Document Detail


Macromelia masquerading as an acromegaloid syndrome in an adult with Klippel-Trénaunay syndrome.
MedLine Citation:
PMID:  18238750     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To describe a case of Klippel-Trénaunay syndrome in an adult patient with symmetric macromelia suggestive of an acromegaloid syndrome. METHODS: We report clinical and laboratory data that were extracted from the medical records of the study patient. We also survey the relevant reports identified through a MEDLINE search of the English-language literature published between January 1, 1996, and June 2, 2007, using the phrase, Klippel-Trénaunay syndrome. RESULTS: A 28-year-old man was admitted to the hospital for weeping lymphedema of the left lower extremity. He had pronounced symmetric hypertrophy of all distal extremities, port-wine stains on his right neck, and varicosities on his left groin. The patient's insulinlike growth factor 1 concentration was 92 ng/mL (reference range, 117-329 ng/mL), which did not support the diagnosis of acromegaly. Klippel-Trénaunay syndrome is a rare congenital condition that belongs to a family of disorders characterized by tissue overgrowth. Classically, the syndrome presents as a triad of vascular malformations, cutaneous hemangiomas, and bone or soft-tissue hypertrophy usually affecting one extremity. The tissue hypertrophy in this syndrome is typically localized and asymmetric. CONCLUSION: The recognition that the tissue hypertrophy in Klippel-Trénaunay syndrome can occur symmetrically will help avoid unnecessary and extensive workup for acromegaly.
Authors:
Erik Lowman; Arshag D Mooradian
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists     Volume:  14     ISSN:  1934-2403     ISO Abbreviation:  Endocr Pract     Publication Date:    2008 Jan-Feb
Date Detail:
Created Date:  2008-02-01     Completed Date:  2008-03-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9607439     Medline TA:  Endocr Pract     Country:  United States    
Other Details:
Languages:  eng     Pagination:  109-11     Citation Subset:  IM    
Affiliation:
Department of Medicine, University of Florida College, Jacksonville, Florida 32209-6511, USA.
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MeSH Terms
Descriptor/Qualifier:
Acromegaly / complications,  diagnosis*
Adult
Diagnosis, Differential
Humans
Klippel-Trenaunay-Weber Syndrome / diagnosis*
Lymphedema / complications,  diagnosis*
Male
Port-Wine Stain / complications,  diagnosis*
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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