Abstract
Introduction Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations,
such as intestinal rotation abnormalities. However, there is no standard evaluation
and treatment of intestinal rotation during initial CDH repair. This study evaluates
intestinal malrotation in CDH patients.
Materials and Methods All patients with a CDH treated in a high-volume center between 2000 and 2015 were
retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical
treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus)
were described.
Results A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation
was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but
82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2–16
years), 12 additional malrotations were diagnosed, leading to a prevalence of at least
45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which
three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery,
compared to 3% of the 76 initially diagnosed patients (p < 0.001).
Conclusion Malrotation is associated with CDH, with a prevalence of at least 45% and in 21%
a normal intestinal rotation. “Missed” malrotations have a higher risk on need for
acute surgery later in life.
Keywords
congenital diaphragmatic hernia - malrotation - small bowel obstruction
