Abstract
Heterotaxy syndrome (HS) is a complex disorder involving thoracic and abdominal asymmetries. Congenital heart disease is often accompanied by an intestinal rotation abnormality (IRA) that may predispose to bowel ischemia and infarction. There is controversy in the literature whether asymptomatic infants with HS must be screened for IRA and, if present, whether a prophylactic Ladd procedure should be performed. We performed a retrospective chart review of all patients who underwent a Ladd procedure from January 2007 to December 2010 at Stollery Children’s Hospital, Edmonton, Canada. Twenty-nine patients underwent a Ladd procedure, 22 without HS but with symptomatic malrotation and 7 with HS and asymptomatic malrotation. Asymptomatic HS patients had a complication rate of 57 % after a prophylactic Ladd procedure compared with a complication rate of 9 % in the symptomatic non-HS population. The management of asymptomatic IRA in patients with HS remains controversial. We suggest that HS patients be screened for IRA and that asymptomatic patients be managed conservatively.
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Choi M, Borenstein SH, Hornberger L, Langer JC (2005) Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities. Arch Dis Child 90:813–815
Cohen MS, Schultz AH, Tian ZY, Donaghue DD, Weinberg PM, Gaynor JW et al (2006) Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival? Ann Thorac Surg 82:1629–1636
Ditchfield MR, Hutson JM (1998) Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol 28:303–306
Festen MD (1982) Postoperative small bowel obstruction in infants and children. Ann Surg 196:580–583
Filston HC, Kirks DR (1981) Malrotation—the ubiquitous anomaly. J Pediatr Surg 16:614–620
Kouwenberg M, Severijnen RSVM, Kapusta EL (2008) Congenital cardiovascular defects in children with intestinal malrotation. Pediatr Surg Int 24:257–263
Langman J, Sadler T (2003) Langman’s medical embryology, 9th edn. Lippincott Williams & Wilkins, Philadelphia, pp 304–313
Nakada K, Kawaguchi F, Wakisaka M, Nakada M, Enami T, Yamate N (1997) Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg 32:91–94
Tashjian DB, Weeks B, Brueckner M, Touloukian RJ (2007) Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia. J Pediatr Surg 42:528–531
Torres AM, Ziegler MM (1993) Malrotation of the intestine. World J Surg 17:326–331
Wilkins BM, Spita L (1986) Incidence of postoperative adhesion obstruction following neonatal laparotomy. Br J Surg 73:762–764
Yu DC, Thiagarajan RR, Laussen PC, Laussen JP, Jaksic T, Weldon CB (2009) Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg 44:1089–1095
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Pockett, C.R., Dicken, B., Rebeyka, I.M. et al. Heterotaxy Syndrome: Is a Prophylactic Ladd Procedure Necessary in Asymptomatic Patients?. Pediatr Cardiol 34, 59–63 (2013). https://doi.org/10.1007/s00246-012-0385-6
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DOI: https://doi.org/10.1007/s00246-012-0385-6