1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-1-28-b 10.15844/pedneurbriefs-1-4-9 Congenital CNS Defects Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Arnold-Chiari with Myelomeningocele http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 09 1987 01 08 2016 1 4 28 29 Copyright: © 1987 The Author(s) 1987 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Management of Chiari II complications in infants with myelomeningocele

The outcome of 19 infants with complications of Arnold-Chiari malformation and meningomyelocele was reviewed at the Depts.

 Stridor Alone Cyanotic Spells Ventricular Shunt

The outcome of 19 infants with complications of Arnold-Chiari malformation and meningomyelocele was reviewed at the Depts. of Pediatrics, Pathology, and Neurosurgery, University of Pennsylvania School of Medicine and the Children’s Hospital of Philadelphia. Vocal cord paralysis and inspiratory stridor alone occurred in 10 (grade I), apnea was an additional symptom in 4 (grade II), and cyanotic spells and dysphagia were associated in 5 (grade III).

Ventricular shunt was performed in 14 infants, with resolution of symptoms in 7 (in 5 of 8 with grade I, 2 of 4 with grade II, and none of 2 with grade III symptoms). Of 10 with posterior fossa decompressions, symptoms resolved in only 2 (in 1 of 4 with grade I, one of 2 with grade II, and none of 4 with grade III symptoms). Within 6 months after symptoms began, one infant with grade II and 3 with grade III died. No deaths occurred with the grade I group. Infants with grade II or III symptoms have more extensive brain stem damage, such as hemorrhage, infarction and necrosis, and carry a poor prognosis whereas those with grade I symptoms often improve after neurosurgical procedures. [1]

COMMENT: The grading of cases according to complications is useful in investigation, treatment and prognosis. In a previous study from the University of Toronto [2], decompression was recommended before rapid neurologic deterioration takes place, even if a functioning shunt is present. Of 45 infants with surgical decompression of the Chiari malformation, 28 survived and showed improved neurologic function and in 24 of these, recovery was complete. About 71% died of those patients who developed cardiorespiratory arrest, vocal cord paralysis, or arm weakness within 2 weeks before decompression, compared with 22% of those with more gradual neurologic deterioration.

 Charney EB Rorke LB Sutton LN Schut L Management of Chiari II complications in infants with myelomeningocele J Pediatr 1987 Sep 111 3 364 71 3625403 10.1016/S0022-3476(87)80455-9 Park TS Hoffman HJ Hendrick EB Humphreys RP Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele Neurosurgery 1983 Aug 13 2 147 52 6888694 10.1227/00006123-198308000-00007