title: Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.
abstract: BACKGROUND
  The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease.
  
  
  METHODS
  Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed.
  
  
  RESULTS
  Of a total of 92 patients, 16 (17.4%) cases underwent foregut imaging studies. Three examinations were performed in 'symptomatic' patients. Twelve studies reported 'abnormal anatomy' with only five patients undergoing surgical correction. No complication(s) were recorded after Ladd's operation to correct defects. A single fatality occurred within 30 days postoperatively from cardiac failure. In 87 patients in whom Ladd's operation was not undertaken, no single patient developed intestinal volvulus (median length of follow-up 27.2 months, total 446.1 person years).
  
  
  CONCLUSIONS
  This study strongly supports a 'watchful waiting' policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.
authors.affiliation: Department of Surgical Paediatrics, The Royal Hospital for Sick Children, Yorkhill, Glasgow, UK.
authors.forename: Paul S
authors.initials: PS
authors.lastname: Cullis
authors.affiliation: Department of Paediatric Surgery, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Sotirios
authors.initials: S
authors.lastname: Siminas
authors.affiliation: Department of Paediatric Surgery, Alder Hey Childrens Hospital NHS Foundation Trust, Liverpool, UK.
authors.forename: Adeline
authors.initials: A
authors.lastname: Salim
authors.affiliation: Department of Paediatric Cardiology, Alder Hey Childrens Hospital NHS Foundation Trust, Liverpool, UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Johnson
authors.affiliation: Department of Paediatric Surgery, Alder Hey Childrens Hospital NHS Foundation Trust, Liverpool, UK. paul.losty@liverpool.ac.uk.|Institute of Child Health, University of Liverpool, Liverpool, UK. paul.losty@liverpool.ac.uk.
authors.forename: Paul D
authors.initials: PD
authors.lastname: Losty
mesh_terms: D059446:Heterotaxy Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D045822:Intestinal Volvulus / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D016019:Survival Analysis; D016896:Treatment Outcome; D006113:United Kingdom
keywords: Cardiac anomalies; Heterotaxy; Intestinal rotation; Ladd’s operation; Outcomes