Cases reported "Spina Bifida Occulta"

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1/31. 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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2/31. Fetal neurenteric cyst causing hydrops: case report and review of the literature.

    Routine prenatal ultrasound revealed a unilocular cystic mass associated with upper thoracic hemivertebrae that grew to 6 cm at 28 weeks and was associated with hydrops. A thoraco-amniotic shunt decompressed the cyst and resolved the hydrops, but the shunt occluded 17 days later. Preterm labour led to vaginal delivery at 31 2/7 weeks. Postnatally, the cyst was decompressed by thoracentesis due to respiratory distress. It was resected on day four of life. Severe tracheobronchomalacia was present post-operatively, presumably due to prenatal mass effect of the cyst. At one year of age, the child has recovered completely without adverse respiratory or neurological sequelae.
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3/31. 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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4/31. Plexiform neurofibroma of the cauda equina. Case report.

    Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.
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5/31. Neurenteric cyst of the craniocervical junction--case report.

    A 60-year-old female presented with occipital headache and limitation of neck movement. Neurological examination showed weakness of the right sternocleidomastoid muscle. magnetic resonance imaging revealed a cystic lesion at the craniocervical junction and posterior compression of the brain stem. The lesion was totally removed through the transcondylar approach. The histological diagnosis was neurenteric cyst. The transcondylar approach provides a direct operative view of the clivus and anterior craniovertebral junction.
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6/31. Lumbar meningeal hamartoma and epidermoid cyst associated with spinal dysraphism in an elderly patient.

    Meningeal hamartoma associated with spinal dysraphism in an elderly patient is an extremely rare clinical presentation. A 62-year-old woman who progressively developed gait disturbance and urinary retention was admitted to our hospital. magnetic resonance imaging (MRI) revealed a large cystic mass associated with a dermal sinus at the L4-L5 level. MRI was useful in the diagnosis and determination of this lesion. However, preoperative differential diagnosis of the tumor was not possible based on neuroimaging evidence. The diagnosis of meningeal hamartoma was based on the histological and immunohistological findings of the specimen obtained from the tumor. Meningeal hamartoma associated with spinal dysraphism should be kept in mind, even in adult cases. In this article, we also discuss the radiological and pathological appearance of this rare clinical entity.
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7/31. teratoma in human tail lipoma.

    We report a case of a rare congenital teratoma that developed in a lipoma attached to a remnant human tail. A male newborn baby presented with a large, 3-cm mass with an open margin, which pedunculated from a tail attached to the midline skin of the coccygeal area. Magnetic resonance images demonstrated multiple sacral spinal bifida without cord tethering, and also showed neural roots and a lipoma and teratoma with peripheral homogeneous high density and internal low density on T(2)-weighted images. Intraoperatively, we found and dissected two nerve roots from the filum terminale which extended into the mass. Pathologic examination of the mass revealed abnormal differentiation of respiratory epithelium and squamous cell metaplasia along the open margin of the mass, and mainly lipoma in the rest of the mass. We suggest that this case could support the hypothesis of transient teratomatous cells in the pathogenesis of the spina bifida with lumbosacral lipoma.
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8/31. Retrograde intraventricular hemorrhage caused by a traumatic sacral pseudomeningocele in the presence of spina bifida occulta. Case report.

    The authors present the case of a large, posttraumatic sacral pseudomeningocele in the presence of spina bifida occulta. A pseudomeningocele in the sacral region is associated with trauma and with marfan syndrome, but only one occurrence has been reported in association with spinal dysraphism. Trauma resulted in bleeding into the pseudomeningocele and retrograde passage of blood and fat into the ventricles. An oculomotor nerve palsy subsequently developed in the patient. The authors suspected a subarachnoid hemorrhage caused by a posterior communicating artery aneurysm, although this hypothesis was refuted on further investigation. The pseudomeningocele was drained by direct exposure of the neck and opening of the sac. Postoperatively, communicating hydrocephalus developed and the patient underwent ventriculoperitoneal shunt placement, resulting in resolution of the cranial nerve palsies. This first report of intradural bleeding from direct trauma to a pseudomeningocele illustrates the rare phenomenon of retrograde passage of blood from the sacral region to the brain. It also illustrates a possible but unlikely differential diagnosis of intraventricular blood and fat.
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9/31. Holocord intramedullary abscess due to dermal sinus in a 2-month-old child successfully treated with limited myelotomy and aspiration. Case report.

    This 2-month-old child presented with paraplegia. The authors observed a dermal sinus with purulent discharge in the lumbosacral area. Magnetic resonance (MR) imaging of the spine revealed an intramedullary enhancing cavity spanning C-1 to the conus medullaris. Intraoperatively the dermal sinus was seen to infiltrate the lower end of the conus medullaris, and it also communicated directly with the central canal. The L2-5 laminae were removed, and a myelotomy was undertaken on the conus medullaris. A No. 8 French pediatric feeding tube was passed into the abscess cavity and advanced rostrally to the level of C-1. Aspiration was applied via the feeding tube to drain the intramedullary abscess of the spinal cord (IASC). Postoperatively, a 6-week course of intravenous cloxacillin was instituted. Follow-up MR imaging revealed complete resolution of abscess. When the patient was 26 months of age, examination showed complete neurological recovery. The authors describe what, to their knowledge, is the first case of a holocord IASC treated successfully by the aforementioned technique, and review of the related literature.
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10/31. Cerebellar dermoid cyst with occipital dermal sinus. Report of two pediatric cases.

    Intracranial dermoid cyst is a rare entity accounting for 0.1-0.7% of all intracranial tumors. The most common location is in the posterior fossa, at or near the midline. We present 2 pediatric cases with dermal sinus. The first case presented with clinical signs of increased intracranial pressure and cerebellar symptoms. CT scan showed a large and regular midline posterior fossa cyst without contrast enhancement. The second case was revealed by recurrent meningitis. CT scan showed a midline vermis low-density mass with capsular contrast enhancement. Dermal sinus was found in 2 cases. Complete surgical removal was performed followed, in a second operation, by ventriculoperitoneal shunt in 2 cases. There was no postoperative complication in our patients. The aim of this study is to discuss the clinical aspects of dermoid cyst, especially in cases with dermal sinus, and to review the therapeutic strategies in case of associated hydrocephalus.
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