総排泄腔外反に合併した脊髄病変の検討

The Study of Spinal Abnormalities Associated with Cloacal Exstrophy

Ⅰ．はじめに

　総排泄腔外反は20～25万人に1人発生する非常に稀な疾患である^13,14,16)． 同疾患には尿路，性器，消化器系とともに神経系にも異常が合併することが知られている^7,13-15)．通常，脊髄病変の状態により手術適応を決定するが，総排泄腔外反に合併する脊髄病変は症例数が少なく自然経過も知られていない．今回，われわれは自験症例について，文献的考察を加えて報告する．

　The treatment of spinal abnormalities with cloacal exstrophy is controversial. Ten cases of this complex treated at Hyogo Prefectural Kobe Children's Hospital between 1991 and 2010 are presented. In our series, all 10 patients had tethered spinal cords. In addition, there were 3 terminal myelocystoceles, 2 meningoceles, 7 lipomas, 5 thickened filums and 3 syrinxes. Eight of 10 patients underwent surgery, and no patient deteriorated. All 3 patients with terminal myelocystocele had lower extremity weakness, but motor functions in two patients improved after surgery. All 4 patients with lipoma and/or thickened filum were asymptomatic. Two of them were conservatively treated, and they remain asymptomatic.

　Terminal myelocystocele and symptomatic syrinx should be surgically treated.