Spina Bifida and Cranium Bifidum: Papers Reprinted from the New England Journal of Medicine, with the Addition of a Comprehensive Bibliography [Reprint 2014 ed.] 9780674599086

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Spina Bifida and Cranium Bifidum Papers reprinted from the New England Journal of Medicine with The Addition of a Comprehensive Bibliography From the Department of Surgery of The Children's Hospital, and Harvard Medical School

Boston

BY

F R A N C D. INGRAHAM, M.D. Assistant Professor of Surgery, Harvard Medical School Neurosurgeon, The Children's Hospital Senior Associate in Neurosurgery, Peter Bent Brigham Hospital

WITH THE COLLABORATION OF H E N R Y SWAN, M . D . ,

Fellow

HANNIBAL HAMLIN, M . D . , JOHN J. L O W R E Y , M . D . ,

in

Fellow

DONALD D . MATSON, M . D . ,

C H I L D R E N ' S

in

in

Fellow

H . W I L L I A M SCOTT, JR., M . D . ,

T H E

Neurosurgery

Fellow

Neurosurgery Neurosurgery

in

Resident

HOSPITAL,

HARVARD U N I V E R S I T Y PRESS, CAMBRIDGE,

Neurosurgery in

Surgery

B O S T O N MASSACHUSETTS

COPYRIGHT, 1 9 4 3 ,

I944

B Y T H E MASSACHUSETTS MEDICAL SOCIETY FRANC DOUGLAS INGRAHAM

PRINTED AT T H E HARVARD UNIVERSITY PRINTING O F F I C E CAMBRIDGE, MASSACHUSETTS, U.S.A.

CONTENTS I.

A

SURVEY OF F I V E H U N D R E D AND FORTY-SIX CASES

I

FRANC D . INGRAHAM AND HENRY S W A N II.

SURGICAL T R E A T M E N T

17

FRANC D . INGRAHAM AND HANNIBAL H A M L I N III.

O C C U L T S P I N A L DISORDERS

42

FRANC D . INGRAHAM AND JOHN J. LOWREY IV.

AN

U N U S U A L NASOPHARYNGEAL E N C E P H A L O C E L E

57

FRANC D . INGRAHAM AND DONALD D . MATSON V.

T H E ARNOLD-CHIARI MALFORMATION

69

FRANC D . INGRAHAM AND H . WILLIAM SCOTT, JR. BIBLIOGRAPHY

87

I. A S U R V E Y O F F I V E H U N D R E D FORTY-SIX

AND

CASES

U R I N G the last two decades, 546 infants and children have been seen at the Children's Hospital with spina bifida or cranium bifidum. Associated with these conditions in the minds of both the laity and the medical profession is a rather deep pessimism. It seems worth while to analyze this group of patients, therefore, to see whether there can be established any reasonably definite criteria for prognosis, for operative therapy and for operative technics and whether such an attitude of hopeless pessimism should not be modified by examination of the actual end result in a large series of cases.

D

INCIDENCE Race, sex and economic status seem to have little bearing on the incidence of spina bifida. All our patients were members of the white race. Females seem slightly more liable than males, 296 patients being girls and 250 boys. T h e incidence in the different economic levels seemed proportionate to the total number of patients on each level. T h e ratio of incidence to the number of new patients seen at the hospital during the last twenty years is 1:4150. 1

RELATION TO FAMILIAL OR ASSOCIATED ANOMALIES

T h e familial incidence of spina bifida is shown in Table 1. In 16 (6 per cent) of the 277 cases in which information was available, the patients admitted spina bifida in the family. Approximately an equal number admitted anomalies other than spina bifida. TABLE

1.

Incidence

of Familial FAMILY

HISTORY

Congenital anomaly denied

Congenital

Anomalies. NO.

OF

CASES

246

Spina bifida

16

Other anomalies

15

No data

269

Total

546

T h e frequency with which spina bifida is associated with other congenital anomalies in the same patient is impressive. In 232 patients, or slightly more than half the series, 570 associated anomalies were recorded. Even this large number is probably an underestimate, for two reasons. First, in a number of the records no statement is made concerning the presence or absence of associated lesions; these are chiefly the records of patients who were seen only in the Out Patient Department (often on only one or two occasions) and extensive examination and skeletal x-ray studies were not done and in w h o m the presence of hernial sacs, genitourinary anomalies or skeletal defects might well have remained undetected. Secondly, many of the internal anomalies can be definitely established only by post-mortem examination, and this was done in relatively few cases. 2

It is with some hesitation, then, that we give Table 2, fearing that misunderstandings may arise. It must be realized that this is a mere tabulation of all the lesions noted and recorded from such diverse sources as records from the Out Patient Department and autopsy protocols. It is probably incomplete, particularly regarding internal anomalies, and to use it to demonstrate the comparative frequency of the associated anomalies is unwarranted. For example, only 20 cases of Arnold-Chiari malformation are listed. This apparently low incidence of brain-stem anomalies must be largely a factor of the method of the compilation of the data. When the ArnoldTABLE

2.

Associated Congenital

Anomalies.

ANOMALY

NO.

OF C A S E !

Hydrocephalus

208

Clubfoot

102

Bones Vertebras ( i n c l u d i n g 7 cases of K l i p p e l - F e i l syndrome) Ribs Skull Sprengel's deformity O t h e r bones C e n t r a l n e r v o u s system Cerebrum C e r e b e l l u m a n d b r a i n stem ( i n c l u d i n g 20 cases of A r n o l d - C h i a r i m a l f o r m a t i o n ) Other portions Hernia

94 49 23 16 3 3 58 20 26 12 27

Dislocatcd

hip

23

P i l o n i d a l sinus

13

Genitourinary anomaly

11

C o n g e n i t a l heart disease

4

Other anomalies

30

Total

570

Chiari malformation is found, in our experience it is usually associated with the presence of a my3

elocele. T h e patients with myelocele, however, are usually the poor-risk patients and few come to operation, more being institutionalized or cared for at home, and thus not dying in the hospital or receiving post-mortem examination. Thus, relatively few patients with myelocele have been autopsied, but of these a high percentage have shown associated cerebellar or brain-stem anomalies. It seems likely that if more autopsy material were available, more such anomalies would have been found. It will be noted that several of the commoner associated anomalies may have been secondary to the spinal-cord lesions, for example, club feet, dislocated hips and hydrocephalus. CLASSIFICATION

W e have utilized the commonly accepted terminology but have added three subdivisions of our own. W e have been constantly impressed by the association of spina bifida and meningeal defects with an overgrowth of fatty tissue. These lipomatous tumors consist of lobules of gritty, firm fat, bound together by fibrous septums, and are usually adherent to adjacent fascial (or dural) planes by firm and often vascular attachments. If a meningeal defect coexists, these tumors may extend intrathecally for some distance, even causing intrinsic compression on the nerves or cord. If such a tumor is present with spina bifida occulta, we have used the term "lipoma." If a meningeal sac is present as well but no nerve elements are involved, we have called the condition a "lipomeningocele." If nerve elements are also involved, we have used the term "lipomyelomeningocele." 4

A l l m e n i n g e a l protrusions, w h e t h e r or not associated with nerve tissue, whose osseous defect was in the skull ( c r a n i u m b i f i d u m ) w e have grouped together as " e n c e p h a l o c e l e . " S i m p l e derm o i d cysts arising f r o m the dura and extending through the bone have been excluded. O f t e n classification of a lesion f r o m the data available in the record is difficult. W h e n surgical or pathological data are available, diagnosis can be accurate, but the clinical distinction between a meningocele and a m y e l o m e n i n g o c e l e is often perplexing. W e have therefore adopted the following criteria. I f a sac exists clinically and nerve elements cannot be seen, and if there is n o evidence of neurologic disability as demonstrated by muscular weakness or paresis, loss of sphincter tone or aberration in cutaneous sensation, the lesion is a m e n i n g o c e l e . If nerve elements are visible or neurologic disability exists, the lesion is a myelomeningocele. W i t h these criteria, the relative frequencies o f the different types of lesion are shown in T a b l e 3. TABLE 3. TYPE

Incidence OF

of

LESION

Lesions. NO.

OF

CASES

Spina bifida occulta (13 with lipoma)

65

Meningocele

98

Lipomeningocele

14

Myelomeningocele

279

Lipomyelomeningoccle

18

Encephalocele

84

Total

558

S I T E AND A G E

T a b l e 4 records the site in w h i c h these lesions were found. S i x patients suffered f r o m multiple 5

defects, and 2 of these had three separate lesions. TABLE 4. TYPE

Site of Lesion.

