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 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An extreme case of Klippel-Trenaunay-Parkes Weber syndrome is presented. The patient had extensive cutaneous naevus involving the left side of the body and consisting of naevus flammeus, hemangioma cavernosum, and naevus verrucosus. The left extremities were longer and there were multiple arteriovenous connections between a and v subclavia and a and v radialis. Secondary symptoms were sciatica, varicosity and osteoporosis.
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PMID:A case of the Klippel-Trenaunay-Parkes Weber syndrome. 5 Jun 99

"Angioma"-situations in the newborn are common, but even in adults one may find more or less common situations, more or less difficult to manage. - In any way, like in the early childhood, the differentiation between inborn teleangiectatic or angiokeratotic nevi and the capillary angioma or angiomatous nevi is very important concerning the prognostic and therapeutic consequences. The real newborn capillary angiomas are selfhealing. Nearly they are not found in adults and otherwise surgically treated in the same simple matter as little teleangiectasias and the eruptive "granuloma" teleangiectaticum. On the other hand late tuberous alterations of non selfhealing naevus flammeus, naevus angiokeratoticus and the teleangiectatic "angioma" racemosum of the orofacial region give a slot of surgical problems and no sufficient effect to different kinds of radiotherapy. - These situations are presented, discussed and documented, moreover some rare uncommon situations, e.g. blue rubber-bleb nevus-syndroma, and angiokeratotic nevus together with different well checked therapeutic methods and results.
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PMID:[Angiomatous diseases in the maxillofacial region and their treatment in adults]. 16 17

A patient with a rare case of Klippel-Trenaunay-Weber syndrome presented with paraplegia due to compression by a vertebral and epidural cavernous hemangioma. The metameric distribution of the large cutaneous vascular nevus provided the clinical clue to the nature of the spinal lesion. The association of the two lesions is explained in the basis of developmental anomaly.
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PMID:Vertebral and epidural hemangioma with paraplegia in Klippel-Trenaunay-Weber syndrome. Case report. 20 34

The case is presented of a recurrent subarachnoid haemorrhage caused by an angiographically-proven spinal A. V. angioma of glomus type II at the C 1--2 level supplied by both vertebral arteries. The angioma was accompanied by a median naevus flammeus over the occipito-cervical region. The clinical differentiation between subarachnoid haemorrhage of cerebral and spinal origin is discussed and emphasis is placed on the significance of complete cerebral angiography for the detection of the sources of recurrent subarachnoid haemorrhage of undetermined origin.
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PMID:[Recurrent subarachnoid haemorrhage due to upper cervical A. V.-angioma associated with metameric naevus flammeus (author's transl)]. 42 37

A 7-year-old white boy had multiple angiokeratomas, connective tissue nevus of the right foot, and cavernous hemangioma with hemiatrophy of the right lower extremity. The triad of abnormalities is distinct from previously described multiple malformation syndromes.
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PMID:Angiokeratomas, connective tissue nevus, hemangioma. 51 73

A unique association of Sturge-Weber syndrome and atlanto-occipital assimilation is presented. A 18-year-old male was admitted in emergency because of the sudden severe headache and vomiting. He had vascular nevus in the right half of the face at birth and several episodes of generalized convulsive seizures. On admission craniogram demonstrated calcification in the right occipital area. Neurological examination revealed tenderness in the nuchal region, moderate limitation of cervical mobility in a antero-posterior direction, Bruns-Cushing type nystagmus, bilaterally diminished gag reflex, and positive Romberg's test. Spinal tap showed crystal clear CSF with normal pressure. EEG showed paroxysmal slowing focus in the right parieto-occipital area. Polytomography of the craniovertebral junction demonstrated the unilateral atlanto-occipital assimilation on the left associated with the aplasia of the right posterior arch. Myelography was negative. A right carotid angiography disclosed the dilatation of the basal vein of Rosenthal and abnormal venous vasculature. CT-scan demonstrated the calcified region of the right occipital area more distinctly than the plain roentogenogram, but the enhancement study of the leptomeningeal angioma of the Sturge-Weber syndrome was negative. Never been found this rare association in a review of the literature, the authors discussed the clinical and radiological findings of both diseases.
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PMID:[Sturge-Weber syndrome associated with atlanto-occipital assimilation: a report of a case (author's transl)]. 68 66

A patient is described with the dermatological features of blue rubber bleb nevus syndrome (BRBNS), focal seizures, and lateralized neurological signs. CAT scan demonstrated a nonenhanced density in the region of the vein of Galen. Neuropathological examination showed that this density was a clot within a vein of Galen malformation. Hemangiomas that grossly resembled the skin lesions of BRBNS were seen on the cerebral surface. Many of these hemangiomas were thrombosed and overlay patchy zones of infarction. Numerous vascular malformations of varying histological types were also found within the brain and systemic organs. Bluish, compressible, often raised hemangiomas of the skin should alert physicians to the BRBNS and the potential for vascular malformations ot occur within the brain as well as systemic organs. Diagnosis of BRBNS involving the brain may assist in interpretation of radiographic findings. The tendency of these malformations to thrombose may account for focal neurological deficits.
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PMID:Blue rubber bleb nevus syndrome with CNS involvement and thrombosis of a vein of galen malformation. 72 30

A 53-year-old white man was noted to have bilateral retinitis pigmentosa associated with a left nevus flammeus, a left choroidal hemangioma and unilateral glaucoma. No central nervous system manifestations of Sturge-Weber syndrome were noted. The association of retinitis pigmentosa and the nevus flammeus is probably a chance occurrence.
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PMID:Retinitis pigmentosa associated with nevus flammeus and unilateral glaucoma. 90 Jul 9

Cobb syndrome consists of a vascular skin nevus associated with an angioma in the spinal cord. We describe a young man with this condition. To our knowledge, this is the 28th case reported. Cobb syndrome must be differentiated from other syndromes characterized by vascular cutaneous lesions associated with central nervous system abnormalities, such as Sturge-Weber, Osler-Weber-Rendu, Fabry-Anderson, von Hippel-Lindau, and ataxia telangiectasia.
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PMID:Cobb syndrome. 93 2

Nevus flammeus (cutaneous hemangioma, port-wine stain) may develop proliferative hamartomatous changes and go on to form monstrous deforming tumors. Excision is feasible, and it may be best to do it early. The mentally retarded, institutionalized patient can also benefit from the reconstruction. In 3 cases it was possible to preserve function of the muscles of facial expression, because of the superficial location of the tumor. Techniques to decrease blood loss are of variable value during the operative procedure.
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PMID:Excision of massive hemangioneurofibroma of the face. 95 19


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