1/99. A child with neurofibromatosis-1 and a lumbar epidural arteriovenous malformation.A 10-year-old child with neurofibromatosis-1 was evaluated for progressive lumbar scoliosis, back pain, and foot numbness. magnetic resonance imaging showed several lumbar intraspinal and extraspinal masses consistent with neurofibromas. The mass at L3-L5 compressed the thecal sac and was thought to be the source of the symptoms. On operative exploration, a lumbar epidural arteriovenous malformation was found, which was removed in its entirety. The child's back pain and foot numbness resolved. Epidural arteriovenous malformations in patients with neurofibromatosis-1 are rare and have been reported only in the cervical spine. Our finding of a lumbar epidural arteriovenous malformation in a child with neurofibromatosis-1 demonstrates that vascular anomalies can be present throughout the spine of patients with neurofibromatosis-1 and should be considered in the differential diagnosis of any neurofibromatosis-1-related epidural mass.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/99. Malignant triton tumor in the thoracic spine.We present a 15-year-old patient diagnosed with peripheral neurofibromatosis (NF-1), who was admitted with paraparesis caused by a large intrathoracic tumor with an intracanalicular component that affected the spinal cord. After surgery his condition improved, but a year later he suffered a relapse and died. Histologically the tumor was diagnosed as malignant with neurogenic and myogenic differentiation ("malignant triton tumor"). Malignant triton tumors (MTT) are infrequent; those found in the head and neck and the upper or lower extremities have a better prognosis than those in the retroperitoneum, buttock, or trunk. It is not clear whether this variation is due to a difference in tumor grade, stage, or resectability, or whether it is a consequence of therapy.- - - - - - - - - - ranking = 0.53824261351553keywords = spinal, canal (Clic here for more details about this article) |
3/99. An unusual pterygopalatine meningocele associated with neurofibromatosis type 1. Case report.The authors describe an unusual meningocele of the lateral wall of the cavernous sinus and the anterior skull base in a young patient with typical stigmata of neurofibromatosis Type 1 (NF1). This lesion was discovered during evaluation for recurrent meningitis. It represented an anterior continuation of Meckel's cave into a large cerebrospinal fluid space within the lateral wall of the cavernous sinus, extending extracranially through an enlarged superior orbital fissure into the pterygopalatine fossa adjacent to the nasal cavity. It was successfully obliterated, via an intradural middle fossa approach, with fat packing and fenestration into the subarachnoid space. This meningocele most likely represents a variant of cranial nerve dural ectasia occasionally seen in individuals with NF1. It has as its basis the same mesodermal defect responsible for the more common sphenoid wing dysplasia and spinal dural ectasias identified with this condition. Involvement of the trigeminal nerve with expansion of the lateral wall of cavernous sinus has not been reported previously. The authors surmise, however, that it may be present in some cases of orbital meningocele associated with sphenoid wing dysplasia.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
4/99. forearm pseudarthrosis--neurofibromatosis: case report.A 3 1/2-year-old white girl with neurofibromatosis sustained left radius and ulna fractures. The radius was sclerotic with no medullary canal at the fracture site, and the ulna was hypoplastic distal to the fracture. The fractures failed to unite when immobilized in a long arm plaster cast for 5 months and pseudarthrosis developed. Three subsequent operative attempts to obtain union of the pseudarthrosis by means of internal fixation and bone grafting over the next 30 months were also unsuccessful, and the pseudarthrosis persisted. The forearm was supported in a custom molded leather brace until the child was 13 1/2 years old and had reached skeletal maturity. Osseous union was then operatively obtained using dual onlay tibial cortical and cancellous bone grafts. There has been no recurrence of the pseudarthrosis 3 years and 2 months after bone grafting. The author recommends postponing surgical attempts to achieve union of the forearm bone pseudarthrosis associated with neurofibromatosis until the patient reaches skeletal maturity.- - - - - - - - - - ranking = 0.038242613515532keywords = canal (Clic here for more details about this article) |
