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Gorham-Stout Syndrome in a Male Adolescent—Case Report and Review of the Literature

Pfleger, Andreas MD*; Schwinger, Wolfgang MD; Maier, Alfred MD; Tauss, Josef MD§; Popper, Helmut H. MD; Zach, Maximilian S. MD*

Journal of Pediatric Hematology/Oncology: April 2006 - Volume 28 - Issue 4 - p 231-233
doi: 10.1097/01.mph.0000203721.83566.e6
Clinical and Laboratory Observations
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Gorham-Stout syndrome is a rare disease characterized by vascular proliferation in the bones, effecting osteolysis. When it is complicated by chylothorax, the prognosis is poor. The present case illustrates successful management of chylothorax by a combination of surgery (thoracic duct ligation and excision of lymphangiomatous tissue combined with pleurodesis) and treatment with α-2b interferon. Treatment in 38 published cases is reviewed.

*Respiratory and Allergic Disease Division

Division of Hematology and Oncology, Pediatric Department

Division of Thoracic Surgery, Surgical Department

§Department of Radiology

Laboratory for Environmental and Respiratory Pathology, Institute of Pathology, Graz, Styria, Austria

Reprints: Dr Andreas Pfleger, MD, Klinische Abteilung für Pädiatrische Pulmonologie und Allergologie, Univ.-Klinik für Kinder- und Jugendheilkunde, Auenbruggerplatz 30, 8036 Graz, Austria (e-mail: Andreas.Pfleger@meduni-graz.at).

Received for publication May 19, 2005; accepted December 20, 2005

© 2006 Lippincott Williams & Wilkins, Inc.