{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T22:37:33Z","timestamp":1773787053109,"version":"3.50.1"},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T00:00:00Z","timestamp":1763510400000},"content-version":"vor","delay-in-days":322,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Ophthalmological Medicine"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The aim of this study is to report a case of bilateral foveal cysts in MPS I\u2010H resolving with oral acetazolamide and to highlight the diagnostic value of multimodal retinal imaging and electrophysiological testing.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Hurler syndrome (mucopolysaccharidosis Type I\u2010H) is a lysosomal storage disorder that can cause progressive multisystem complications. Retinal involvement often mimics retinitis pigmentosa (RP), and pathology may progress even after early hematopoietic stem cell transplantation (HSCT) due to limited enzyme penetration into ocular tissues.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Summary<\/jats:title>\n                    <jats:p>A 17\u2010year\u2010old female with MPS I\u2010H, post\u2010HSCT at 21 months, presented with bilateral visual decline despite a normal clinical fundus exam. Evaluation included spectral\u2010domain OCT (SD\u2010OCT), fundus autofluorescence (FAF), multifocal electroretinography (mfERG), full\u2010field ERG (ffERG), and visual evoked potential. SD\u2010OCT revealed bilateral intraretinal foveal cysts without leakage on fluorescein angiography. FAF showed a bull\u2032s eye maculopathy pattern; mfERG showed bilateral macular dysfunction. ffERG revealed rod\u2013cone dystrophy. Two 3\u2010month courses of oral acetazolamide (125\u2009mg three times daily) led to complete cyst resolution and visual improvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case supports the role of systemic carbonic anhydrase inhibitors in treating nonleaking macular cysts in MPS I, similar to RP\u2010related cystoid macular pathologies, and highlights the value of integrating electrophysiological and multimodal imaging, especially in occult retinal disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1155\/crop\/5349202","type":"journal-article","created":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T16:34:18Z","timestamp":1763570058000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Bilateral Foveal Cysts in Mucopolysaccharidosis Type I (Hurler Syndrome): Response to Acetazolamide With Insights From Multimodal Retinal Imaging and Electrophysiology"],"prefix":"10.1155","volume":"2025","author":[{"given":"Rashmi","family":"Lobo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmed","family":"Al-Hinai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0241-0209","authenticated-orcid":false,"given":"Aisha","family":"Al Busaidi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,19]]},"reference":[{"key":"e_1_2_12_1_2","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/8866837"},{"key":"e_1_2_12_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2005.11.007"},{"key":"e_1_2_12_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-9071.2010.02366.x"},{"key":"e_1_2_12_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2024.11.013"},{"key":"e_1_2_12_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-015-2953-y"},{"key":"e_1_2_12_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2015.01.015"},{"key":"e_1_2_12_7_2","doi-asserted-by":"publisher","DOI":"10.1080\/13816810.2022.2103836"},{"key":"e_1_2_12_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/s0161-6420(86)33537-1"},{"key":"e_1_2_12_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajoc.2017.10.006"},{"key":"e_1_2_12_10_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0079447"},{"key":"e_1_2_12_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2017.09.009"},{"key":"e_1_2_12_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004170050175"},{"key":"e_1_2_12_13_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-015-3058-3"},{"key":"e_1_2_12_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0039-6257(02)00294-1"}],"container-title":["Case Reports in Ophthalmological Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crop\/5349202","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/crop\/5349202","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crop\/5349202","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T18:59:17Z","timestamp":1773773957000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/crop\/5349202"}},"subtitle":[],"editor":[{"given":"Stephen G.","family":"Schwartz","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":14,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1155\/crop\/5349202"],"URL":"https:\/\/doi.org\/10.1155\/crop\/5349202","archive":["Portico"],"relation":{},"ISSN":["2090-6722","2090-6730"],"issn-type":[{"value":"2090-6722","type":"print"},{"value":"2090-6730","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]},"assertion":[{"value":"2025-07-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-09-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"5349202"}}