Plastic Repair of Congenital Defects Associated with Spina Bifida and Cranium Bifidum

Plastic Repair of Congenital Defects Associated with Spina Bifida and Cranium Bifidum MILTON T. EDGERTON, M.D. *

THIS report is prompted by a double purpose. Today it seems that a strong note of optimism is warranted in the surgical approach to the treatment of myelomeningoceles and encephaloceles; in addition there is very little material in the medical literature dealing with the reconstructive aspects of these major congenital deformities. In particular, we have found most heJpful the use of homogenous bank bone for large cranial defects and the use of immediate large transverse pedicle flaps to cover extensive skin defects with myeloceles of the cord. ' A very rare type of encephalocele involving the orbit is reported and its repair, utilizing the above principles, is described (Figs. 420 to 422).

BACKGROUND Spina bifida and cranium bifidum are excellent descriptive terms and include a number of congenital deformities. These lesions have in common a bony defect in some portion of the skeletal case about the central nervous system. As a result, meninges or neural tissue tend to herniate toward the body surface. Nerve damage, hypoplasia of the skin, or hydrocephalus may be present in certain of these children. That this lesion is not rare may be seen from the fact that Ingraham was able to report 546 cases from Children's Hospital in Boston alone. 6 From the Divi8ion of Pla8tic Surgery, The John8 Hopkin8 H08pital and The John8 Hopkin8 Univer8ity School of Medicine, Baltimore, Maryland.

* A88i8tant Profe880r of Surgery (Pla8tic), The John8 Hopkin8 Univer8ity School of Medicine; Surgeon (Pla8tic) and Surgeon, Outpatient Department, The John8 Hopkins H08pital. 1327

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Etiology

In 1827 Geoffrey St. Hillaire published the "adhesive theory." He felt that spina bifida and cranium bifidum resulted from adhesions of the skin (? intrauterine pressure) to the dura and brain or cord. These adhesions were thought to produce arrest of the bony growth of the spine or cranium in that area. This theory, although 125 years old, was astonishingly accurate, for we have since learned from experimental embryology that, as the neural tube closes dorsally in the fetus, the anterior and posterior neuropores remain open for about three weeks after closure of the rest (Stern berg). Normally these neuropores then close and are separated from the skin by the migration inward of mesoderm from either side. This mesoderm forms the muscle and bone that completes the closure. In the adult human, the base of the nose represents the anterior neuropore and the sacral region represents the location of the embryonic posterior neuropore. A failure of closure of one of these neuropores would explain the common location of a myelocele in the sacral region or an encephalocele at the base of the n~)8e. Similar delays in closure of the central portions of the neural tube could account for upper thoracic, cervical, or occipital herniations. P. K. Duraiswami of New Delhi (personal communication) has recently produced spina bifida experimentally in the chicken by injection of insulin into the yolk on the second day of embryonic life. Landauer21 reported in 1945 on the use of insulin and other chemicals to produce similar congenital deformities in the chick embryo. Quite recently, Butt, Pearson and Simonsen20 have reported on the experimental production of meningoceles and cranioschises in chick embryos by the injection of lead nitrate into the albumen of fertile eggs. They noted that the lesions were usually present in the occipital regions and rarely in the region of the anterior neuropore of the skull. In these chicks, lack of bone development in the region of the meningocele was quite obvious. The nonleadedcontrols in which copper, mercury or salt solutions were used, did not develop meningoceles in a single case. These same authors previously reported the finding of abnormal amounts of lead and mercury in some cases of congenital disease of the newborn. Myeloceles will, on rare occasions, present anteriorly into the pelvis or peritoneal cavity. In these locations they may be misdiagnosed as abdominal tumor, hydronephrosis or presacral teratoma. If the lesion is not recognized at operation, spinal fluid leak, meningitis and death are almost sure to follow. A firm closure of the dura is needed in the repair of these anterior defects just as in the case of posterior lesions. Several reported attempts to "marsupialize" these lesions to the peritoneum resulted in death. 6

