Supplementary Material for: Persistent Hydrocephalus, Shunt and Subglottic Stenosis in a Newborn with Plasminogen Deficiency due to Delayed Treatment with Plasminogen Concentrates - a case report

Here we present the case of a newborn baby boy with severe plasminogen deficiency (PD) causing occlusive hydrocephalus and ligneous conjunctivitis. Shortly after birth, the hydrocephalus was treated with a ventriculo-peritoneal (VP) shunt implantation. However, the child had to be readmitted soon afterwards because of shunt obstruction. Tracheal microtrauma caused by the necessary intubations, then led to another life-threatening complication - subglottic pseudomembrane formation and obstruction. Microsurgical removal had to be performed to secure the airway. Initially, regular plasma transfusions achieved slightly elevated plasminogen activity levels and short-term improvement of the respiratory situation. However, shunt dysfunction reoccurred, and alternative treatment options were needed. . Since therapy with plasminogen concentrate is already available in the USA with encouraging results, this treatment option was organized in hopes of equally good results for this patient. Fortunately, under short term substitution with plasminogen concentrates, the implantation of a new VP shunt was successful and respiratory problems resolved. Plasminogen concentrates are critically needed in Europe and other parts of the world to improve the care of and prevent complications among patients with plasminogen deficiency.