Heterotaxy syndromes and abnormal bowel rotation

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• 作者：Beverley Newman (1)
  Raji Koppolu (2)
  Daniel Murphy (3)
  Karl Sylvester (2)
  
• 关键词：Heterotaxy ; Malrotation ; Non ; rotation ; Children ; Computed tomography ; Magnetic resonance imaging ; Upper gastrointestinal series
• 刊名：Pediatric Radiology
• 出版年：2014
• 出版时间：May 2014
• 年：2014
• 卷：44
• 期：5
• 页码：542-551
• 全文大小：1,101 KB
• 参考文献：1. Daneman A (2009) Malrotation: the balance of evidence. Pediatr Radiol 39:S164鈥揝166 CrossRef
  2. Choi M, Borenstein SH, Hornberger L et al (2005) Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities. Arch Dis Child 90:813鈥?15 CrossRef
  3. Strouse PJ (2004) Disorders of intestinal rotation and fixation ("malrotation"). Pediatr Radiol 34:837鈥?51 CrossRef
  4. Taylor GA (2011) CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation. Pediatr Radiol 41:1378鈥?383 CrossRef
  5. Yousefzadeh DK (2009) The position of the duodenojejunal junction: the wrong horse to bet on in diagnosing or excluding malrotation. Pediatr Radiol 39:S172鈥揝177 CrossRef
  6. Tashjian DB, Weeks B, Brueckner M et al (2007) Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia. J Pediatr Surg 42:528鈥?31 CrossRef
  7. Lee SE, Kim HY, Jung SE et al (2006) Situs anomalies and gastrointestinal abnormalities. J Pediatr Surg 41:1237鈥?242 CrossRef
  8. 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鈥?69 CrossRef
  9. 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鈥?095, discussion 1095 CrossRef
  10. Watanabe T, Nakano M, Yamazawa K et al (2011) Neonatal intestinal volvulus and preduodenal portal vein associated with situs ambiguus: report of a case. Surg Today 41:726鈥?29 CrossRef
  11. Pockett CR, Dicken B, Rebeyka IM et al (2013) Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients? Pediatr Cardiol 34:59鈥?3 CrossRef
  12. Applegate KE (2009) Evidence-based diagnosis of malrotation and volvulus. Pediatr Radiol 39:S161鈥揝163 CrossRef
  13. Long FR, Kramer SS, Markowitz RI et al (1996) Radiographic patterns of intestinal malrotation in children. Radiographics 16:547鈥?56, discussion 556-560 CrossRef
  14. Karmazyn B (2013) Duodenum between the aorta and the SMA does not exclude malrotation. Pediatr Radiol 43:121鈥?22 CrossRef
  
• 作者单位：Beverley Newman (1)
  Raji Koppolu (2)
  Daniel Murphy (3)
  Karl Sylvester (2)
  
  1. Department of Radiology, Stanford University, Lucile Packard Children鈥檚 Hospital, 725 Welch Road, MC 5913, Stanford, CA, 94305, USA
  2. Department of Surgery, Lucile Packard Children鈥檚 Hospital at Stanford, Stanford, CA, USA
  3. Department of Cardiology, Lucile Packard Children鈥檚 Hospital at Stanford, Stanford, CA, USA
  
• ISSN：1432-1998

文摘

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.