title: Acute abdominal symptoms and signs in children and young adults with spina bifida: ten years' experience.
abstract: OBJECTIVE
  Diagnosis and management of the acute abdomen in patients with spina bifida (SB) can be problematic. There are at least 4 clinical factors that can predispose to the development of acute abdominal symptoms and signs, and patients with a thoracic level lesion can have a partially insensate abdomen. The authors analyzed their accumulated experience to determine the annual incidence of acute abdominal signs and symptoms in children and young adults with spina bifida, the differential diagnosis, the operative management, and the outcome. The pertinent literature was reviewed.
  
  
  METHODS
  Cases were ascertained during a 10-year period at 1 institution and reviewed retrospectively.
  
  
  RESULTS
  Twenty-two episodes of acute abdominal symptoms and signs in 19 children and young adults with SB were ascertained over 10 years at 1 institution, for an annual incidence of 0.74%. More patients had a thoracic level lesion (n = 12; 60%) than in the clinic population as a whole (27%; P =.04), but the gender distribution was similar (58% girls), as was the prevalence of ventriculoperitoneal shunts (VPS; 95%). The median age was 13 years (range, 1 year to 26 years). Hospitalization was necessary for 19 (86%) of the 22 episodes. The duration of symptoms before diagnosis was a median of 3 days (range, 1 to 14 days). Most patients (82%) presented with abdominal pain. Fever was present in 27%, shock in 23%, and peritoneal signs in 23%. There were 14 different final diagnoses, 10 (71%) of which were associated with a predisposing factor. Of the 22 episodes, 18 (82%) could be attributed to an underlying factor: (1) neurogenic bladder (9; 41%); (2) neurogenic bowel (3; 14%); (3) VPS (4; 18%); (4) complications from previous surgery (2; 9%). Thirteen patients (59%) underwent a total of 20 surgical procedures of 12 different kinds. Despite awareness of the complexities involved, 3 patients (14%) died: 1 from complications resulting from bladder perforation; 1 from urosepsis and shock; and 1 from peritonitis caused by VPS infection.
  
  
  CONCLUSIONS
  The differential diagnosis of the acute abdomen in patients with SB is broad, conditions requiring surgery are frequently diagnosed, and the mortality rate is substantial, despite aggressive management.
authors.affiliation: Program in Neurodevelopmental Pediatrics and Division of Genetics and Metabolism, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA.
authors.forename: G
authors.initials: G
authors.lastname: Worley
authors.forename: J S
authors.initials: JS
authors.lastname: Wiener
authors.forename: T M
authors.initials: TM
authors.lastname: George
authors.forename: H E
authors.initials: HE
authors.lastname: Fuchs
authors.forename: J F
authors.initials: JF
authors.lastname: Mackey
authors.forename: R D
authors.initials: RD
authors.lastname: Fitch
authors.forename: K T
authors.initials: KT
authors.lastname: Oldham
mesh_terms: D000006:Abdomen, Acute / Q000175:diagnosis* / Q000453:epidemiology*; D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D011379:Prognosis; D018570:Risk Assessment; D012307:Risk Factors; D017678:Sex Distribution; D016135:Spinal Dysraphism / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery