Management of Imperforate Anus

Abstract

Anorectal malformations, or imperforate anus, represent a wide range of congenital defects and are associated with other congenital anomalies, including cardiovascular, urologic, gastrointestinal, and musculoskeletal disorders. Appropriate work-up for associated anomalies should be performed prior to definitive management. After the diagnosis of an imperforate anus is made on newborn examination, evaluation for a fistula should be performed, and oftentimes the definitive anatomy is not known until after a fistulogram. In females with a single perineal opening or cloaca, the common length channel should be assessed using cystoscopy, vaginoscopy, and possibly magnetic resonance imaging (MRI). Hydrocolpos should be drained if found. In a patient without a fistula, an abdominal X-ray should be performed to evaluate the distance between the distal gas and the skin. In patients with more complicated anomalies, such as cloaca, recto-urethral fistulas and patients with rectal gas above the coccyx on abdominal X-ray, colostomy should be performed first and definitive repair delayed until further work-up can be performed. Otherwise, superficial fistulas may be managed with anoplasty and deeper fistulas managed with posterior sagittal anorectoplasty in males or posterior sagittal anorectovaginourethroplasty in females.