Highlights
- •Screening and management practices of intestinal rotational abnormalities in heterotaxy syndrome are debated.
- •Watchful waiting in a multidisciplinary pathway permits safe and close follow-up with intervention when clinically indicated.
Summary
Purpose
Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children
with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities
(IRA) that differ from typical malrotation. In this prospective study, we examine
the development of a management pathway, imaging findings, and clinical course of
patients with HS and IRA.
Methods
After literature review, a multispecialty focus group designed a pathway for HS. Participants
from a single institution were prospectively enrolled from 2016 to 2019. They underwent
an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic
IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating,
severe reflux, and intermittent pain or abdominal distention. Screening for symptoms
occurred every three months for the first two years and then annually.
Results
18 participants were followed for a median of 5.0 years. Three clinical categories
were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic
with feeding intolerance or failure to thrive requiring gastrostomy placement and
diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent
exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily
correlate with US and/or UGI findings.
Conclusions
In children with HS and IRA, postnatal imaging did not inform the need for intestinal
surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated,
and symptomatic patients should be offered surgical intervention. A multidisciplinary
care pathway for patients with HS and IRA can coordinate care and help counsel families
on the likelihood of needing intestinal surgery for IRA.
Keywords
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References
Dassinger MS, Smith SD. Disorders of Intestinal Rotation and Fixation. In: Coran AG, Adzick, NS, editors. Pediatric Surgery (Seventh Edition), Elsevier Mosby; 2012, p. 1111-1125
- Laparoscopic repair of acute volvulus in a neonate with malrotation.Surg Endosc [Internet]. 1995; 9 (Available from:): 1123-1124https://doi.org/10.1007/bf00189001
- Laparoscopic treatment of intestinal malrotation in children.Surg Endosc [Internet. 1998; 12 (Available from:): 1314-1316https://doi.org/10.1007/s004649900848
- Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review.J Pediatr Surg [Internet]. 2015; 50 (Available from:): 1783-1790https://doi.org/10.1016/j.jpedsurg.2015.06.019
Intestinal Rotational Abnormalities [Internet]. Pedsurglibrary.com. [cited 2022 Mar 14]. Available from: https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829042/all/Intestinal_Rotational_Abnormalities
- Intestinal rotation abnormalities and midgut volvulus.Surg Clin North Am [Internet]. 2017; 97 (Available from:): 147-159https://doi.org/10.1016/j.suc.2016.08.011
- Heterotaxy syndrome and intestinal rotation abnormalities.Pediatrics [Internet]. 2018; 142 (Available from:)https://doi.org/10.1542/peds.2017-4267
- Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?.Pediatr Cardiol [Internet]. 2013; 34 (Available from:): 59-63https://doi.org/10.1007/s00246-012-0385-6
- Is screening of intestinal foregut anatomy in heterotaxy patients really necessary?: A systematic review in search of the evidence.Ann Surg [Internet]. 2016; 264 (Available from:): 1156-1161https://doi.org/10.1097/SLA.0000000000001563
- Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.Arch Dis Child [Internet]. 2005; 90 (Available from:): 813-815https://doi.org/10.1136/adc.2004.067504
- Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial.J Pediatr Surg [Internet. 2013; 48 (Available from:): 164-169https://doi.org/10.1016/j.jpedsurg.2012.10.033
- Ultrasound for malrotation and volvulus - point.Pediatr Radiol [Internet]. 2021; (Available from:)https://doi.org/10.1007/s00247-021-05154-0
- Making the diagnosis of midgut volvulus: Limited abdominal ultrasound has changed our clinical practice.J Pediatr Surg [Internet]. 2020; 55 (Available from:): 2614-2617https://doi.org/10.1016/j.jpedsurg.2020.04.012
- Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia.J Pediatr Surg [Internet]. 2007; 42 (Available from:): 528-531https://doi.org/10.1016/j.jpedsurg.2006.10.060
- Ladd’s procedure in functional single ventricle and heterotaxy syndrome: does timing affect outcome?.Ann Thorac Surg [Internet]. 2013; 95 (discussion 1407-8. Available from:): 1403-1407https://doi.org/10.1016/j.athoracsur.2012.11.018
- Mesenteric blood flow response to feeding after systemic-to-pulmonary arterial shunt palliation.Ann Thorac Surg [Internet]. 2003; 75 (Available from:): 947-951https://doi.org/10.1016/s0003-4975(02)04627-1
- Congenital cardiovascular defects in children with intestinal malrotation.Pediatr Surg Int [Internet]. 2008; 24 (Available from:): 257-263https://doi.org/10.1007/s00383-007-2086-4
- Racial disparities in heterotaxy syndrome: Racial and Ethnic Disparities in Heterotaxy Syndrome.Birth Defects Res A Clin Mol Teratol [Internet]. 2015; 103 (Available from:): 941-950https://doi.org/10.1002/bdra.23416
- Patient and family experience in a multidisciplinary clinic for children with single-ventricle heart disease.J Patient Exp [Internet]. 2020; 7 (Available from:): 1384-1390https://doi.org/10.1177/2374373520904214
Article info
Publication history
Accepted:
January 3,
2023
Received in revised form:
December 22,
2022
Received:
September 11,
2022
Publication stage
In Press Accepted ManuscriptIdentification
Copyright
© 2023 Elsevier Inc. All rights reserved.
