Miki Fujimura1, Yasuko Kusaka, Reizo Shirane. 1. Department of Neurosurgery, Tohoku University, Graduate School of Medicine, 1-1 Seiryo-machi, 980-8574 Aoba-ku, Sendai, Japan. fujimur@nsg.med.tohoku.ac.jp
Abstract
CASE REPORT: A 2-year-old girl presented with repaired cloacal exstrophy and a subcutaneous mass at the midline of the lumbosacral region. Neurological examination revealed mild paraparesis with bilateral lower-extremity hyperreflexia and sensory disturbance below the level of L4. A CT scan showed asymmetrical spina bifida on the right side extending from L2 to L5, and symmetrical spina bifida below S1. Magnetic resonance imaging showed a low-set conus with terminal syringohydromyelia, lumbosacral lipoma, and the extradural arachnoid cysts at Th12-L4 compressing the spinal dural sac from behind. Surgical removal of the arachnoid cyst and lipoma relieved spinal dural sac compression and the tethered spinal cord. The patient showed an improvement in the lower-extremity hyperreflexia postoperatively. CONCLUSION: The rare association of the cloacal exstrophy with the multiple spinal cord abnormalities as shown in the present case indicates that detailed routine screening of the spinal cord is necessary for the careful planning of treatment in patients with anorectal anomalies.
CASE REPORT: A 2-year-old girl presented with repaired cloacal exstrophy and a subcutaneous mass at the midline of the lumbosacral region. Neurological examination revealed mild paraparesis with bilateral lower-extremity hyperreflexia and sensory disturbance below the level of L4. A CT scan showed asymmetrical spina bifida on the right side extending from L2 to L5, and symmetrical spina bifida below S1. Magnetic resonance imaging showed a low-set conus with terminal syringohydromyelia, lumbosacral lipoma, and the extradural arachnoid cysts at Th12-L4 compressing the spinal dural sac from behind. Surgical removal of the arachnoid cyst and lipoma relieved spinal dural sac compression and the tethered spinal cord. The patient showed an improvement in the lower-extremity hyperreflexia postoperatively. CONCLUSION: The rare association of the cloacal exstrophy with the multiple spinal cord abnormalities as shown in the present case indicates that detailed routine screening of the spinal cord is necessary for the careful planning of treatment in patients with anorectal anomalies.