Çocukluk Çağı Ender Hastalığı: Gorham-Stout Sendromu

Abstract

Gorham-Stout sendromu lokalize lenfatik damarların endotelial proliferasyonuna bağlı kemikte absorbsiyon ve destruksiyonun geliştiği düşünülen nedeni tam olarak bilinmeyen ender bir hastalıktır. Ateş ve öksürük yakınması ile başvuran 6 yaşında kız hastanın akciğer grafisinde kostalarda ve inceleme alanına giren humerusda litik lezyonlar saptandı. Öyküsünde 3 yaşındayken küçük bir travma sonrası sağ humerus, her iki klavukulada kırık tarifleyen hastanın direk kafa grafisi, pelvis grafisi, üst ve alt ekstremite kemiklerinde yaygın osteolitik lezyonlar izlendi. Hastanın ekstremite magnetik rezonans görüntülemeleri Gorham-Stout hastalığı ile uyumlu bulundu. Gorham-Stout hastalığın ender görülmesi, diğer hastalıklar ile karışabilmesi ve başlangıçta tanı konmasının zor olması nedeniyle vakamızı sunduk.

Keywords

• Gorham-Stout sendromu,kemik kırıkları,kemikte litik lezyonlar

A Rare Childhood Disease: Gorham-Stout Syndrome

Abstract

Gorham-Stout syndrome is a rare disorder characterized by endothelial proliferation of vascular vessels that results in the development of destruction and resorption of osseous matrix. A 6-year-old girl admitted to hospital with fever and cough. Lytic bone lesions were detected on ribs chest radiograms, and humerus within region of examination. In medical history we learned that right humerus and both of clavicles were broken before when she was 3 years of age at different times from each other with minor traumas. Extensive lytic lesions were observed on radiograms of pelvis, cranium, upper and lower extremities. Magnetic resonance imaging of the patient’s extremities were consistent with Gorham-Stout disease. We presented this case because Gorham-Stout disease is a rare disease and the diagnosis is difficult at its onset, and the disease can easily be confused with other diseases.

Keywords

• Gorham-Stout syndrome,bone fractures,lytic bone lesions

References

1. Patel dv. Gorham’s disease or massive osteolysis. Clin Med Res 2005;3(2):65-74.
2. https://doi.org/10.3121/cmr.3.2.65
3. Gorham lW, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disap- pearing bone); its relation to hemangiomatosis. J Bone Joint Surg Am 1955;37(5):985-1004.
4. https://doi.org/10.2106/00004623-195537050-00008
5. lala S, Mulliken JB, Alomari Aı, Fishman SJ, kozakewich HP, Chaudry G. Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiolo- gic, and histologic differentiation. Skeletal Radiol 2013;42(7):917-24.
6. https://doi.org/10.1007/s00256-012-1565-4
7. radhakrishnan k, rockson SG. Gorham’s disease: an osseous disease of lymphangiogenesis? Ann N Y Acad Sci 2008;1131:203-5.
8. https://doi.org/10.1196/annals.1413.022

9. Scalzetti EM, Heitzman Er, Groskin SA, randall PA, katzenstein Al. Developmental lymphatic disor- ders of the thorax. Radiographics 1991;11(6):1069-85.
10. https://doi.org/10.1148/radiographics.11.6.1749850
11. Prasanna r, Sankar J, ramachandran P. Gorhams disease: vanishing bone syndrome. Indian Pediatr 2009;46(3):255-6.
12. Heyd r, Micke O, Surholt C, Berger B, Martini C, Füller J, et al; German Cooperative Group on radiotherapy for Benign diseases (GCG-Bd). Radiation therapy for Gorham-Stout syndrome: results of a national patterns-of-care study and literature revi- ew. Int J Radiat Oncol Biol Phys 2011;81(3):e179-85.
13. https://doi.org/10.1016/j.ijrobp.2011.01.006
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(3): E47-50.
15. https://doi.org/10.1016/j.jpedsurg.2004.11.015
16. 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(6):613-5.
17. https://doi.org/10.1002/ppul.20849
18. zheng MW, yang M, Qiu JX, nan XP, Huang ly, zhang Wd, et al. Gorham-Stout syndrome presenting in a 5-year-old girl with a successful bisphosphonate therapeutic effect. Exp Ther Med 2012;4(3):449-51.