Servelle-martorell syndromeQ85.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

angiomatosis osteolytica; Gorhams disease; Gorham's Syndrome; Gorham-Stout Syndrome; Hemangiomatosis osteolytic; Osteolytic hemangiomatosis; Peripheral vascular malformation (Servelle-Martorell); Vanishing bone disease

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HistoryThis section has been translated automatically.

Servelle 1948; Gorham and Stout 1955; Martorell and Monserrat 1962; Servelle et al. 1976

DefinitionThis section has been translated automatically.

Very rare, congenital angiodysplasia characterized by extensive venous, mostly cavernous hemangiomas and disproportionate skeletal hypoplasia, occasionally with bone destruction of a limb. No hemodynamically active arteriovenous fistulas.

Differential diagnosisThis section has been translated automatically.

Klippel-Trénaunay syndrome; Parkes-Weber syndrome; Cobb syndrome.

TherapyThis section has been translated automatically.

Symptomatic, especially by means of compression therapy in congested areas. In addition, orthopaedic treatment as far as possible.

Progression/forecastThis section has been translated automatically.

Bone is resorbed over several years, which can lead to its complete dissolution. From the initially affected area, the infection can spread across joints, or it can remain local. It can persist, but it can also be progressive. Lost bone is not replaced.

LiteratureThis section has been translated automatically.

  1. Gorham LW, Wright AW, Shultz HH, Maxon FC (1954) Disappearing bones: A rare form of massive osteolysis. Report of two cases, one with autopsy findings. On J Med 17: 674-682
  2. Gorham LW, Stout AP (1955) Massive osteolysis (acute spontaneous absorption of bone, phantom bone, dissapearing bone): Its relation to hemangiomatosis. J Bone Joint Surg (Am) 37: 985-1004
  3. Lo CP et al Disappearing calvarium in Gorham disease: MR imaging characteristics with pathologic correlation. AJNR Am J Neuroradiol 25: 415-418
  4. Martorell F, Monserrat J (1962) Aplasia of the iliac vein and Klippel Trenaunay syndrome. Angiologia 14: 2-4
  5. Servelle M, Bastin R, Loygue J, et al (1976) Hematuria and rectal bleeding in the child with Klippel and Trenaunay syndrome. Annals of Surgery 183: 418-428
  6. Servelle M (1985) Klippel and Trenaunay's Syndrome. Annals of Surgery 201: 365-373
  7. Wolf CH (1987) Angiodysplasias of the extremities. dermatologist 38: 645-651

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer