Boston keratoprosthesis for visual rehabilitation in porphyria cutanea tarda

Abstract

A 56-year-old man, presented with bilateral chronic visual loss associated with generalised skin lesions. He had undergone multiple penetrating keratoplasties in his right eye for recurrent corneal infections and perforations. On ocular examination, his left eye was phthisical and his right eye had light perception vision owing to a failed and vascularised corneal graft. Dermatological evaluation revealed multiple hyperpigmented and hypopigmented lesions along with thickening of skin on nose, scalp and dorsum of hands. Skin biopsy showed focal areas of deposition of faint periodic acid Schiff-positive diastase-resistant perivascular material. The high-performance liquid chromatography assessment revealed increased presence of porphyrins in blood and urine, thus confirming a diagnosis of porphyria cutanea tarda. The patient's vision in the right eye improved after undergoing Boston type 1 keratoprosthesis along with general photoprotective measures for the exposed parts of the body.

Ocular manifestations in patient with congenital erythropoietic porphyria

“…However, we took indirect evidence from the typical clinical presentations in previously reported cases and tear studies to establish our diagnosis. [345810] Such atypical presentations of CEP can mimic other inflammatory causes of scleritis, thereby demanding careful history taking and examination. It must be understood that unlike conventional thinking, these patients may progress to corneal perforation, especially in the presence of other risk factors.…”

“…[12] However, sight-threatening complications, like keratolysis, leading to total corneal melt in the presence of other risk factors have rarely been reported. [345] We report a case of a woman diagnosed with CEP, presenting a serious variant of ocular disease.…”

Complications Oculaires De La Porphyrie Cutanee Tardive : A Propos D’un Casocular Complications in Porphyria Cutanea Tarda : A Case Report .

“…La prise en charge des manifestations cornéennes dépend aussi du type d'atteinte: greffe de membrane amniotique en cas d'ulcère creusant ou présentant un retard de cicatrisation, colle cyano-acrylate, voire parfois greffe de cornée transfixiante en urgence en cas de perforation de grand diamètre. Plus récemment, certaines équipes ont proposé la mise en place de kératoprothèses, avec des résultats à moyen et long terme très encourageants (9).…”