ACCORDING

Cranial Nasal Nasopharyngeal Frontal Parietal Occipital

TO

SITE

NO.

OF

CASES

84 5 1 6 9 63

Cervical

23

Thoracic

39

Thoracolumbar

43

Lumbar

205

Lumbosacral

87

Sacral

46

Thoracolumbosacral

10

Undesignated

8

Pelvic

1 Total

546

T h e age at which the patient was first seen is of some interest (Table 5). As would be expected, the majority of the patients were seen in the first year of life. In these the local mass or deformity was the presenting complaint. In 42 patients, the chief trouble was orthopedic, whereas TABLE 5.

Age of Patients when First Seen. AGE

No.

Less than 1 week 1-3

OF

CASES

102

weeks

180

1-11 months

179

1 year

29

2 - 4 years

30

5 - 1 1 years

26

Total

546

14 complained initially of urinary difficulties. Many of these patients were well along into childhood 6

before increasing neurologic disability led them to seek aid. In 16 patients, spina bifida occulta was found as a fact unrelated to the admitting complaint. Among the patients with encephalocele there was a great deal of variation in the size, site and amount of neural tissue involved, and these factors are of prognostic significance. If a protrusion consists only of dura containing fluid attached by a pedicle, its size is of slight importance, large ones being as readily removed as smaller ones. If, however, the sac contains a neural mass, and particularly if the ventricular system is demonstrated by pneumoencephalographic studies to extend into this mass, the prognosis is usually poor. Nevertheless, occasionally a sac contains anomalous, abnormal neural tissue that can be excised with impunity. We have found that lesions in the occipital region more frequently contain neural elements and if so offer a poorer prognosis than do lesions in other sites, even though they are small. This is apparently because such lesions are frequently associated with a malformation at the base of the brain, which leads to a block of the flow of cerebrospinal fluid and to hydrocephalus, and the latter proves fatal whether or not the lesion is removed. Associated anomalies of the brain are occasionally found even with apparently hopeful lesions in all sites, so that prognosis must be guarded. In our operative technic the sac is developed to its base, excised together with its neural contents, and the dura tightly closed. A firm closure of the soft tissues and skin is considered imperative. No attempt is made to repair the bony defect. The results obtained by operation for enceph7

alocele are, on the whole, encouraging. In our series 59 patients had adequate follow-up studies. Of these, 52 underwent operation, and 17 (33 per cent) of them subsequently died, either in the immediate postoperative period or at some later date. Twenty-one (34 per cent) of the 59 patients are alive, well and entirely normal. One of these when last heard from was playing football on his high-school team. When one sees a photograph of this boy as a baby with a sac almost as large as his head protruding from the occiput, this result becomes even more impressive. Other results are equally striking. Table 6 lists the Present Status of Patients with Cranium

T A B L E 6.

STATUS

Bifidum. NO.

Alive

OF

CASES

37

Operated

on

35

Normal Mental retardation Increasing hydrocephalus Associated anomaly Unoperated

20 6 4 5

on

2

Dead

22

Operated on Unoperated on

17 5

Unknown

25 Total

84

present status of our 84 patients with encephalocele. From these data it seems fair to conclude that about a third of the patients with encephalocele can, with the aid of operative therapy, look forward to an entirely normal existence. Spina Bifida Because the complaints and the degree of disability of patients with spina bifida are so varied, 8

classification for analysis of a large group presents many difficulties. It seems as if each patient were almost a law unto himself. It appeared, however, that in the absence of a leak in spinal fluid, with its concomitant danger of infection, there are two major factors that constitute a threat to the patient's life and health: hydrocephalus and injury to, or the lack of, the neural elements of the vertebral canal. T h e former is recognized by an enlargement of the head, undue separation of sutures and tense fontanelies; the latter by muscular paresis or weakness, lack of sphincter tone and loss of cutaneous sensation. T h e presence of any one of the latter or a combination of them we have termed "neurologic disability." W e have found patients who have had all possible combinations of these two factors, but by utilizing them as a means to classification we can introduce some order into an otherwise chaotic situation. Nor is this division unduly arbitrary, since it parallels, to a certain extent, clinical thought in evaluating these patients as operative risks. In the operative mortality and postoperative results any series of cases of spina bifida depends on the indications for operation. Does the presence of increasing neurologic disability — progressive incontinence, for example — constitute an indication for or against operation? Does the presence of hydrocephalus contraindicate operation? What aspects of the local lesion constitute operative indications? On the answer to these questions there seems to be no general agreement. At the Children's Hospital in recent years all cases of spina bifida have been under the care of the Neurosurgical Service and certain concepts of operability have been elaborated. 9

T h e presence of a mass constitutes an indication for operation in most cases. Occasionally a small lipomatous tumor is found with occult spina bifida and no neurologic disability, and in such cases operative interference is not necessary. T h e age of choice for operations in infants is between twelve and eighteen months. This allows time for the development and recognition of disabilities and hydrocephalus, for the local growth of skin adequate to permit closure and for the child to develop in stature and nutrition into a better operative risk. However, certain local considerations may alter the desirability of waiting. If the sac is broken but uninfected, or if it is so thin as to threaten rupture at any moment, and the patient appears to be otherwise relatively normal, immediate operation is demanded to save his life. T h e presence of infection of the sac or of meningitis contraindicates operation, but local dressings and chemotherapy may suffice to overcome the infection. Assuming that the mass does not threaten rupture, the patient can be cared for adequately on the following regime. T h e mass and surrounding skin are thoroughly washed with boric solution and a sheet of perforated Cilkloid applied. A "doughn u t " of sheet wadding wrapped in gauze is placed around the lesion for protection. Over the top is placed one or two gauze pads, and the whole is bound to the infant by a flannel or linen binder. T h e Cilkloid is replaced by the mother as necessary but not too often, since frequent changing nullifies the stimulative effect on epithelial proliferation. T h e lesion is washed daily with boric solution and clean gauze is reapplied. Every one 10

or two months the patient visits the hospital, where progress is evaluated. T h e presence of progressive hydrocephalus constitutes a contraindication to operation. Occasionally hydrocephalus stabilizes, and if this occurs, the contraindication n o longer exists. T h e presence of neurologic disability does not necessarily constitute a contraindication; indeed, particularly in the older age group, if such disability is progressive, we believe that it is a strong argument for operation. In many of these patients, owing to their defect, the spinal cord becomes anchored at this site. As the child grows tension is placed on the cord, since it increases in length less rapidly than the vertebral canal. There may also be local compression by lipoid tumor growth. Both these elements contribute to the development of the signs of nerve dysfunction. This usually occurs between the ages of six and ten. In such cases, the wisest procedure is exploratory laminectomy with a view to alleviating, if possible, the local situation. This attempt is occasionally successful. T h e decision to operate having been made, certain technical phases of the operative procedure are of paramount importance. T h e skin preparation must be wide and thorough. W e use a colored chemical disinfectant, such as diluted tincture of iodine or Tincture Merthiolate, as the final step in the preparation, thus making the cleansed field visible and assuring a wide enough area. T h e incision, which is usually elliptical, is made with the long axis transverse. W h e r e the lesion is low, the incision must be placed high enough to avoid the intergluteal fold. W e always wall of? the lower limit of the field from the region of the 11

anus by a sheet of sterile gutta percha before the drapes are placed, and postoperatively by a sheet of rubberized silk attached by adhesive strapping. The sac is traced to its emergence from the bony canal, as much as possible is excised, and a tight closure of the dura is made. For this we use interrupted sutures of fine silk, and make the closure as near watertight as possible. Meticulous care must be taken not to cut or injure nerve elements that may be adherent to or contained within the sac. If need be, these are carefully dissected free and returned to their position within the canal. No attempt whatsoever is made to repair the bony defect. If the cut edges of the fascia of the erector Spinae muscle come together easily, they are approximated, but the repair of this layer is of no great importance, and under no circumstances should such great effort be made as to cause compression on the underlying dural closure. A firm repair of the subcutaneous tissues and skin is of great importance. The skin must be made to approximate without undue tension by means of wide undermining of flaps and even relaxing incisions at a distance, if such are necessary. Silver foil is placed over the wound, and a compression bandage applied. For this we have found several layers of gauze held in place by Elastoplast bandage quite satisfactory. For several days postoperatively the patient is held prone on a Bradford frame with the head end about 10 cm. lower than the foot. This position probably minimizes the pressure of cerebrospinal fluid on the dural suture line during the healing period, and thus reduces the possibility of the development of a leak. Unless there is some indication for doing so, the wound is not disturbed 12

for six or seven days, at which time the sutures can be removed. Table 7 gives a somewhat detailed listing of the present status of our 462 patients with spina bifida. TABLE

7.