5/99. Lumbosacral dural ectasia in type 1 neurofibromatosis. Report of two cases.Dural ectasia denotes circumferential expansion or dilatation of the dural sac, and has been frequently reported in association with type 1 neurofibromatosis (NF1). The pathogenesis has not been defined, but its correlation with NF1 infers a congenital malformative hypothesis. The neural elements in the dilated sleeve typically are not enlarged or abnormal, nevertheless the enlarged area contain an increased amount of cerebrospinal fluid. The dura in the area of ectasia is extremely thin and fragile, and erodes the surrounding bony structures destabilising the spine and permitting spectacular spinal deformities. We present two cases with lumbosacral dural ectasia, enlargement of the intervertebral foramina and posterior scalloping of vertebral bodies. Neurological examination showed sciatic nerve irritation. As the etiology of this malformation remains uncertain and dysplastic changes of the spine may be intrinsic or secondary controversies remain about optimum treatment. We conclude that patients affected by NF-1 require an accurate neuroradiological study of the whole spine in order to detect possible dural and spinal anomalies. Surgical treatment is indicated only in patients with progressive neurological deterioration. The thin dural sac predisposes to a high morbidity if surgery is undertaken.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
6/99. Retroperitoneal malignant peripheral nerve sheath tumor associated with scoliosis in neurofibromatosis.neurofibromatosis 1 is an autosomal dominant disorder characterized by distinctive clinical problems including scoliosis and malignant peripheral nerve sheath tumors. We present two cases of retroperitoneal malignant peripheral nerve sheath tumor associated with scoliosis in neurofibromatosis. Presence of spinal deformity resulted in delay of the diagnosis of the sarcoma.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
7/99. Recurrent pyogenic meningitis: an unusual presentation in a case of neurofibromatosis-1.We report a case of recurrent pyogenic meningitis with cerebrospinal fluid rhinorrhoea in a patient with neurofibromatosis-1, which has not been reported previously.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
8/99. The association of neurofibromatosis 1 and spinal deformity with primary hyperparathyroidism and osteomalacia: might melatonin have a role?A 35-year-old woman with neurofibromatosis 1 and thoracic kyphoscoliosis had incomplete paraplegia. She had a history of hyperparathyroidism due to a parathyroid adenoma which had been excised 4 years previously. Plain radiographs of the spine revealed kyphoscoliosis from the third to sixth thoracic vertebrae. kyphosis and scoliosis angles were 86 degrees and 28 degrees, respectively. Radiographs of the skull and hands showed radiological changes suggestive of hyperparathyroidism. Laboratory tests showed low-normal serum calcium, hypophosphatemia, elevated serum alkaline phosphatase, and low serum 25-hydroxyvitamin D. Retrospective review of the patient's laboratory data showed that she had osteomalacia at the time of diagnosis of primary hyperparathyroidism. The patient had been treated by anterior and posterior decompression and fusion with posterior instrumentation through a single posterior approach. The postoperative kyphosis and scoliosis angles were 30 degrees and 12 degrees, respectively. Neurological recovery and spinal fusion had been achieved. osteomalacia responded well to vitamin d therapy. This is the first case of coexisting neurofibromatosis 1, primary hyperparathyroidism due to parathyroid adenoma and osteomalacia to be reported in the literature. The osteomalacia in this patient could be related to primary hyperparathyroidism, and not to neurofibromatosis 1. A drop in melatonin level after parathyroidectomy may have been the cause of spinal curvature progression in this patient.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
9/99. Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
10/99. paraparesis after excision of intrathoracic meningoceles in a patient with neurofibromatosis.Intrathoracic meningocele associated with neurofibromatosis is a rarity. We treated a 16-year-old boy with neurofibromatosis, marked kyphoscoliosis, and two right-sided intrathoracic meningoceles. Because his chief complaints of cough and chest pain were thought to be caused by the meningoceles, resection of these lesions was performed prior to correction of the spinal deformity. On the day after the resection, complete paraplegia developed, followed by recovery to paraparesis. Decompressive lumbar puncture was performed, but intraspinal pressure was normal. Postoperative spinal cord damage and consequent paresis may have resulted from a loss of pressure buffering by the meningocele, which rendered the cord vulnerable to injury. The possibility of a similar unusual complication should be borne in mind when treating patients with intrathoracic meningocele associated with neurofibromatosis.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
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