전체층황반원공을 동반한 망막색소변성환자의 수술적 치료: 증례보고
Surgical Repair of a Full-thickness Macular Hole in Retinitis Pigmentosa: a Case Report
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목적: 망막색소변성환자에서 발생한 전체층황반원공에 대해 수술적 치료 후 장기 결과를 보고하고자 한다. 증례요약: 5년 전 망막색소변성을 진단받은 55세 남자가 6개월 전 시작한 좌안의 시력저하를 주소로 내원하였다. 내원 시 최대교정시력은 우안 1.0, 좌안 0.3이었고, 세극등검사상 양안 후방의 인공수정체 이외의 특이 소견은 없었다. 안저검사상 양안 망막 중간주변부에 광범위한 골소체 모양의 색소침착 및 좌안 황반부에 시신경 크기 1/3 정도의 황반원공이 확인되었고, 빛간섭단층촬영검사상 유리체황반견인을 동반한 전체층황반원공과 원공둘레의 망막내액이 관찰되었다. 이에 수술적 치료를 계획하였으며, 유리체절제술 및 인도시아닌그린을 이용한 내경계막벗김술, 과불화프로판을 이용한 안내가스충전술을 시행하였다. 술 후 1주일에 시행한 빛간섭단층촬영검사상 황반원공은 해부학적으로 폐쇄된 것이 확인되었으며, 술 후 3개월째 좌안 최대교정시력은 0.63까지 호전되었다. 마지막 방문(술 후 6년)까지 시력과 황반원공의 폐쇄는 안정적으로 유지 중이다. 결론: 망막색소변성환자에서 전체층황반원공이 합병된 경우 내경계막벗김술을 동반한 유리체절제술이 치료 방법으로 고려될 수 있다.
Abstract
Purpose: To report the long-term outcome after surgical repair of a full-thickness macular hole (FTMH) in a patient with retinitis pigmentosa (RP). Case summary: A 55-year-old male who had been diagnosed with retinitis pigmentosa in both eyes 5 years earlier presented with decreased visual acuity in his left eye over the last 6 months. On examination, his Snellen best-corrected visual acuity (BCVA) was 1.0 in the right eye and 0.3 in the left eye. Slit-lamp examination of the anterior segment was remarkable only for posterior chamber intraocular lenses in each eye. Fundus examination demonstrated extensive bony spicule-like pigmentation in the mid-peripheral region in both eyes and a FTMH with approximately one-third disc diameter in the left eye. The optical coherence tomography (OCT) findings confirmed a FTMH with a surrounding cuff of intraretinal fluid and vitreomacular traction in the left eye. The patient underwent 23-gauge pars plana vitrectomy (PPV) with indocyanine green-assisted internal limiting membrane peeling and gas tamponade. One week postoperatively, an anatomically well-sealed macular hole was confirmed by OCT. At the 3-month postoperative follow-up, the BCVA improved to 0.63 and the hole remained closed until his last follow-up (postoperative 6 years). Conclusions: Although macular hole is a rare occurrence in RP patients, it should be considered as a cause of significant visual loss in patients with this disorder. Our case suggested that over the long-term, PPV may be tolerable in the management for FTMH in RP.
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