Ligneous Conjunctivitis: A Challenging Case Of Recurrent Membrane Formation And Treatment Strategies

Ligneous conjunctivitis (LC) is a rare, chronic, recurrent condition characterized by the formation of fibrin-rich (pseudo)membranes on the conjunctiva, which can significantly impair vision and lead to long-term ocular complications. It is caused by a deficiency in plasminogen. LC typically presents with chronic conjunctivitis, and untreated cases can lead to corneal involvement and potential visual loss. Treatment for LC remains challenging due to the lack of a standardized protocol and the high rate of recurrence following treatment. This case report aims to detail the clinical course, treatment strategies, and outcomes of a patient with LC, highlighting the difficulties in managing this rare condition.

This case report was conducted at the Department of Ophthalmology, Antwerp University Hospital, Edegem, Belgium, within a tertiary care setting for pediatric and corneal ocular diseases.

A two-year-old female was referred for chronic bilateral conjunctivitis with flare-ups since the young age of one. The left eye was predominantly affected. The patient had a medical history of hydrocephalus, respiratory issues, and a family history of consanguinity. Examinations revealed mechanical ptosis, swollen upper eyelids, and fibrous pseudomembranes on the tarsal conjunctiva. Histopathological analysis and plasminogen testing confirmed the diagnosis of ligneous conjunctivitis. Genetic testing revealed a likely pathogenic variant in the PLG gene. Several surgical excisions were performed, followed by different intensive topical treatments. Despite multiple surgeries, recurrences were observed. A total of six surgeries were required, with varying success in preventing recurrence.

This case illustrates the challenges of managing ligneous conjunctivitis that requires a tailored, often trial-and-error approach to treatment. Despite surgical excision and intensive topical therapy, recurrence remains common. Early diagnosis and treatment are crucial to prevent permanent ocular damage and to preserve vision. In this case, a combination of surgical excision, topical therapies (including 50% and 20% heterologous serum, ciclosporin, and corticosteroids), and fresh frozen plasma infusion helped manage the disease, although recurrences still occurred. The treatment strategy was adjusted according to the patient's response, and longer intervals between surgeries were possible due to ongoing therapy.