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Treatment of EARLY‐ONSET Gorham Syndrome with 8‐Year Follow‐Up
Author(s) -
Maruani Annabel,
Thimon Sylvie,
de Courtivron Benoît,
de Pinieux Gonzague,
Baulieu Françoise,
Machet Laurent,
Lorette Gérard
Publication year - 2012
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2011.01712.x
Subject(s) - medicine , lymphedema , osteolysis , histopathology , tibia , radiography , thigh , surgery , anatomy , pathology , cancer , breast cancer
A case of Gorham disease with several years of follow‐up is reported. At birth he had a mass in the thigh which was had pathology demonstrating a lymphangioma. By age 3 years, he had lymphedema of the ipsilateral foot and discrepant leg lengths. Radiography revealed heterogenous dystrophy of the bones and osteolysis of the hallux. At age 6, a spontaneous fracture of the right tibia was treated with surgery. Histopathology of a bone sample demonstrated bone remodelling, fibrous tissue, and large vascular lacunas within bone tissue, bordered by cells expressing the lymphaticmarker D2–40. At 8 years old, lymphedema of the right inferior leg had increased, leg lengths still differed, but other clinical signs were absent.