1/12. dermoid cyst with dermal sinus tract complicated with spinal subdural abscess. Spinal subdural abscess caused by spread of infection with the dermal sinus tract is rare in children. This article reports on a 1-year-old male with prolonged fever, progressive paraplegia, and bowel and bladder dysfunction resulting from a spinal subdural abscess secondary to an infected spinal dermoid cyst with a dermal sinus tract. This is the youngest patient to be reported having this condition. Surgical intervention was performed to find a tumor that had capsule and keratinlike contents. culture of the abscess was positive for escherichia coli and bacteroides vulgatus. He received 6 weeks of parenteral antibiotic treatment. This patient illustrates the importance of urgent radiologic examination, immediate surgical resection, and appropriate antibiotic therapy for spinal subdural abscess. ( info) |
2/12. Detection of skin over cysts with Spina bifida may be useful not only for preventing neurological damage during labor but also for predicting fetal prognosis. Spina bifida is one of the most common open neural tube defects. There are two common types of spina bifida cystica, myelomeningocele and meningocele. Special attention to the thickness of the cystic sac (presence of intact skin and subcutaneous tissue) on magnetic resonance imaging is advantageous for determination of whether the child will profit from cesarean section in order to prevent neurological change (infection and drying of nerve tissue) and for management of spina bifida (most meningocele) during the perinatal period. Furthermore, skin detection may help to predict the prognosis of spina bifida after birth. meningocele, with intact skin over the cyst, has a better clinical course than myelomeningocele. Some myelomeningoceles with neural tube defects in a lower position, also frequently having an intact skin over the cyst, have almost the same clinical course as a meningocele. From this, we hypothesize that a baby with spina bifida who has intact skin over the cyst might have a good prognosis neurologically. In this report, we concentrate attention on the skin over cysts in 3 cases (1 meningocele and 2 myelomeningoceles). ( info) |
3/12. Diastematomyelia with terminal lipomyelocystocele arising from one hemicord: case report. Diastematomyelia is an uncommon spinal dysraphism with midline dorsoventral clefting of the spinal cord. Formation of syrinx cavity within the hemicord, vertebral anomalies and lipomas is known to be associated with diastematomyelia. We present an unusual case of a 4-month-old girl, who presented with diastematomyelia and terminal myelocystocele, and dilatation of the terminal portion of the spinal canal with formation of a cystic structure. Findings were confirmed by subsequent surgery and histopathology. Ectopic right kidney, Chiari I malformation and changes of partial sacral agenesis were other findings noted in this case. No case report of formation of terminal myelocystocele arising from one hemicord was described in the literature we reviewed. ( info) |
4/12. Sacral hemangioma with sinus tract in an infant. Congenital midline cutaneous lesions should always alert the clinician to the possibility of spinal dysraphism. These lesions can take many different forms. The physician should be cognizant of such lesions in order to avoid potential neurologic complications. We present a patient with a midline sacral hemangioma associated with a congenital enteric sinus cyst, a previously unreported association. ( info) |
5/12. Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment. spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment. ( info) |
6/12. Split cord malformation as a cause of tethered cord syndrome in a 78-Year-old female. A 78-year-old woman presented for evaluation of back pain, urinary dysfunction, leg weakness and progressive equinovarus foot deformity. She reported that shortly after her birth in 1924, she underwent resection of a subcutaneous 'cyst' in the lower lumbar area. Seven years prior to evaluation at our institution, she had undergone bilateral total knee arthroplasty for osteoarthritis. After the procedure, she began to experience severe low back pain that radiated into her legs. Weakness of the foot inverters, urinary dysfunction and worsening bilateral equinovarus foot deformity developed in the years following the surgery. MRI revealed a split cord malformation with a tethered spinal cord. Because of the patient's age and poor medical condition, her symptoms were managed conservatively. This case demonstrates symptomatic deterioration in an elderly patient with a tethered spinal cord after many years of clinical stability. ( info) |
