Cases reported "Neural Tube Defects"

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1/40. A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst.

    We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.
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2/40. cerebrospinal fluid pseudocyst: a postoperative complication of augmentation ileocystoplasty in myelodysplastic children.

    Peritoneal cerebrospinal fluid pseudocyst (CSFoma) formation is a rare postoperative complication of augmentation cystoplasty in children, with only three other cases being reported in the world literature. We describe two patients with CSFoma formation following augmentation ileocystoplasty. The management of these cases and a brief review of the pathogenesis and management of this condition are presented.
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3/40. F wave monitoring during surgery for adult tethered cord syndrome--a case report.

    We would like to report our first attempt in intraoperative study of F wave response electromyography (EMG) to monitor the spinal motor function during a spinal surgery for excision of a giant lumbosacral lipoma.
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4/40. Intramedullary neurenteric cysts of the spine. Case report and review of the literature.

    This case of a 68-year-old woman with a low-thoracic intramedullary neurenteric cyst is notable for clinical presentation, cyst location, intraoperative findings, and imaging characteristics. The patient's postoperative course was complicated by neurological deterioration and a neuropathic pain syndrome. Potential causes of these complications are discussed, as are possible ways to reduce the risk of their occurrence.
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ranking = 2
keywords = operative
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5/40. Thoracic intradural extramedullary lipomas. Report of three cases and review of the literature.

    BACKGROUND: Indication for surgery and the surgical strategy for treatment of spinal intradural lipomas are still discussed controversially. Among spinal lipomas the cervicothoracic region is rarely affected. We report on our experience with spinal decompression for the treatment of three intradural thoracic lipomas. FINDINGS: Three adult patients with extramedullary intradural lipomas of the thoracic spinal cord demonstrated progressive neurological symptoms and signs without evidence of growth of the lipoma. All patients underwent surgery with decompression of the affected spinal levels. No attempt was made to reduce the size of the lipomas. Postoperatively, each patient demonstrated significant clinical improvement. Interpretation. Attempts to remove lipomas are associated with significant risks of surgical morbidity. Lipomas are hamartomas which change their size according to alterations of body fat. Therefore, decompression of the affected spinal levels is sufficient to achieve significant neurological improvement.
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6/40. A case of diastematomyelia associated with myeloschisis in a hemicord.

    We report a case of myeloschisis on the right hemicord in a patient with diastematomyelia. The patient was a female neonate with myeloschisis visible at birth in the upper lumbar region. Radiological examination, including three-dimensional CT and MRI, clearly revealed a bony septum as well as the myeloschisis on the right hemicord, which enabled us to make a precise preoperative diagnosis of this complex anomaly. Closure of the myeloschisis and removal of the septum were successfully accomplished in one stage to prevent subsequent infection and neurological deterioration. The presence of combined diastematomyelia and myeloschisis is consistent with the hypothesis of an ontogenic basis of development and emphasizes the importance of early imaging for diagnosis in this complex anomaly.
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7/40. Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review.

    The authors report a case of a 3-month-old male child with paraplegia in whom magnetic resonance imaging (MRI) revealed a nonenhancing intramedullary cystic lesion extending from the level of D1 to D7 without any other associated anomaly. Intraoperatively, these findings were confirmed and the spinal cord was found to have splayed circumferentially into a papery thin rim. The patient underwent marsupialization of the cyst with subtotal excision of the cyst wall. Histopathological examination revealed ciliated pseudostratified columnar epithelium consistent with the diagnosis of a neurenteric cyst. Intramedullary neurenteric cysts are rare developmental malformations, and out of the 13 previously reported cases, only 3 were evaluated by MRI. This is the first case report in the literature of an intramedullary neurenteric cyst presenting as infantile paraplegia. In the present report, the embryology, etiopathogenesis, radiological imaging and management of this rare clinical entity are discussed and a detailed literature review is presented.
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8/40. Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult. Case report.

