황반원공 망막박리로 나타난 베체트병
Behçet’s Disease Initially Presenting as a Macular Hole with Exudative Retinal Detachment
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목적: 삼출망막하액을 동반한 황반원공 형태로 나타난 베체트 포도막염 증례를 보고하고 그 발생 기전을 고찰하고자 한다. 증례요약: 19세 여자가 한 달 전부터 시작된 우안 시력저하를 주소로 내원하였다. 우안 시력은 안전수지, 전방 염증과 유리체혼탁, 삼출망막하액을 동반한 황반원공이 관찰되었고, 형광안저혈관조영술에서 망막혈관염이 관찰되었다. Kg당 1 mg의 경구 prednisolone 복용 후 혈관염은 조절되었다. 우안 수정체 보존 유리체절제술, 내경계막제거술, 내경계막절편술, 액체공기교환술을 시행 후 황반원공은 폐쇄되었다. 치료 시작 3개월 후 prednisolone을 10 mg 복용한 상태에서 회음부 궤양, 구강 궤양이 발현되었고 베체트병으로 진단되었다. 경구 colchicine과 cyclosporine을 추가하여 염증을 조절하였고, 약물 감량 중 재발하여 adalimumab 피하주사를 사용하여 조절 중이다. 결론: 황반원공 망막박리 형태로 발현된 베체트병 증례이며, 후포도종이 없는 젊은 여성에서 삼출망막박리를 동반한 황반원공 망막박리가 관찰되는 경우 베체트병 등 포도막염 동반을 의심해야 한다.
Abstract
Purpose: To report a case of Behçet’s disease presenting as a macular hole with exudative retinal detachment and an examination of its mechanism. Case summary: A 19-year-old woman presented with decreased visual acuity in her right eye for a month. The visual acuity of the right eye was finger count. Inflammation was evident in the anterior chamber cells and vitreous. Optical coherence tomography revealed a macular hole with retinal detachment. Retinal vasculitis was observed in fluorescein angiography imagery. Vasculitis was well controlled with administration of 1 mg per kg oral prednisolone. Vitrectomy and internal limiting membrane (ILM) peeling were performed with ILM flap creation. The macular hole was closed after the surgery. Behçet’s disease was diagnosed based on a manifestation of genital and oral ulcers during tapering of prednisolone. Subcutaneous adalimumab has been used to control uveitis due to repeated relapse. Conclusions: This is a case of Behçet’s disease initially presenting as macular hole retinal detachment. Uveitis such as that occurring with Behçet’s disease should be considered if the macular hole retinal detachment is noticed in a young patient without posterior staphyloma.
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