1/12. Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism.Neurofibromatosis has been known to involve blood vessels throughout the body. Pulmonary involvement with interstitial fibrosing alveolitis has been described but no case of pulmonary vascular involvement has been reported to date. A 51 year old patient with cutaneous neurofibromatosis is described who presented with severe pulmonary hypertension and radiographic, scintigraphic, and angiographic evidence of chronic thromboembolic pulmonary hypertension. Severe intimal fibrosis consistent with vascular involvement with neurofibromatosis was found on endarterectomy with no evidence of pulmonary thromboembolism. Neurofibromatosis of pulmonary arteries should be considered as a possible cause of pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. 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 = 1keywords = vessel (Clic here for more details about this article) |
3/12. 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 = 1keywords = vessel (Clic here for more details about this article) |
4/12. 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 = 2keywords = vessel (Clic here for more details about this article) |
5/12. Meningioangiomatosis occurring in a young male without neurofibromatosis: with special reference to its histogenesis and loss of heterozygosity in the NF2 gene region.A 16-year-old young male experienced persistent headache, and brain computed tomography and magnetic resonance imaging showed an abnormal mass with calcification in the right temporal lobe of the cerebrum. The tumor was located in the leptomeninges and cerebral cortex. In the leptomeninges, multiple calcified-fibrous nodules were noted. In this area spindle-shaped cells were arranged in a fascicular or storiform pattern. A few meningioma-like nodules were also present. With continuity of this leptomeningeal lesion, a diffuse infiltrative lesion composed of proliferating perivascular cells and hyalinized small vessels was also present in the cerebral cortex. The proliferating vessels were small and narrowed by proliferation of surrounding spindle-shaped cells. Immunohistochemically, the spindle-shaped cells had strong to moderate positivity for vimentin and CD34 and weak positivity for epithelial membrane antigen and S-100 protein. The maximum Ki67 labeling index was 0.3%. The spindle-shaped cells showed loss of heterozygosity on D17S929 and D17S282 microsatellite markers flanking the NF2 gene. These histopathologic and genetic findings are consistent with meningioangiomatosis, and meningioangiomatosis has been thought to be a neoplastic lesion of meningothelial cells. This is the first report of a genetic alteration in a case of meningioangiomatosis.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
6/12. Neurofibromatosis cerebral vasculopathy in an infant: clinical, neuroradiographic, and neuropathologic studies.An infant with neurofibromatosis developed recurrent cerebrovascular accidents beginning at 3 months of life. Cerebral arteriography demonstrated large-vessel occlusive disease and bilateral severe moyamoya. At autopsy these lesions were apparent on gross examination, and advanced intimal and medial dysplasia was seen on microscopic examination of both the anterior and posterior circulations. Although rare, neurofibromatosis cerebrovasculopathy may occur in young infants and may account for some strokes in this age group.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/12. Severe hypotension in the prone position in a child with neurofibromatosis, scoliosis and pectus excavatum presenting for posterior spinal fusion.A 34-mo-old boy with neurofibromatosis, scoliosis, and pectus excavatum developed severe hypotension when positioned prone. A magnetic resonance image study revealed neurofibromas encircling the great vessels. During the next anesthetic the patient was placed in the prone position on transverse bolsters and hypotension ensued again. A transesophageal echocardiogram (TEE) revealed compression of the right ventricle by the sternum. When the child was turned supine, the blood pressure returned to baseline. The patient was returned to the prone position, this time with bolsters placed longitudinally, without problem. This case supports a cardiac evaluation, possible intraoperative TEE, and avoidance of sternal pressure in patients with chest wall deformities requiring prone positioning. IMPLICATIONS: A child with neurofibromatosis, scoliosis, and a chest wall deformity presenting for spinal fusion developed severe hypotension while prone. This was due to compression of the heart by the sternum, not compression of the great vessels by neurofibromas. Sternal pressure in prone patients with chest wall deformities should be avoided. Unique management included the use of transesophageal echocardiography to determine the cause of the hypotension.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/12. skin ulcerations as manifestation of vasculopathy in neurofibromatosis.Vascular involvement in neurofibromatosis sometimes occurs and usually affects larger blood vessels of internal organs. We describe an unusual case of a now 41-year-old man with extensive skin involvement with ulcerations and histopathologic features of fibromuscular dysplasia, which has not been previously reported in the skin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/12. aneurysm of the popliteal artery in neurofibromatosis.The neurofibromatoses are a heterogeneous set of genetic disorders having clinical manifestations that involve the skin, the nervous system, or both. In addition, the disease can be confounded by a broad spectrum of complications, such as various kinds of osseous lesion, vascular lesions, aqueduct stenosis, optic glioma, and learning disabilities. Neurofibromatosis results in vascular involvement in approximately 10% of cases. Stenotic lesions predominate, but aneurysms have been documented as well. Rarely noted, however, have been peripheral aneurysms. In this report, we discuss the case of a 66-year-old woman with type 1 neurofibromatosis and a popliteal artery aneurysm who was operated upon because of threatened limb ischemia. Histological findings confirmed neurofibromatous invasion of the vessel wall.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/12. Superficial femoral vein invasion by a benign neurofibroma in a non-neurofibromatosis patient: case report.OBJECTIVE AND IMPORTANCE: Neurofibromas are benign neural sheath tumors arising from intraneural supporting cells. Such tumors are characteristic of neurofibromatosis Type I (von Recklinghausen disease) but also occur sporadically. Vascular involvement by neurofibromata is rare, but has been described in the past in the context of neurofibromatosis. There is, to our knowledge, no description of vascular involvement by a neurofibroma in a non-neurofibromatosis patient. CLINICAL PRESENTATION: A 40-year-old woman presented with a 4 year history of a right thigh mass associated with diffuse lower extremity pain. She had no other clinical manifestations of neurofibromatosis and no known family member with neurofibromatosis Type I. magnetic resonance imaging scans revealed a well-defined solid mass in the anteromedial aspect of the right thigh closely associated with the superficial femoral vein. INTERVENTION: The vessel segment and encapsulated mass were resected "en bloc" after proximal and distal ligation of the vein. The pathological appearance of the mass was consistent with a benign neurofibroma that had infiltrated all layers of the vessel. CONCLUSION: Vessel invasion by a benign sporadic neurofibroma is a rare occurrence with potentially severe implications for the patient. It suggests that surgical removal of asymptomatic benign-appearing lesions of that type should be considered if they are adjacent to important anatomical structures.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
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