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Pathology

Frazier 5 has given an excellent review of the anatomy and pathology of spina bifida. Some studies 6 have shown that up to 25 per cent of all children possess some form of occult vertebral bony defect. It is well known that spina bifida occulta may sometimes be associated with neurological changes in the legs or bladder with no evidence of external tumor or with only a patch of hairy skin over the midline of the back to indicate the site of trouble. Other children will show maldevelopment of the feet (Ingraham) or scoliosis, or disturbances in gait with spina bifida occulta. Examination may reveal only a palpable bony defect or a lipoma over the dorsal spinto In some of these patients the symptoms may progress until operation. If at that time adhesions to the neural elements are divided the symptoms may subside somewhat or remain irreversible. This would be suggestive evidence to support early operation. Spina bifida may be accompanied by a meningocele without herniation of actual neural elements. This type is most readily treated surgically but unfortunately is only about one-sixth as common as the much graver myelomeningocele. The myelomeningocele represents a complete failure of the neural tube edges to roll together and close. 6 As a result there is direct communication between the external environment and the central canal of the cord. In such cases the pocket of distending fluid which produces the tumor actually lies anterior to the cord and the latter is flattened out in the central part of the tumor. The nerve roots thus run anteriorly from this neural plate to pass through the spinal fluid in the sac and hence reach their spinal foramina. It may be seen that the more distended such a sac becomes the greater will be the damage from stretching of the nerve roots. The danger of a midline incision through the medullary neural elements can be readily understood. Frazier refers to this central area as the area vasculosa or medullary layer. It is surrounded by another thin band of scar called the area serosa. This is lateral to the neural elements but is not covered by healthy skin. It may frequently become ulcerated. Peripheral to that one finds the area dermatica. Here the skin is thick but is inflamed and hypertrichotic. Even this area is not often of use in the repair of these deformities. In rachischisis or syringomyelocele the distending fluid is in the central canal of the cord and the neural elements are badly splayed out. In such cases the condition is universally fatal. Incidence

Myeloceles and encephaloceles have been reported in something over a thousand children but this undoubtedly represents only a fraction of

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the incidence as only a few of the larger clinics have surveyed their cases. The patients are mostly of the white race and females are definitely more prone to the anomaly than males. Ingraham 6 and others have shown that approximately 50 per cent will show other congenital defects. Over 15 per cent show clubfoot but some of these foot deformities may be secondary to the neurological damage associated with cord myeloceles. In support of this fact, harelip or cleft palate is rarely present. The incidence of congenital cleft lip or palate is normally about equal to that of clubfoot in most sections of this country and the same ratio should exist in the group of children with spina bifida except for the fact that the spina bifida itself must increase the number of clubfeet by neurological damage. Cord meningoceles (spina bifida cystica) and meningomyeloceles are about five times as common as encephaloceles. Very rarely a child will be seen with multiple herniations of the central nervous system. One of these may be an encephalocele and the other a cord myelocele. We have found that collections of abnormal gritty fat are not uncommon in association with cord defects. Ingraham reported approximately one in fourteen of his cases showed such lipomas. A multiloculated lipoma of the lumbosacral region should always raise the diagnostic possibility of a cord herniation. By common consent the term "encephalocele" has come to encompass all types of cranium bifidum. Dermoids may be attached to the durabut these, of course, are not encephaloceles. About 75 per cent of the encephaloceles lie in the occipital region. The remainder are anterior and may be sincipital (frontal, nasal or orbital) or basal (nasal, or pharyngeal). Mood 9 has been able to find only forty anterior encephaloceles in the literature and notes that they are about half sincipital and half basal. A number of the "hidden" or basal encephaloceles have been mistaken for large polyps, gliomas or· juvenile nasopharyngeal angiofibromas. If basal encephaloceles are removed through the nose or mouth and dural closure is neglected, the usual result is rhinorrhea, meningitis and death or recurrence (McGillicuddy 8). The encephalocele presenting in the nasopharynx probably results from a fusion failure of the cribriform plates. This brain mass may then prevent mechanical closure of the palatal processes and a cleft palate (usually not a cleft lip) may result. Most of the sincipital encephaloceles present at the fontanelles or suture lines and show as external tumors of the nose, orbit or forehead. The most common sincipital type is the nasofrontal variety. The frontal bone arises· from a right and left center of ossification. Each center ap-

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pears about the eighth week of intrauterine life and although these centers do not usually unite until about the eighth year, they approach each other quite closely in the prenatal period. 9 A failure of closure of the anterior neuropore would be associated with interruption in this normal sequence of bony development. The encephalocele involving the orbit is the rarest variety of all. In fact, except for the case presented in this paper, the only orbital encephaloceles described in clear detail are the ones treated by Dandy,3 and that reported by Siris from Bellevue in 1936. 18 The defect in this orbital type is located at the junction of the lacrimal, frontal and nasal bones. Exophthalmos, diplopia and destruction of the attachments of the extraocular muscles will usually result. These problems will be considered below under "treatment." THE PROBLEM OF HYDROCEPHALUS

