An entire spectrum of rotational and fixation disturbances that can occur during embryonic development.
The anatomical variant that poses the highest risk of volvulus is a narrow midgut mesenteric base accompanied by lack of retro-peritoneal midgut fixation. This variant cannot be reliably determined from any radiological studies.
Malrotation predisposes patients to a risk of midgut volvulus.
Emergency surgical consultation is appropriate before obtaining any diagnostic studies if midgut volvulus is suspected. Upper GI contrast studies should be ordered in patients who are stable.
Treatment is surgical correction, the procedure of choice being the Ladd procedure.
Intestinal malrotation is a term used to describe an entire spectrum of rotational and fixation disturbances that can occur during embryonic development.
Abnormal caecal attachments to the right upper peritoneal cavity (i.e., Ladd’s bands) can cross the second portion of the duodenum creating an extraluminal low-grade obstruction of the duodenum, which may present with signs of partial obstruction. However, the most significant pathological concerns in malrotation are a lack of intestinal fixation to the retro-peritoneum and a narrow midgut mesenteric base that predisposes to a twisting of the small bowel in the form of midgut volvulus. This condition creates an abrupt obstruction of the duodenum, resulting in bilious vomiting.
If the twisting at the base also obstructs flow in the superior mesenteric artery, the entire small intestine and proximal colon may become acutely ischaemic and subsequently necrotic within a few hours.
Patients with malrotation are at risk for volvulus and should be identified whenever possible to allow for proper therapy.
History and exam
Fizan Abdullah, MD, PhD
Pediatric Surgery Division Head & Vice Chair Department of Surgery
Program Director, Fellowship in Pediatric Surgery
Ann & Robert H. Lurie Children's Hospital of Chicago
Professor of Surgery
Northwestern University Feinberg School of Medicine
FA declares that he has no competing interests.
Omar Karim, MD
Clinical Fellow in Surgery
Harvard Medical School
OK declares that he has no competing interests.
Dr Fizan Abdullah and Dr Omar Karim would like to gratefully acknowledge Dr Stephen Shew and Dr S.D. St Peter, previous contributors to this monograph. SS is the author of two references cited in this monograph. SDSP is the co-author of one reference cited in this monograph.
Casey M. Calkins, MD
Assistant Professor of Pediatric Surgery
The Medical College of Wisconsin
Children's Hospital and Health System
CC declares that he has no competing interests.
Steve Rothenberg, MD
Chief of Pediatric Surgery
Chairman Department of Pediatrics
The Rocky Mountain Hospital for Children at Presbyterian/St. Luke's
SR declares that he has no competing interests.
KuoJen Tsao, MD
Department of Pediatric Surgery
University of Texas Health Science Center at Houston
KT declares that he has no competing interests.
Eric Nicholls, MD
Consultant Paediatric Surgeon
St George's Hospital
EN declares that he has no competing interests.
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