Present

Status

of Patients

with

Spina No.

Alive

...

Unoperated

on

Normal W i t h s p i n a bifida o c c u l t a W i t h associated s m a l l lipoid mors

tu-

Awaiting operation; no neurologic d i s a b i l i t y Awaiting operation; neurologic disability Operation not advised N e u r o l o g i c s t a t u s n o r m a l (associated anomaly) Operated

on

(Children's

3 17 38 5

Hospital)

148

N o hydrocephalus and no neurologic ability preoperatively Entirely normal Developed hydrocephalus (now stabilizing) Developed neurologic disability.. (Severely incapacitated, 8; mildly incapacitated, 15; d i s a b i l i t y i m m e d i a t e l y posto p e r a t i v e l y , 10; d i s a b i l i t y m o n t h s or years l a t e r , 13) Developed both hydrocephalus and neurologic disability . . . N o r m a l as r e g a r d s s p i n a b i f i d a ; associated a n o m a l y No hydrocephalus but neurologic ty p r e o p e r a t i v e l y

dis84 45 5 23

disab 48

I m p r o v e d by o p e r a t i o n M a d e w o r s e by o p e r a t i o n Neurologic disability persists (severe, 25; m i l d , 6) Developed hydrocephalus Hydrocephalus but no neurologic ty p r e o p e r a t i v e l y

disab

A r r e s t e d ( n o r m a l , 1; m i l d n e u r o logic d i s a b i l i t y , 5) Progressing

13

6 1

Bifida. OF

CASES

234

Hydrocephalus and preoperatively

neurologic

disability

Arrested (severe, 7; mild neurologic disability, 2) Progressing

9

9 0

Operated on elsewhere (severe, 10; mild neurologic disability, 2)

12

Dead

167

Unoperated on

127

Operated on

40

Operative deaths (meningitis,

12)

22

Subsequent deaths (hydrocephalus, 16) . . . .

18

Status unknown

61

Total

462

Any further simplification, however, would deprive it of analytic value. A portion of this material is presented in condensed form in Table 8, which gives only the present status of the living patients, grading them as normal, slightly handicapped or severely handicapped. T h e data presented in Tables 7 and 8 allow some interesting conclusions. There are 401 patients whose status is known and whose follow-up studies are considered adequate. Of these, 234 (58 TABLE 8.

Present Status of Surviving

PATIENTS

NORMAL

Patients.

MILD

SEVERE

NEUROLOGIC

NEUROLOGIC

DISABILITY

DISABILITY

Operated on (Children's Hospital)

46

42

60

Others

14

19

53

60

61

113

Totals

per cent) are alive. T h e remainder have died of a variety of causes. Operation was performed on 188, and 20 more are awaiting operation. Thus, 14

208 (52 per cent) were or are thought to be suitable for operation. T h e immediate operative mortality in our hands was 12 per cent. Of the patients who survived, 60 are considered normal and 61 are suffering from neurologic mild disability. T h e patients in both these groups may be considered as capable of leading a normal human existence. Thus, about 30 per cent of the patients with spina bifida may look forward to a life unhampered by any significant incapacity resulting from their anomaly. Congenital lack of neural elements or destruction by long-standing compression results in neurodisability of an irreversible nature. One of these situations prevails, unfortunately, in the majority of cases where disability is present. However, occasionally local compression or traction on the cord is the cause of the progression of symptoms. If these are recognized, laminectomy offers promise of relief if the local situation permits operation. In our series there were 10 patients in whom relief or improvement in neurologic disability occurred following such an exploration. In the light of the data presented by this review, we believe that an outlook of extreme pessimism in the presence of spina bifida is unwarranted. Each patient must be evaluated as an individual problem. SUMMARY

A series of 462 cases of spina bifida and 84 of cranium bifidum (encephalocele) is presented, with data on the incidence, sex, race, type, site, associated anomalies and clinical manifestations. Therapeutic indications and end results are tabulated and discussed. 15

Thirty per cent of patients with spina bifida and 34 per cent with encephalocele may expect a relatively normal life. A n unduly pessimistic prognosis is therefore unwarranted until individual evaluation has been carefully pursued.

16

II. S U R G I C A L

TREATMENT

T

H E treatment of myelomeningocele and encephalocele constitutes one of the major problems of pediatric neurosurgery. T h e lesion m a y occur at any point of the dorsal axis, usually but not invariably associated with bifid vertebral anomalies or defects in the skull. These protrusions exhibit great diversity in appearance and histologic components. T h e distribution, frequency of various types and so forth have been tabulated in a previous communication 1 and the clinical features have been repeatedly described. Pathologically, myelomeningocele is regarded as representing the result of incomplete and abnormal dorsal fusion that takes place relatively early in embryologic life. T h e defect or defects thereby produced involve structures inherent to and surrounding the central canal and its contents in variable degree at one or several segments, which may be conjoined or separated by normal intervening anatomical units. T h e selection of patients who are likely to be amenable to long-range rehabilitation and the choice of the proper time for operation are of paramount consideration before the question of surgical procedure arises. In some cases the m e m 17

brane may be so thin and fragile that operation must be carried out immediately to avoid rupture and subsequent meningitis. Occasionally we have removed such protrusions the first day of life, in spite of the fact that it was impossible to give an adequate prognosis at such an early age. Usually, however, there is no need for haste and careful evaluation of the infant, including roentgenologic examination, should be made. The size of the lesion and the nature of its protective coverings are noted. Many of the more formidable ones have a central sac or loculation that is covered externally by a relatively large area of delicate membrane, and one is obliged to await peripheral epithelialization to obtain sufficient tissue for closure, even though the skin eventually available may be cicatrized, uneven and of poor tensile strength. There may be additional scoliosis, vertebral fusion, absence of ribs or other skeletal abnormalities. Predominating in lumbosacral lesions, talipes equinovarus or valgus is often present and is likely to be accompanied by faulty innervation of the sphincters and lower limbs. Inept control of the latter may not be apparent for weeks or months. Clubfoot deformity and paralysis presage a long course of orthopedic treatment, which can be instituted immediately but often cannot be satisfactorily continued until the tumefaction of the lower back is reduced and given adequate skin protection. These factors combine to produce confusion and indicate that temporization with home care and out-patient visits forms the proper basis of treatment until the infant can be fairly appraised with respect to future promise. There are patients with open marsupialization of the lesion 18

who will not survive long. Other handicaps, particularly poor mental endowment, render surgical success highly improbable. On the other hand, certain patients in w h o m the outlook seems to be almost hopeless during the early months of life will in time progress to a point where surgery is both feasible and advisable. F o r example, an infant is examined at a few weeks of age and is found to have a myelomeningocele surrounded by barely enough skin for closure; there are also clubfoot deformity, m a r k e d neurologic disorders and hydrocephalus. A t the age of two the hydrocephalus has stabilized and the child shows adequate sphincter control and a chronological mental status. Epithelialization of the lesion has greatly reduced the operative risk; after successful repair the residual physical disability can receive full benefit of orthopedic surgery. O n e must be very slow to give a definite prognosis in such cases, even in the severe grades of the deformity. A m o n g the congenital anomalies associated with myelomeningocele, hydrocephalus offers the greatest difficulty from the standpoint of selection of cases for surgery and their prognosis. T h e abnormality may be recognizable at birth with palpable diastasis of fontanelies and cranial sutures and craniolacunia ( L i i \ e n s c h ä d e l ) present roentgenographically. Conservative h o m e care is obviously to be recommended as the initial phase of treatment in such cases. Sometimes, however, the disturbance is insidious, enlargement of the head becoming apparent shortly after the myelomeningocele has been well repaired. Repetition of this sequence of events is probably responsible for the myth that removal of a meningocele and its spinal fluid reservoir is liable to cause an 19

