title: Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis.
abstract: OBJECTIVE
  To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy.
  
  
  METHODS
  PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic.
  
  
  RESULTS
  From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Postoperative mortality after Ladd procedure mainly was associated with cardiac insufficiency, and overall it was significantly greater in the emergency group compared with the prophylactic group (18% vs 5.6%). The complication rate also was greater in case of emergency vs prophylactic abdominal surgery (27% vs 16%); adhesional small bowel obstruction was the most common complication overall (6%).
  
  
  CONCLUSIONS
  The screen-detected incidences of IRA and acute midgut volvulus were significantly greater in heterotaxy than the normal population. Prophylactic Ladd procedure was associated with less morbidity and mortality compared with emergency surgery. A long-term prospective randomized trial is needed to define the indication for screening and prophylactic treatment of IRA in heterotaxy.
authors: Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom.
authors: Yew-Wei
authors: YW
authors: Tan
authors: Fetal Medicine Unit, Division of Developmental Science, St George's University of London, London, United Kingdom.
authors: Asma
authors: A
authors: Khalil
authors: Department of Pediatric and Neonatal Surgery, King's College Hospital NHS Foundation Trust, London, United Kingdom.
authors: Madhavi
authors: M
authors: Kakade
authors: Fetal Medicine Unit, Division of Developmental Science, St George's University of London, London, United Kingdom; Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.
authors: Julene S
authors: JS
authors: Carvalho
authors: Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom.
authors: Sarah
authors: S
authors: Bradley
authors: Department of Pediatric and Neonatal Surgery, Royal London Hospital, London, United Kingdom.
authors: Stewart
authors: S
authors: Cleeve
authors: Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom. Electronic address: Stefano.giuliani@nhs.net.
authors: Stefano
authors: S
authors: Giuliani
mesh_terms: D004065:Digestive System Abnormalities / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D013505:Digestive System Surgical Procedures / Q000009:adverse effects / Q000379:methods; D059446:Heterotaxy Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D015994:Incidence; D045822:Intestinal Volvulus / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D007422:Intestines / Q000002:abnormalities*; D008403:Mass Screening / Q000379:methods*; D011183:Postoperative Complications; D011184:Postoperative Period; D012399:Rotation