title: Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.
abstract: Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case.
authors.affiliation: Department of ENT, R. G. Kar Medical College, Kolkata, West Bengal, India.
authors.forename: Debangshu
authors.initials: D
authors.lastname: Ghosh
authors.forename: Somnath
authors.initials: S
authors.lastname: Saha
authors.forename: Sumit Kumar
authors.initials: SK
authors.lastname: Basu
mesh_terms: D000293:Adolescent; D002971:Cleft Lip / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D002972:Cleft Palate / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D003608:Dacryocystorhinostomy; D004476:Ectodermal Dysplasia / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D005402:Fistula / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007767:Lacrimal Duct Obstruction / Q000151:congenital* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D009301:Nasolacrimal Duct / Q000002:abnormalities*