International Abstracts of Pediatric Surgery

Abstract

Congenital plasminogen deficiency is a rare disorder that usually becomes symptomatic as ligneous conjunctivitis. However, pseudomembranous lesions in the pharyngeal mucosa, the tracheobronchial tree, and the peritoneum may also occur. An accompanying hydrocephalus is extremely rare, with only 16 cases reported in the literature. The existing reports indicate that hydrocephalus, even treated by ventriculo-peritoneal (VP) cerebrospinal fluid shunting, worsens the prognosis substantially. Thus, VP cerebrospinal fluid shunting does not seem the optimal therapy for hydrocephalic children with plasminogen deficiency. The authors are adding 2 cases to the literature and, on the basis of their experience, propose a management strategy for the hydrocephalus. Both children were initially treated with VP shunts and had a very similar clinical course with multiple shunt malfunctions because of nonabsorption by the peritoneum. In the first child, the attempt to treat the hydrocephalus with a ventriculoatrial shunt failed because of catheter thrombosis. Finally, a ventriculo-cholecystic shunt was placed in both children. Thus, in patients with plasminogen deficiency and associated hydrocephalus, special care must be taken. The absorptive capacity of the peritoneum is reduced by pseudomembrane formation, which results in shunt malfunction. The plasminogen deficiency also results in early thrombus formation if atrial catheters are used. Therefore, it is recommended that ventriculo-cholecystic shunting should be considered early in the course of the disease.