title: Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele.
abstract: Between January, 1981, and July, 1991, 17 infants under 1 month of age were admitted to The Hospital for Sick Children with the signs and symptoms of a Chiari II malformation. These patients' presentation included swallowing difficulty (71%), stridor (59%), apneic spells (29%), aspiration (12%), weakness of cry (18%), and arm weakness (53%). Decompression of the Chiari II malformation was performed in all patients, with a time interval between onset of symptoms and surgery ranging from 1 to 121 days. Fifteen patients (88%) remain alive, all of whom have shown a complete recovery. The mean follow-up period in this group of patients was 65 months. Two patients died, one due to respiratory arrest 8 months after decompression and the other because of shunt infection and peritonitis 7 years after decompression. These results support the concept that compressive forces, rather than a primary intrinsic disorder of the brain-stem nuclei, play a crucial etiological role in the development of a symptomatic Chiari II malformation. Early recognition of the symptoms of Chiari II malformation should be followed by immediate decompressive laminectomy in order to promote a prompt and full neurological recovery.
authors.affiliation: Division of Neurosurgery, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: W P
authors.initials: WP
authors.lastname: Vandertop
authors.forename: A
authors.initials: A
authors.lastname: Asai
authors.forename: H J
authors.initials: HJ
authors.lastname: Hoffman
authors.forename: J M
authors.initials: JM
authors.lastname: Drake
authors.forename: R P
authors.initials: RP
authors.lastname: Humphreys
authors.forename: J T
authors.initials: JT
authors.lastname: Rutka
authors.forename: L E
authors.initials: LE
authors.lastname: Becker
mesh_terms: D001139:Arnold-Chiari Malformation / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008591:Meningomyelocele / Q000209:etiology / Q000601:surgery*; D011183:Postoperative Complications / Q000401:mortality; D015996:Survival Rate