The treatment of spina bifida or cranium bifidum cannot be rational without an understanding of the nature of the often associated hydrocephalus. Much interesting work at the present time deals with this problem. Originally it was felt that hydrocephalus with spina bifida was progressive and led to death or imbecility of the child. Such a view obviously discouraged operative intervention. This idea was soon found to be quite false when a few of these children showed an arrested hydrocephalus and lived to develop excellent mental faculties in spite of the continuing existence of a dangerous, thin-walled myelomeningocele. This myelocele may have been denied surgery for many years because of surgical pessimism. Surgical repair was then attempted on some of these children and a few showed an actual increase in their hydrocephalus in the months following surgery. This resulted in the theory that correction of the myelocele with removal of the sac led to increase in the hydrocephalus. The sac was considered to be a "safety valve" during this period of surgical thought. Penfield and Coburn13 next came forward with their suggestion that if the sac were saved in the repair of these defects, the hydrocephalus would be unlikely to increase following surgery. They believed the internal lining of the sac had something to do with the absorption of spinal fluid. Since their publication in 1932 many other workers have tried this "sac saving" procedure and most have reported no decrease in the amount of hydrocephalus following operation. 6 Here at Johns Hopkins the neurosurgeons feel that hydrocephalus will increase or develop without regard to operation on the myelocele, and that one is not justified in withholding operation for that reason alone. 12

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Scoville17 has recently suggested that some of the previous instances of postoperative hydrocephalus may in truth have been due to continued partial herniation with poor plastic repair and nerve root adhesions. It should be emphasized that the degree of hydrocephalus may not be correlated at all with mental retardation. Putnam16 believes that this mental retardation in many hydrocephalics is due to a primary cortical atrophy. And he feels that operation should not be withheld from patients with hydrocephalus, and even with paralysis. Hydrocephalus is present in 25 per cent of patients with a bulging spina bifida and some slight continuing increase in the hydrocephalus should be expected in most of these patients. Some will show, however, no increase in head size. Siris18 has reported a child with increasing hydrocephalus even before surgery. The child had a very thin-walled sac that was repaired and seemed normal in every way two years later except for a slight and decreasing amount of vesical incontinence. This patient and others would have surely been lost had the repair been withheld because of increasing hydrocephalus. Figures 418 and 419 illustrate a case of ours with a similar history. The danger of allowing one of these thin sacs to rupture is illustrated by the fact that syringomyelocele (congenital cord opening) is universally fatal. In 1914 Weed and Dandy showed that most of the cases of hydrocephalus associated with spina bifida are of the communicating variety. The communication is between the lumbar subarachnoid space and the ventricles. If the foramina of Luschka and Magendie are patent, then fluid may escape from the fourth ventricle into the spinal subarachnoid space-----but if this fluid at the same time is blocked from getting into the subarachnoid space of the posterior fossa it will then be unable to reach the cranial arachnoid villi where Dandy felt that about threefourths of the cerebrospinal fluid is absorbed. In this manner an internal hydrocephalus develops and the cord defect does not close. In some such children meningitis has developed and at autopsy the exudate has been found confined to the subarachnoid space below the foramen magnum. This would be further evidence for a block at that location. Russell and Donald16 reported in 1935 that hydrocephalus is quite rare in association with simple meningocele as opposed to its high incidence with meningomyelocele. They examined the brain stem in cases of spina bifida occulta and found it normal. They then reported ten consecutive cases of Arnold-Chiari malformation of the brain stem in meningomyeloceles with hydrocephalus. Ink injections into the lateral ventricles (in vivo) supported the suggestions of Dandy that a block of the subarachnoid space existed at the level of the foramen magnum. The ink could be recovered, however, from the lumbar subarachnoid space. The association of this interesting deformity of the brain stem with

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spina bifida led Penfield I4 to suggest that traction on the cord from below pulled the cerebellum downward into the foramen magnum and caused the block at that level. He reasoned that, since the vertebral column grows more rapidly than the cord after the third month of intrauterine life, adhesion between these two structures would result in a pulling down of the cord (and cerebellum) with increasing length of the vertebral column. It was suggested that this "adhesion" would lie in the region of the spina bifida. Recently it has been suggested that a diastematomyelia with a bony spur may cause the "anchoring." The original Arnold-Chiari syndrome shows typically two tongues of cerebellar hemispheres and one-half to two-thirds of the medulla lying below the level of the foramen magnum. * The cerebellum is hypoplastic. The fourth ventricle is usually elongated and flattened by the foramen magnum. The foramina of Magendie and Luschka lie below the foramen magnum and internal hydrocephalus is usually present.u Russell feels this anatomical arrangement may block flow into basilar cisternae but allow fluid into spinal canal. 16 About half of the cases of hydrocephalus are definitely of the communicating type. The cervical nerve roots are found to pass upward to reach their foramina, thus further suggesting a relative downward displacement of the cord. Ingraham 6 has reported some 20 cases of Arnold-Chiari deformity in a series of some 546 cases of spina bifida. He suggests the relationship between this and myelocele, but points out that the Arnold-Chiari malformation has not yet been produced experimentally. He does feel strongly that patients with Arnold-Chiari deformity should have a surgical exploration of the posterior fossa approximately two weeks after freeing the spinal myelocele. In determining which patients have this malformation, the importance of craniolacunia on roentgen studies of the skull should be emphasized. We have not noted these radiolucent areas on patients with myeloceles in the absence of brain stem malformation. There are, however, a number of observations that suggest that this attractive theory of a mechanism for the hydrocephalus is not wholly accurate. It is well known that hydrocephalus often occurs in patients with meningomyelocele in whom no brain stem deformity can be demonstrated. There are already 6 reported cases of typical Arnold-Chiari malformations in adults with no associated evidence of myelocele or meningocele. Dr. F. J. OtenasekI2 of this hospital has had 2 additional cases of this type recently. Drs. Bachs and Walker! report 4 cases of

