Evaluation of tethered cord syndrome associated with anorectal malformations

The clinical courses of four patients with occult spinal dysraphism who were also found to have anorectal malformations and tethered cord syndrome are evaluated in this report. These patients were among 27 patients with occult spinal dysraphism treated in the Division of Neurosurgery of Tohoku University School of Medicine during the last 3 years. All four patients experienced urinary disturbances early in life, and initially, these disturbances were considered to be caused by urogenital malformations. As a result, treatments were delayed. Only one patient with male Turner's syndrome exhibited mental retardation. Other symptoms, such as sensory disturbance and pain in the lower extremities, became apparent from complaints by the patients without atresia ani. When surgery was performed earlier in our patients with occult spinal dysraphism, the outcome was better. We believe in early surgery for tethered cord syndrome, although there is no consensus for the timing of this surgery. Unfortunately, the diagnosis of tethered cord syndrome was delayed in our four patients. However, because occult spinal dysraphism and anorectal malformations seem to be closely related, the presence of anorectal malformations may result in an earlier diagnosis of tethered cord syndrome.