Anorectal malformations (ARMs) commonly co-occur with other congenital anomalies, particularly VACTERL (vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb, and duodenal) associations. However, this collection of associations is not comprehensive, and other concurrent anomalies may exist that can be missed during the standard work-up of patients with ARMs. We present a rare case of a neonate with a low ARM with concurrent jejuno-ileal atresia that was diagnosed after the correction of the ARM when the patient developed segmental volvulus. This case illustrates the importance of having a high index of suspicion when deviation from a classic presentation occurs.

A 57-year-old woman with non-insulin-dependent diabetes mellitus and no other medical history had worsening left-sided back pain over 4 months and no other symptoms. Physical examination findings were significant for mild left flank pain, but there was no costovertebral angle tenderness. Laboratory results were within normal limits. Axial imaging demonstrated an exophytic, retroperitoneal mass with solid and cystic components involving the left kidney, posterior abdominal wall, and mesocolon (Figure 1). The mass measured 20 cm in its greatest dimension. Findings on colonoscopy and mammography were negative. Positron emission tomography-computed tomography did not reveal any other abnormality.Quiz at jamasurgery.com