Long-Term Clinical And Visual Outcomes In Ligneous Conjunctivitis: A 10-Year Retrospective Case Series

Ligneous conjunctivitis is a rare, chronic ocular surface disorder associated with plasminogen deficiency. Because of its low prevalence, most published data are limited to case reports or short-term follow-up studies. We aimed to evaluate the long-term clinical course, surgical burden, and visual outcomes of patients with plasminogen deficiency–associated ligneous conjunctivitis followed for approximately 10 years at a tertiary referral center.

Department of Ophthalmology, Cerrahpaşa Medical Faculty, Istanbul University-Cerrahpaşa, Istanbul, Turkey.

This retrospective case series included 15 patients with plasminogen deficiency–associated ligneous conjunctivitis managed in collaboration with the Department of Pediatric Hematology. Demographics, parental consanguinity, sibling history, follow-up duration, surgical excision history, and extraocular involvement were recorded. Ophthalmic evaluation included best-corrected visual acuity (logMAR), slit-lamp examination, fundus examination, and tear film assessment with tear break-up time (TBUT) and Schirmer testing. Long-term clinical course and associations between surgical burden and visual outcomes were analyzed. Statistical analyses were performed using appropriate nonparametric tests, with p<0.05 considered significant.

All patients demonstrated bilateral ocular involvement, often asymmetric. Recurrent membranes required repeated excisions in 92.3%. Excision burden showed a moderate negative correlation with logMAR visual acuity (Spearman r=−0.45, p=0.15). Mean worse-eye BCVA was 0.70±0.26. Severe visual loss occurred in three eyes (two hand motion, one light perception). Corneal vascularization and stromal haze were common. Tear film instability was evident with reduced TBUT (4.7±1.1 s). Extraocular involvement occurred in 53.8%, mainly gingival. Parental consanguinity was present in 46.2% and affected siblings in 53.8%. Active plasminogen replacement therapy showed stable vision with a trend toward fewer recurrences, without statistical significance.

Plasminogen deficiency–associated ligneous conjunctivitis demonstrated a chronic, relapsing course with substantial surgical burden and potential for severe visual impairment. Although overall visual acuity was relatively preserved, a moderate association between excision burden and visual decline was observed. The high rates of consanguinity and affected siblings underscore the strong genetic background of the disease. Early recognition and multidisciplinary management remain essential to limit progressive ocular surface damage and long-term visual morbidity.