Cases reported "Spinal Dysraphism"

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1/69. An unusual case of dorsally situated bony spur in a lumbar split cord malformation.

    A case of lumbar split cord malformation (SCM) with a bony spur situated dorsally is presented. This was associated with a hypertrophied posterior arch. The ventral dura was totally intact, and there was no fibrous septum connecting the bony arch to the dura. To our knowledge, such a case has not been reported earlier. In view of this unique finding, we propose a slight modification in Pang's unified theory of embryogenesis in the development of SCM.
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2/69. Bladder cancer arising in a spina bifida patient.

    We report the case of a 52-year-old patient with spina bifida, neurologic bladder, and a history of recurrent urinary tract infections (UTIs) in whom a bladder cancer was incidentally discovered. Cytology, cystoscopy, and cystography showed nonspecific, extensive inflammatory lesions. Cystography demonstrated a complex of diverticulae and cellules. Pathologic examination of a diverticulectomy specimen revealed a grade III pT3b transitional and squamous cell carcinoma. Because of the similar disease causation (recurrent UTIs, stones, and indwelling catheterization), we suggest extension of the guidelines proposed for patients with spinal cord injuries (ie, annual serial bladder biopsies) to patients with nontraumatic neurogenic bladder.
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3/69. Intradural spinal lipoma of the conus medullaris without spinal dysraphism.

    A 42-year-old man suffering from progressive left radicular sensory motor loss (L4 level) underwent neurosurgical repair. neuroimaging (RMI) had led to the diagnosis of schwannoma of the filum terminale with lipomatous component. Histological examination visualized a true mature lipoma associated with numerous bundles of more or less dystrophic nerve fibers. This histological benign tumor raised the problem of the genesis of intradural lipomas of spinal cord.
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4/69. Terminal Myelocystocele:an unusual presentation.

    Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin-covered lumbosacral spina bifida. An arachnoid-lined meningocele, continuous with the spinal subarachnoid space, is traversed by the hydromyelic cord. Clinically, this presents with a skin-covered lumbosacral mass, but often no neurological deficit is present. We present a case of terminal myelocystocele in a child born without deficit and without an obvious back mass. diagnosis was delayed until sphincter disturbance and lower limb inequalities developed. We discuss the presentation, imaging and operative findings in this case.
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5/69. Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report.

    STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. methods: An adult patient presented with symptoms of neurogenic claudication in the left leg. magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.
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6/69. Infantile arachnoid cyst compressing the sacral nerve root associated with spina bifida and lipoma--case report.

    A 2-year-old boy presented with a rare sacral arachnoid cyst manifesting as gait disturbance. neuroimaging revealed an intradural cyst in the sacral nerve root sheath associated with spina bifida occulta and a lipoma at the same level. At surgery, the conus medullaris was situated at the L-1 level and not tethered. The highly pressurized arachnoid cyst had exposed the dural sheath of the left S-2 nerve root and compressed the adjacent nerves. An S-2 nerve root pierced through the cyst. There was no communication between the cyst and spinal arachnoid space. We thought the one-way valve mechanism had contributed to the cyst enlargement and the nerve compression. Radical resection of the cyst was not attempted. A cyst-subarachnoid shunt was placed to release the intracystic pressure. Postoperatively, his gait disturbance improved and no deterioration occurred during the 4-year follow up. Both tethered cord syndrome and sacral arachnoid cyst in the nerve root sheath should be considered in pediatric progressive gait disturbance. Cyst-subarachnoid shunt is an alternative method to cyst resection or fenestration to achieve neurological improvement.
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keywords = cord syndrome, cord
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7/69. Sonographic diagnosis of diastematomyelia in utero: a case report and literature review.

    Fetal diastematomyelia is a rare form of spinal dysraphism that is characterized by a complete or incomplete division of the spinal cord by an osseous or fibrocartilaginous septum. A case of diastematomyelia, which was detected on the routine third trimester detailed ultrasound scan, is presented. The diagnosis was based on the detection of an echogenic focus in the posterior aspect of the spine in association with widening of the interpedicular vertebral space. The case illustrates that diastematomyelia can occur in the absence of overt spina bifida and that prenatal detection will allow timely postnatal investigation and treatment. Prenatal literature is further reviewed to assess the clinical significance of this finding.
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8/69. Congenital midline cleft of the posterior arch of atlas: a rare cause of symptomatic cervical canal stenosis.

