Agageldi ANNAYEV, İsmail YILDIZ, Muhammet Ali VARKAL, Oğuz Bülent EROL, Ayşe KILIÇ, Fatma OĞUZ, Emin ÜNÜVAR

Çocukluk Çağı Ender Hastalığı: Gorham-Stout Sendromu

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.

Anahtar Kelimeler:

Gorham-Stout sendromu, kemik kırıkları, kemikte litik lezyonlar

A Rare Childhood Disease: Gorham-Stout Syndrome

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,

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