Article
Long-term outcome of hydrocephalus due to plasminogen deficiency type I treated with ventriculo-biliary CSF diversion
Langzeitergebnis bei Hydrocephalus verursacht durch einen Plasminogen-Mangel Typ I mit ventrikulo-biliärer Liquorableitung
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Published: | May 25, 2022 |
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Objective: While hydrocephalus patients are commonly treated with ventriculo-peritoneal (VP) or ventriculo-atrial (VA) shunt systems, recurrent shunt failure may necessitate consideration of alternative CSF-diversion sites. Occasionally, CSF diversion into the gallbladder has been reported, but larger patient series and long-term experiences are lacking.
Methods: We conducted a retrospective chart review on a patient treated with ventriculo-biliary (VB) CSF diversion with a long-term follow-up over 19 years under consideration of the current literature.
Results: We report the case of a 22 yr old patient with neonatal hydrocephalus occlusus due to pseudomembranous adhesions, caused by plasminogen deficiency type I, a rare autosomal recessive hereditary disorder. After failure of ventriculocisternostomy and multiple VP shunt revisions in early childhood, the patient developed severe ascites and resorption failure of the intraperitoneal cavity at the age of 3. VA shunting was prohibited due to thrombophilia, so that the peritoneal shunt catheter was finally placed into the gallbladder. 9 yrs later, the distal catheter had to be extended due to growth in body length. After an otherwise clinically stable interval of 18 yrs, the patient developed abdominal pain with hydrops of the gallbladder (see figure) with moderate inflammatory signs and secondary headache. With presumed cholecystitis secondary to choledocholithiasis, cholecystectomy was considered, but finally antibiotic treatment initiated. CSF analysis was not conducted due to patient´s refusal. After 5 days of antibiotic treatment, symptoms and gallbladder hydrops resolved completely. Apart from infectious complications (in the literature 24% of reported cases), gallbladder atony has previously been reported as most common cause for VB shunt failure.
Conclusion: While obstructive and infectious complications seem to constitute relevant risks in VB-shunt therapy, CSF diversion into the gallbladder may still be a viable treatment option in selected patients, potentially allowing a beneficial longterm-outcome. To our knowledge, this is the longest reported follow-up of an adult patient with plasminogen typ I deficiency and VB-shunt therapy since early childhood.