.11 (a–c) Fourteen-year-old born with unilateral cleft lip and palate. (a) Occlusal view demonstrating class 3 malocclusion. (b) Facial and profile views before and

3 years after Lefort I maxillary advancement (c) Occlusal views 3 years after Lefort I maxillary advancement, demonstrating canine substitution

3  Clinical and Diagnostic Findings During Mixed Dentition

3.2 Diagnostic Findings After obtaining a comprehensive history and physical examination from patient and family, diagnostic studies should be considered. There are various studies that can be helpful, but not all will be necessary for each patient. Only objective, standardized tests with results that will impact decision making should be undertaken. If the test result will not affect change, the burden to patient and risk of burn out outweighs the benefit of the study.

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Fluoroscopy is simultaneously employed, and the velum is assessed in the coronal and sagittal planes. Closure patterns as well as distance of velum to pharyngeal wall can be measured. Velar movement angle and change in genu angles can give information regarding closure function. Due to radiation, this evaluation is less common than the nasendoscopic evaluation [31–33].

3.2.4 Speech Bulb

A child born with cleft lip and palate, who presents during mixed dentition for alveolar bone grafting, often has persistent abnormal speech articulation or resonance issues that may be related to the cleft anatomy. A formal speech evaluation by a certified speech and language pathologist may be helpful to determine if a fistulas, velopharyngeal insufficiency, or malocclusion may be contributing factors that can be simultaneously addressed during this phase of reconstruction.

Typically reserved for adults, a speech bulb can be helpful in older children with complex speech anomalies. It is commonly used for diagnostic purposes but can be therapeutic if tolerated. A speech bulb is an appliance created by a dentist or prosthodontist that looks like a maxillary denture with extra material that projects beyond the vibrating line and into soft palate. The acrylic extension allows for support of the soft palate, for assistance in cases of neuromuscular weakness (as in patients with 22Q deletion anomalies), or in multiply operated patients with thick scar tissue and dysfunctional palates who may not be good surgical candidates [34].

3.2.2 Nasopharyngoscopy

3.2.5 Palatal Obturator

Nasendoscopic evaluation of the velopharyngeal port during speech is helpful in patients with hypernasality or nasal air emission. Distance to the soft palate, lateral wall movement, posterior wall architecture, and size of tonsils and adenoids can all be assessed. It can be stressful for children to participate in the speech exam while an endoscope is in place, but with lots of patience and positive reinforcement, the study can be accomplished in most 5-year-olds [28–30].

These appliances can be made by a dentist or orthodontist and can be valuable in obliterating palatal fistulas to assist with feeding or with comprehensive speech analysis. At times they can also be used to treat speech disorders either temporarily until surgery can be planned or permanently in complex situations [35]. If needed, teeth can be incorporated into the design of the obturator as well. Palatal obturators have many disadvantages, including patient compliance, need for revisions, irritation to tissues, and hygiene concerns. However, in some instances they remain an integral part of cleft rehabilitation [36, 37].

3.2.1 Clinical Speech Evaluation

3.2.3 Videofluoroscopic Evaluation A videofluoroscopic evaluation of the nasopharynx is another way to assess velopharyngeal function. A small amount of contrast is placed into the nose, and as it bathes the nasopharynx the child is asked to go through a speech sample.

3.2.6 Radiographic Studies The gold standard for assessment of cleft alveolar defects during the mixed dentition is a maxillary

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Examination Rectangular collimation posterior bitewing Rectangular collimation FMX Round collimation FMX (F-speed film) Panoramic Cephalometric Cone beam CT head CT abdomen Chest X-ray

Effective dose (μSv) 5.0

Equivalent Background Exposure (days) 0.6

35

4

171

21

9–26 3–6 20–599 2000 10,000 20

1–3 0.5–1 3–75 105 3 years 2

occlusal radiograph [38]. Many centers opt for cone beam computed tomography, which has the advantage of visualizing the defects in three dimensions but has a disadvantage of greater radiation exposure. The differences in radiation dosage using various modalities are seen in Table 3.1.

3.2.7 Two-Dimensional Imaging Two-dimensional imaging modalities include panoramic, periapical, and occlusal radiographs. Cleft patients are usually monitored closely by their pediatric dentist, and all three may be available. Generally, the gold standard for evaluating a patient’s alveolar cleft is the maxillary occlusal radiograph. This image allows us to view the size of the cleft, presence of supernumerary or malformed teeth, as well as the eruption of the permanent dentition. The maxillary occlusal radiograph is taken with the patient seated upright, and the film placed across the occlusal surfaces of the teeth. The X-ray beam is then angled perpendicular to a line that bisects the long axis of the tooth and the axis of the film (Fig. 3.12). The beam should be centered through the tip of the nose and use a large enough sensor or film

to capture the entirety of the anterior dentition. This method of evaluation has been used for decades, with multiple countries standardizing the postoperative and preoperative evaluation to include a maxillary occlusal film. In addition, there are multiple standardized assessment methods based around the maxillary occlusal radiograph to evaluate the grafting outcomes. The most common methods are the Bergland Index, the Kindelan Scale, the Chelsea Scale, and the Standardized Way to Assess a reconstructed alveolar cleft ridge (SWAG scale) [38, 39] The Bergland Index grades the graft outcome into four types compared to normal alveolar bone height: Type I is normal alveolar bone height; Type 2 is >75% of normal bone height; Type III is  75%, Grade II 75–50%, Grade III 75% from an apical direction. Group D includes presence of bone tissue across >50% of both roots from an apical direction. Group E includes the presence of a bridge-like bone tissue in any area of the cleft except apically and coronally. Group F includes