* Arnold, J.: Zieg. Beitr. 16: 105, 1894. Chiari: Quoted by Gredig, M. and Schusable, K. D.: Am. J. Dis. Child. 8: 406,1934.

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patients with Arnold-Chiari malformation and all showed a complete block with a noncommunicating type of hydrocephalus. RESULTS OF TREATMENT IN PREVIOUS REPORTS

The momentum of surgical pessimism is hard to arrest. For many years surgeons have been disinclined to operate on the child with a severe spina bifida. Yet Frazier reports that this condition is 81 per cent fatal if not operated on within the first year of life.· Moore, only twentyfive years ago, reported a 50 per cent operative mortality. Cutler reported some 65 children operated on with a 53 per cent mortality. Siris18 made a careful study of the patients seen in Bellevue from 1919 to 1934. Fifty-six per cent were not offered surgery and died within a few months or years. Forty-four per cent were operated on and this group showed a hospital mortality of 37.3 per cent. These children were operated on at an early age and attempt was made to preserve the lining of their sacs. Even at that time Siris felt that mild hydrocephalus or sac rupture was not per se a contraindication to repair. In judging the feasibility of operation he stressed (a) the condition of the covering sac, (b) the extent of nerve tissue involvement, (c) the extent of the bony defect, (d) the degree of hydrocephalus and (e) the nature of any other anomalies that might be present. Many of the older reports stress the "lack of practical possibilities in the relief of these conditions" (myeloceles and encephaloceles) and merely describe the number and types of cases seen. In many instances where surgery was undertaken, the tumor was actually excised instead of saving all neural tissue and returning it to its bony case. Pathological reports of "normal brain tissue" or "normal spinal cord" were described. Almost invariably no reconstructive principles were employed and many of these wounds broke open from tension. Some continued to leak spinal fluid, or simply could not be closed because of the size of the defect. Meningitis was common postoperatively and prior to the days of antibiotics this usually meant death. Mixter was pessimistic enough to state, "I am opposed to surgical interference in patients in whom neurological signs indicate irreparable and complete damage to nerve roots supplying bladder, rectum and legs, even if repair of the defect seems possible." What happens to these children that are passed over? Siris in 193618 reported on 12 children with myelomeningoceles. Seven of these were thought to be inoperable. All 7 died. Such reports are typical and illustrate both how little is to be lost by surgical effort and how important it may be to increase the percentage of operability by use of immediate plastic closures. Of 37 patients on whom Siris did operate, 67.7 per cent survived up to a

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ten-year follow-up. Operative and immediate postoperative mortality was 32.3 per cent and wound breakdown was important as a cause of death. Improved soft tissue repair should reduce this immediate postoperative mortality appreciably. The outlook of the encephalocele was equally black in 1936. Siris reported a rather typical series of some patients with 10 encephaloceles. Five were thought inoperable and all of these died within a few monthsusually of meningitis. Of the 5 operated on, 3 died of overwhelming meningitis and 2 of shock. IS With discouraging results of this kind as a background, however, Kolodny7 in 1933 gave us one of the first reports showing favorable results of operation on children with myelomeningoceles and paraplegia. In 1944 Ingraham 6 gave further cause for optimism by pointing out that the prognosis was not as bad as originally taught. In a very large series he found that the hydrocephalus often tended to stabilize. He was unable to substantiate Penfield's "sac saving" method of minimizing hydrocephalus and he returned to excision of all excess sac. He utilized a "head low" position after operation and placed a needle in the dural canal to keep intracranial pressure low. He described 30 children with myelomeningoceles that had developed normally without clinical evidence of neurological impairment following surgical repair. He suggested that progression of neurological dysfunction may in some cases result from vertebral column growth pulling on the cord due to anchoring of the cord at the site of the defect. Apparently Ingraham's group did not shift flaps to close cord defects. They did not state the number that showed leakage of spinal fluid after operation or the number of wound separations but the immediate operative mortality was 12 per cent. They found a congenital lack of neural elements in some children. A low percentage of their patients actually improved neurologically after operation-possibly due to release of traction or compression on the cord. 6 Nerve tissue that was found ending in the sac wall was sacrificed. Rarely anomalous abnormal neural tissue may be present in the sac and this may be excised. Caution must be exercised, however, as we have found occasional movement of the legs on electrical stimulation of nerve fibers that apparently were splayed out into the wall of the myelocele sacs. Recently optimistic reports 6 • 7 have led to more surgical effort in the repair of encephaloceles as well as myeloceles. This effort has been rewarding. About one-third of infants with cranium bifidum die soon after birth or possibly before operation. But 35 to 40 per cent of those treated in recent years have developed into children normal in all respects. 6 In the more serious cases neurologic disability may result in paresis, loss of sensation, lack of sphincter tone, or occasional convulsions. Most neurosurgeons state that they operate on these children by

