A Rare Cause of Chylothorax in Children: Gorham-Stout Syndrome

Year 2019, Volume: 13 Issue: 1, 36 - 39, 21.03.2019

Engin Aydın

https://doi.org/10.12956/tjpd.2018.375

Abstract

 Gorham-Stout disease is a complex disease in which lytic lesions of bone tissue are accompanied by chylothorax and abdominal lymphangioma. The etiology is still unknown. The diagnosis is made by the patient’s clinical and laboratory findings and biopsy results. It may be fatal depending on the organs involved. Although many treatments have been described, successful results have been reported with interferon alpha 2b in recent years. We discuss the clinical diagnosis and treatment of a 5-year-old female patient with osteolytic lesions in the bones after she was referred with chylothorax 

Keywords

Child, Gorham-Stout Syndrome, Interferon Alfa-2b

Çocuklarda Nadir Bir Şilotoraks Nedeni: Gorham-Stout Sendromu

Abstract

 Gorham-stout hastalığı kemik dokusunun litik lezyonları ile birlikte şilotoraks ve abdominal lenfangiomanın olabildiği kompleks bir hastalıktır. Etiyolojisi halen bilinmemektedir. Tanısı hastanın kliniği, laboratuvar bulguları ve biyopsi ile konulur. Tutulan organlara göre ölümcül seyredebilir. Birçok tedavi tariflenmiş olmakla birlikte son yıllarda interferon alfa 2b ile başarılı sonuçlar bildirilmiştir. Biz burada şilotoraks ile başvuran, kemikte litik lezyonları olan 5 yaşındaki kız hastanın klinik tanısı ve tedavisini tartıştık. 

Keywords

Çocuk, Gorham-Stout Sendromu, Interferon Alfa-2b

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