Malrotation after infancy-can we do better? A 10-year multi-centre retrospective review

Malrotation is classically a neonatal condition, but it can present in older children. This population has a non-specific presentation that can delay diagnosis with fatal consequences. We aimed to identify presentation patterns and efficacy of imaging modalities to inform the management of children with suspected malrotation. Children aged 1-18 who had surgery for malrotation between 2014 and 2024 at four centres in the south of England were retrospectively reviewed. Hospital presentations, radiology, operative findings and outcomes were reviewed. Seventy-five. patients were identified: 35 (47%) had emergency surgery, and 40 (53%) had elective surgery. The median symptom onset was 3 years (IQR 1.0-6.0). The median age at hospital presentation was 4.9 (IQR 3.4-7.9), and the median age at surgery was 6 years (IQR 2.7-9.9). The median time from presentation to surgery was 1.9 years in the emergency group and 1 year in the elective group (p = 0.18). Common symptoms were abdominal pain (69%), vomiting (52%) and bilious vomiting (27%). Sixty-three percent of emergency patients had prior presentations. Upper-GI contrast (UGI) was the most common radiological study (72%; sensitivity (SN), 89%; false negative rate, (FNR) 3.7%). Ultrasound was used in 33% of cases (SN, 56%; FNR, 44%). Cross-sectional imaging was used in 20% of cases: eleven CT scans (SN, 81%; FNR, 0.0%) and four MRI scans (SN, 50%; FNR, 0.0%). Of the three mortality and nine bowel ischaemia cases, 67% had presented previously.

Our cohort demonstrated delays between presentation and surgery. Malrotation must be considered in cases of recurrent abdominal pain or vomiting. USS is not reliable in this cohort. We recommend these children undergo UGI or cross-sectional imaging to exclude malrotation. Timely diagnosis and intervention will prevent morbidity and mortality.

• Malrotation in older children is known to have a less specific presentation than classical neonatal malrotation associated with bilious vomiting. This can delay diagnosis and intervention.

• This would be the largest cohort in the European literature comprehensively reporting presentations, investigations and outcomes for children with malrotation over the age of 1 year. In providing this information, our study helps clinicians identify patients in need of investigation for malrotation and what investigations can be relied upon in this context. This should help provide timely investigation and management of these children, reducing their risk of morbidity and mortality.