title: Critical analysis of the Chiari malformation Type I found in children with lipomyelomeningocele.
abstract: OBJECTIVE
  Occasional comments are found in the literature regarding patients with lipomyelomeningocele and concomitant Chiari malformation Type I (CM-I). The object of this study was to explore the association between these two conditions.
  
  
  METHODS
  The authors performed a retrospective database analysis of lipomyelomeningocele cases to identify cases of concomitant CM-I. Analysis of posterior fossa volume (based on the Cavalieri principle) was performed in all identified cases in which appropriate neuroimages were available, and the results were compared with those obtained in age-matched controls. Seven (13%) of 54 patients with lipomyelomeningocele were found to also have CM-I. Two of these were symptomatic (cervicothoracic syrinx and occipital headaches) and required posterior fossa decompression. No correlation was found between the amount of hindbrain herniation and the level of the conus medullaris or the type of lipomyelome-ningocele (for example, caudal or transitional). Volumetric studies of the posterior fossa revealed normal age-matched volumes in all but one patient (who had asymptomatic CM-I).
  
  
  CONCLUSIONS
  The incidence of CM-I in patients with lipomyelomeningocele appears to be significantly greater than that of the general population and the association rate is too high for the finding to be a chance occurrence. Decreases in the volume of the posterior cranial fossa were not found in the majority of patients in this small cohort; therefore, the cause of the concomitant occurrence of lipomyelomeningocele and CM-I remains undetermined. Clinicians should consider obtaining imaging studies of the entire neuraxis in patients with lipomyelomeningoceles and should investigate other causes for syringes found in association with lipomyelomeningoceles.
authors.affiliation: Section of Pediatric Neurosurgery, University of Alabama at Birmingham and Children's Hospital, Alabama 35233, USA. rstubbs@uab.edu
authors.forename: R Shane
authors.initials: RS
authors.lastname: Tubbs
authors.forename: Cuong J
authors.initials: CJ
authors.lastname: Bui
authors.forename: William C
authors.initials: WC
authors.lastname: Rice
authors.forename: Marios
authors.initials: M
authors.lastname: Loukas
authors.forename: Robert P
authors.initials: RP
authors.lastname: Naftel
authors.forename: Michael Paul
authors.initials: MP
authors.lastname: Holcombe
authors.forename: W Jerry
authors.initials: WJ
authors.lastname: Oakes
mesh_terms: D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000453:epidemiology* / Q000473:pathology; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003388:Cranial Fossa, Posterior; D016208:Databases, Factual; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008159:Lumbar Vertebrae; D008297:Male; D008591:Meningomyelocele / Q000150:complications* / Q000473:pathology; D012189:Retrospective Studies