Plasminogen deficiency, a rare autosomal recessive disorder, is classified as type I (hypoplasminogenemia) or type II (dysplasminogenemia). Hypoplasminogenemia is characterized by impaired wound healing while ligneous conjunctivitis (LC) is its main manifestation presenting with redness of the conjunctivae and progression to pseudomembranes' formation on the palpebral surfaces. A 4-year-old girl with LC in her left eye and impaired vision was referred to our unit. The conjunctival membranes had been already excised twice, followed by recurrences. Soon after the third recurrence, a probable diagnosis of LC was suggested, confirmed by a reduced plasminogen activity at 20% (normal values 80–120%). Both of her parents have slightly reduced plasminogen levels (50–60%) without any relevant symptom. Fresh frozen plasma (FFP) was administered systemically and topically, initiating 2 days before surgical removal of pseudomembranes with electrocautery under general anaesthesia. Systemic FFP was administered for 12 days postoperatively, along with topical use; the later was continued thereafter for 3 months. No recurrence was noticed. The vision was improved. Two weeks after cessation of the topical treatment, pseudomembranes reappeared. Topical application of FFP was reinitiated soon thereafter, and the girl underwent a second operation to have the conjunctival pseudomembranes removed. The perioperative therapeutic management was as previously described. Systemic treatment was stopped at the end of the tenth day while topical application of FFP was being continued until now, 10 months postoperatively. No recurrence has been observed and the vision remains at 9/10. Conclusion: Since surgical excision of the conjunctival pseudomembranes alone in patients with LC does not protect from recurrences, the perioperative administration of FFP, both systemically and topically improves the outcome. Furthermore, the long-term application of topical FFP preparations seems to prevent recurrences and has a protective effect on the vision of these patients.
This is a preview of subscription content, log in to check access.
Buy single article
Instant access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Borel MG (1933) Un nouveau syndrome palpebral. Bull Soc Fr Ophthalmol 46:168–180
Bouisson M (1847) Ophthalmie sur-aigue avec formation de pseudomembranes a la surface de la conjoctive. Ann Ocul 17:100–104
Caputo R, Pucci N, Mori F, Secci J, Novembre E, Frosini R (2008) Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops. Thromb Haemost 100:1196–1198
Gürlü VP, Demir M, Alimgil ML, Erda S (2008) Systemic and topical fresh-frozen plasma treatment in a newborn with ligneous conjunctivitis. Cornea 27(4):501–503
Lijό Pavia J (1924) Tumor inflammatorio fungoso recidivante de la conjunctiva palpebral. Semana Med 31:326–331
Mehta R, Shapiro AD (2008) Plasminogen deficiency. Haemophilia 14:1261–1268
Mirshahi S, Soria J, Nelles I et al (1996) Plasminogen activators in human corneal fibroblasts: secretion, cellular localization and regulation. Fibrinolysis Proteolysis 10:255–262
Schott D, Dempfle C-E, Beck P, Liermann A, Mohr-Pennert A, Goldner M et al (1998) Therapy with purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency. N Engl J Med 339(23):1679–1686
Schuster V, Hügle B, Tefts K (2007) Plasminogen deficieny. J Thromb Haemost 5:2315–2322
Schuster V, Seregard S (2003) Ligneous conjunctivitis. Surv Ophthalmol 48:369–388
Tabbara KF (2004) Prevention of ligneous conjunctivitis by topical and subconjunctival fresh frozen plasma. Am J Ophthalmol 138(2):299–300
The authors stated that they have no interests which might be perceived as posing a conflict or bias.
About this article
Cite this article
Pergantou, H., Likaki, D., Fotopoulou, M. et al. Management of ligneous conjunctivitis in a child with plasminogen deficiency. Eur J Pediatr 170, 1333–1336 (2011). https://doi.org/10.1007/s00431-011-1483-9
- Ligneous conjunctivitis
- Plasminogen deficiency