Approximately 75% of patients with classic malrotation and midgut volvulus present in neonatal age-group; another 15% may present up to one year. In these, the diagnosis is made because of typical presentation. However, there are many cases in which malrotation has been detected after infancy. In-spite of the variable case reports and studies in literature, there is no specific data on the incidence of malrotation after infancy, its presentation and management guidelines. With this systematic review, we aim to coalesce these findings from various individual case reports and studies. This systematic review was performed as per the PRISMA (preferred reporting items for systematic review and meta-analyses) guidelines. The authors conducted thorough searches in databases like PubMed/MEDLINE, SCOPUS, Google Scholar and Web of Science for relevant articles. Studies were reviewed for clinical presentation and outcome for malrotation diagnosed beyond infancy. Clinical presentation of malrotation after infancy can be acute as well as chronic; acute presentation being common under 2 years of age and chronic symptoms present as age increases. Sudden onset of abdominal pain and bilious vomiting are commonest acute symptoms and intermittent episodes of recurring vague abdominal pain associated with vomiting and diarrhoea are the commonest chronic symptoms. The diagnostic modalities vary from UGI Contrast series to USG with colour Doppler and CECT scan. Management is operative open as well as laparoscopic Ladd's procedure is being favoured.
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