ensuing hydrocephalus. Delay in surgical intervention, therefore, is the wisest general course to pursue even in some of the simpler forms of the syndrome. T h e small meningoceles, especially the cervicodorsal protrusions, in infants w h o are neurologically normal and show no roentgen defect other than a localized spina bifida can be operated on during the first days or weeks of life with a good outlook. T h e complicated cases, however, in which the myelomeningocele usually is lumbosacral, compel a decision to wait until accurate appraisal of the problem can be formulated during the early period of rapid growth and development. T h u s , the best surgical procedure for the given situation and its aftermath, which may be the most perplexing part of the treatment, is carried out with confidence. T h e most important feature of the actual operation is the adaptation of whatever technic is used to the case in hand. T h e elaborate approach necessary to deal safely with nasopharyngeal protrusions will be described in a later communication. T h e specialized treatment of orbital protrusions has been described by Dandy. 2 Anterior protrusions into the thoracic and abdominal cavities should not be difficult to deal with if a meticulous closure of dura is made and is reinforced by bringing stronger adjacent tissues together over the closure. T h e disastrous result of failing to m a k e a secure dural closure is illustrated by the following case history. CASE 1 (C. H. 168203). A 5-month-old male infant was transferred to the Surgical Service on February 14, 1933, for treatment of a retrorectal tumor. T h e patient had been admitted with a history of dysuria and oliguria of 16 days' duration. During this time there had been 20

progressive distention of the abdomen and increasing constipation. Catheterization had been necessary at frequent intervals. Physical examination showed a well-developed and well-nourished infant in no apparent distress. A soft, smooth, r o u n d e d mass the size of a small orange was palpable by rectal examination about 6 cm. above the anal sphincter. X-ray examination showed marked distortion of the large-bowel shadow by the pelvic mass (Fig. 1). T w o days later, abdominal exploration was carried out and the cystic mass f o u n d to fill the entire pelvis. It was separated f r o m the surrounding structures d o w n to the sacrum, where it was f o u n d to be densely adherent. A t this point the cyst was aspirated and clear, faintly yellow fluid was removed. It was thought that total removal was impossible, and a portion of the wall was excised, permitting the fluid to drain into the peritoneal cavity. Following operation there were severe vomiting and diarrhea, which gradually subsided. As the general condition improved rectal examination showed that the cyst had reformed. Nineteen days after the first exploration a secondary operation was done and the sac was sutured to the peritoneum. T h e patient's condition was extremely poor at the end of this procedure and he died 28 hours later. Permission for autopsy was not obtained, but sections of the cyst wall showed it to be undoubtedly the wall of a myelomeningocele containing typical dura and a small, irregular mass of glial tissue. In

retrospect

it s e e m s

relatively

certain

that

the

v i t a l s t e p i n t h e o p e r a t i v e p r o c e d u r e i n t h i s case — t h a t is, firm c l o s u r e of t h e sac — w a s e n t i r e l y o m i t ted.

T o t a l r e m o v a l of t h e sac w a s a p p a r e n t l y i m -

possible b u t w a s also u n n e c e s s a r y , a n d

firm

closure

of t h e r e m a i n i n g p o r t i o n , w i t h t h e f o r m a t i o n of a small secondary meningocele, would probably have given a satisfactory result. Protrusions

through

the cranial vault are

tively s i m p l e t o d e a l w i t h . unusually large, a n d

firm 21

rela-

T h e b o n y d e f e c t is n o t c l o s u r e of t h e d u r a

and

1. Barium Enema Showing Marked Displacement of the Large Bowel by a Pelvic Anterior Meningocele.

FIGURE

galea, with the incision adapted to the size and shape of the defect, are all that is necessary (Fig. 2). Low occipital encephaloceles are likely to present a much more complicated problem. These masses are sometimes extremely large, and the decision whether or not operation should be done may be difficult. If marked abnormality of the brain or poor function below the lesion is demonstrable, operation should not be considered. Occasionally one sees an extremely large occipital meningocele or encephalocele that gives the impression of a hopeless outlook when the brain is actually normal (Fig. 3 ) . It is advisable to postpone any estimate of the prognosis until a complete study has been carried out. T h e two essential features of any operation on the commoner protrusions — that is, cervical, dorsal, lumbar and lumbosacral (Figs. 4, 5, 6 and 7) — are tight closure of the sac and firm closure of the skin and subcutaneous tissue. Reinforcement of the closure with fascial leaves is desirable if this can be done without prolonging the operation unduly. Reconstruction of the bony canal should never be attempted at this time, but if the defect is such that the bony spine is seriously weakened, a secondary procedure with a bone graft may be done. If the patient is given proper orthopedic care, this should seldom be necessary. In the typical case an elliptical incision (Figs. 8 and 9) is made around the base of the mass, preferably preserving enough healthy skin to be brought together without tension. If this is impossible and operation cannot be postponed until there is enough skin, counterincisions may be made or a Ζ flap of skin brought over the defect. In the case of lumbar and lumbosacral lesions, the inci23

sion should always be made transversely so as to keep the wound as far as possible from the intergluteal fold.

FIGURE

3.

Large Occipitocervical Encephalomeningocele Removed at the Age of Two Weeks.

Dissection is carried down - to the neck of the sac, carefully avoiding any nerve elements that may be running out from the sac. T h e sac is then opened through an area, preferably midline, 25

FIGURE

8.

Type of Incision and Dissection Used for Most Lumbar and Lumbosacral Protrusions.

9. Closure Re-enforced by Fascial Flaps. This step must frequently be omitted.

FIGURE

that can be shown to be free from nerve tissue. The interior of the cavity is inspected, nerve tissue ending in the wall is sacrificed, and nerves that may possibly be functioning are carefully preserved. T h e major portion of the sac is then cut away, leaving sufficient tissue to overlap the edges enough to make a secure closure. If there is a large amount of tissue that may have potential function, it may be necessary to create a small secondary meningocele to preserve it. In our experience, plication of the sac, as advocated by Penfield, 3 has had no bearing on the development of hydrocephalus postoperatively. In fact, the meningoceles with sufficient skin to make plication of the sac possible are those milder grades of maldevelopment in which one would not expect hydrocephalus to be present. The first postoperative week is an extremely critical period because of the danger of spinal-fluid leakage, infection at the closure site and possibly meningitis. T h e hazard increases with the size and complexity of the lesion. If there is any doubt about the possibility of making a firm closure, prophylactic administration of a sulfonamide, preferably sulfadiazine, is recommended starting forty-eight hours prior to anesthesia and maintaining an effective blood level for at least one week. Ideal aftercare consists of proper initial and subsequent dressing of the wound and the use of specialized methods for regulating cerebrospinalfluid pressure. Diligent nursing is essential. Complications, if they arise, should be recognized and treated quickly. The particular problems in the postoperative care of the lumbar and lumbosacral types of protrusion are discussed in some detail below. 32

Before the wound itself is dressed following operation, an apron of sheet rubber or strong oiled silk is glued across the buttocks below the surgical field to protect this area from contamination by excreta. A routine dressing of sterile sponges over silver foil covering the skin is satisfactory for the small, well-approximated incision, which is the result in many lesions. Anxiety about delayed healing and spinal-fluid leakage is more likely to be felt in the lumbosacral type. When good approximation is achieved following the evacuation and obliteration by suture of a large subcutaneous meningocele, the skin flaps, despite exact alignment, are sometimes loose. In this situation, dead space between the fascial planes can be minimized during the important stage of primary union by taping a small sandbag on the dressing and leaving it there for twenty-four to forty-eight hours. The pad of sponges should be fastened to the skin with adhesive strips running at right angles to one another, leaving interstices of gauze exposed for circulation of air. In addition to meticulous dressing, conscientious nursing in the supervision of a few specialized technics, adapted to the aftercare of these patients, contributes toward shortening their immobilization, which is usually prolonged under the best conditions. Figure 10 shows the basic setup used postoperatively at the Children's Hospital for cases of myelomeningocele. A Bradford frame is supported by blocks front and back on the mattress of an ordinary crib bed with side drops. The usual rubber sheet crosses the midportion of the mattress, upon which a bedpan rests beneath an overlying aperture in the canvas. 33

