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Rieger's syndrome: (oligodontia and primary mesodermal dysgenesis of the iris) clinical features and report of an isolated case.
J Pediatr Ophthalmol Strabismus. 1978 Mar-Apr; 15(2):67-70.JP

Abstract

An 11-year-old female manifested bilateral prominent Schwalbe's line, iridal adhesions, displaced and irregular pupils. She had no evidence of juvenile glaucoma. The major nonocular finding in this patient was the congenital absence of her primary and permanent maxillary central and lateral incisors. Although missing or malformed teeth may occur in other conditions such as Down's syndrome, focal dermal hypoplasia syndrome, anhidrotic ectodermal dysplasia, chondroectodermal dysplasia, and incontinentia pigmenti, the clinician should include Rieger's syndrome in his differential diagnosis in patients with oligodontia in combination with malformation of the anterior chamber of the eye. Rieger's syndrome is a rare condition characterized by ocular and dental abnormalities, namely mesoectodermal dysgenesis of the iris and cornea of the eyes and hypodontia of the dentition. This report presents a sporadic case of Rieger's syndrome in an 11-year-old female and discusses the pertinent clinical features of this condition.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

105118

Citation

Wesley, R K., et al. "Rieger's Syndrome: (oligodontia and Primary Mesodermal Dysgenesis of the Iris) Clinical Features and Report of an Isolated Case." Journal of Pediatric Ophthalmology and Strabismus, vol. 15, no. 2, 1978, pp. 67-70.
Wesley RK, Baker JD, Golnick AL. Rieger's syndrome: (oligodontia and primary mesodermal dysgenesis of the iris) clinical features and report of an isolated case. J Pediatr Ophthalmol Strabismus. 1978;15(2):67-70.
Wesley, R. K., Baker, J. D., & Golnick, A. L. (1978). Rieger's syndrome: (oligodontia and primary mesodermal dysgenesis of the iris) clinical features and report of an isolated case. Journal of Pediatric Ophthalmology and Strabismus, 15(2), 67-70.
Wesley RK, Baker JD, Golnick AL. Rieger's Syndrome: (oligodontia and Primary Mesodermal Dysgenesis of the Iris) Clinical Features and Report of an Isolated Case. J Pediatr Ophthalmol Strabismus. 1978 Mar-Apr;15(2):67-70. PubMed PMID: 105118.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Rieger's syndrome: (oligodontia and primary mesodermal dysgenesis of the iris) clinical features and report of an isolated case. AU - Wesley,R K, AU - Baker,J D, AU - Golnick,A L, PY - 1978/3/1/pubmed PY - 1978/3/1/medline PY - 1978/3/1/entrez SP - 67 EP - 70 JF - Journal of pediatric ophthalmology and strabismus JO - J Pediatr Ophthalmol Strabismus VL - 15 IS - 2 N2 - An 11-year-old female manifested bilateral prominent Schwalbe's line, iridal adhesions, displaced and irregular pupils. She had no evidence of juvenile glaucoma. The major nonocular finding in this patient was the congenital absence of her primary and permanent maxillary central and lateral incisors. Although missing or malformed teeth may occur in other conditions such as Down's syndrome, focal dermal hypoplasia syndrome, anhidrotic ectodermal dysplasia, chondroectodermal dysplasia, and incontinentia pigmenti, the clinician should include Rieger's syndrome in his differential diagnosis in patients with oligodontia in combination with malformation of the anterior chamber of the eye. Rieger's syndrome is a rare condition characterized by ocular and dental abnormalities, namely mesoectodermal dysgenesis of the iris and cornea of the eyes and hypodontia of the dentition. This report presents a sporadic case of Rieger's syndrome in an 11-year-old female and discusses the pertinent clinical features of this condition. SN - 0191-3913 UR - https://www.unboundmedicine.com/medline/citation/105118/Rieger's_syndrome:__oligodontia_and_primary_mesodermal_dysgenesis_of_the_iris__clinical_features_and_report_of_an_isolated_case_ L2 - https://www.diseaseinfosearch.org/result/6329 DB - PRIME DP - Unbound Medicine ER -