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Surgical management of congenital malrotation presenting in adulthood
Jacob Holzemer1, Krishna Patel1, Oscar Ruiz2
1General Surgery, Riverside Methodist Hospital, Columbus, Ohio, USA.
Insights
Intestinal malrotation, a congenital abnormality, affects neonates but can present in adults with intermittent symptoms. Early diagnosis in adults is crucial to prevent increased morbidity from delayed detection.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Embryology
Background:
- Intestinal malrotation stems from incomplete embryonic development and abnormal fixation of the intestines.
- It is characterized by a failure in the normal 270° rotation around the superior mesenteric artery axis.
Observation:
- While common in neonates (1 in 500), symptomatic cases are rare (1 in 6000), often presenting in the first year.
- Adult presentation (0.2% incidence) typically involves intermittent postprandial symptoms like abdominal pain, bilious emesis, and malabsorption.
Findings:
- Adults with malrotation rarely experience acute obstruction or volvulus, with fewer than 100 cases documented.
- A high index of suspicion is necessary for diagnosing adult intestinal malrotation.
Implications:
- Delayed diagnosis in adults significantly increases morbidity due to the potential for acute complications.
- Recognizing adult malrotation is vital for timely intervention and improved patient outcomes.
Abstract:
Intestinal malrotation is characterised by positional and congenital fixation abnormalities resulting from a failure in embryonic development in the normal 270° rotation around the axis of the superior mesenteric artery. Intestinal malrotation is primarily thought to affect neonates with an incidence of 1 in 500, however, only 1 in 6000 live births are symptomatic, and these usually present within the first month of life in 40% of cases and within the first year in over 5% of cases as an obstructive pathology or volvulus. In adults, however, the incidence has been documented at 0.2%. These patients usually present with postprandial symptoms that are intermittent such as bilious emesis, abdominal pain and malabsorption. Rarely, adults with congenital malrotation may present with acute obstruction due to volvulus, however, less than 100 adult cases are described in the literature. A high index of suspicion is required to identify cases of malrotation in the adult patient which leads to delays in diagnosis and therefore increases in morbidity can be seen.