A half-cylinder f r a m e keeps the bedclothes away from the surgical area. A clean diaper is kept under the protective apron, which is sealed off above the buttocks. Diapers are applied in the usual fashion when there is no risk of contaminating the dressing or when healing is well advanced. Fitted on the supportive swathes of the Bradford frame are mattress covers over which are stretched and taped layers of Pliofilm or cellophane, which afford excellent protection to infant skin. T h e patient lies prone as illustrated, and is so maintained by cloth binders around the trunk and upper thighs and a combination of wrist, shoulder and ankle restraints, well padded with sheet wadding. T h e restraints are varied according to the horizontal plane of the frame but with constant regard for maximum comfort. Feeding, bathing and other nursing details are easily accomplished, since the whole f r a m e can be picked up and moved whenever necessary without disturbing the patient. As convalescence progresses the child is allowed gradual freedom of posture by altering the restraints until he is completely mobilized. If there is undue tension or other reason for examining the wound at frequent intervals, a transparent dressing can be easily prepared (Fig. 11). By this means the operative field can be inspected without removing the dressing and given immediate care should the need arise. Overtight stitches are removed before necrosis starts; any point of the suture line that appears weak may be sealed by a cutaneous silver clip and an incipient leak perhaps prevented. If superficial infection develops, it can be checked at the onset. A wall of gauze at least 5 cm. deep is built 35

a r o u n d the w o u n d by layering sponges folded or t r i m m e d to appropriate size. T h e s e are secured to the skin and to o n e another by means of sterile glue, such as Mastisol. A piece of transparent plas-

11. Postoperative Wound Twenty-jour Hours after Application of Transparent Dressing.

FIGURE

tic material is then cut to overlap generously the o p e n i n g in the dressing. F i n a l l y the w h o l e is bound down with adhesive bandage and tape. If spinal-fluid leakage is detected d u r i n g the critical 36

stage of healing, the development of a fistula is preventable by prompt measures such as applications of warm physiologic saline solution and the reduction of intracranial pressure by lumbar puncture or ventricular drainage. If all goes well, the window is left for eight to ten days, at the end of which time a conventional dressing can be substituted. Occasionally, in spite of all precautions, temporarily increased cerebrospinal-fluid pressure becomes a serious menace. In these cases gravity can be utilized to a certain degree by keeping the head end of the Bradford frame several centimeters lower than the foot with proper-sized blocks. In lumbosacral wounds the head-down position requires that extra vigilance be maintained over excretory cleanliness. W h e r e the pressure is not controllable by periodic tapping, we have used a simple system of constant lumbar drainage that has proved effective. A small-gauge (No. 20) needle is thrust through a sterile cork measuring approximately 3 by 4 cm. and is inserted in the caudal sac at a convenient interspace relative to the dressing. W h e n a free flow of spinal fluid is obtained, the needle is connected by an adapter to an autoclaved gravity apparatus, as shown in Figure 12. T h e apparatus consists of narrow rubber tubing, broken at one point by a medicine dropper for inspection of patency and connected to a 25-cc. burette. T h e needle is held perpendicular by taping the cork to the skin. T h e burette is suspended on the side gate of the crib so that hydrostatics can be regulated to produce the desired rate of flow by raising or lowering the reservoir as indicated by its fluid meniscus. Daily output 38

is measured, and varies according to the time required for stabilization of cerebrospinal-fluid dynamics. An apparatus similar in principle and design was originally described by Ingraham and Campbell 4 for constant ventricular drainage. It can be kept in continuous operation, however, longer by the spinal route than by the ventricular without the risk of meningeal contamination that goes with the latter. This assumes that the dynamics will ultimately stabilize at a physiologic level, which is usually the case if there has not been progressive hydrocephalus preoperatively. If there has been increasing pressure, operation should not have been carried out. The following summary of the first case of postoperative myelomeningocele in which this technic was applied demonstrates how constant spinal drainage solved a difficult problem of leakage and fistula. CASE 2 (C. H. 256381). A 2-year-old boy was admitted on October 15, 1941, with the complaint of paralysis of the lower extremities and a lump on the back (Fig. 13). There was no hydrocephalus and the patient was normally alert and responsive. On the 9th hospital day the meningocele was excised and closure was carried out under tension. Seven days later a cerebrospinal-fluid leak developed from the central portion of the incision. Sulfadiazine was started by mouth and an attempt was made to close the leak by resuturing the wound. Three weeks later a third attempt was made 'and the leak was satisfactorily closed. A lumbar-puncture needle was passed into the upper lumbar subarachnoid space according to the technic described above and 200 to 300 cc. of cerebrospinal fluid was drained every 24 hours for 7 days. At the end of this time the wound was firmly healed. There have been no further complications. It seems reasonable to assume that, without the continuous drainage above the site of the incision, healing would not have occurred and infection would finally have developed. 40

SUMMARY

T h e essential features of the satisfactory treatment of congenital cranial and spinal protrusions are the selection of patients suitable for operation, simple removal and repair of the defect adapted to the location, size, and shape of the protrusion and meticulous postoperative care. It should be emphasized that a definitely hopeless prognosis should very rarely be given at the first examination. REFERENCES 1. I n g r a h a m , F . D., and Swan, H . Spina bifida and c r a n i u m b i f i d u m . I. Survey of five h u n d r e d and forty-six cases. New Eng. / . Med. 228:559-563, 1943. 2. Dandy, W . E. O p e r a t i v e treatment for certain cases of meningocele (or enccphalocele) into orbit. Arch. Ophth. 2:123-132, 1929. 3. Penfield, W . , and Cone, W . Spina bifida and cranium bifidum: results of plastic repair of meningocele and myelomeningocele by n e w m e t h o d . / . A. M. A. 98:454-461, 1932. 4. I n g r a h a m , F. D., and Campbell, J. B. Apparatus for closed drainage of ventricular system.. Ann. Surg. 114:1096-1098, 1941.

41

III. O C C U L T S P I N A L D I S O R D E R S

OUGHLY 25 per cent of normal children have occult defects in the vertebral laminas and this incomplete closure is frequently demonstrated in x-ray films taken for some other reason. The defect is likeliest to occur in the lumbar or sacral region and can be shown to have persisted in many normal adults. Over a twelve-year period at the Children's Hospital we have selected the much smaller group of patients who have had symptoms associated with occult spina bifida. Probably too few of these patients have been given the benefit of local exploration, but it is only by careful study and selection that operation can be offered to the proper group. The variations of symptoms and physical findings are unlimited. In some cases there may be such faulty development of the lower portion of the spine and cauda equina that surgical treatment will not be helpful, and those patients who might possibly be worse after operation must be carefully excluded.

R

PRESENTING S Y M P T O M S AND SIGNS

Of the 65 cases studied, 31 were first seen in the orthopedic clinic and the most frequent present42

ing complaint was maldevelopment of the feet or lower extremities with some disturbance in gait, which was present in 29 cases. The findings included valgus, varus and cavus deformities of a foot, a small foot and a limp due to muscle weakness. In the large majority of cases the deformity was unilateral. The second commonest complaint was some local abnormality, such as a swelling, a bony lump or defect, a scoliosis or a local overgrowth of hair. These accounted for 22 cases. Enuresis or incontinence brought 7 patients to the hospital. In 9 cases the spina bifida was an incidental finding. The neurologic disabilities are not easily classified because of the difficulty of deciding whether or not a given abnormality is on a neurogenic basis. If a patient on physical examination shows anesthesia over the distribution of the fifth lumbar root and at operation is found to have a tumor pressing on that root, the cause and effect are apparent. In most of our cases, however, the findings at physical examination were various degrees of muscular weakness in a lower extremity and an abnormality of posture presumably dependent on this imbalance of muscle groups. The findings at operation suggest that the muscle weakness and imbalance may be on a neurogenic basis. The age group dealt with made neurologic examination difficult, but abnormal reflexes were recorded in 15 cases, a positive Babinski response in 7, poor sphincter tone in 6, and abnormal sensation in the lower extremities in 5. Besides the neurologic or developmental changes in the lower -extremities, which may or may not have been produced by lesions of the cord or cauda equina at the site of the spina bifida, there 43

were a few local findings of importance. As determined by physical examination or x-ray or both, the lesion was present most frequently in the lumbosacral region (25 cases). Eighteen cases showed sacral involvement alone, 4 dorsal, 3 dorsolumbar and sacral, 3 dorsolumbar, 1 cervicodorsal, and 1 cervical. As stated above, some local abnormality, such as a soft or bony swelling, a local overgrowth of hair or a palpable defect, was the presenting complaint in 22 cases. Routine physical examination showed a palpable bony defect in 28 cases, and a local overgrowth of hair in 14. Of these 65 cases, in 13 the abnormality was associated with a socalled "lipoma." As described in a previous paper, 1 lobules of gritty, firm fat, bound together by fibrous septa, usually adherent to adjacent fascial (or dural) planes by firm and often vascular attachments are frequently found associated with spina bifida. They may be at any tissue level from the cord itself to the subcutaneous tissue or may extend directly from one layer to another, binding the various tissues together and thereby producing adhesions and preventing the normal movement of the caudal end of the spinal cord with growth. It should be emphasized, however, that spina bifida may be present without any symptoms or signs. It was present in 11 cases without any neurologic disability or abnormality of the lower extremities, such as valgus or small foot, that might cause a disturbance in gait. In 15 cases, there was no local visible or palpable abnormality that would suggest the spinal defect.

