Congenital Hydrocephalus as a Rare Association with Ligneous Conjunctivitis and Type I Plasminogen Deficiency

Abstract

Severe type I plasminogen deficiency is the underlying cause of ligneous conjunctivitis (LC). Furthermore, pseudomembranes may also be found on other mucous membranes (gastrointestinal tract, bronchial system, genital tract). In very rare cases, congenital hydrocephalus has been associated with the more severe forms of the disease and may even precede LC. The pathophysiological mechanism is unclear at present. It is advisable to look for plasminogen deficiency in patients with congenital hydrocephalus, because obstruction of ventriculoperitoneal shunts is possible when such a condition is overlooked. Here, we report a case of LC with hydrocephalus. This report reemphasizes the association of LC with hydrocephalus which is not well known.

Key words

Ligneous conjunctivitis - type I plasminogen deficiency - congenital hydrocephalus

References

• 1 Chai F, Coates H. Otolaryngological manifestations of ligneous conjunctivitis.  Int J Pediatr Otorhinolaryngol. 2003;  67 189-194
• 2 Ciftci E, Ince E, Akar N, Dogru U, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency.  Eur J Pediatr. 2003;  162 462-465
• 3 Demarmels Biasiutti F, Sulzer I, Stucki B, Wuillemin W A, Furlan M. et al . Is plasminogen deficiency a thrombotic risk factor? A study on 23 thrombophilic patients and their family members.  Thromb Haemost. 1998;  80 167-170
• 4 Heinz C, Kremmer S, Externbrink P, Steuhl K P. Ligneous conjunctivitis in a patient with plasminogen type I deficiency-case report with review of literature.  Klin Monatsbl Augenheilkd. 2002;  219 156-158
• 5 Martinovic E, Ells A. Ligneous conjunctivitis related to a defect in the fibrinolytic system.  Can J Ophthalmol. 2001;  36 147-149
• 6 Ozcelik U, Akcoren Z, Anadol D, Kiper N, Orhon M, Gocmen A. et al . Pulmonary involvement in a child with ligneous conjunctivitis and homozygous type I plasminogen deficiency.  Pediatr Pulmonol. 2001;  32 179-183
• 7 Schott D, Dempfle C E, Beck P, Liermann A, Mohr-Pennert A, Goldner M. et al . Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency.  N Engl J Med. 1998;  339 1679-1686
• 8 Schuster V, Mingers A M, Seidenspinner S, Nussgens Z, Pukrop T, Kreth H W. Homozygous mutations in the plasminogen gene of two unrelated girls with ligneous conjunctivitis.  Blood. 1997;  90 958-966
• 9 Schuster V, Zeitler P, Seregard S, Ozcelik U, Anadol D, Luchtman-Jones L. et al . Homozygous and compound-heterozygous type I plasminogen deficiency is a common cause of ligneous conjunctivitis.  Thromb Haemost. 2001;  85 1004-1010
• 10 Schuster V, Seregard S. Ligneous conjunctivitis.  Surv Ophthalmol. 2003;  48 369-388
• 11 Watts P, Suresh P, Mezer E, Ells A, Albisetti M, Bajzar L, Marzinotto V. et al . Effective treatment of ligneous conjunctivitis with topical plasminogen.  Am J Ophthalmol. 2002;  133 451-455