title: Porphyria: varied ocular manifestations and management.
abstract: On review of past 10 years medical records, we could find four typical cases of porphyria with rare ocular manifestations. Cases 1, 2 and 4 have presented with features suggestive of acute scleritis. Based on clinical, biochemical and dermatological evaluation, all these three cases were diagnosed to have congenital erythropoietic porphyria. Case 1 was initially managed with scleral patch graft which on subsequent melt was managed with double layered amniotic membrane grafting along with conjunctival advancement and lateral paramedian tarsorrhaphy in both the eyes. Cases 2 and 4 were managed conservatively with artificial tear drops and general protective measures. Case 3 was presented with multiple failed grafts due to repeated ulceration and infection. Owing to multiple failed grafts, Boston keratoprosthesis was done and the patient is doing well with stable kertaoprosthesis at the last follow-up visit.
authors.affiliation: Eye Department, Command Hospital (EC), Kolkata, West Bengal, India.
authors.forename: Alok
authors.initials: A
authors.lastname: Sati
authors.forename: Virender S
authors.initials: VS
authors.lastname: Sangwan
authors.forename: Sayan
authors.initials: S
authors.lastname: Basu
mesh_terms: D000293:Adolescent; D000328:Adult; D000650:Amnion / Q000637:transplantation; D015817:Eye Infections / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D017119:Porphyria Cutanea Tarda / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D017092:Porphyria, Erythropoietic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D019736:Prostheses and Implants; D012590:Sclera / Q000473:pathology*; D015423:Scleritis / Q000175:diagnosis* / Q000209:etiology