Treatment with plasma transfusion and plasma-derived human plasminogen in a newborn with plasminogen deficiency and recurrent hydrocephalus: A Case Report.

Introduction: Plasminogen deficiency is a congenital autosomal recessive disorder. Ligneous conjunctivitis is pathognomonic to the disease, however hydrocephalus is a rare complication.

Case presentation: A neonate with prenatal diagnosis of hydrocephalus associated with Dandy-Walker Syndrome was sent to our observation. Surgery was followed by multiple shunt malfunctions in the early postoperative period. An ophtalmological evaluation suggested the diagnosis of ligneous conjunctivitis. Laboratory tests lead to the diagnosis of a systemic PLGD. The disease caused the formation of multiple membranes, which prevented the proper functioning of the ventricular shunts. We started an alternated treatment with plasma transfusions and plasma-derived human plasminogen. This has allowed the control of the complications related to the treatment of the hydrocephalus.

Discussion: Ligneous conjunctivitis was pivotal in the diagnosis of pathology. It is currently difficult to get sufficient doses of Plasma-Derived Human Plasminogen for e.v. administration, considering that it is still not officially authorized in Europe.