Intestinal malrotation is 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 gastrointestinal contrast studies should be ordered in patients who are stable.
Treatment is surgical correction, the procedure of choice being the Ladd's procedure.
Intestinal malrotation is a term used to describe an entire spectrum of rotational and fixation disturbances that can occur during embryonic development.
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. Abnormal caecal attachments to the right upper peritoneal cavity (i.e., the 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.
Patients with malrotation are at risk for volvulus and should be identified whenever possible to allow for proper therapy.
History and exam
Key diagnostic factors
- bilious vomiting
- abdominal pain
Other diagnostic factors
- infant age <1 year
- normal abdominal examination
- abdominal distension
- abdominal tenderness
- tachycardia with hypertension
- tachycardia with hypotension
- weight loss
- dark blood in nappy
- rebound tenderness and guarding
- embryological abnormality
1st investigations to order
- upper gastrointestinal contrast series
- CT abdomen (with oral and intravenous [IV] contrast)
- abdominal plain x-rays
Investigations to consider
- lower gastrointestinal (GI) contrast series
obstruction with ischaemia
obstruction without ischaemia
intermittent or partial volvulus or obstructing Ladd's bands
questionable malrotation or asymptomatic finding
Michael Stanton, MB BS, MD, FRCS (Paed Surg)
Consultant Paediatric Surgeon and Honorary Senior Lecturer
Department of Paediatric Surgery
University Hospital Southampton
MS acts as an expert witness and writes reports for medico-legal cases on an ad hoc basis.
Dr Michael Stanton would like to gratefully acknowledge Dr Stephen Shew, Dr S.D. St Peter, Dr Fizan Abdullah, and Dr Omar Karim, previous contributors to this topic.
SS is the author of two references cited in this topic. SDSP is the co-author of one reference cited in this topic. FA and OK declared that they had no competing interests. FA and OK declared that they had no competing interests.
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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