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choice at 12 to 18 months of age. Such a delay will allow other congenital anomalies to manifest their presence, will permit some measurement of hydrocephalus and mental development, and will allow surrounding normal structures to enlarge and be more readily handled at operation. It would seem to us, however, that if the encephalocele covering is thin, the dangers of rupture and meningitis would outweigh the convenience of delayed operation. Ingraham stated that with large bulging spina bifida or cranium bifidum he has had to wait for "peripheral epithelialization" to gain adequate skin for closure. 6 The plastic £lap procedures described below (Figs. 418 and 419) should make unnecessary this undesirable delay and accomplish much more certainly the type of secure repair desired. The treatment of clubfoot and other orthopedic deformities may have'to await firm closure of the vertebral or cranial defect. This is an additional reason for seeking an early repair. It is obvious that one should deliberately withhold heroic surgical repair with encephaloceles in which there is marked lack of function of the entire cord. But it should be stressed that the mere size of the mass or tumor may not be correlated at all with the degree of neurological dysfunction. This is notoriously true of occipital encephaloceles. Bachs and Walker point out that "hydrocephalus due to congenital atresia of the foramina of Luschka and Magendie and Arnold-Chiari malformation may be relieved in the majority of cases."l THE QUESTION OF OPERABILITY

It may be seen from the above that although there is an increasing tendency to attempt salvage of more and more infants with encephaloceles and meningomyeloceles, there are still many differences of opinion as to the criteria of operability. It must be remembered that the extent and reversibility of paralysis and incontinence are difficult to determine in the infant. Operation should not be withheld solely on the fear that neurological damage might become more severe later. One need not wait until an accurate prognosis can be given to offer operation to these deformed children. Death, meningitis or chronic nursing care may be the only outcomes of . refusing to operate. Hydrocephalus may be recognized at birth by craniolacunia (Lukenschadel), separation at the cranial sutures, and diastasis of the fontanelles. It may progressively increase, become arrested either before or after operation, or not manifest itself at all. This hydrocephalus does not seem to be increased by operation on the myeloceles and we have seen examples of patients operated on in the presence of increasing hydrocephalus who became "arrested" and developed normal mentality' (Figs. 418, 419).

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Extreme mental retardation is probably a contraindication to major surgery-but here again mental development may be difficult to assess in the infant and the nursing problem presented by such a child may be greatly simplified by a single operative procedure. At present, the principal difficulty in early operative repair is the technical problem of gaining a one-stage reconstruction of the larger defects. Utilization of well-known plastic methods makes this feasible (Figs. 418 to 422). The generous use of blood transfusions and chemotherapy has reduced the hazards of operative shock and meningitis tremendously. Complete or partial paralysis of extremities or bladder is certainly not a definite contraindication to operation. These symptoms will sometimes improve after surgery. In the case of encephaloceles, however, it must be recognized that if the cranial sac contains part of the ventricular system on pneumoencephalographic studies that the prognosis will be poor. With most other encephaloceles, any spasticity or difficulty in gait may show improvement on return of brain substance to the cranial cavity. TREATMENT

I have come to feel that newborn infants with spina bifid a or cranium bifidum should have operation deferred until the child is free of jaundice, and has regained its birth weight. There s):lOuld be absence of congenital heart disease or other major deformity, and a normal hemoglobin should be present. If there is any rash of the perineum, or gluteal folds associated with anesthesia from the cord lesion, this rash should be cleared up by placing the child face down on a Bradford frame and giving diligent nursing to reduce the danger of meningitis at operation (Fig. 418, upper). Preoperative chemotherapy is routinely employed and drug sensitivity studies are made of any bacteria found in the gluteal ulcerations. At the time of operation it has been our practice to isolate the operative area from the anal region with skin glue. Warming pads may be placed beneath the child to avoid chilling. The head is usually placed low to avoid excessive sudden loss of spinal fluid on opening the sac. Nerve filaments that end in the sac wall probably have no useful function but our policy has been to return them to the neural canal when possible. Any that are to be sacrificed should be first touched with a neurostimulator to determine possible motor function in the legs or hips. No attempt is made to utilize the thin scar or surrounding inflamed epithelium in the repair. The bony defect with meningoceles and myeloceles will rarely require bone grafting unless the defect i.s quite large and the vertebral column

