Heterotaxy syndromes and abnormal bowel rotation

• Published in: Pediatric radiology 2014-05, Vol.44 (5), p.542-551
• Main Authors: Newman, Beverley, Koppolu, Raji, Murphy, Daniel, Sylvester, Karl
• Format: Article
• Language: English
• Subjects: Female; Heterotaxy Syndrome - diagnosis; Humans; Imaging; Infant; Infant, Newborn; Intestinal Volvulus - diagnosis; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Neuroradiology; Nuclear Medicine; Oncology; Original Article; Pediatrics; Radiology; Reproducibility of Results; Sensitivity and Specificity; Stomach Volvulus - diagnosis; Tomography, X-Ray Computed; Ultrasound
• ISSN: 0301-0449; 1432-1998
• DOI: 10.1007/s00247-013-2861-4

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.