Cloacal exstrophy: the neurological implications

Congenital anomalies commonly accompany the abdominal wall defects of cloacal exstrophy with an incidence as high as 100% in some series. The urological literature cites an incidence of vertebral and/or spinal cord anomalies in 75% of cases. We reviewed the records of the 17 patients with cloacal exstrophy treated at our institution since 1978. Abnormalities of the vertebral column or spinal cord were identified in 16 patients, including 11 of 12 (92%) fully evaluated by myelography, computerized tomography or magnetic resonance imaging of the lumbosacral spinal cord. Nine patients had a lipomeningocele and 4 had a lipomyelocystocele. Spinal exploration, repair and cord untethering were done in 11 patients of whom 2 subsequently underwent reoperation for re-tethering the cord. A total of 15 patients is ambulatory although 5 require lower extremity braces. The incidence of spinal cord or vertebral anomalies in our patients with cloacal exstrophy approaches 100%, which is higher than commonly cited in the urological literature. We recommend magnetic resonance imaging of the spinal cord as part of the initial evaluation of newborns with cloacal exstrophy. Lifelong attention to the risk of spinal cord tethering can optimize neurological outcome in affected individuals.