44

OPERATED

CASES

Of the 65 cases reported, 26 had exploration of the region of the spina bifida. In 4 of these, the procedure was minor in nature and consisted only in local excision of a subcutaneous lipoma or dermoid, or incision and drainage of an infected dermoid. In 22 cases, a laminectomy was performed. O n e of these was in a five-day-old infant with bilateral brachial palsy. At operation acute angulation of the cord due to hemivertebras was found. T h e patient died two days after operation. Eliminating this case as being unusual and complicated by the other vertebral anomalies in addition to the spina bifida, there were 21 cases. Some of these patients had two-stage operations. Many of them had previous or subsequent orthopedic operations to correct the deformities of the extremities. T h e findings at operation were variable. T h e most frequent finding was a combined intradural and extradural lipoma, which was present in 5 cases. Three cases showed extradural lipoma, 3 a fibrous tract leading from the skin to the dura, 2 subdural lipoma, 2 extradural lipoma with a bifid spinal cord, 2 no gross lesion of the spinal cord or the nerve roots, 1 an abnormal spicule of bone with pressure on the nerve roots, 1 a fibrous tract from the skin to the ligamentum flavum, 1 a constriction of the cord due to adhesions, and 1 a meningocele entirely contained within the sacral canal. T h e criteria on which success of operations of this sort must be based are difficult to determine because of the multiple complaints. Also, where permanent nerve damage has been present it is 45

not possible to expect improvement. It therefore seems justifiable to consider a result as good if a definite cause of progressive symptoms can be found and relieved and if no further progression of symptoms occurs. There was no operative mortality in these cases. In some cases there was striking recovery, such as healing of a trophic ulcer with return of sensation, or marked improvement in urinary and fecal control. It is important that patients who are developing equinus or cavus deformities be operated on early, because such deformities once present cannot be expected to disappear following operation. Using the criteria defined above, it can be said that a good result was obtained from operation in 13 cases. In 3 cases the cord or roots showed an abnormality and the disturbance in gait persisted. Three cases have not been followed long enough to determine the result, although all these showed obvious disease at operation. One showed a bifid spinal cord. One patient (Fig. 1) operated on because of incontinence, with the finding of a fibrous cord from the subcutaneous tissue to the dura, exerting tension on the latter, showed improvement in the hospital but lapsed to practically her preoperative state when left with unco-operative parents. One patient awaits reoperation because complete removal of an extradural lipoma was not possible at the first operation. CASE

REPORTS

CASE 1 (C. H. 75872). This patient was first seen in the hospital at 2 months of age because of bilateral club feet. The feet were treated with manipulation and then casts. At the age of 4 he was admitted because of a 46

trophic ulcer of the right small toe (Fig. 2). This failed to heal and the toe was amputated. Spina bifida occulta was noted at that time. He was followed in the Out Patient Department and did fairly well except for occasional trophic ulcers. At the age of 8 he had a right arthrodesis for persistent varus deformity of the right foot. At that admission reflexes in the right leg were absent and there was diminished sensation over the lower half of the lateral surface of the lower leg and the dorsum and sole of the foot. At 11 years of age the patient was admitted for a trophic ulcer of the right fourth toe, which subsided following treatment with wet packs. Four months later he was admitted for a trophic ulcer of the right third toe that had developed cellulitis. Physical examination showed a large crater over the third toe discharging purulent material, with surrounding cellulitis. The diminished sensation over the lower leg and foot was still present, with absent temperature sense. T h e spine showed a dorsal angulation over the 2nd lumbar vertebra with a palpable bony defect. X-ray examination showed abnormal development of the 12th dorsal vertebra and all the lumbar and sacral laminas, with incompletely formed, defective and incompletely fused arches. It was decided that there might be pressure on the spinal cord associated with the spina bifida, so laminectomy was carried out. On opening the dura definite constriction of the cord due to adhesions associated with the defective closure of the spinal cord was found at the level of the 11th dorsal vertebra. The cord was freed at that point. Postoperative recovery was uneventful. A small piece of bone was removed from the base of the ulcer 7 days postoperatively, following which healing rapidly occurred. Postoperatively there was return of sensation and improvement in control of the anal sphincter. When the patient was last seen, 10 months after operation, his toes were still in good condition. CASE 2 (C. Η. 263163). This girl first entered the hospital on the Neurological Service at 9 years of age with a chief complaint of progressive talipes equinovarus deformity of both feet of 4 years' duration. T h e feet tended to become cold. Otherwise there were no complaints. T h e patient had had treatment with corrective shoes and 48

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arch supports without relief. The family and past histories were not remarkable. Physical examination showed diffuse mottling of both lower extremities, with atrophy of the legs from the knees down, talipes equinovarus deformity of the feet and thick calluses on the toes. Neurologic examination was negative except for diminution of pain and touch sensation in the toes bilaterally. X-ray films of the spine showed a spina bifida of the 3rd lumbar vertebra and of the sacral segments, with a poorly formed sacrum. Laminectomy was performed in two stages owing to the extensive dissection necessary, and a meningocele (Fig. 3) was found extending from the lumbosacral junction through the sacral canal to the undersurface of a dimple in the skin just above the anus. The sac contained darkyellow fluid. No connection with the dura proper could be demonstrated except for a tiny fibrous stalk. The sac was freed from the nerve roots and excised. Postoperatively the patient did well. Six months after operation she showed return of sensation to the toes, and the temperature of the lower legs seemed normal. She walked better than before the operation. Muscle examination showed essentially the same picture as preoperatively. CASE 3 (C. H. 244120). This girl was first seen in the Orthopedic Clinic at 9 years of age for a deformed left foot, which had been present at birth. Physical examination showed atrophy of the left lower leg and equinovarus deformity of the foot, associated with a cock-up of the toes (Fig. 4). The left foot was considerably smaller than the right and there was some diminished sensation over it. The spinous process of the 3rd lumbar vertebra appeared double. X-ray examination showed congenital anomalies of the laminas of the 2nd and 3rd lumbar vertebras, with partial fusion of the laminas and indentation of the posterior aspects of the vertebral bodies. There were also defects in the laminas of the 5th lumbar vertebra and the 1st sacral segment. Lumbar puncture was done, with normal spinal-fluid findings, and the patient was discharged home. She was followed in the Out Patient Department over the next 50

FIGURE 3. Case 2. These drawings show the intraspinal meningocele connected with the dura by a small stalk.·

year and developed further loss of sensation in the left foot and also loss of reflexes on the left. Lumbar laminectomy was therefore carried out. A lipoid mass was found in the subcutaneous tissue, and extending from this was a fibrous stalk that penetrated the edge of the 3rd lumbar vertebra and entered the neural canal, but without any connection with the dura (Fig. 5). On opening the dura, small swellings giving the appearance of neuromas, the maximum being twice the diameter of the nerve trunk, were seen on the 2nd, 3rd and 4th lumbar roots. These swellings could not be isolated and no attempt was made to resect them. The canal was left wide open. The patient had a smooth postoperative course and was discharged home on the 16th postoperative day immobilized in a plaster jacket. At the time of discharge the area of hypoesthesia had diminished so that instead of half the dorsum of the left foot only the toes of the left foot were affected. Muscle examinations before operation and 5 months postoperatively showed no change in muscle strength. C A S E 4 (C. H. 239250). This girl was first seen in the Orthopedic Clinic at 5 years of age, with a complaint of weakness of the left foot. Five months previously it had first been noted that the left foot was smaller than the right and that the patient seemed to drag it while walking. These complaints had progressed to entry. Physical examination was normal except for the spine and lower extremities. There was a growth of long, dark hair over the lower lumbar and upper sacral segments, with a suggestive underlying spinal defect (Fig. 6). The left foot was smaller than the right and showed a cavus deformity. The left calf was 0.5 cm. and the left thigh 1.0 cm. smaller than the right. Reflexes and sensation were normal in both lower extremities. X-ray films showed defects involving the laminas of the first three lumbar and upper sacral segments, with widening of the interpeduncular spaces. The spinal fluid was normal. At laminectomy it was found that the spinous processes of the 3rd and 4th lumbar vertebras were absent and

