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