{"id":"2dfce790-b700-4ee8-bce6-03034c93e6bd","slug":"brain-overgrowth-associated-with-megalencephaly-capillary-malformation-syndrome-causing-progressive-chiari-and-syringomyelia","title":"Brain overgrowth associated with megalencephaly-capillary malformation syndrome causing progressive Chiari and syringomyelia","authors":["Tom Deleu","Katrien Jansen","Frank Van Calenbergh"],"abstract":"Background: Megalencephaly-capillary malformation (M-CM) syndrome is a rare overgrowth syndrome characterized by macrocephaly, port-wine stains, asymmetric brain growth, hydrocephalus, and developmental delay. Cerebellar tonsil herniation is often seen, but rarely with syringomyelia. Case Description: A newborn with M-CM syndrome developed a progressive Chiari malformation type I (CM-I) with syringomyelia. At 4 months, he was treated for subdural hematomas, while at 10 months, he required a shunt for hydrocephalus. At 16 years of age, he newly presented a left hemiparesis and ataxia. Notably, successive volumetric measurements of the posterior fossa/cerebellum showed disproportionate cerebellar growth over time that correlated with the appearance of a CM-I. Following a suboccipital craniectomy with C1-laminectomy and duraplasty, he neurologically improved. Conclusion: M-CM with CM-I and syringomyelia rarely present together. Here, we treated an infant with M-CM who developed a progressive CM-I malformation and syringomyelia reflecting disproportionate growth of the cerebellum/posterior fossa over a 16-year period.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/2dfce790-b700-4ee8-bce6-03034c93e6bd/featured/hero-1781561343638.png","publishDate":"2022-05-20T00:00:00.000Z","doi":"10.25259/SNI_1016_2021","categories":["Pediatric Neurosurgery","Case Report"],"fullTextUrl":"https://surgicalneurologyint.com/wp-content/uploads/2022/05/11605/SNI-13-211.pdf"}