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Uterine didelphys and occult spinal dysraphism: an unusual case.

This report describes a case of uterine didelphys, lipomeningocele, meatal stenosis, and inguinal hernia presenting in a single individual. This unusual group of birth defects suggests a common mechanism of malformation in tailbud maturation which involves both the müllerian duct and the distal spinal cord.

Adult↗

Split cord malformations of the lumbar region. A model for the neurosurgical management of all types of 'occult' spinal dysraphism?

From a group of 84 patients with split cord malformations presenting to our Department between 1976 and 1990, we have selected 47 cases in whom the split cord was confined to the lower dorsal-lumbar region and in whom there were no other dysraphic features such as meningocele, lipoma or dermoid cyst. We have studied these cases of 'pure split cord malformation' in an attempt to decipher its natural history and the effects of surgical procedures designed to untether the spinal cord. We conclude that the 'neuro-orthopaedic syndrome' (which includes lower limb asymmetry, talipes and modest sensory and motor problems) is, in most cases, an inevitable consequence of the abnormal functional anatomy of the split cord and is not due to any mechanical effects of tethering of the spinal cord. Its emergence as a child grows (assuming that it was not obvious at birth) is not influenced by surgery. True neurological deterioration (defined as the loss of a previously established neurological or urological function) is a rarer event. It may occur at any age--including well into adulthood--but in children the average age at presentation is 6.8 years (range 2 years 9 months to 11 years). It is due to the mechanical effects of tethering of the spinal cord and is more likely to be arrested than improved by surgery. 'True' deterioration did not occur in any of our cases who had undergone prophylactic untethering of the spinal cord and we have concluded, therefore, that surgery has a role in the prevention of late neurological problems affecting the lower limbs and bladder. Surgery to untether the spinal cord will not, however, have any affect upon the emergence of the neuro-orthopaedic syndrome.

Adolescent↗

Spinal dysraphism: experience with 250 cases operated upon.

During a period of 4 years (December 1983-December 1987) 250 children were operated upon by the authors. At the outpatient clinic of the New Children's Hospital 313 infants and children suffering a myelodysraphic lesion were seen, 63 of whom were not treated surgically owing to the presence of severe hydrocephalus in 16 cases and complete flaccid paraplegia with marked sphincteric disturbances in 47. All of the patients came from poor families. Malnutrition, many siblings per family, poor maternal care, and repeated pregnancies and abortions have added to the large incidence of such a handicap in Egypt. The clinical features, diagnosis, and management of 200 cases of spinal bifida aperta (SBA) and 50 of spina bifida occulta (SBO) are summarized. SBA cases were operated upon as soon as possible from the time of their presentation; their preliminary assessment included an exhaustive general, neurological, urological, and orthopedic examination; and investigations such as plain X-rays to the back and skull, CT scanning of the cranium, spinal myelogram, and metrizamide CT scanning to the back were performed. The surgical outcome is far from being satisfactory in the sense of producing an ambulatory, intelligent, healthy individual, especially in a meningomyelocele patient having a high-level lesion. The authors believe that there is still much to be done for these children marked by fate, and the neurosurgeon should be one among several other physicians devoted to the amelioration or at least the prevention of worsening, if possible, of the condition in such unfortunate and handicapped children.

Child↗

Experimental study of the embryogenesis of open spinal dysraphism.

This article discusses the experimental studies that have been performed to make clear the embryogenesis of myeloschisis or spina bifida. Some of these theories include: Simple nonclosure; overgrowth and nonclosure; so-called reopening; overgrowth and reopening; and primary mesodermal insufficiency. The authors also discuss recent investigations and give a view into the future.

Animals↗

Surgical complications of open spinal dysraphism.

The embryogenesis, closure technique, and preoperative preparation of open myelomeningocele are described in this article. Both early and late complications of myelomeningocele closure are discussed with respect to predisposing factors, diagnosis, treatment, and prevention. These complications include worsened neurological level, wound dehiscence, wound infection, cerebrospinal fluid leak, postoperative ileus, symptomatic Chiari malformation, shunt infection, necrotizing enterocolitis, and problems related to kyphectomy.

Female↗

Cystic spinal dysraphism of the cervical and upper thoracic region.

BACKGROUND: Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles. METHODS: A retrospective study of 18 patients was conducted. RESULTS: In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles. CONCLUSIONS: Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.

Abnormalities, Multiple↗

Congenital lumbosacral lipomas presenting as a form of occult spinal dysraphism. A report of 9 surgically treated cases.

Nine cases of congenital lumbosacral lipoma have been diagnosed and treated surgically at the Hacettepe University School of Medicine, Department of Neurosurgery during the last 16 years. Five patients were male and four female with ages ranging from 18 months to 30 years. The most frequent complaints were mass in the back (in five cases), motor weakness in the lower extremities (in five), urinary incontinence (in five), and lumbosacral hypertrichosis (in five). On neurological examination, four patients had deep tendon reflex changes, five had motor changes and four were normal. There was significant improvement in seven cases. Two had no improvement. In this report, we review nine patients retrospectively, and discuss the surgical intervention and the results achieved.

Adolescent↗

Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale.

A highly select group of 31 patients presenting with urinary incontinence failed to respond to conservative management and were found to have unstable bladders and spina bifida occulta. After thorough evaluation they were suspected of having neurogenic bladder dysfunction possibly due to a tethered cord. Following division of the filum terminale daytime incontinence resolved in 72%, urodynamic detrusor hyperreflexia disappeared in 59% and bladder compliance improved in 66% of the patients. The operation was well tolerated and did not result in any neurological complications. The clinical, radiological and urodynamic characteristics of these patients before and after treatment are reported.

Adolescent↗