Uncontrolled corneal melting in a Xeroderma pigmentosum patient

Session Details

Session Title: Presented Poster Session: Surgical Cornea

Venue: Poster Village: Pod 2

First Author: : M.Garat Chifflet SPAIN

Co Author(s): :    D. Ortiz-Vaquerizas                       

Abstract Details

Purpose:

To describe the evolution of a Xeroderma Pigmentosum (XP) patient who was operated on for a symblepharon and a severe pseudopterygium. We describe the surgical techniques used to control the corneal melting that caused three corneal perforations in a period of two months.

Setting:

Xeroderma is a rare, hereditary, autosomal, recessive disorder with photo hypersensitivity of tissues exposed to the sun and a several-fold increased risk of malignant changes. XP results in an impaired ability to repair UV-induced DNA damage, and ocular involvement is very frequent.

Methods:

A 50-year-old man with decreased visual acuity came to our clinic with symblefaron and severe pseudopterygium in his right eye. We performed an extensive conjunctival dissection leaving a slimed corneal residual bed (300u) and we covered the surface with an amniotic membrane graft. Two weeks later he came back with a spontaneous corneal perforation and we covered it with a scleral patch. Three weeks later he came in with a new, spontaneous perforation in the upper periphery. We performed another scleral graft but two weeks later, a third corneal perforation appeared in the lower graft, secondary to an important melting.

Results:

Due to the failure of the scleral graft, we decided to perform a partial lamellar keratoplasty on the lower half of the cornea with an extensive tarsorrhaphy. We added a temporary, systemic, immunosuppressive treatment with mycophenolate, 720mg/12h. Two weeks later he had another central corneal melting. Fortunately, the vascularization and epithelization prevented a new perforation. The small upper scleral graft epithelized and repaired the upper perforation perfectly.

Conclusions:

With XP patients, one has to avoid touching the surface unless absolutely necessary. If one decides to intervene, it is important to consider performing an extensive tarsorrhaphy. The scleral graft can be a good option for small peripheral corneal perforation but it will fail if the epithelization is incomplete. Finally, the immunosuppression should be for a limited time only, because it can increase the risk of neoplasms in this kind of patients.

Financial Disclosure:

None