Bilateral congenital agenesis of lacrimal glands in a Tunisian family

Abstract

Background: Chronic corneal ulcers still pose etiological and therapeutic challenge. They are serious complications and often associated with poor functional prognosis.
Aim: We report the case of a patient with bilateral and chronic corneal ulcer revealing a rare   familial   form   of   bilateral   agenesis   of   the   lacrimal   gland.
Cases report: A 39-year-old man was referred to our department for bilateralchronic and sterile ulcer. The tear break-up time was less than one second and the schirmer test detected no wetting in 5 minutes. He mentioned that lacrimation had been totally absent even when crying as was the case of his brother and his sister. Orbital echography showed absence of lacrimal gland. Orbital magnetic resonance imaging revealed absence of both lacrimal glands. Our patients were treated with permanent topical artificial tears. We performed also permanent occlusion of lower lachrymal poncti to preserve basic tear flow.
Conclusion: Congenital lacrimal gland agenesis is rare. We report, to ourknowledge, the first case of Tunisian family with three patients suffering from bilateral lacrimal gland agenesis and the first documented familiarly cases diagnosed in adulthood.