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Severe corneal hydrops with good response to treatment in a patient with Leber congenital amaurosis and keratoglobus

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Session Details

Session Title: Presented Poster Session: Cornea III

Venue: Poster Village: Pod 2

First Author: : M.Srur SPAIN

Co Author(s): :    C. Carranza Neira   G. Garces Monsalve   S. Finianos Mansour   C. Gutierrez Gutierrez   R. Blanco Soler   B. Garcia-Valcarcel Gonzalez

Abstract Details


Leber Congenital Amaurosis (LCA) is an infrequent desease that affects 3 out of 100.000 Newborns. It’s a genetic desease of autosomal recessive inheritance that produces an interruption in the phototransducer cascade, leading to blindness at an early age. Franceschetti oculodigital sign is a characteristic but infrequent finding, thought to be associated to the development of Corneal Dystrophies. We want to present the rare case of an adult patient with severe corneal hydrops and his successful medical treatment, in the context of Keratoglobus (KTG) associated to LCA.


55 year old patient with known history of LCA and KTG, nystagmus of great amplitude and intense photophobia, light perception as basal visual acuity, who senses a growing painless lump in the left eye that deform the lower eyelid. Diagnosed as a Corneal Hydrops after biomicroscopic evaluation, we started treatment.


We investigated his medical history and performed a complete ophtalmologic study including Visual Acuity, Biomicrospcopy and Eye Fundoscopy, as well as photographs, and collected his previous medical research: Corneal Topography and molecular studies. We started treatment of the Hydrops with anti-edema eye drops and ointment, Combigan® and Maxidex®, and started biomicroscopic and photographic periodic follow up.


On his first visit the patient had a severe corneal hydrops that deformed the lower eyelid and extended through the lower third of the cornea. Conservative treatment was started with topical anti-edema, intraocular pressure regulators and corticosteroids, with good initial and sustained response during a four month period follow up, until almost complete resolution of the corneal decompensation in the last control.


We present the atipical case of an adult man with corneal decompensation in the context of an uncommon disease. The relation between corneal ectasia and posterior pole disease is known, as is the present case. The oculodigital sign (eye rubbing in order to produce mechanical retinal stimulation) is determinant. The severity of the corneal decompensation and the extraordinary response to medical treatment is atypical. We reinforce the need to opt for conservative meassures as a first aproximation in the management of severe corneal hydrops before aggressive invasive treatments that can lead to unwanted complications.

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