{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:23:33Z","timestamp":1787840613044,"version":"build-2784847793"},"reference-count":14,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose:<\/jats:title>\n                    <jats:p>To describe a particular form of posterior microphthalmos pigmentary retinopathy syndrome (PMPRS) with an atypical clinical presentation of pigment retinal dystrophy and an association to an inconstant complication which is angle-closure glaucoma (ACG).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>A 40-year-old male patient with ACG on maximal topical treatment was referred to our department for uncontrolled intraocular pressure. Best-corrected visual acuity was 2\/10 in the right eye and light perception in the left eye. Intraocular pressure was 36 mmHg bilaterally. He had 360\u00b0 peripheral anterior synechiae on gonioscopy. Fundus examination revealed total cupping with pale retinal lesions in both eyes and a few pigment deposits in the midperiphery of the right eye. Multimodal imaging was done.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Fundus autofluorescence revealed patchy areas of hypoautofluorescence. Optical coherence tomography (OCT) showed bilateral foveoschisis and macular folds. Anterior segment OCT showed a circumferential iridocorneal angle closure. Axial length measured with ultrasound biomicroscopy was 18.4 mm in the right eye and 18.1 in the left eye. Electroretinogram revealed attenuated scotopic responses. The patient was diagnosed with nanophthalmos\u2013retinitis pigmentosa (RP)\u2013foveoschisis syndrome complicated with ACG. A combined surgery with phacoemulsification - anterior vitrectomy - intraocular lens implantation and trabeculectomy was performed in both eyes with a satisfactory outcome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>In its typical forms, PMPR syndrome is an association of nanophthalmos - RP - foveoschisis and optic nerve head (ONH) drusen. Incomplete phenotypes may lack ONH drusen or foveoschisis. Patients with PMPRS have to be screened for iridocorneal angle synechia and ACG.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/joco.joco_145_22","type":"journal-article","created":{"date-parts":[[2024,6,1]],"date-time":"2024-06-01T18:38:30Z","timestamp":1717267110000},"page":"474-477","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["Posterior Microphthalmos Pigmentary Retinopathy Syndrome with Angle-Closure Glaucoma"],"prefix":"10.4103","volume":"34","author":[{"given":"Hela","family":"Sassi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khaled","family":"Ammar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meriem","family":"Ouederni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monia","family":"Cheour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-15-20240910","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1007\/s10633-011-9266-1","article-title":"Posterior microphthalmos pigmentary retinopathy syndrome","volume":"122","author":"Pehere","year":"2011","journal-title":"Doc Ophthalmol"},{"key":"R2-15-20240910","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1080\/13816810.2019.1633547","article-title":"Posterior microphthalmos, retinitis pigmentosa, and foveoschisis caused by a mutation in the MFRP gene: A familial study","volume":"40","author":"Morillo","year":"2019","journal-title":"Ophthalmic Genet"},{"key":"R3-15-20240910","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/1471-2415-12-23","article-title":"Angle-closure glaucoma in a patient with the nanophthalmos-ocular cystinosis-foveoschisis-pigmentary retinal dystrophy complex","volume":"12","author":"Sonmez","year":"2012","journal-title":"BMC Ophthalmol"},{"key":"R4-15-20240910","doi-asserted-by":"crossref","first-page":"2735465","DOI":"10.1155\/2018\/2735465","article-title":"Nanophthalmos: A Review of the Clinical Spectrum and Genetics","volume":"2018","author":"Carricondo","year":"2018","journal-title":"J Ophthalmol"},{"key":"R5-15-20240910","first-page":"42","article-title":"[Associated microphthalmia: microphthalmia-pigmentary retinitis-glaucoma syndrome]","volume":"1","author":"Hermann","year":"1958","journal-title":"Bull Soc Ophtalmol Fr"},{"key":"R6-15-20240910","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1136\/bjo.69.8.624","article-title":"Bilateral nanophthalmos, pigmentary retinal dystrophy, and angle closure glaucoma \u2013 A new syndrome?","volume":"69","author":"Ghose","year":"1985","journal-title":"Br J Ophthalmol"},{"key":"R7-15-20240910","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1001\/archopht.1987.01060030086032","article-title":"Retinal degeneration with nanophthalmos, cystic macular degeneration, and angle closure glaucoma. A new recessive syndrome","volume":"105","author":"MacKay","year":"1987","journal-title":"Arch Ophthalmol"},{"key":"R8-15-20240910","first-page":"1483","article-title":"A new autosomal recessive syndrome consisting of posterior microphthalmos, retinitis pigmentosa, foveoschisis, and optic disc drusen is caused by a MFRP gene mutation","volume":"12","author":"Ayala-Ramirez","year":"2006","journal-title":"Mol Vis"},{"key":"R9-15-20240910","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/j.ajo.2008.04.029","article-title":"A novel mutation confirms MFRP as the gene causing the syndrome of nanophthalmos-renititis pigmentosa-foveoschisis-optic disk drusen","volume":"146","author":"Cresp\u00ed","year":"2008","journal-title":"Am J Ophthalmol"},{"key":"R10-15-20240910","first-page":"2623","article-title":"Membrane frizzled-related protein gene-related ophthalmological syndrome: 30-month follow-up of a sporadic case and review of genotype-phenotype correlation in the literature","volume":"18","author":"Neri","year":"2012","journal-title":"Mol Vis"},{"key":"R11-15-20240910","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1136\/bjophthalmol-2016-309376","article-title":"Retinitis pigmentosa-associated cystoid macular oedema: Pathogenesis and avenues of intervention","volume":"101","author":"Strong","year":"2017","journal-title":"Br J Ophthalmol"},{"key":"R12-15-20240910","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.ajoc.2018.08.002","article-title":"Prophylactic anterior vitrectomy during cataract surgery in eyes at increased risk for aqueous misdirection","volume":"12","author":"Thompson","year":"2018","journal-title":"Am J Ophthalmol Case Rep"},{"key":"R13-15-20240910","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1177\/1120672120971187","article-title":"Goniosynechialysis combined with multiple surgeries for secondary glaucoma in nanophthalmos: A case report","volume":"32","author":"Li","year":"2022","journal-title":"Eur J Ophthalmol"},{"key":"R14-15-20240910","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/s13643-022-01889-5","article-title":"Prevalence of complications in eyes with nanophthalmos or microphthalmos: Protocol for a systematic review and meta-analysis","volume":"11","author":"Ally","year":"2022","journal-title":"Syst Rev"}],"container-title":["Journal of Current Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/joco.joco_145_22","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T13:25:54Z","timestamp":1787837154000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/joco.joco_145_22"}},"subtitle":["A Case Report"],"short-title":[],"issued":{"date-parts":[[2022,10]]},"references-count":14,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/joco.joco_145_22","relation":{},"ISSN":["2452-2325"],"issn-type":[{"value":"2452-2325","type":"print"}],"subject":[],"published":{"date-parts":[[2022,10]]}}}