AI Article Synopsis

  • Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder that causes oculocutaneous albinism, bleeding issues, and can lead to serious complications like lung fibrosis and colitis.
  • A case report discusses a 63-year-old woman with HPS type 1 who experienced acute angle closure glaucoma after cataract surgery, resulting in severe pain in her right eye.
  • The preoperative care included aminocaproic acid to reduce bleeding risks, and a YAG laser iridotomy was performed to lower eye pressure and stabilize her condition.

Article Abstract

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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11456148PMC
http://dx.doi.org/10.7759/cureus.68752DOI Listing

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