Adult Presentations of Congenital Midgut Malrotation: A Systematic Review

Background Adult midgut malrotation is a rare cause of an acute abdomen requiring urgent intervention. It may also present in the non-acute setting with chronic, non-specific symptoms. The objective of this study is to identify the clinical features, appropriate investigations and current surgical management associated with adult malrotation. Methods A systematic review was conducted according to PRISMA guidelines, identifying confirmed cases of adult malrotation. Patient demographics, clinical features, investigation findings and operative details were analysed. Results Forty-five reports met the inclusion criteria, totalling 194 cases. Mean age was 38.9 years (n = 92), and 52.3% were male (n = 130). The commonest presenting complaints were abdominal pain (76.8%), vomiting (35.1%) and food intolerance (21.6%). At least one chronic symptom was reported in 87.6% and included intermittent abdominal pain (41.2%), vomiting (12.4%) and obstipation (11.9%). Computerised tomography scanning was the most frequent imaging modality (81.4%), with a sensitivity of 97.5%. The whirlpool sign was observed in 30.9%; abnormalities of the superior mesenteric axis were the commonest finding (58.0%). Ladd's procedure was the most common surgical intervention (74.5%). There was no significant difference in resolution rates between emergency and elective procedures (p = 0.46), but length of stay was significantly shorter for elective cases. (p = 0.009). There was no significant difference in risk of mortality, or symptom resolution, between operative and conservative management (p = 0.14 and p = 0.44, respectively). Conclusion Malrotation in the adult manifests with chronic symptoms and should be considered as a differential diagnosis in patients with abdominal pain, vomiting and food intolerance.