Inoue Mikihiro, Uchida Keiichi, Otake Kohei, Nagano Yuka, Shimura Tadanobu, Hashimoto Kiyoshi, Matsushita Kohei, Koike Yuhki, Matsubara Toshio, Kusunoki Masato
Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Edobashi 2-174, Tsu, Mie, 514-8507, Japan.
Department of Neurosurgery, Mie University Graduate School of Medicine, Edobashi 2-174, Tsu, Mie, 514-8507, Japan.
Pediatr Surg Int. 2017 Sep;33(9):995-999. doi: 10.1007/s00383-017-4127-y. Epub 2017 Aug 4.
Anorectal malformation (ARM) is associated with a tethered spinal cord (TSC). Long-term functional outcome of untethering surgery for TSC in patients with ARM has not been well evaluated.
Patients aged 7 years and older who underwent repair of ARM and spinal magnetic resonance imaging from January 1995 to December 2008 were reviewed retrospectively. Untethering surgery was performed in all patients who were diagnosed with TSC, regardless of the presence or of neurological symptoms. Clinical symptoms reflecting anorectal, urinary, and lower limb function were compared between patients complicated with TSC (TSC group, n = 17) and those without TSC (non-TSC group, n = 14).
The median age at functional evaluation was 11.7 and 12.9 years in the TSC and non-TSC groups, respectively (p = 0.52). Untethering surgery for TSC was performed at a median age of 1.3 years. Preoperative urinary and lower limb dysfunction, except for vesicoureteral reflux in the TSC group in one patient, was improved after surgical detethering. Current anorectal function was comparable between the groups.
Long-term functional outcome in patients with ARM and TSC undergoing untethering surgery is equivalent to that in those without TSC. Prophylactic surgical detethering for patients with ARM and TSC can be a treatment of choice to maximize neurological functional outcome.
肛门直肠畸形（ARM）与脊髓栓系（TSC）有关。对于患有ARM的患者，TSC松解手术的长期功能预后尚未得到充分评估。
回顾性分析1995年1月至2008年12月期间接受ARM修复和脊髓磁共振成像检查的7岁及以上患者。所有诊断为TSC的患者均接受了松解手术，无论是否存在神经症状。比较了合并TSC的患者（TSC组，n = 17）和未合并TSC的患者（非TSC组，n = 14）之间反映肛门直肠、泌尿和下肢功能的临床症状。
TSC组和非TSC组功能评估的中位年龄分别为11.7岁和12.9岁（p = 0.52）。TSC松解手术的中位年龄为1.3岁。手术松解后，除TSC组有1例患者存在膀胱输尿管反流外，术前的泌尿和下肢功能障碍均得到改善。两组目前的肛门直肠功能相当。
接受松解手术的ARM合并TSC患者的长期功能预后与未合并TSC的患者相当。对于ARM合并TSC的患者，预防性手术松解可能是使神经功能预后最大化的一种治疗选择。
