Literature DB >> 24419494

Heterotaxy syndromes and abnormal bowel rotation.

Beverley Newman1, Raji Koppolu, Daniel Murphy, Karl Sylvester.   

Abstract

BACKGROUND: Bowel rotation abnormalities in heterotaxy are common. As more children survive cardiac surgery, the management of gastrointestinal abnormalities has become controversial.
OBJECTIVE: To evaluate imaging of malrotation in heterotaxy with surgical correlation and provide an algorithm for management.
MATERIALS AND METHODS: Imaging reports of heterotaxic children with upper gastrointestinal (UGI) and/or small bowel follow-through (SBFT) were reviewed. Subsequently, fluoroscopic images were re-reviewed in conjunction with CT/MR studies. The original reports and re-reviewed images were compared and correlated with surgical findings.
RESULTS: Nineteen of 34 children with heterotaxy underwent UGI, 13/19 also had SBFT. In 15/19 reports, bowel rotation was called abnormal: 11 malrotation, 4 non-rotation, no cases of volvulus. Re-review, including CT (10/19) and MR (2/19), designated 17/19 (90%) as abnormal, 10 malrotation (abnormal bowel arrangement, narrow or uncertain length of mesentery) and 7 non-rotation (small bowel and colon on opposite sides plus low cecum with probable broad mesentery). The most useful CT/MR findings were absence of retroperitoneal duodenum in most abnormal cases and location of bowel, especially cecum. Abnormal orientation of mesenteric vessels suggested malrotation but was not universal. Nine children had elective bowel surgery; non-rotation was found in 4/9 and malrotation was found in 5/9, with discrepancies (non-rotation at surgery, malrotation on imaging) with 4 original interpretations and 1 re-review.
CONCLUSION: We recommend routine, early UGI and SBFT studies once other, urgent clinical concerns have been stabilized, with elective laparoscopic surgery in abnormal or equivocal cases. Cross-sectional imaging, usually obtained for other reasons, can contribute diagnostically. Attempting to assess mesenteric width is important in differentiating non-rotation from malrotation and more accurately identifies appropriate surgical candidates.

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Mesh:

Year:  2014        PMID: 24419494     DOI: 10.1007/s00247-013-2861-4

Source DB:  PubMed          Journal:  Pediatr Radiol        ISSN: 0301-0449


  14 in total

Review 1.  Disorders of intestinal rotation and fixation ("malrotation").

Authors:  Peter J Strouse
Journal:  Pediatr Radiol       Date:  2004-09-04

2.  Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?

Authors:  Charissa R Pockett; Bryan Dicken; Ivan M Rebeyka; David B Ross; Lindsay M Ryerson
Journal:  Pediatr Cardiol       Date:  2012-05-29       Impact factor: 1.655

Review 3.  Malrotation: the balance of evidence.

Authors:  Alan Daneman
Journal:  Pediatr Radiol       Date:  2009-04

4.  Situs anomalies and gastrointestinal abnormalities.

Authors:  Seung Eun Lee; Hyun-Young Kim; Sung-Eun Jung; Seong-Cheol Lee; Kwi-Won Park; Woo-Ki Kim
Journal:  J Pediatr Surg       Date:  2006-07       Impact factor: 2.545

5.  Neonatal intestinal volvulus and preduodenal portal vein associated with situs ambiguus: report of a case.

Authors:  Toshihiko Watanabe; Miwako Nakano; Kazuki Yamazawa; Katsuhiro Maeyama; Masao Endo
Journal:  Surg Today       Date:  2011-05-01       Impact factor: 2.549

6.  Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.

Authors:  M Choi; S H Borenstein; L Hornberger; J C Langer
Journal:  Arch Dis Child       Date:  2005-05-12       Impact factor: 3.791

7.  CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation.

Authors:  George A Taylor
Journal:  Pediatr Radiol       Date:  2011-05-19

8.  Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial.

Authors:  Stephanie Papillon; Catherine J Goodhue; Osnat Zmora; Shalini S Sharma; Winfield J Wells; Henri R Ford; Jeffrey S Upperman; Kasper S Wang; Gerald A Bushman; Richard Kim; James R Pierce
Journal:  J Pediatr Surg       Date:  2013-01       Impact factor: 2.545

9.  Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation.

Authors:  David C Yu; Ravi R Thiagarajan; Peter C Laussen; James P Laussen; Tom Jaksic; Christopher B Weldon
Journal:  J Pediatr Surg       Date:  2009-06       Impact factor: 2.545

Review 10.  The position of the duodenojejunal junction: the wrong horse to bet on in diagnosing or excluding malrotation.

Authors:  David K Yousefzadeh
Journal:  Pediatr Radiol       Date:  2009-04
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  7 in total

1.  Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without.

Authors:  Rohit S Loomba; Peter C Kouretas; Robert H Anderson
Journal:  Pediatr Cardiol       Date:  2016-07-08       Impact factor: 1.655

2.  Identifying intestinal malrotation on magnetic resonance examinations ordered for unrelated indications.

Authors:  Jill S Fay; Victoria Chernyak; Benjamin H Taragin
Journal:  Pediatr Radiol       Date:  2017-06-16

3.  Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.

Authors:  Paul S Cullis; Sotirios Siminas; Adeline Salim; Robert Johnson; Paul D Losty
Journal:  Pediatr Surg Int       Date:  2015-08-05       Impact factor: 1.827

4.  Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes.

Authors:  Xiaofang Wang; Yifan Shi; Shi Zeng; Jiawei Zhou; Jia Zhou; Hongxia Yuan; Lin Wang; Weiyuan Shi; Qichang Zhou
Journal:  BMC Pregnancy Childbirth       Date:  2017-11-23       Impact factor: 3.007

5.  A problem at any age: a case report of congenital malrotation with bowel ischemia in an 84-year-old.

Authors:  Marisa E Schwab; Sage P Kramer; Aya Bashi; Taehyun P Chung; Claudia M Mueller
Journal:  BMC Surg       Date:  2022-01-29       Impact factor: 2.102

Review 6.  [Gastrointestinal Emergencies in Neonates: What We Should Know].

Authors:  Hyun-Hae Cho; So Mi Lee; Sun Kyoung You
Journal:  Taehan Yongsang Uihakhoe Chi       Date:  2020-07-30

7.  Congenitally Malformed Hearts: Aspects of Teaching and Research Involving Medical Students.

Authors:  Catherine C Pickin; James Castle; Vibha Shaji; Adeolu Banjoko; Aimee-Louise Chambault; Anna N Seale; Anthony Lander; Chetan Mehta; Adrian Crucean
Journal:  J Cardiovasc Dev Dis       Date:  2021-03-28
  7 in total

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