1/45. Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/45. Surgical treatment of multiple neurofibromas of the ulnar nerve in segmental neurofibromatosis. Case report.The case of an 18-year-old man with numerous neurofibromas along his left ulnar nerve is described. The patient had a painful mass in the medial third of the internal aspect of his left forearm, and two additional symptomatic painful masses were identified during clinical examination: one in the distal portion of the retroepitroclear groove and another near the Guyon tunnel in the wrist. The main symptom was neurogenic pain; however, sensory and motor disturbances were also present. No other stigma of neurofibromatosis (NF) was found, and no cases of NF were known in the patient's family. During surgery many neurofibromas were found; the three painful neurofibromas and some of the other larger lesions were microsurgically excised. The patient's symptoms fit the criteria for segmental NF or NF5. This is a very rare form of NF characterized by lesions located in a particular area of the body.- - - - - - - - - - ranking = 0.71428571428571keywords = nerve (Clic here for more details about this article) |
3/45. A case of familial angiolipomatosis with Lisch nodules.Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.- - - - - - - - - - ranking = 0.0058358652055534keywords = neoplasm (Clic here for more details about this article) |
4/45. Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis. Case report.Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. patients with NF5 present with cafe-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain. neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.- - - - - - - - - - ranking = 20.950443492506keywords = nerve sheath, sheath, nerve (Clic here for more details about this article) |
5/45. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation.Neurofibromatosis is rare in the general population. Its clinical manifestations are systemic and variable. The clinical presentation of cutaneous lesions is even more variable. Some patients have giant tumors in the trunk or limbs (so-called "elephant neurofibromatosis"). The pathological findings are diffuse neurofibromatosis of the nerve trunk associated with overgrowth of subcutaneous tissue and skin. The associated vascular malformations make most surgeons hesitant to address them because bleeding to death is possible if the bleeding is not well controlled. According to the authors' experience in treating this complication of neurofibromatosis, they noted that there are three key points to reducing the amount of hemorrhage to a minimal level: (1) hypotensive anesthesia, (2) preliminary sutures around the lesion, (3) ligation of the limited numbers of feeding vessels in the vascular malformation of the neurofibroma. Ligating these pedicles can decrease bleeding during resection of the neurofibroma, as demonstrated in their patient.- - - - - - - - - - ranking = 0.14285714285714keywords = nerve (Clic here for more details about this article) |
6/45. Deep-seated segmental neurofibromatosis without cafe au lait spots.Segmental neurofibromatosis is a rare disease characterized by neurofibromas with or without cafe au lait spots localized to one segment of the body. The majority of reported cases have had cutaneous neurofibromas, and patients with deep involvement have rarely been described. We report on two patients with deep-seated segmental plexiform neurofibromatosis and review the literature. All reviewed cases including the present two had no cafe au lait spots, axillary freckling, Lisch nodules, family history or malignant progression of disease. Differential diagnoses from neuro-fibromatosis 1 (von Recklinghausen disease) and malignant peripheral nerve sheath tumor are important for genetic counseling and avoiding overtreatment.- - - - - - - - - - ranking = 4.1900886985012keywords = nerve sheath, sheath, nerve (Clic here for more details about this article) |
7/45. Ruptured internal carotid aneurysm resulting from neurofibromatosis: treatment with intraluminal stent graft.PURPOSE: This report shows a method of treatment for life-threatening hemorrhage due to rupture of an aneurysm in the cervical internal carotid artery caused by neurofibromatosis. methods: Ten days after delivery of healthy twins, a 28-year-old woman with known neurofibromatosis had sudden massive swelling in the left neck. After initial tracheostomy, angiography confirmed rupture of the mid cervical internal carotid artery as well as contribution to the resultant pseudoaneurysm from external carotid branches. Treatment began with coil embolization of the external carotid branches. The internal carotid lesion, a defect approximately 1 cm in length, was then closed through use of two stent grafts, each made from Palmaz stents and 3-mm polytetrafluorethylene grafts predilated to 6 mm. The neck hematoma was then evacuated surgically. RESULTS: Completion angiography and computed tomographic scanning confirmed control of the hemorrhage. The patient survived neurologically intact with the exception of cranial nerve deficits caused by the hemorrhage. The tracheostomy tube was removed 3 weeks postoperatively. Follow-up computed tomographic scanning showed a gradual decrease in the size of the cervical soft tissue and no recurrent aneurysm. CONCLUSION: Neurofibromatosis is a rare cause of aneurysmal degeneration of blood vessels. Repair of a ruptured cervical internal carotid artery aneurysm, though feasible, is difficult with stent grafts; however, this is a better option than surgical intervention in inaccessible vessels.- - - - - - - - - - ranking = 0.14285714285714keywords = nerve (Clic here for more details about this article) |
8/45. Functional reconstruction of the lateral face after ablative tumor resection: use of free muscle and musculocutaneous flaps.BACKGROUND: Wide resection of tumors of the middle third of the face often results in complex three-dimensional defects and facial paralysis either due to removal of the facial nerve within the tumoral tissue or to extensive resection of the facial muscles. methods: We report the cases of three patients who underwent wide excision of tumors of the cheek region, operations that resulted in tissue defects and facial palsy. Defect reconstruction and facial reanimation was accomplished in one stage through functional muscle transplantation. RESULTS: Follow-up of more than 1 year showed good symmetry at rest and reanimation of the corner of the mouth in all cases, but one patient, in which the ipsilateral facial main trunk was used as motor nerve supply to the transplanted muscle, developed significant muscle contracture and binding of the cheek skin. CONCLUSIONS: Every effort should be made to optimize the functional and cosmetic outcomes of neurovascular muscle transfers through precise planning and careful execution of the intricate details of the surgical technique for muscle transplantation.- - - - - - - - - - ranking = 0.28571428571429keywords = nerve (Clic here for more details about this article) |
9/45. Segmental neurofibromatosis-induced spinal cord compression. Case report.Spinal segmental neurofibromatosis (NF) is a rare entity. To date, patients in reported cases of segmental NF (or NF5) have harbored neurofibromas involving the peripheral nerves only. The author reports a rare case of segmental NF that caused spinal cord compression in a 40-year-old woman who presented with a 6-month history of intercostal neuralgia. Examination revealed mild lower-extremity weakness and dysesthesia in the right-sided T-9 dermatome. magnetic resonance imaging revealed three neurofibromas involving the T-9 region, which were excised, and the patient's neuralgic pain was resolved postoperatively. Traditionally, it has been believed that segmental NF involved only the peripheral nerves. The present case illustrates that although rare, spinal cord compression can also occur in patients with segmental NF.- - - - - - - - - - ranking = 0.28571428571429keywords = nerve (Clic here for more details about this article) |
10/45. A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.- - - - - - - - - - ranking = 2.5743465040313keywords = nerve, neoplasm (Clic here for more details about this article) |
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