53

FIGURE 5. Case 3. This drawing is a reconstruction of the firm lipoma from the dura to the subcutaneous tissue.

extending

FIGURE 6 . Case 4. This photograph shows the abnormal growth of hair over the site of the spina bifida.

that the laminas were overlapped, those on the right lying under the left so as to cause constriction of the dura and absence of epidural fat. The dura was exposed and opened, and chronic arachnoiditis was found. On the left, in the dura at the level of the 4th interspace, there was a funnel-shaped depression that was attached to a piece of bone coming from the ventral surface. The nerve roots on the left were deflected by this bone and were adherent to it. The nerve roots were freed, and the bony prominence removed. Postoperatively the patient made a good recovery and was discharged in a plaster jacket on the 14th postoperative day. With orthopedic shoes she did well. The gait was better and the deformity did not progress. SUMMARY

An analysis of 65 cases of spina bifida occulta is presented. The symptoms and usual findings are listed. Twenty-one cases in which laminectomy was performed are discussed, and the operative findings summarized. The indications for operation were specific, and operation was not performed without a definite history of progressive difficulty. In only 2 out of the 21 cases did operation fail to show a lesion that might account for the presenting complaint. In the large majority operation showed a definite lesion affecting the cauda equina or nerve roots, that could be remedied surgically. The findings suggest that probably more of the questionable cases should be offered the benefit of exploration. REFERENCE 1. I n g r a h a m , F. D., and Swan, H . Spina bifida and cranium bifidum. I. Survey of five hundred and forty-six cases. Neu/ Eng. J. Med. 228:559-563, 1943.

56

IV. AN UNUSUAL NASOPHARYNGEAL ENCEPHALOCELE

A

T T H E Children's Hospital 546 patients have been seen with meningoceles distributed throughout the central nervous system. 1 Of these cases, 84 may be classified as encephaloceles, that is, herniation through a defect in the cranium. Among the encephaloceles, 63 were occipital, 9 were parietal, 6 were frontal, and 5 protruded into the nose. T h e case reported here is the only one in which anterior herniation of the brain occurred through a defect in the cribriform plate and presented in the nasopharynx. Dandy 2 described a patient with protrusion of the cranial content into the orbit, and outlined a satisfactory operative procedure for such cases. Anterior encephaloceles must be extremely rare. Mood 3 reported 40 cases collected from the literature, dividing them into basal and sincipital varieties, which occurred with about equal frequency. T h e sincipital types present as external tumors, whereas the basal varieties are intranasal, intrapharyngeal or intraorbital. This author pointed out that herniation through the skull is made possible because of clefts between the various bones that are present either normally or pathologically during early development. 57

More recently, McGillicuddy 4 reported the case of a thirteen-year-old boy with an encephalomeningocele filling the left nostril and antrum but not visible in the nasopharynx. Partial removal had been done at three months of age, with no untoward sequelae. Intranasal removal with a tonsil snare was carried out and recovery was uneventful, but drainage from the nostril persisted. One year after operation the patient developed severe meningitis, which responded to chemotherapy, -but he showed a slight recurrence in the upper anterior nares and continued to have rhinorrhea. McGillicuddy suggests that an intracranial approach to the pedicle was probably indicated. T h e clinical problems of diagnosis and especially of neurosurgical management of this rare anomaly seem to make a report of the following case desirable. R . A. N. (C. H . 242961) was first seen on June 7, 1940, at the age of 23 days. He was referred to the Children's Hospital because of a right incomplete harelip, cleft palate and a redundant skin tab over the tip of the coccyx. N o family history of congenital anomalies could be elicited. H e was delivered normally at full term and there were no postnatal disturbances. Examination revealed the above findings, and was otherwise negative except for a midline dimple in the skin of the sacral region. Plastic repair of the harelip was carried out uneventfully and the patient was discharged home. H e re-entered the hospital for repair of the cleft palate on September 28, 1942, the operation having been twice postponed in the interval because of mild upper-respiratory infection associated with nasal discharge. T h e interval history was otherwise negative and the patient's development appeared to be normal. Physical examination revealed a healthy child whose head was of normal size and shape; the fontanelles were closed. T h e nose showed flattening of the right external nares. T h e left nostril 58

was almost completely blocked by a smooth, bluish-gray, fluctuant, soft-tissue mass medial to the inferior turbinate and extending posteriorly. A probe could be passed around the mass on all sides, so that it seemed to be attached superiorly. The mass did not shrink after the topical application of adrenalin. There was a well-healed scar of the harelip repair with a good cosmetic result. A wide cleft of the palate extended from the uvula to within 1 cm. of the alveolar ridge anteriorly. A moderate growth of adenoid tissue was visible on the posterior pharyngeal wall. On either side of the nasal septum, smooth, bluish-gray, fluctuant masses were seen protruding over the margins of the cleft palate, that on the left measuring 1 by 1.5 cm. and that on the right 0.5 by 1.0 cm. (Fig. 1). These masses were observed to vary somewhat in size at different examinations. Laboratory studies revealed normal urine and blood. A blood Hinton test and an intracutaneous tuberculin (1:1000 dilution) test were negative. Spinal-fluid studies were within normal limits. Roentgenograms of the skull and sinuses showed absence of the normal bone structures in the region of the cribriform plate, with obliteration of most of the ethmoid cells (Fig. 2). A pneumoencephalogram showed the ventricular system to be quite normal in size, with no evidence of an unusual collection of air near the cribriform plate and no shift or defect in the lateral or third ventricles (Fig. 3). On October 9, 1942, under Avertin and ether anesthesia, exploration of the frontal fossa was carried out through a right frontal bone flap exposed by a coronal incision. Extradural and intradural exploration over the right frontal lobe visualized a projection of dura-covered brain through a defect in the cribriform plate, and the diagnosis of anterior encephalocele was thus confirmed. However, this exposure was obviously inadequate for surgical removal of the lesion, so that routine closure was carried out. The postoperative course was uneventful and on the 6th postoperative day, again under Avertin and ether anesthesia, the coronal incision was reopened. The right frontal bone flap was again elevated on a lateral hinge 59

FIGURE

Sinus roentgenogram showing tures of the cribriform plate on the left.

2.

the absence of bony and of the ethmoid

struccells

FIGURE

3.

Lateral pneumoencephalogram showing a normal system and subarachnoid spaces.

ventricular

of periosteum. T w o m o r e burr holes were placed in the left frontotemporal region and a left frontal bone flap extending across the midline was elevated on a left periosteal hinge (Fig. 4 ) . T h e d u r a was then opened on either side of the sagittal sinus as far anteriorly as possible (Fig. 4A) a n d the sinus was ligated a n d divided. As the falx was divided down to the crista galli, a n unusual n u m b e r of large tributary veins leading into the anterior end of the sagittal sinus were divided between silver clips. Both frontal poles were retracted posteriorly, a n d it became easy to visualize a herniation of brain extending f r o m the left frontal lobe d o w n w a r d a n d forward through a defect in the cribrif o r m plate, which displaced a rudimentary crista galli toward the right (Fig. 4B). T h e herniated brain could not be delivered f r o m its extracranial compartment, so that it was amputated f r o m the frontal pole and subsequently removed piecemeal f r o m the meningeal sac (Fig. 4 C ) . This sac, which could then be invaginated into the frontal fossa, was, however, left intact in the position in which it was f o u n d in order to avoid communication of the meninges with the nasal cavity. T h e inner surface of the meningocele sac was superficially coagulated with endothermy current in the hope of speeding its obliteration. A flap of dura and periosteum was next raised f r o m the floor of the frontal fossa laterally, turned on a hinge, and sutured across the defect to the crista galli (Fig. AD). Routine closure of the dura, bone flaps and scalp was carried out. T h e postoperative course was again uneventful. O n the 7th postoperative day, examination under Avertin anesthesia revealed the masses in the nasopharynx to have shriveled markedly (Fig. 5). T h e mass in the left nostril was smaller but not necrotic. T h e nasopharyngeal airway was m u c h improved. O n the 14th postoperative day, the patient was discharged to the Wellesley Convalescent H o m e for 4 weeks, at the end of which time repair of the cleft palate was planned.

