Acquired Chiari I malformation and syringomyelia after valveless lumboperitoneal shunt in infancy
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Updated: Jul 6, 2026
Neuronavigation and Laparoscopy Guided Ventriculoperitoneal Shunt Insertion for the Treatment of Hydrocephalus
Published on: October 14, 2022
Laurent Riffaud1, Carolina Moughty, Pierre-Louis Henaux
1Department of Neurosurgery, Pontchaillou University Hospital, Rennes, France. laurent.riffaud@chu-rennes.fr <laurent.riffaud@chu-rennes.fr>
Insights
A valveless lumboperitoneal shunt in a child led to acquired Chiari I malformation and syringomyelia. Adjusting the shunt resolved symptoms, highlighting risks of CSF leakage from such devices.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Lumboperitoneal (LP) shunts are used to treat conditions like hydrocephalus and macrocephaly in children.
- Valveless LP shunts are sometimes preferred for their simplicity.
- Acquired Chiari I malformation and syringomyelia are serious neurological conditions.
Observation:
- A 13-year-old boy with a history of a valveless LP shunt developed disabling postural headaches.
- Neurological examination revealed Chiari I malformation, syringomyelia, and cervical spinal cord edema.
- Clamping the existing shunt temporarily worsened symptoms, indicating increased intracranial pressure.
Findings:
- The patient's symptoms were attributed to a symptomatic acquired Chiari I malformation and syringomyelia.
- These conditions were likely caused by chronic cerebrospinal fluid (CSF) leakage from the valveless LP shunt.
- Insertion of an adjustable valve shunt (110 mm Hg) successfully resolved all neurological symptoms.
Implications:
- This case highlights the potential risk of symptomatic acquired Chiari I malformation and syringomyelia associated with valveless LP shunts.
- Careful consideration and monitoring are necessary when using LP shunts in pediatric patients, particularly those without valves.
- Management of CSF shunts requires precise pressure regulation to prevent complications such as spinal CSF leaks and secondary malformations.
Abstract:
The authors present the case of a 13-year-old boy who experienced frequent disabling episodes of typical postural induced headaches, which revealed a Chiari I malformation, syringomyelia and cervical spinal cord edema. This boy had received a valveless lumboperitoneal (LP) shunt at the age of 18 months for macrocephaly. At this time, cranial CT scan showed moderate enlargement of the subarachnoid spaces without hydrocephalus or tonsillar herniation. The LP shunt was clamped but the patient experienced signs of brain stem dysfunction and increased intracranial pressure. Finally, an adjustable valve (110 mm Hg) was inserted and all the symptoms resolved. Although some authors consider LP shunt as a safe and effective procedure even in the pediatric population, our case reminds us that these shunts, especially when valveless, may expose the patient to the risk of symptomatic acquired Chiari I malformation and syringomyelia consecutive to a chronic spinal CSF leakage.