7/12. Thoracic myelocystoceles--two variants. Myelocystoceles are rare lesions. Rarer still are thoracic myelocystoceles. Two patients with thoracic myelocystoceles are being reported. The first patient presented with swelling in the thoracic region with paraplegia and incontinence. MR with MR myelography revealed the malformation to be a thoracic myelocystocele. The second patient presented with a midline cutaneous mass lesion without neurological deficit. MRI revealed a Type I split cord malformation, a CSF containing sac in the thoracic region that communicated through a stalk-like structure to the hydromyelic hemicord. Surgery in both these patients did not produce any change in the neurological status. The importance of recognizing thoracic myelocystoceles, their clinical presentation, embryology, value of MR myelography in the diagnosis and role of surgery are discussed. The differences between terminal myelocystoceles and thoracic myelocystoceles are also highlighted. ( info) |
8/12. Percutaneous fetoscopic patch coverage of spina bifida aperta in the human--early clinical experience and potential. OBJECTIVE: The current operative approach for fetal repair of spina bifida aperta requires maternal laparotomy and hysterotomy. Following technical feasibility studies in sheep, we performed percutaneous fetoscopic patch coverage of this lesion in 3 human fetuses between 23 4 and 25 3 weeks of gestation. methods AND RESULTS: Whereas the patch detached in the first case 3 weeks after the procedure, it covered the exposed neural tissue in the 2 other fetuses beyond their delivery. Two of the three children survived, but 1 unexpectedly died from a ventilation problem in its 3rd week of life. In 1 of the 2 survivors, ventriculoperitoneal shunt insertion was delayed. CONCLUSIONS: Percutaneous fetoscopic patch coverage of spina bifida aperta is feasible in human fetuses and offers a substantial reduction of maternal trauma compared to open fetal repair. Further clinical experience is now required before the efficacy of the new approach to protect the exposed neural tissue from mechanical and chemical damage and to improve hindbrain herniation can be evaluated. ( info) |
9/12. love and loss. Conversations with parents of babies with spinal bifida managed without surgery, 1971-1981. Looking back over the span of years surveyed, it appears that a sad experience, even many years ago, commonly leaves residual pain. This can be modified by sympathetic support enabling parents and baby to interact, although such interaction is not without painful as well as pleasurable effects. Coming to terms with loss may take longer than was previously thought. This study highlights the need for bereavement care, which aims to leave families with positive rather than negative feelings. Perhaps, for mental health, the eventual feelings about past happenings are more important than the happenings themselves, although we have also shown that a greater insight into the needs of parents at the time modified their long-term feelings for the good, some even indicating that they had been enriched by the whole experience. The interviews suggested that parents who were involved with their baby's care were more able to accept the reality of their loss and to adjust to the experience of pain and grief than was possible for those who had been kept apart from their children. This supports conclusions by other grief counsellors (e.g. Warden 1983). ( info) |
| neural tube defects (NTD) are among the most prevalent congenital malformations in man. Based on the molecular defect of Splotch, an established mouse model for NTD, and on the clinical association between NTD and waardenburg syndrome (WS), mutations in the PAX3 gene can be expected to act as factors predisposing to human NTD. To test this hypothesis, 39 patients with familial NTD were screened by SSC analysis for mutations in exons 2 to 6 of the human PAX3 gene. One patient with lumbosacral meningomyelocele was identified with a 5 bp deletion in exon 5 approximately 55 bp upstream of the conserved homeodomain. The deletion causes a frameshift with a stop codon almost immediately after the mutated site. Clinical investigation of the index patient indicated mild signs of WS type I. Varying signs of this syndrome were found to cosegregate with the mutation in the family. Our results support the hypothesis that mutations in the gene for PAX3 can predispose to NTD, but also show that, in general, mutations within or near the conserved domains of the PAX3 protein are only very infrequently involved in familial NTD. ( info) |