Literature DB >> 184345

[Combination of the syndrome of Sturge-Weber and the syndrome of Klippel-Trénaunay (author's transl)].

J Deutsch, G Weissenbacher, K Widhalm, G Wolf, B Barsegar.   

Abstract

Up until now 39 cases of combined Klippel-Trénaunay syndrome and Sturge-Weber syndrome have been described. Here follows the report of a girl, now 4 years of age, displaying a full combination of these syndromes. Only a small part of the body surface is not covered with naevi teleangiectatici laterales. The patient has clear hypertrophy of the left cheek and of the left lower extremity, less noticeable on the left upper extremity. For therapeutic reasons the left side of the head and the left lower extremity were thoroughly angiographically examined--this revealed typical abnormalities. The vessel-alteration of the lower extremity are not extremely far developed and arteriovenous fistulas on a large scale are also absent. This allows us to dismiss the F.P. Weber syndrome on the one hand, while it explains the absence of complications of the Klippel-Trénaunay syndrome, as described in literature, on the other. The significance of the alterations of lymph nodes in this disease, which we are the first to describe, is at present not fully clear. The cerebral attacks have until now showed only a temporary response to medication.

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Year:  1976        PMID: 184345

Source DB:  PubMed          Journal:  Klin Padiatr        ISSN: 0300-8630            Impact factor:   1.349


  4 in total

1.  Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations.

Authors:  C Adamsbaum; F Pinton; Y Rolland; C Chiron; O Dulac; G Kalifa
Journal:  Pediatr Radiol       Date:  1996-11

2.  Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations.

Authors:  A Bisdorff; J B Mulliken; J Carrico; R L Robertson; P E Burrows
Journal:  AJNR Am J Neuroradiol       Date:  2007-02       Impact factor: 3.825

3.  An infantile case of Sturge-Weber syndrome in association with Klippel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis.

Authors:  Chang-Woo Lee; Du-Young Choi; Yeon-Geun Oh; Hyang-Suk Yoon; Jong-Duk Kim
Journal:  J Korean Med Sci       Date:  2005-12       Impact factor: 2.153

4.  Overlap of sturge-weber syndrome and klippel-trenaunay syndrome.

Authors:  Radheshyam Purkait; Tryambak Samanta; Tapankumar Sinhamahapatra; Mridula Chatterjee
Journal:  Indian J Dermatol       Date:  2011-11       Impact factor: 1.494

  4 in total

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