In this case, several associated developmental defects in the facial area were present. A cleft 63

FIGURE 4.

In the insert, the solid line indicates the site of incision, and the dotted lines the limits of the right and left frontal bone flaps. In A, the dura has been opened on either side of the longitudinal sinus at the anterior limit of the exposure. In B, the pedicle of the herniated brain at its origin from the left frontal pole has been exposed after division of the longitudinal sinus and falx and retraction of the frontal lobes. C shows the inside of the meningocele sac after amputation and removal of the herniated brain. In D, the dural flap has been reflected from the floor of the frontal fossa across the defect in the cribriform plate and has been sutured to the crista galli.

FIGURE

5.

This shows the shrin\age of the meningocele sac in the nasopharynx, with resultant improvement of airway, as seen on the seventh postoperative day.

palate resulted from failure of the lateral palatine processes to unite properly. Failure of the right maxillary process to fuse with the median nasal process resulted in a right incomplete harelip. According to Arey, 5 the embryonic ethmoidal cartilage consists of a mesial mass and of paired masses lateral to the olfactory sacs. The lateral masses ossify first into the spongy bone of the ethmoidal labyrinths. Fibers of each olfactory nerve pass between the unjoined mesial mass and the adjacent lateral mass. Later, cartilaginous trabeculae surround these bundles of nerve fibers and interconnect the three masses. When these perforated parts of the completed ethmoid become ossified, they are designated the cribriform plates. Presumably, failure of these cartilaginous trabeculae to form or to ossify resulted, in this case, in a defect in the cribriform plate that permitted herniation of the brain into the nose and nasopharynx. This case proved interesting not only because of the rarity of the lesion but because the even more uncommon association of the cleft palate provided an excellent view of what might otherwise have been a hidden anomaly. The possibility and danger of mistaking such a lesion for a polyp or tumor of extracranial origin and of attacking it surgically through the nose or mouth are self-evident. The development of a spinal-fluid leak into a contaminated field followed by meningitis would be an almost certain complication. The case is also interesting because the patient was an otherwise apparently normal boy (Fig. 6) with a series of congenital anomalies, all suitable for satisfactory surgical therapy. The importance of delaying the repair of the cleft palate until an 66

adequate airway had been established by removal of the encephalocele should be emphasized. The surgical approach outlined and illustrated in this report proved highly satisfactory, and is recommended for attacking lesions of this kind in the region of the cribriform plate. REFERENCES 1. I n g r a h a m , F. D., and Swan, H. Spina bifida and cranium bifidum: survey of five h u n d r e d and forty-six eases. New Eng. I. Med. 228:559-563. 1943. 2. Dandy, W . L·. Operative treatment for certain cases of meningocele (or encephalocele) into orbit. Arch. Ophth. 2:123-132, 1929. 3. Mood, G. F. Congenital anterior herniations of brain. Rhin. & Laryng. 47:391-401, 1938. 4. McGillicuddy, Ο. B. Encephalomeningoceles Otol., Rhin, ό- Laryng. 51:516-525, 1942.

Ann.

in nasal cavities.

Otol., 4nn.

5. Arey, L. B. Developmental Anatomy: A textbook, and laboratory manual of embryology. 593 p p . Philadelphia: W. B. Saunders and Company, 1934.

68

V.

T H E ARNOLD-CHIARI

MALFORMA-

T I O N : A S T U D Y O F 20

CASES

T

H E A r n o l d - C h i a r i malformation is a congenital anomaly of the hindbrain characterized by a downward elongation of the cerebellum and brain stem into the cervical portion of the bony spinal canal. T h e condition has been considered a rarity and is unknown to a large percentage of the medical profession. T h e purpose of this study is to present evidence that it is a frequent accompaniment of myelomeningocele and to discuss the clinical and pathological significance of this relation and other associated findings. REVIEW OF LITERATURE T h e literature on the malformation is limited. T h e anomaly was originally described by Arnold 1 in 1894 and by Chiari 2 in 1895. Schwalbe and Gredig 3 published an anatomic and embryologic description of it in 1907, including reference to an earlier study by Solovtzoff. 4 In 1935, Russell and Donald 3 reported 10 cases of Arnold-Chiari malformation occurring in infants with myelomeningocele. T h e y showed that the anomaly of the cerebellum and medulla in these cases was the prime factor in the production 69

of the complicating hydrocephalus, and suggested that this anomaly might be the mechanism of hydrocephalus in all cases of myelomeningocele and in some cases of simple meningocele. Penfield and Coburn 6 in 1938 presented an attractive explanation of the development of the malformation in a case of theirs observed at operation and autopsy. They considered the malformation to be brought about by traction on the brain stem in embryonic life resulting from fixation of the spinal cord at the meningocele site. Normally, growth of the vertebral column and spinal cord occurs at an equal rate until the third month of intrauterine life. After this the bony vertebral canal outstrips the cord in growth, and owing to the anchoring of the cerebrospinal axis by the position of the cerebellum above the foramen magnum, the spinal cord is normally pulled upward so that the conus medullaris comes to lie opposite the first lumbar vertebra. When spina bifida and meningocele are present, the cord may become fixed by herniation and adhesion at the site of the defect so that traction is exerted on the brain stem by the continued growth of the vertebral column and a portion of the hindbrain drawn downward through the foramen magnum. In support of this theory is the observation of Penfield and Coburn that the cerebellar "tails" retracted upward a distance of 3 cm. on being freed from their attachments to the medulla at operation. In their case, and in those of Russell and Donald, the spinal nerve roots in the cervical region were found to pass upward to the intervertebral foramens, rather than horizontally. This was interpreted as additional evidence of a downward traction on the cerebrospinal axis. 70

In 1938, Aring 7 reported a case of cerebellar and brain-stem malformation in an adult with cerebellar symptoms without associated hydrocephalus or meningocele. This resembled the ArnoldChiari malformation anatomically. McConnell and Parker 8 in 1938 published a report of 5 cases of hindbrain malformation similar to that described by Arnold and Chiari, but apparently not associated with spina bifida or meningocele. These cases, and that of Aring, tend to cast doubt on the traction theory of the genesis of hindbrain malformation, or else suggest that other mechanisms than traction may be concerned in its production. It is interesting that, in Aring's case and in 4 of the 5 cases of McConnell and Parker, spina bifida occulta was not excluded. X-ray photographs of the spine were apparently taken in only 1 case and in that one only the lumbar spine was studied and found normal. In this same report McConnell and Parker described autopsies on 6 infants with myelomeningoceles, 4 of whom had the Arnold-Chiari malformation. In the other 2 there was a "prolongation of flattened cerebellar tonsils through the foramen magnum associated with a nodule on the dorsal surface of the medulla." D'Errico 9 in 1939 discussed the surgical treatment of hydrocephalus associated with spina bifida and emphasized the importance of the Arnold-Chiari malformation in the production of hydrocephalus in such cases. He reported 10 cases of myelomeningocele, all of which had an associated ArnoldChiari malformation. Two cases were reported by Adams, Schatzki and Scoville10 in 1941. One of these was associated with myelomeningocele. The other case 71

was similar to those of McConnell and Parker, and x-ray films of the entire spine failed to show spina bifida. In 1941, List 1 1 included the Arnold-Chiari malformation in an extensive discussion of the neurologic syndromes accompanying bony developmental defects in the occipital region. Ogryzlo 12 presented 7 cases in 1942. Of these, 3 were associated with myelomeningoceles, and 1 with simple meningocele, whereas the remaining 3 had no overt spina bifida. Again spina bifida occulta was apparently not excluded. REPORTED

CASES

Twenty consecutive cases of Arnold-Chiari malformation that came under observation at the Children's Hospital from 1929 to 1943 were examined. Nineteen of these were studied at autopsy as well as clinically. Their principal clinical and anatomical features are presented in Table 1. All these patients were infants between two days and eighteen months of age. Meningocele, with or without hydrocephalus, was the reason for hospitalization in each case. The malformation of the cerebellum and medulla in these 20 cases was anatomically identical with Arnold's original case and with those of Russell and Donald. T w o parallel tongue-like processes from the inferior poles of the cerebellar hemispheres extended downward through the foramen magnum and were closely applied to the posterior surface of the medulla (Fig. 1). In each case from one half to two thirds of the medulla lay below the level of the foramen magnum. The medulla was somewhat elongated, slightly narrower than usual and flattened anteroposteriorly. 72

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