Vázquez-Folch Sebastián J, Jiménez-Berríos Gabriel A, Izquierdo Natalio
School of Medicine, Universidad Central del Caribe, Bayamón, PRI.
Department of Surgery, School of Medicine, University of Puerto Rico, Medical Sciences Campus, San Juan, PRI.
Cureus. 2024 Sep 5;16(9):e68752. doi: 10.7759/cureus.68752. eCollection 2024 Sep.
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism, bleeding diathesis, and ceroid deposition leading to complications such as pulmonary fibrosis and colitis. This case report describes a 63-year-old female with HPS type 1 (HPS1) who developed acute angle closure glaucoma following cataract surgery with anterior intraocular lens implantation. The patient presented with severe acute pain in the right eye, which was diagnosed as a pupillary block leading to acute angle closure glaucoma. Preoperative planning included the administration of aminocaproic acid to mitigate bleeding risk, given the patient's underlying bleeding diathesis. Yttrium aluminium garnet (YAG) laser iridotomy was performed, resulting in a significant reduction of intraocular pressure and stabilization of the patient's condition.
赫尔曼斯基-普德拉克综合征(HPS)是一种罕见的常染色体隐性疾病,其特征为眼皮肤白化病、出血素质以及蜡样质沉积,可导致诸如肺纤维化和结肠炎等并发症。本病例报告描述了一名63岁患有1型HPS(HPS1)的女性,她在白内障手术联合前房型人工晶状体植入后发生了急性闭角型青光眼。患者右眼出现严重急性疼痛,被诊断为瞳孔阻滞导致急性闭角型青光眼。鉴于患者潜在的出血素质,术前计划包括给予氨基己酸以降低出血风险。实施了钇铝石榴石(YAG)激光虹膜切开术,眼压显著降低,患者病情稳定。