Imperforate anus: the neurologic implication of sacral abnormalities

The association of imperforate anus with bony sacral abnormalities and neurologic deficits is well recognized. These neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction. To investigate the frequency of such lesions, routine myelography of imperforate anus patients with sacral anomalies was undertaken. The extraordinarily high incidence of unsuspected lesions known to cause progressive bowel, bladder, and musculoskeletal dysfunction is the basis of this interim report. Thirty percent of patients with anorectal abnormalities had sacral dysplasia. Of the nine patients undergoing myelography to date, eight have been abnormal. Six children had a tethered spinal cord, one had narrowing of the bony spinal canal and dural sac stenosis, and one an anterior meningocele. Spinal cord conditions that may cause deterioration of bowel, bladder, and extremity function should be defined and corrected before irreversible damage occurs. Because of the high incidence of spinal cord lesions detected in these patients with coexisting anorectal and sacral anomalies, routine screening for spinal cord lesions is recommended.