    Developmental symptomatic C1 canal stenosis is very rare. We describe the computed tomography (CT) and magnetic resonance imaging (MRI) appearances in a 8-year-old child who presented with progressive upper and lower limb neurological symptoms and in whom imaging revealed the medial posterior hemiarches of a bifid C1 to be inturned and compressing the cervical cord. This particular configuration of the posterior arch of atlas is frequently associated with other craniocervical bony anomalies and presents with neurological symptoms early in life. Early CT or MRI examination of patients with symptomatic posterior arch of C1 defects is necessary, in order to detect such an appearance, since surgical treatment may prevent neurological deterioration.
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9/69. Lumbar canal stenosis: a cause of late neurological deterioration in patients with spina bifida.

    BACKGROUND: patients diagnosed with spina bifida may show late deterioration. This worsening in their clinical symptoms has been attributed to a multiplicity of causes such as secondary tethering of the spinal cord, Chiari II anomaly, hydromyelia, diastematomyelia, arachnoid cysts, and dermoid tumors. methods: We searched the clinical records of patients diagnosed with spina bifida who were treated at our hospital for a period of 25 years for the purpose of ascertaining the number and etiology of cases of late neurological deterioration. RESULTS: Six of 144 patients with open spina bifida presented with late neurological deterioration. In one of these cases and in another patient with occult spina bifida the most relevant factor noted during surgery was the presence of marked lumbar canal stenosis. CONCLUSION: We suggest that certain cases of late clinical worsening in spina bifida patients are because of lumbar canal stenosis and that this condition should be added to the list of causes that may produce delayed neurological deterioration in patients with spinal dysraphism.
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10/69. Unpredicted spontaneous extrusion of a renal calculus in an adult male with spina bifida and paraplegia: report of a misdiagnosis. Measures to be taken to reduce urological errors in spinal cord injury patients.

    BACKGROUND: A delay in diagnosis or a misdiagnosis may occur in patients with spinal cord injury (SCI) or spinal bifida as typical symptoms of a clinical condition may be absent because of their neurological impairment. CASE PRESENTATION: A 29-year old male, who was born with spina bifida and hydrocephalus, became unwell and developed a swelling and large red mark in his left loin eighteen months ago. pyonephrosis or perinephric abscess was suspected. X-ray of the abdomen showed left-sided staghorn calculus. Since ultrasound scan showed no features of pyonephrosis or perinephric abscess, he was prescribed a prolonged course of antibiotics for infection presumed to arise from the site of metal implant in spine. He developed a discharging sinus, following which the loin swelling and red mark subsided. About three months ago, he again developed a red mark and minimal swelling in the left loin. Ultrasound scan detected no abnormality in the renal or perinephric region. Therefore, the red mark and swelling were attributed to pressure from the backrest of his chair. Five weeks later, the swelling in the left loin burst open and a large stone was extruded spontaneously. An X-ray of the abdomen showed that he had extruded the central portion of the staghorn calculus from left kidney. With hindsight, the extruded renal calculus could be seen lying in the subcutaneous tissue of left loin lateral to the 10th rib in the X-ray of abdomen, which was taken when he presented with red mark and minimal swelling. CONCLUSION: This case illustrates how mistakes in diagnosis could occur in spinal cord injury patients, and highlights the need for corrective measures to reduce urological errors in these patients. Voluntary reporting of urological errors is recommended to facilitate learning from our mistakes. In the patients who have marked spinal curvature, ultrasonography of kidneys and perinephric region may not be entirely reliable. As clinical symptoms and signs may be non-specific in SCI patients, they require prompt, detailed and occasionally, repeated investigations. A joint team approach by health professionals belonging to various medical disciplines, which is strengthened by frequent, informal and honest discussions of a patient's clinical condition, is likely to reduce urological errors in SCI patients.
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