    Diastematomyelia is a rare entity in which some portion of the spinal cord is split into two by a midline septum. Most cases occur in childhood, but some develop in adulthood. A variety of concurrent spinal anomalies may be found in patients with diastematomyelia. The authors describe a 38-year-old right-handed woman who presented with a 7-month history of lower-extremity pain and weakness on the right side. She denied recent trauma or illness. Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. neuroimaging revealed diastematomyelia extending from T-1 to T-3, an expanded right hemicord from T-2 to T-4, and a C6-7 syrinx. The patient underwent T1-3 total laminectomies, resection of the septum, untethering of the cord, and excision of the hemicord lesion. The hemicord mass was determined to be an intramedullary epidermoid cyst; on microscopic evaluation the diastematomyelia cleft was shown to contain fibroadipose connective tissue with nerve twigs and ganglion cells. Postoperatively, the right lower-extremity pain, weakness, and sensory deficits improved. Diastematomyelia can present after a long, relatively asymptomatic period and should be kept in the differential diagnosis for radiculopathy, myelopathy, tethered cord syndrome, or cauda equina syndrome. Numerous spinal lesions can be found in conjunction with diastematomyelia. To the authors' knowledge, this is the first case in which a thoracic epidermoid cyst and cervical syrinx occurred concurrently with an upper thoracic diastematomyelia. Thorough neuraxis radiographic evaluation and surgical treatment are usually indicated.
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ranking = 1
keywords = operative
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9/40. Intramedullary neurenteric cyst with a false mural nodule: case report.

    OBJECTIVE AND IMPORTANCE: Spinal neurenteric cysts are rare congenital lesions that may occur either alone or in the context of a complex malformative disorder including typical vertebral and cutaneous abnormalities. The interest of the case of a spinal neurenteric cyst described here lies in its rare intramedullary location and in the false mural nodule image on the preoperative magnetic resonance imaging scan. A further distinctive feature is the association with a cleft spinal cord. CLINICAL PRESENTATION: A 28-year-old woman presented with a 2-year history of progressive paraparesis and urinary retention. A magnetic resonance imaging study disclosed a T8-T9 intramedullary cystic lesion with a mural nodulelike formation on the posterior face. INTERVENTION: A posterior midline myelotomy exposed a cystic lesion that had translucent walls and contained a milky fluid. No mural nodules were found. Once the cyst had been emptied, a collateral finding was a cleft that was clearly observed in the anterior spinal cord. The histological diagnosis was a neurenteric cyst. Retrospectively, the nodular lesion found on the preoperative scan was attributed to mucinous clots deposited at the bottom of the cyst. CONCLUSION: This case report demonstrates that neurenteric cysts can vary widely in radiological appearance, depending on the contents of the cyst. These differences become especially important if the associated stigmata are lacking and the preoperative diagnosis rests on magnetic resonance imaging scan appearance alone.
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ranking = 3
keywords = operative
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10/40. A recurrent intradural cervical neurenteric cyst operated on using an anterior approach: a case report.

    The neurenteric cyst is an uncommon congenital lesion. In most reported cases, it has been operated on via a posterior approach using a laminectomy, despite the fact that the cyst is usually located ventral to the spinal cord. Reports have shown that early postoperative results have been good with the posterior approach, but very few studies of the long-term postoperative recurrence of neurenteric cysts have been conducted. Here, we report on a case of recurrent neurenteric cyst that was operated on using an anterior approach.A 42-year-old woman presented with a cervical neurenteric cyst that had recurred eight years after its partial removal via a posterior approach. The patient complained of pain on the lateral side of her upper arms, and an magnetic resonance imaging showed that the recurrent cyst was located ventral to the spinal cord and compressed the cord dorsally at the C4-6 level.The patient was operated on via an anterior approach using a vertebrotomy at the lower half of C5 and the upper half of C6. The cyst was attached to the spinal cord firmly and was subtotally removed, with the thickest portion adhering to the cord not being removed. The caudal end of the cyst was observed with the assistance of a rigid endoscope.A neurenteric cyst may recur after partial removal, and the patient's condition may deteriorate during postoperative follow-up. The anterior surgical approach provides good visualization and facilitates safe removal of the lesion.
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ranking = 3
keywords = operative
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