목적 : 색소녹내장이 동반된 홍채각막내피증후군 1예를 경험하였기에 보고하고자 한다. 대상과 방법 : 우안의 간헐적인 시력저하를 주소로 내원한 44세 남자 단안 녹내장 환자에게 감별진단을 위해 안과적 검사와 전신 검사를 시행하였다. 결과 : 양안 교정시력은 1.0이었고, 골드만압평안압계 검사상 우안 50 mmHg, 좌안 18 mmHg이었다. 우안에서 세극등검사상 각막내피에 크루켄버그방추(Krukenberg spindle)와 유사한 색소의 침착, 앞방각경 검사상 쉬발베선을 넘어선 주변홍채앞유착과 섬유주 과색소침착, 경면현미경검사상 각막내피세포의 다형태성, 크기의 다양성과 숫자의 감소, 세포중심부의 밝은 반점과 주변은 어둡게 보이고 세포 가장자리는 밝게 나타나는 특징적 세포의 관찰되어 색소녹내장이 동반된 홍채각막내피증후군으로 진단하였다. 약물치료에도 불구하고 시신경유두입체촬영, 망막신경섬유층촬영 및 시야검사상 녹내장성 시신경손상이 진행하여 섬유주절제술을 시행하였다. 결론 : 약물치료에 반응하지 않아 녹내장 수술을 시행한 홍채각막내피증후군과 단안 색소녹내장이 동반된 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Purpose: To report a case of iridocorneal endothelial syndrome with pigmentary glaucoma. Methods: We encountered a 44-year-old unilateral glaucoma patient who complained of intermittently decreased visual acuity in the right eye. For differential diagnosis, we carried out ophthalmic and systemic examination. Results: Binocular corrected visual acuity was 1.0. His intraocular pressure, measured using a Goldmann applanation tonometer, was 50 mm Hg in the right eye and 18mm Hg in the left. Upon examination of the right eye, we found pigments with a Krukenberg's spindle appearance on the corneal endothelium, peripheral anterior synechia extending beyond Schwalbe's line, trabecular hyperpigmentation, endothelial pleomorphism, polymegathism, cell loss, dark area within the cells, a light central spot and light peripheral zone, retinal nerve fiber layer defects, and visual field defects, which together led to the diagnosis of iridocorneal endothelial syndrome with pigmentary glaucoma. We observed progressed glaucomatous injury upon examination of the disc stereo photograph, retinal nerve fiber layer photograph, and visual field test, even though we used topical IOP reducers. Eventually, we performed a trabeculectomy in the right eye. Conclusions: We experienced a case of iridocorneal endothelial syndrome with unilateral pigmentary glaucoma. The glaucoma was not well-controlled with topical IOP reducers. The glaucoma was then treated by a trabeculectomy. We report this case with a review of the literature.

Purpose: To report a case of iridocorneal endothelial syndrome with pigmentary glaucoma. Methods: We encountered a 44-year-old unilateral glaucoma patient who complained of intermittently decreased visual acuity in the right eye. For differential diagnosis, we carried out ophthalmic and systemic examination. Results: Binocular corrected visual acuity was 1.0. His intraocular pressure, measured using a Goldmann applanation tonometer, was 50 mm Hg in the right eye and 18mm Hg in the left. Upon examination of the right eye, we found pigments with a Krukenberg's spindle appearance on the corneal endothelium, peripheral anterior synechia extending beyond Schwalbe's line, trabecular hyperpigmentation, endothelial pleomorphism, polymegathism, cell loss, dark area within the cells, a light central spot and light peripheral zone, retinal nerve fiber layer defects, and visual field defects, which together led to the diagnosis of iridocorneal endothelial syndrome with pigmentary glaucoma. We observed progressed glaucomatous injury upon examination of the disc stereo photograph, retinal nerve fiber layer photograph, and visual field test, even though we used topical IOP reducers. Eventually, we performed a trabeculectomy in the right eye. Conclusions: We experienced a case of iridocorneal endothelial syndrome with unilateral pigmentary glaucoma. The glaucoma was not well-controlled with topical IOP reducers. The glaucoma was then treated by a trabeculectomy. We report this case with a review of the literature.

Iridocorneal endothelial syndrome, Pigmentary glaucoma