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is unstable. Lordosis will usually be excessive in these patients. Albee suggested the use of tibial grafts to bridge the gap. Bobroff2 used iliac crest grafts and left the bone attached to the soft tissues to preserve its

Fig. 418. Upper, Preoperative view of large sacral meningomyelocele in a 3! month old white female (History No. 512686). Spinal fluid actually "exuded" through the sac. Note the pruritic rash associated with perianal anesthesia. Muscle paralysis was present in both lower extremities. Lower, At operation the sac. has been removed and the neural elements reduced. Fascial flaps were turned in from either side and a large double pedicle skin flap has been shifted down from the small of the back. A split graft has just been taken from the left buttock, and is being applied to the dorsal donor defect.

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circulation. Zenenko 19 described the use of local bone from the adjacent vertebral arches and tuberosities. All of these authors suggesting osteoplastic procedures recognize the fact that the repair should be of sufficient stability to resist continuous pressure. Frazier" states that if the defect is greater than 2 em., the repair may require the utilization of rigid tissues for permanent results.

A

B

Fig. 419. A, Postoperative photograph showing operative area in same child as shown in Figure 418. The split graft has thickened so that it is hardly visible. B, The same child 3 years after operation. The hydrocephalus is completely arrested and mental development is normal. The child is able to walk unsupported, and even without the aid of the reinforced shoes shown in the photograph. There is a decreasing amount of urinary incontinence which we hope to correct surgically when the child is older.

The bone flaps and grafts do add appreciably more surgical trauma and shock to the operative procedure and we have not often employed them for spina bifida (in contrast to cranium bifidum). Recently N osikll has reported the use of tantalum mesh to repair the bony gap. This offers the advantage of being readily obtained and inserted but it fails to enlarge with the bones as the child grows larger and is probably more susceptible to trauma than autogenous tissue. If bone repair is needed a homograft of thin rib or cranial bank bone is easily applied, readily accepted by the tissues, and is naturally curved to accommodate the neural elements beneath (Figs. 420 to 422). Fascial

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flaps may be turned over the bony defect using musculofascial pedicles on either side of the midline. The cutaneous repair is probably the most important layer in the closure of cord defects. If the sac is excised in a transverse axis a single, broad, double pedicle flap can be raised from the normal skin just cephalad to the defect (Fig. 418, lower). There is no objection to having this flap cross the midline. The flap is undermined at the level of muscle fascia and then shifted downward so as to lie over the muscle flaps without tension. A split-thickness graft may then be taken from one buttock and immediately sutured into the donor defect left by the movement of the skin flap. A firm pressure dressing is then applied and the child is returned to the ward and kept face down for forty-eight hours. We have not found it necessary to keep these children in a head-down position. Ingraham has stated: "the first postoperative week is an extremely critical period because of the danger of spinal fluid leakage, infection at the closure site, and meningitis."6 He suggested that Z-flaps might be of value in the plastic repair but we have not been able to gain enough lateral mobility of the skin to use this type of closure. Using the method described above, in some 8 cases with large skin defects, we have had no postoperative fistulas or meningitis. Of course, the smaller defects may not require a formal pedicle flap. In none of these patients have we inserted a needle to reduce intracranial pressure. Spinal fluid leak is probably a result of skin closures under tension. Moore lO has previously suggested the use of vertical relaxing incisions but did not state whether he had actually used the method and with what successes he may have met. N osikl l states that simple closure is still attempted most commonly (with many failures). A troublesome pseudomeningocele may then follow. Ingraham 6 describes a case in which the patient was returned to the operating room at least three times in attempts to resuture the wound and prevent cerebrospinal fluid leakage. In recent years-thanks to chemotherapy-it has become quite uncommon to see a case of early postoperative meningitis in these children. Once the myelocele is repaired, if hydrocephalus is arrested or absent, attention may be turned toward orthopedic correction of any foot deformities and at a later age one of the plastic operations to control urinary or rectal incontinence may be employed. The actual number of such children requiring this complex program is quite small. A certain number of these patients will also show the Arnold-Chiari malformation.13 There are now two possible modes of treatment available to them. In 1938 D'Erric04 described 3 such children who had obtained good results from operative removal of the occipital bone and posterior portion of the foramen magnum. There was 1 additional operative death and 1 child who died of meningitis a year later. In recent years this bone

