Association of anorectal malformation and intestinal malrotation.

Background: Patients born with anorectal malformations (ARM) frequently have other congenital anomalies that are well-defined; however, limited data exist examining the relationship of ARM with malrotation.

Methods: A 10-year retrospective review was performed to examine all patients treated at a regional children's medical center with a diagnosis of ARM. Data were collected to identify malrotation, vertebral, anorectal, cardiac, tracheo-esophageal fistula, renal, radial, limb (VACTERL) anomalies, the type of ARM, operative procedures performed, and long-term bowel management.

Results: One hundred forty-six patients were identified. Upper gastrointestinal evaluation was performed in 21 patients (14.4%), while contrast enemas were performed in 22 patients (15.1%). Seven patients were found to have malrotation (4.8%) and 6/7 of these patients had 2 or more VACTERL anomalies.

Conclusions: Patients with ARM and 2 or more VACTERL anomalies should undergo screening for malrotation. Patients with intestinal malrotation, ARM, and poor potential for bowel control should have their appendix preserved during a Ladd's procedure.