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).
Conclusion
This study strongly supports a ‘watchful waiting’ policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.
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References
Jacobs JP, Anderson RH, Weinberg PM et al (2007) The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 17(S2):1–28
Bartz PJ, Driscoll DJ, Dearani JA et al (2006) Early and late results of the modified Fontan operation for heterotaxy syndrome: 30 years of experience in 142 patients. J Am Coll Cardiol 48:2301–2305
Anagnostopoulos PV, Pearl JM, Octave C et al (2009) Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg 35:871–878
Nakada K, Kawaguchi F, Wakisaka M et al (1997) Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg 32:91–94
Ditchfield MR, Hutson JM (1998) Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol 28:303–306
Murphy FL, Sparnon AL (2006) Long-term complications following intestinal malrotation and the Ladd’s procedure: a 15 year review. Pediatr Surg Int 22:326–329
El-Gohary Y, Alagtal M, Gillick J (2010) Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int 26:203–206
Biko DM, Anupindi SA, Hanhan SB, Blinman T, Markowitz RI (2011) Assessment of recurrent abdominal symptoms after Ladd procedure: clinical and radiographic correlation. J Pediatr Surg 46:1720–1725
Ferdman B, States L, Gaynor JW, Hedrick HL, Rychik J (2007) Abnormalities of intestinal rotation in patients with congenital heart disease and the heterotaxy syndrome. Congenit Heart Dis 2:12–18
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
Yu DC, Thiagarajan RR, Laussen PC et al (2009) Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg 44:1089–1095
Newman B, Koppolu R, Murphy D, Sylvester K (2014) Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol 44:542–551
Choi M, Borenstein SH, Hornberger L et al (2005) Heterotaxia syndrome : the role of screening for intestinal rotation abnormalities. Arch Dis Child 90:813–815
Papillon S, Goodhue CJ, Zmora O et al (2013) Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial. J Pediatr Surg 48:164–169
Pockett CR, Dicken B, Rebeyka IM, Ross DB, Ryerson LM (2013) Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients? Pediatr Cardiol 34:59–63
Hill SJ, Heiss KF, Mittal R et al (2014) Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg 49:934–937
Elder CT, Metzger R, Arrington C, Rollins M, Scaife E (2014) The role of screening and prophylactic surgery for malrotation in heterotaxy patients. J Pediatr Surg 49:1746–1748
Pockett CR, Dicken BJ, Rebeyka IM, Ross DB, Ryerson LM (2013) Heterotaxy syndrome and intestinal rotation abnormalities: a survey of institutional practice. J Pediatr Surg 48:2078–2083
Malek MM, Burd RS (2006) The optimal management of malrotation diagnosed after infancy: a decision analysis. Am J Surg 191:45–51
Mehall JR, Chandler JC, Mehall RL et al (2002) Management of typical and atypical intestinal malrotation. J Pediatr Surg 37:1169–1172
McVay MR, Kokoska ER, Jackson RJ, Smith SD (2007) The changing spectrum of intestinal malrotation: diagnosis and management. Am J Surg 194:712–719
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Cullis, P.S., Siminas, S., Salim, A. et al. Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre. Pediatr Surg Int 31, 1127–1131 (2015). https://doi.org/10.1007/s00383-015-3755-3
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DOI: https://doi.org/10.1007/s00383-015-3755-3