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decompression operation has been tried by a number of neurosurgeons in an effort to relieve the hydrocephalus. Some have obtained good results whereas others prefer to attack the hydrocephalus itself. Putnam 15 has attempted coagulation of the choroid plexus to reduce spinal fluid formation. In his first 16 patients 7 were alive and improved some months later, but there were 8 deaths within forty-eight hours of operation. Here in Baltimore there has been more enthusiasm for the use of ureterosubarachnoid anastomoses to relieve the hydrocephalus. 1 With regard to the treatment of the encephaloceles, much the same principles are followed. The neural tissue is returned to' thE! cranium whenever possible. A careful dural closure is sought. This may require a graft of fascia lata. If the skin covering is inadequate, pedicle flaps may be needed here, just as in the cord defects. Due to the anatomical requirements of the skull, and unlike the cord myeloceles, bone repair is usually required. Scoville17 reported recently 2 cases of encephalocele in which he attempted to close the defect with tantalum mesh. He tried to insert the mesh on a bias to allow it to "stretch with growth." I have already seen 2 patients (see Figs. 420 to 422) in whom tantalum was employed for encephaloceles who showed recurrence of the brain herniations as the children grew and the defects enlarged. This necessitated secondary repairs complicated by scar from the previous operation. The use of bone grafts is not a new idea in the repair of these defects and the soundness of the use of such tissue in reconstruction is too well known to require comment. Yet the small child may not have a readily available donor site for a sufficiently large autogenous bone graft to close some skull defects. In addition, the taking of such a graft may add greatly to the extent of the operation. For these reasons we have used homogenous bank bone. And in one case (Fig. 421, lower) we have used homogenous cranial bone preserved in aqueous merthiolate for fourteen months prior to the repair of the encephalocele. Because of the almost purely cortical nature of this cranial bone it has been suggested that the graft might not become vascularized and might fail to unite. This has certainly not been the case. The graft has remained firmly in place and shows excellent bony union. The child has grown considerably and there had been no evidence of recurrence of the encephalocele. This patient is of additional interest because she represents only the third reported case we have been able to find of an encephalocele involving the orbit. 3 • 18 The only previous description of therapeutic effort to correct such a condition is that of Dandy in 1929.3 He described a patient with pulsating exophahalmos caused by a congenital defect of the posterior part of the roof of the left orbit. The globe in his case (like

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ours) was grossly depressed downward and outward. There was no diplopia but subsequent operations were carried out on the extraocular muscles. Because of the posterior location of that bony defect, Dandy felt it wise to repair it intracranially and used an autograft of the outer table of the patient's skull to cover the orbital roof. The patient was~'a 16 year old girl who showed no apparent hypertelorism. -

Fig. 420. Preoperative"photographs of a 19 month old Negro female (History No. 562913). This:child had a recurrent left naso-orbital encephalocele. The hypertelorism wasJincreasing each month, and generalized right sided convulsions were becoming quite frequent. A previous unsuccessful attempt to repair this with tantalum mesh had been undertaken by a neurosurgeon in her home town.

CASE REPORT

Our patient (JHH No. 562913) was a small Negro child of 19 months with a pulsating exophthalmos and clinical hypertelorism. Shortly after birth a surgeon in her home town inserted tantalum mesh to control a large encephalocele of the orbital ftnd nasolacrimal region on the left side. The herniation promptly recurred and the patient developed increasing exophthalmos and began to have periodic generalized convulsions (Fig. 420). Because of the anterior and orbital location of this defect, Dr. A. Earl Walker felt that this lesion was not suitable for intracranial repair. We accordingly approached the tumor through the previous incision in the nose and forehead. The dura was freed and the frontal lobe returned to the cranial cavity. There was a 3.5 cm. defect in the roof and medial walls of the left orbit, extending around into the glabella regions (Fig. 421). The cribriform plate was depressed to about the level of the infraorbital ridges. There were no

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nasal bones on the left side. The normal pulley of the left superior oblique muscle was missing. The preserved cranial bone was quickly shaped with a power saw and ronguers, and was hinged with small loops of stainless steel wire so that a portion would extend back along the medial wall of the orbit·and a small

- Fig. 421. The upper figure shows the anterior portion of the bony defect with frontal lobe protruding into it. The previously applied tantalum mesh has broken away from the bone edges and may be seen near the base of the nose. In the lower figure, repair has been carried out utilizing a homograft of preserved cranial bone removed and preserved for 14 months. The bone graft has been hinged so that a portion of it will extend back along the roof and medial aspect of the left orbit to close the defect in that region. This unphysiological incision was utilized only because of its preexistence due to the former operation.

