Conjunctivitis lignea: komplikace vrozeného deficitu plazminogenu (kazuistika)

Ligneous Conjunctivitis: Complication of Inborn Plasminogen Deficiency (a Case Report)

Authors refer about detailed analysis of ten years follow up of a child patient with ligneous conjunctivitis. They document presence of all typical eye findings (especially recurrent formation of granulomatous pseudotumors in affected mucous membrane) related to this rare pseudomembranous conjunctivitis. The clinical picture of the disease includes plasminogen deficiency, a factor newly considered as primary cause of the disease. The diagnosis was confirmed histologicaly by repeated probatory excisions. Pseudomembranous inflammation with mixed inflammatory infiltrate containing large amount of elements of chronical as well as acute inflammatory reaction is typical. Granuloma with the accumulation of the PASpositive amorphous matrix with high content of the fibrin and with the network of newly formed vessels is the component of the inflammatory picture. The treatment was based on successively discovered knowledge of the possible etiopathogenesis of the disease. Only the ninth, last surgical procedure induced longer-term remission, which lasts sixteen months until now. The granuloma excision was combined with the mitomicin application on the wound surface and a conjunctivoplasty. During the postoperative period, the ointment with heparin, corticosteroid and antibiotic was applied. The purpose of the mitomicin use is to slow down the fibroproliferative reparative reaction. Covering of the wound surface decreases the number of microtraumas on the uneven postoperative scleral surface. Heparin in the interstitial tissue of the wound surface blocks the conversion of fibrinogen to fibrin.

Key words:
ligneous conjunctivitis, plasminogen, heparin, mitomycin C, aberrant healing, pseudomembranous inflammation