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Persistent Hydrocephalus, Shunt, and Subglottic Stenosis in a Newborn with Plasminogen Deficiency due to Delayed
Benedikt Stephan Biesinger1, Christoph Male2, Veronika Kranebitter3
1Division of Neonatology, Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics, Intensive Care and Neuropediatrics, Medical University of Vienna, Vienna, Austria, dr.benedikt.biesinger@gmail.com.
Insights
Severe plasminogen deficiency in a newborn caused hydrocephalus and ligneous conjunctivitis. Plasminogen concentrate therapy successfully treated complications, improving shunt function and airway issues.
Area of Science:
- Biochemistry
- Pediatrics
- Medical Genetics
Background:
- Severe plasminogen deficiency is a rare genetic disorder.
- It can lead to serious complications such as hydrocephalus and ligneous conjunctivitis.
- Current treatment options are limited and often insufficient.
Observation:
- A newborn male presented with severe plasminogen deficiency, occlusive hydrocephalus, and ligneous conjunctivitis.
- The patient experienced shunt obstruction and subglottic stenosis requiring multiple interventions.
- Initial plasma transfusions provided only temporary improvement.
Findings:
- Treatment with plasminogen concentrate resolved respiratory problems and enabled successful ventriculoperitoneal shunt implantation.
- Plasminogen concentrate therapy offers a promising alternative for managing severe plasminogen deficiency.
- This case highlights the efficacy of plasminogen concentrates in preventing and treating complications.
Implications:
- Plasminogen concentrates are crucial for improving patient care globally.
- Wider availability of plasminogen concentrates can prevent severe outcomes in plasminogen deficiency.
- This treatment can significantly reduce morbidity and mortality associated with this rare condition.
Introduction:
Here we present the case of a newborn baby boy with severe plasminogen deficiency causing occlusive hydrocephalus and ligneous conjunctivitis.
Case Presentation:
Shortly after birth, the hydrocephalus was treated with a ventriculoperitoneal shunt implantation. However, the child had to be readmitted soon afterward because of shunt obstruction. Subglottic microtrauma caused by the necessary intubations then led to another life-threatening complication - subglottic stenosis with pseudomembrane formation. 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 ventriculoperitoneal shunt was successful, and respiratory problems resolved.
Conclusion:
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.
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