Literature DB >> 7949488

Angiodysplastic syndrome with capillary and venous malformation associated with soft tissue hypotrophy.

A J Bircher1, J Y Koo, I J Frieden, T G Berger.   

Abstract

Angiodysplastic syndromes include a vascular malformation which may often be associated with secondary changes such as further vascular abnormalities, soft tissue and bone hypertrophy. One of the best known is the syndrome triad originally described by Klippel and Trenaunay, which includes a unilateral capillary malformation, ectatic veins and osseous and soft tissue hypertrophy. A female patient is reported who had an extensive capillary malformation, discrete ectatic veins overlying an extensive venous malformation and soft tissue hypotrophy without bone involvement. Our patient and cases from the literature illustrate the occurrence of 'atypical', hypotrophic variants of angiodysplastic syndromes. Discrete superficial angiodysplasias may overlie deep, extensive malformations, and identification of such cases is important with regard to management and prognosis. The current classification of angiodysplasias is based on the primary vascular malformation. The use of eponyms should be avoided, because they do not contribute to the diagnosis and management of such cases.

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Year:  1994        PMID: 7949488     DOI: 10.1159/000246865

Source DB:  PubMed          Journal:  Dermatology        ISSN: 1018-8665            Impact factor:   5.366


  3 in total

1.  Oromaxillofacial osseous abnormality in Sturge-Weber syndrome: case report and review of the literature.

Authors:  D D M Lin; P Gailloud; E F McCarthy; A M Comi
Journal:  AJNR Am J Neuroradiol       Date:  2006-02       Impact factor: 3.825

2.  Axial structures control laterality in the distribution pattern of endothelial cells.

Authors:  S Klessinger; B Christ
Journal:  Anat Embryol (Berl)       Date:  1996-04

3.  Focal venous hypertension as a pathophysiologic mechanism for tissue hypertrophy, port-wine stains, the Sturge-Weber syndrome, and related disorders: proof of concept with novel hypothesis for underlying etiological cause (an American Ophthalmological Society thesis).

Authors:  Cameron F Parsa
Journal:  Trans Am Ophthalmol Soc       Date:  2013-09
  3 in total

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