Engin AYDIN, Turan YILDIZ, Aysel YUCAK, Zekeriya İLÇE, Bahri ELMAS

Çocuklarda Nadir Bir Şilotoraks Nedeni: Gorham - Stout Sendromu

Gorham-stout hastalığı kemik dokusunun litik lezyonları ile birlikte şilotoraks ve abdominal lenfangiomanın olabildiğikompleks 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 2bile başarılı sonuçlar bildirilmiştir. Biz burada şilotoraks ile başvuran, kemikte litik lezyonları olan 5 yaşındaki kız hastanınklinik tanısı ve tedavisini tartıştık

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

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.

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1. Noda M, Endo C, Hoshikawa Y, et al. Successful management of intractable chylothorax in Gorham-Stout disease by awake thoracoscopic surgery. Gen Thorac Cardiovasc Surg. 2013;61(6):356- 8.
2. De Smet K, De Maeseneer M, Huijssen-Huisman E, Van Gorp V, Hachimi-Idrissi S, Ernst C. A rare cause of dyspnea due to chylothorax. Emerg Radiol 2010;17:503-5.
3. Kuriyama DK, McElligott SC, Glaser DW, Thompson KS. Treatment of Gorham-Stout disease with zoledronic acid and interferon-α: A case report and literature review. J Pediatr Hematol Oncol 2010;32:579-584.
4. Brodszki N, Länsberg J-K, Dictor M, et al. A novel treatment approach for paediatric Gorham–Stout syndrome with chylothorax. Acta Paediatr 2011;100:1448-53.
5. Deveci M, Inan N, Çorapçioǧlu F, Ekingen G. Gorham-Stout syndrome with chylothorax in a six-year-old boy. Indian J Pediatr. 2011;78:737-9.
6. Brodszki N, Länsberg JK, Dictor M, et al. A novel treatment approach for paediatric Gorham-Stout syndrome with chylothorax. Acta Paediatr Int J Paediatr 2011;100:1448-53.
7. Nozawa A, Ozeki M, Kuze B, Asano T, Matsuoka K, Fukao T. Gorham-Stout disease of the skull base with hearing loss: Dramatic recovery and antiangiogenic therapy. Pediatr Blood Cancer 2016;63:931-4.
8. Gorham Lw, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone); its relation to hemangiomatosis. J Bone Joint Surg Am 1955;37-A(5):985- 1004.
9. Patel DV. Gorham’s disease or massive osteolysis. Clin Med Res 2005;3:65-74.
10. Kakhaki AD, Khodadad K, Pejhan S, Karimi S, Arab M, Saghebi R, et al. Gorham’s Disease with chest wall involvement: A case report and a review of the literature. Iran Red Crescent Med J 2014;16. e12180.
11. Kose M, Pekcan S, Dogru D, Akyuz C, Ozcelik U, Ozsurekci Y, et al. Gorham-Stout syndrome with chylothorax: Successful remission by interferon alpha-2b. Pediatr Pulmonol 2009;44:613-5.
12. Trenor CC, Chaudry G. Complex lymphatic anomalies. Semin Pediatr Surg 2014;23:186-90.
13. Ezekowitz RAB, Mulliken JB, Folkman J. Interferon Alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992;326:1456-63.
14. Takahashi A, Ogawa C, Kanazawa T, Watanabe H, Suzuki M, Suzuki N, et al. Remission induced by interferon alfa in a patient with massive osteolysis and extension of lymph-hemangiomatosis: A severe case of Gorham-Stout syndrome. J Pediatr Surg 2005;40:E47-E50.
15. Timke C, Krause MF, Oppermann H, Leuschner I, Claviez A. Interferon alpha 2b treatment in an eleven-year-old boy with disseminated lymphangiomatosis. Pediatr Blood Cancer 2007;48:108-11.
16. Hagberg H, Lamberg K, Aström G. Alpha-2b interferon and oral clodronate for Gorham’s disease. Lancet 1997;350:1822-3.
17. Grunewald TGP, Damke L, Maschan M, Petrova U, Surianinova O, Esipenko A, et al. First report of effective and feasible treatment of multifocal lymphangiomatosis (Gorham-Stout) with bevacizumab in a child. Ann Oncol 2010;21:1733-4.