821 Early Surgical Release of Tethered Cord May Benefit Children with a History of Imperforate Anus

Abstract

INTRODUCTION: Anomalies of the caudal spinal cord that cause tethering often present in children with a history of imperforate anus. We examined the role of early versus delayed surgery in these children. METHODS: A retrospective review at our institution between January 2000 and December 2003 identified eight children with a history of imperforate anus who were referred to neurosurgery for evaluation of tethered cord. Age at diagnosis, presenting symptoms, magnetic resonance imaging (MRI) findings, timing of surgery, and postoperative course were analyzed. RESULTS: Two children who underwent delayed surgery (ages, 3 and 8 yr) after presenting with urinary incontinence and radiculopathy showed no improvement in bladder function after surgery. MRI findings revealed a dermoid tumor in one patient and a filum lipoma in the other. Six children underwent early surgery (between 5 and 18 months of age) after diagnosis by screening MRI shortly after birth. Of note, screening ultrasound at birth was normal in all but one patient. MRI findings included fibrolipomatous infiltration of the filum (four patients), intradural lipoma (three patients), and low-lying conus (three patients). One of these children became symptomatic with progressive scoliosis by the time of surgery; this did not progress postoperatively. The other five patients remained neurologically intact after surgery; one of these patients presented with recurrent tethered cord 2 years later. CONCLUSION: Children born with imperforate anus may develop irreversible neurological deficits caused by a tethered spinal cord. Children who presented after developing neurological deficits did not improve with surgery. Children who underwent early surgery remained neurologically intact. These results suggest that children with imperforate anus may benefit from early MRI screening and early release of tethered cord to avoid permanent neurological sequelae.