A Case of Ectrodactyly-ectodermal Dysplasia-cleft Syndrome with Bilateral Epiphora.
10.3341/jkos.2016.57.12.1953
- Author:
Sang Min LEE
1
;
Jong Seo PARK
;
Helen LEW
Author Information
1. Department of Ophthalmology, CHA Bundang Medical Center, CHA University, Seongnam, Korea. eye@cha.ac.kr
- Publication Type:Case Report
- Keywords:
Ectrodactyly-ectodermal dysplasia-cleft syndrome (EEC) syndrome;
Epiphora;
Nasolacrimal duct obstruction
- MeSH:
Adolescent;
Dacryocystorhinostomy;
Humans;
Lacrimal Apparatus;
Lacrimal Apparatus Diseases*;
Male;
Nasolacrimal Duct;
Silicon;
Silicones
- From:Journal of the Korean Ophthalmological Society
2016;57(12):1953-1957
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: In the present study, a case of recurrent nasolacrimal duct obstruction as ectrodactyly-ectodermal dysplasia-cleft syndrome is reported. CASE SUMMARY: An 18-year-old male complained of epiphora in both eyes. By the age of 1, he was diagnosed with nasolacrimal duct obstruction and received left side dacryocystotomy, both sides silicone tube insertion and, right side endoscopic dacryocystorhinostomy. The general findings showed microdontia and, bilateral ectrodactyly. An irrigation test showed ‘regurgitation without pus’ and Jones test showed ‘negative’ in both sides. Complete obstruction was observed on dacryocystography and the patient underwent endoscopic conjunctivodacryocystorhinostomy with Jones tube at right side and endoscopic dacryocystorhinostomy at left side. The chromosome test showed normal findings. CONCLUSIONS: Nasolacrimal duct obstruction in ectrodactyly-ectodermal dysplasia-cleft syndrome is usually caused by dysplasia of the nasolacrimal duct and accompanied by dysplasia of lacrimal punctum and canaliculus. Providing proper care for nasolacrimal duct obstruction in ectrodactyly-ectodermal dysplasia-cleft syndrome is important. Furthermore, the high failure rate should be considered.
