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Posterior embryotoxon may not be a forme fruste of Axenfeld-Rieger's Syndrome.
J AAPOS. 2004 Oct; 8(5):504-6.JA

Abstract

Axenfeld-Rieger Syndrome is a disorder of morphogenesis which is autosomal dominantly inherited. One of the main ocular features associated with Axenfeld-Rieger is posterior embryotoxon, which is a prominent anteriorly displaced Schwalbe's line. This can be found in up to 15% of normal eyes, without any clinical significance or may represent a forme fruste of an anterior segment dysgenesis. Many large case series studies of Axenfeld-Rieger had used the presence of posterior embryotoxon with iris strands extending from the peripheral iris to Schwalbe's line, as inclusion criterion for this condition. We report a case of Axenfeld-Rieger syndrome without posterior embryotoxon and the abnormalities of the angle, but with other features to support the diagnosis of Axenfeld-Rieger Syndrome.

Authors+Show Affiliations

St Paul's Eye Unit, Royal Liverpool University Hospital, UK.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15492748

Citation

Sim, K T., et al. "Posterior Embryotoxon May Not Be a Forme Fruste of Axenfeld-Rieger's Syndrome." Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus, vol. 8, no. 5, 2004, pp. 504-6.
Sim KT, Karri B, Kaye SB. Posterior embryotoxon may not be a forme fruste of Axenfeld-Rieger's Syndrome. J AAPOS. 2004;8(5):504-6.
Sim, K. T., Karri, B., & Kaye, S. B. (2004). Posterior embryotoxon may not be a forme fruste of Axenfeld-Rieger's Syndrome. Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus, 8(5), 504-6.
Sim KT, Karri B, Kaye SB. Posterior Embryotoxon May Not Be a Forme Fruste of Axenfeld-Rieger's Syndrome. J AAPOS. 2004;8(5):504-6. PubMed PMID: 15492748.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Posterior embryotoxon may not be a forme fruste of Axenfeld-Rieger's Syndrome. AU - Sim,K T, AU - Karri,Bhavani, AU - Kaye,Stephen B, PY - 2004/10/20/pubmed PY - 2004/12/16/medline PY - 2004/10/20/entrez SP - 504 EP - 6 JF - Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus JO - J AAPOS VL - 8 IS - 5 N2 - Axenfeld-Rieger Syndrome is a disorder of morphogenesis which is autosomal dominantly inherited. One of the main ocular features associated with Axenfeld-Rieger is posterior embryotoxon, which is a prominent anteriorly displaced Schwalbe's line. This can be found in up to 15% of normal eyes, without any clinical significance or may represent a forme fruste of an anterior segment dysgenesis. Many large case series studies of Axenfeld-Rieger had used the presence of posterior embryotoxon with iris strands extending from the peripheral iris to Schwalbe's line, as inclusion criterion for this condition. We report a case of Axenfeld-Rieger syndrome without posterior embryotoxon and the abnormalities of the angle, but with other features to support the diagnosis of Axenfeld-Rieger Syndrome. SN - 1091-8531 UR - https://www.unboundmedicine.com/medline/citation/15492748/Posterior_embryotoxon_may_not_be_a_forme_fruste_of_Axenfeld_Rieger's_Syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S109185310400134X DB - PRIME DP - Unbound Medicine ER -