J Korean Ophthalmol Soc.  2016 Dec;57(12):1953-1957. 10.3341/jkos.2016.57.12.1953.

A Case of Ectrodactyly-ectodermal Dysplasia-cleft Syndrome with Bilateral Epiphora

Affiliations
  • 1Department of Ophthalmology, CHA Bundang Medical Center, CHA University, Seongnam, Korea. eye@cha.ac.kr

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 "ňúregurgitation without pus' and Jones test showed "ňúnegative' 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.

Keyword

Ectrodactyly-ectodermal dysplasia-cleft syndrome (EEC) syndrome; Epiphora; Nasolacrimal duct obstruction

MeSH Terms

Adolescent
Dacryocystorhinostomy
Humans
Lacrimal Apparatus
Lacrimal Apparatus Diseases*
Male
Nasolacrimal Duct
Silicon
Silicones
Silicon
Silicones
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