title: Intracranial anomalies and cloacal exstrophy--is there a role for screening?
abstract: OBJECTIVE
  Cloacal exstrophy (CE) is a severe multi-system congenital defect. While spina bifida is a defining feature of cloacal exstrophy, patients are not routinely screened for intracranial anomalies (ICAs). We sought to better characterize this risk of ICA in the CE patient.
  
  
  METHODS
  We retrospectively reviewed the medical records of 81 children with CE treated at our institution, identifying intracranial pathology, including hydrocephalus, Chiari malformation and craniosynostosis. Data points included ICA, neural tube defect, surgical procedures, and ambulatory status.
  
  
  RESULTS
  Of the 39 patients with cranial imaging, 31% had an ICA: 6 hydrocephalus, 3 Chiari malformation, 1 craniosynostosis, 1 hydrocephalus and craniosynostosis, and 1 Chiari malformation and craniosynostosis. All patients with ICAs had spina bifida. Patients with ICAs underwent more neurosurgical procedures, including more spinal procedures. Patients with ICAs were much more likely to be wheelchair-bound or ambulate minimally when compared to patients without anomalies.
  
  
  CONCLUSIONS
  In our population of CE patients with available head imaging, 31% had an ICA, thus screening would seem prudent. As all patients with ICAs had spina bifida, it may be less important to screen those rare CE patients without spinal pathology. Patients with ICAs were more likely to be wheelchair-bound, suggesting worse neurologic outcomes.
authors.affiliation: Division of Pediatric Urology, Johns Hopkins University School of Medicine, Baltimore, MD 21287; Pediatric Urology, Children's Hospital of Michigan, Detroit, MI 48201. Electronic address: kdsuson@gmail.com.
authors.forename: Kristina D
authors.initials: KD
authors.lastname: Suson
authors.forename: Paul M
authors.initials: PM
authors.lastname: Colombani
authors.forename: George I
authors.initials: GI
authors.lastname: Jallo
authors.forename: John P
authors.initials: JP
authors.lastname: Gearhart
mesh_terms: D034861:Abdominal Wall / Q000002:abnormalities* / Q000196:embryology; D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000196:embryology / Q000601:surgery; D001139:Arnold-Chiari Malformation / Q000453:epidemiology / Q000601:surgery; D001746:Bladder Exstrophy / Q000453:epidemiology / Q000601:surgery; D002988:Cloaca / Q000002:abnormalities*; D003376:Counseling; D003398:Craniosynostoses / Q000453:epidemiology / Q000601:surgery; D019299:Decompression, Surgical; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000151:congenital / Q000453:epidemiology / Q000601:surgery; D007231:Infant, Newborn; D008297:Male; D051346:Mobility Limitation; D015997:Neonatal Screening; D009436:Neural Tube Defects / Q000453:epidemiology / Q000601:surgery; D059906:Neuroimaging / Q000706:statistics & numerical data; D019635:Neurosurgical Procedures / Q000706:statistics & numerical data; D011296:Prenatal Diagnosis; D011379:Prognosis; D012189:Retrospective Studies; D016135:Spinal Dysraphism / Q000453:epidemiology; D014910:Wheelchairs / Q000706:statistics & numerical data
keywords: Chiari malformation; Cloacal exstrophy; Hydrocephalus