Cases reported "Spina Bifida Occulta"

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1/125. The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.

    OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. methods: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.
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2/125. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.

    A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.
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3/125. Intramedullary spinal teratoma and diastematomyelia. Case report and review of the literature.

    The authors present a patient with diastematomyelia and a spinal intramedullary teratoma, remote from the split cord malformation. A split cord malformation at the L2-L3 level was initially discovered during investigations for thoracic congenital scoliosis, and this was treated surgically. The teratoma, which was at the level of the scoliosis, went undiagnosed until neurological deterioration occurred many years later. Surgical removal of the teratoma resulted in return to normal function. The potential for coexisting congenital anomalies at separate levels of the spinal cord must be considered in radiological investigations of a developmental spinal lesion.
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4/125. Dermal sinus and intramedullary spinal cord abscess. Report of two cases and review of the literature.

    Intramedullary abscesses of the spinal cord are uncommon. Most of them occur in association with heart, pulmonary or urogenital infections. We report two cases of intramedullary spinal cord abscesses secondary to congenital dermal sinus. Only 14 cases of such an association have previously been reported. In our cases, dermal sinus was associated with an epidermoid tumour. The clinical presentation, pathogenesis, magnetic resonance imaging findings, surgical management and outcome are discussed.
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5/125. 3D spiral CT imaging of bone anomalies in a case of diastematomyelia.

    The case of a 48-year-old woman, suffering from a diastematomyelia, is presented. This case and the diagnostic findings are used to demonstrate the demands on imaging methods with respect to a new classification of split cord malformations (SCMs) recently published. Although MRI is the method of choice for imaging of the spinal cord generally, only X-ray methods and especially conventional computer tomography provide the information necessary for correct classification of SCMs. Additional 3D-reconstructions from suitable CT-data are helpful in visualizing complex anomalies of bony structures found in most cases of SCM.
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6/125. Anomaly of the axis causing cervical myelopathy. Case report.

    Although the craniovertebral junction is one of the most common sites at which anomalies develop, spina bifida occulta of the axis (C-2) associated with cervical myelopathy is extremely rare. The authors present the case of a 46-year-old man who developed progressive tetraparesis caused by a cervical canal stenosis at the level of the axis. The spinal cord was compressed by an invaginated bifid lamina of the axis. The patient made a remarkable recovery after undergoing decompressive laminectomy of C-3 and removal of the bifid posterior arch of the axis.
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7/125. Cervical cord tethering mimicking focal muscular atrophy.

    spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. skin lesion at the nuchal area should be carefully looked for.
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8/125. Diastematomyelia in two sisters.

    Diastematomyelia is a rare spinal cord anomaly that usually occurs in a non-syndromal, sporadic manner; however, few familial cases have been reported. We report on diastematomyelia in 2 sisters with variable expressivity. The spinal column is divided by osseous or fibrous tissue. This may be responsible for the variable expressivity. Most cases previously reported were females. This suggests X-linked dominant inheritance with lethality in hemizygous males or female sex limitation of a multifactorial trait.
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9/125. Thoracic neurenteric cyst in an adult: case report.

    OBJECTIVE AND IMPORTANCE: Neurenteric cysts are very rare (particularly in the adult age group) congenital intraspinal cysts of endodermal origin. The patient described was a 48-year-old man who was diagnosed as having an isolated thoracic neurenteric cyst at the T5-T6 level, without a mediastinal enterogenous cyst. Radiological studies demonstrated multiple anterior vertebral column abnormalities. CLINICAL PRESENTATION: The patient presented with a long history of mild midback pain. During the 2 years preceding admission, his midback pain worsened and he experienced right intercostal pain in the midthoracic area. Several months before admission, he noticed right lower extremity weakness, which led him to undergo neurological evaluation and radiological studies. INTERVENTION: Thoracic spine x-rays revealed marked thoracic scoliosis and multiple vertebral abnormalities. magnetic resonance imaging and myelography revealed an intradural extramedullary cystic mass at the T5-T6 level, severely compressing the spinal cord from the ventral side. Thoracic laminectomy was performed and the cystic lesion was completely removed, with disconnection of the ventral tract. CONCLUSION: This is an unusual presentation, at this age, of a congenital intraspinal cyst in the thoracic area. Such cysts must investigated for early diagnosis and treatment. A neurenteric cyst should be suspected particularly if the cyst is associated with anterior vertebral column abnormalities.
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10/125. Primary tethered cord syndrome: diagnosis and treatment of an insidious defect.

    Failure to recognize the signs and symptoms of tethered cord syndrome in patients with spina bifida occulta can result in tragic consequences. Of patients with tethered spinal cord, 35% have bowel and/or bladder dysfunction. scoliosis, foot or leg length discrepancies, pes cavus, and varus or valgus deformities also can occur. Early assessment and intervention by the neuroscience nurse who is familiar with these clinical signs can help prevent significant deformities and irreversible neurological deficits.
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