1/28. Suboccipital meningocele presenting as a huge retropharyngeal mass in a patient with neurofibromatosis Type 1. Case report.The authors report an extremely rare case of neurofibromatosis Type 1 (NF1) with a suboccipital meningocele presenting as a huge retropharyngeal mass. A 73-year-old woman with typical cutaneous manifestations of NF1 presented with nasal obstruction and dysphagia due to a retropharyngeal mass. magnetic resonance imaging revealed a huge mass lesion extending from the right occipital bone defect to the retropharynx through the right paravertebral region. Computerized tomography scanning after intrathecal administration of contrast material confirmed that the mass was a meningocele protruding through a right occipital bone defect. The authors attempted to ligate this meningocele, most of which was excised via a suboccipital approach, but a second transcervical operation was required. Finally, the meningocele resolved and the patient was discharged without symptoms.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
2/28. 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 = 0.875keywords = meningocele (Clic here for more details about this article) |
3/28. Intrathoracic meningoceles and neurofibromatosis.We describe here four additional cases of intrathoracic meningoceles associated with neurofibromatosis, bringing the total number of reported cases of thoracic meningoceles to 88. Seventy-five (85%) have been associated with neurofibromatosis. Possibly, both dural and regional vertebral dysplasia are intrinsic to neurofibromatosis and contribute to the development of the meningocele.- - - - - - - - - - ranking = 0.875keywords = meningocele (Clic here for more details about this article) |
4/28. neurofibroma of the bladder wall in von Recklinghausen's disease.Neurofibromatosis, or von Recklinghausen's disease, is an autosomal dominant disease with multiple neurofibroma and cafe-au-lait spots. However, neurofibroma in the bladder wall is a rare condition in von Recklinghausen's disease. A 31-year-old man with neurogenic voiding dysfunction due to sacral meningocele and acute urinary retention with neurofibroma of the bladder wall is presented with detailed radiologic evaluation. patients with von Recklinghausen's disease should be carefully evaluated if urological symptoms exist.- - - - - - - - - - ranking = 0.125keywords = meningocele (Clic here for more details about this article) |
5/28. 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 = 1.125keywords = meningocele (Clic here for more details about this article) |
6/28. A neurofibromatosis type 1 patient with severe kyphoscoliosis and intrathoracic meningocele.The patient presented with neurofibromatosis and a dystrophic kyphoscoliosis around the cervico-thoracic junction. When the patient was 59 years old, he started to suffer from dyspnea caused by an intrathoracic meningocele in the upper left thoracic cavity. A wide laminectomy from T2 to T5 was performed and the meningocele was resected. Although the dyspnoea disappeared postoperatively, the patient started to neurologically deteriorate. laminectomy alone caused instability around the apex of the kyphosoliosis and spinal cord compression. Halo cast was applied and brought remarkable recovery of neurologic deficits. This result encouraged us to perform posterior fusion in situ from C3 to L2 with bone graft from the iliac crests and the Luque technique in conjunction with the Isola system. This resulted in the patient being able to walk again. The removal of the posterior element predisposes the patient to unstable postlaminectomy kyphosis and removes valuable bone stock required for posterior spinal fusion. For this reason, spinal fusion should have been conducted during surgery for the patient's meningocele.- - - - - - - - - - ranking = 0.875keywords = meningocele (Clic here for more details about this article) |
7/28. Giant intrathoracic meningoceles associated with cutaneous neurofibromatosis type I: case report.BACKGROUND: Intrathoracic meningocele is a rare pathology, almost always associated with neurofibromatosis type I and with a few cases related in the literature. In the majority of cases cysts are small or asymptomatic, and the surgery is indicated when big or symptomatic cysts are present. We report a case of giant intrathoracic cysts surgically extirpated through out thoracotomy. CASE: A 59-year-old male with familiar Von Recklinghausen's disease which developed thoracic radicular pain after a fall. On examination he presented some difficulty in walking fast and dyspneia on small efforts. The chest plain x-ray showed the presence of 3 huge left side intrathoracic cysts (10 to 15cm). The patient was submitted to a surgical treatment and complete extirpation of the cysts was performed through a left side thoracotomy. During the surgery a fourth smaller cyst was detected and also extirpated. Evolution was uneventful and the patient remains well in these last 12 years. This finding of intrathoracic cysts related to neurofibromatosis type I is rare and is probably unique in the literature the presence of 4 huge cysts in one side of the thorax.- - - - - - - - - - ranking = 0.625keywords = meningocele (Clic here for more details about this article) |
8/28. Intrathoracic meningocele.We present the case of a 63-year-old woman and review the literature. The incidence of intrathoracic meningocele is rare, even in known cases of Von Recklinghausen's neurofibromatosis. Cases of isolated intrathoracic meningocele or multiple meningoceles without neurofibromatosis are even more uncommon. The majority of intrathoracic meningoceles have been discovered by chance or after nonspecific symptoms. Although diagnosis by CT scan and MRI has been documented, we are convinced that myelography is still of great value. There is no agreement about the etiology of intrathoracic meningocele. Treatment is also discussed.- - - - - - - - - - ranking = 1.125keywords = meningocele (Clic here for more details about this article) |
9/28. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1.Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles.- - - - - - - - - - ranking = 0.875keywords = meningocele (Clic here for more details about this article) |
10/28. Spinal neurofibromatosis.Neurofibromatosis is a congenital abnormality due to maldevelopment of the neurectoderm and mesoderm. Spinal abnormalities are a frequent occurrence and the roentgenological changes observed in 21 patients are presented. These include scoliosis, posterior scalloping of vertebral bodies, and the roentgenological features associated with intraspinal tumors and thoracic meningoceles.- - - - - - - - - - ranking = 0.125keywords = meningocele (Clic here for more details about this article) |
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