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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This article describes the detailed ocular pathology found in a premature neonate, born at 34 weeks, with multiple congenital anomalies resulting from de novo trisomy 1q32-qter. The ocular defects include goniodysgenesis, persistent tunica vasculosa lentis and hyaloid vessels, hypopigmentation of the posterior iris epithelium, ectopia of the ciliary processes, and abnormal insertion of the ciliary muscle and cataract. This is the first report of the detailed ocular pathology in a case of trisomy 1q and is also unusual in that the chromosomal defect has apparently arisen de novo in the proband.
J Pediatr Ophthalmol Strabismus
PMID:Congenital ocular defects associated with an abnormality of the human chromosome 1: trisomy 1q32-qter. 819 62

A 10-year-old boy with clinically confirmed congenital pupillary-iris-lens membrane with goniodysgenesis underwent light microscopic examination of the enucleated eye. The anterior segment changes consisted of the absence of the iridial pupillary sphincter muscle and dilator muscle processes. Endothelialization and descemetization of the anterior chamber angle and the anterior surface of the iris covered the original eccentric pupillary opening (occlusio pupillae), but grew around the fibrotic edges of the surgically created, patent pseudopupil, probably secondary responses to surgery. The anterior chamber findings in this case establish a localized syndrome that includes absence of the pupillary sphincter and dilator muscle processes. Incomplete development of the iris may be partly attributable to an abnormality of stromal development and inductions by the anterior neuroectodermal layer, together with anomalies of the pupillo-iridial vasculature.
J Pediatr Ophthalmol Strabismus
PMID:Congenital pupillary-iris-lens membrane with goniodysgenesis: histopathologic findings in an enucleated eye. 2050 3

The authors present a case of congenital pupillary-iris-lens membrane with goniodysgenesis, a unilateral, sporadic, and congenital anomaly with the presence of a vascularized pupillary membrane on the iris surface that further adheres to the posterior embryotoxon. Early diagnosis and treatment is vital to prevent complications such as amblyopia and glaucoma.
J Pediatr Ophthalmol Strabismus 2010 May 21
PMID:Another case of congenital pupillary-iris-lens membrane with goniodysgenesis. 2121 49