脊髄空洞症を呈したCurrarino症候群の一手術例

A Surgical Case of Currarino Syndrome with Syringomyelia

Currarino症候群は直腸肛門奇形，仙骨奇形，仙骨前腫瘤を三徴とする希少疾患である。今回，脊髄空洞症を呈したCurrarino症候群の一手術例を報告する。症例は2歳2カ月の女児。4カ月検診で殿部のdimpleの精査の結果，仙骨奇形，脊髄前髄膜瘤を認め，Currarino症候群と診断した。2歳1カ月時に著明な便秘，慢性膀胱炎，下肢の脱力が見られ，脊髄前髄膜瘤の直腸圧迫と脊髄空洞症に伴う症状と判断し，髄膜瘤基部の離断と脊髄係留の解除術を実施した。術後1週間で下肢の脱力，便秘は改善した。Currarino症候群は診断時の症状は軽度だが，画像や神経症状の有無をフォローし適切な時期に手術する必要がある。

Abstract

Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome.

(Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023)