Chiari Malformation (CM) type III surgical management in infant: a case report

Abstract

Background: Chiari Malformations (CM) is a collection of hindbrain abnormalities ranging from simple to complete agenesis of the cerebellum. Type III CM (CM III) is the rarest CM, with herniation of the cerebellum and brainstem into a posterior encephalocele and other intracranial anomalies. Reduction of the viable herniated brain, along with providing intracranial space and inadequate closure, often presents as the main problem. This case study aims to evaluate the Chiari malformation type III surgical management in infants. Case Presentation: A 16-day-old male infant with an intact congenital mass in the occipital, with no neurological deficit. Radiological examination shows a defect in the occipital skull consisting of cystic non-contrast enhancing lesion and parenchymal tissue herniated through the defect.  The cele excision and defect closure were performed, and viable neural tissue was found inside the sac. Blossom flower-like trim expanded the fossa posterior space and put the viable neural tissue inside the intracranial room. The defect was then closed with a dura, periosteal patch, and tension-free two-layered scalp closure. Post-operative follow-up presented a good outcome, and 1-year follow-up showed good recovery, with developmental delay, following developmental rehabilitation. Conclusion: CM III is rare and has poor outcomes because neurological deficits and respiratory problems usually follow it. Operative management patient with CM III was presented with occipital encephalocele with no post-operative complication. Occipital bone reconstruction using a blossomed-flower-like technique could be considered to create adequate room for viable neural tissue.