Surgical Outcome In Ligneous Conjunctivitis Due To Plasminogen Deficiency: A Case Report

Ligneous conjunctivitis is a rare form of chronic conjunctivitis associated with systemic type-1 plasminogen deficiency. Lack of plasminogen, a precursor of plasmin in the fibrinolytic pathway, results in chronic inflammation and formation of fibrin-rich thick pseudomembranes in palpebral conjunctiva often in close contact with cornea. In severe cases, recurrent corneal erosions and scarring may debilitate visual acuity. Surgical excision alone may result in rapid recurrence of the membranes, therefore a careful debridement in combination with proper medical treatment pre- and post-operatively is crucial for a long-term disease-free course.

University of Health Sciences, Beyoglu Eye Training and Research Hospital, Istanbul, Turkey

A 25-year-old Caucasian female with a history of Familial Mediterranean Fever (FMF) and Celiac disease (CD) was admitted due to pain and blurry vision in her right eye following formation of a pink membrane inside her upper eyelid. The best corrected visual acuity was 20/25 in the right and 20/20 in the left eye using the Snellen chart. Anterior segment examination revealed a protruding wood-like pseudomembrane in the upper palpebral conjunctiva of the right eye. A superiorly located 1mm x 1mm epithelial corneal ulcer and diffuse punctate epithelial erosions were noted. Rest of the exam was unremarkable. Her history was significant for previously failed debridement and medical treatment. Surgery and medical treatment was carefully planned.

Membranes were removed by gentle dissection using blunt forceps and no cauterization or suturing was used, opting for minimum microtrauma. Histopathological examination revealed subepithelial fibrin-rich eosinophilic periodic acid Schiff (PAS) positive hyaline material that was negative for amyloid staining with Congo Red.  The tissue showed inflammatory cellular infiltrate and was confirmed as ligneous conjunctivitis. Further investigations revealed type-1 hypoplasminogenemia (free plasminogen level: 31.7 mg/dL).  Topical heparin drops (1000 units/mL) hourly and human plasminogen drops were initiated. Six months postoperatively, the membranes and ocular surface disease still cease to exist with current treatment.

Ligneous conjunctivitis is a rare and recurring disease leading to permanent pathologies in ocular surface. Medical management alone may not be sufficient in cases with severe pseudomembrane formation. Surgery must be carried diligently without the use of sharp tools, cauterization or suturing to ensure a trauma-free environment, as inflammation triggers rapid recurrence. Furthermore, postoperative care should be meticulously continued with topical plasminogen and heparin in addition to anti-inflammatory treatment in hypoplasminogenemia for a disease-free course.