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portion would cover the anterior defect at the root of the nose. The graft was heldJ6.rmly in place by means of additional drill holes and steel wires through the ..!edges of the cranial defect. The medial canthal ligament was then found and brought upward to obtain a level matching the inner canthus on the right side. A Bunnell pull-out wire technic was employed to anchor this ligament to the right nasal bone. It may be seen that these alterations permitted a partial correction of the hypertelorism. There was some accumulation of spinal fluid under the skin in the first week after operation but aspiration and application of a pressure dressing prevented its recurrence. The child has remained well, developed normally, and happily her convulsive episodes have ceased. Additional minor plastic procedures may be needed when she is older.

'Fig. 422. Photographs taken 1 year postoperatively show no recurrence of the encephalocele. No pUlsations can be felt in the region of the left orbit, or base of nose. The hypertelorism has been corrected by an actual distance of 3 mIn. and the inner canthus has been raised. Happily the child's convulsions became less frequent following operation and have finally ceased. Intelligence tests reveal normal development. SUMMARY

A brief review of the present status of spina bifida and cranium bifidum has been undertaken. Special emphasis has been placed on the need for more optimism in the treatment of children with these deformities. It should be stressed, of course, that many children with very severe deformities and permanent loss of function as a result of an extensively damaged central nervous system may not have their lot greatly improved by recent reconstructive methods. However, more of these children may be left relatively normal today than was possible a few years ago. An outline of the general surgical program that is currently available' is described, emphasizing the importance of using well-established reconstructive principles. In particular, we have found large transverse double

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pedicle flaps to be most useful in the immediate closure of large cord defects. The availability and ease of application of homogenous bank bone for the repair of skull defects is stressed. The superiority of human tissue over foreign materials in the repair is suggested. A very rare type of encephalocele involving the orbit is described and its repair is shown. REFERENCES 1. Bachs, A. and Walker, A. E.: Surgical Clinic on Hydrocephalus (this issue). 2. Bobroff. Chir. Laitop Mosk., 1892 II, 468. 3. Dandy, W. E.: Operative Treatment for Certain Cases of Encephalocele into Orbit. Arch. Ophth. 2: 125-132, 1929. 4. D'Errico, A.: A Surgical Procedure for Hydrocephalus Associated with Spina Bifida. Surgery 4: 856, 1938. 5. Frazier, C. H. : Surgery of Spine and Spinal Cord. New York, Appleton, 1918, p.265. 6. Ingraham, F. D.: Spina Bifida and Cranium Bifidumj A Survey of 546 Cases. New England J. Med. 228: 745-750, 1943. 7. Kolodny, A.: Results of Surgery in Spina Bifida. J. A. M. A.l0l: 1626 (Nov.) 1933. 8. McGillicuddy, O. B.: Encephalomeningoceles in Nasal Cavities. Ann. Otol., Rhin. & Laryng. 51: 516-525, 1942. 9. Mood, G. F.: Congenital Anterior Herniations of Brain. Ann. Otol., Rhin. & Laryng. 47: 391-401, 1938. 10. Moore, J. E.: Spina Bifida with the Report of Three Hundred and Eighty-five Cases Treated by Excision. Surg., Gynec. & Obst. 1: 137, 1905. 11. Nosik, W. A.: The Repair of Lumbar and Cranial Meningocele with Tantalum Gauze. J. Neurosurg. 8(5): 540, 1951. 12. Otenasek, F. J.: Personal communication. 13. Penfield, W. and Coburn, D. F.: Arnold-Chiari Malformation and Its Operative Treatment. Arch. Neurol. & Psychiat. 40: 328, 1938. 14. Penfield, W. and Cone, W.: Spina Bifida and Cranium Bifida. J.A.M.A. 98: 454, 1932. 15. Putnam, T. J.: Discussion of Penfield's Paper, "Hydrocephalus and Spina Bifida." Surg., Gynec. & Obst. 60: 369, 1935. 16. Russell, D. and Donald, C.: The Mechanism of Internal Hydrocephalus in Spina Bifida. Brain 58: 203, 1935. 17. Scoville, W. B. and McLaurin, R. L.: Tantalum Mesh Repair of Congenital Bony Defects of Skull and Spine. J.A.M.A. 147(7): 639-640, 1951. 18. Siris, I. E.: Spina Bifida. Ann. Surg. 103: 97-123, 1936. 19. Zenenko: Diss., 1895, 61 pp., St. Petersburg. 20. Butt, E. M. and others: Production of Meningoceles and Cranioschisis in Chick Embryos with Lead Nitrate (19340). Proc. Soc. Exper. BioI. & Med. 79: 247, 1952. 21. Landauer, W. J.: Rumplessness of Chicken Embryos Produced by the Injection of Insulin and Other Chemicals. J. Exp. Zool. 98: 65, 1945.