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Ⅰ.はじめに
先天性皮膚洞(congenital dermal sinus)は神経管の閉鎖不全によって生じる比較的稀な疾患で,硬膜内の感染症の原因となることはよく知られている7).これらを認知せずに放置した場合,重篤な神経脱落症状を来すことがあるため1,12),機能予後の改善のためには早期の発見と積極的な加療が重要である.
今回,われわれはdermal sinus(皮膚洞)に起因した脊髄髄内外巨大膿瘍の1例を経験した.本症例は,先天性dermal sinusを合併した児の神経学的予後を改善する上で重要な症例と考えられたので,若干の文献的考察を加えて報告する.
Congenital dermal sinuses, resulting from abnormal neurulation, are relatively uncommon and cause the patients to contract intraspinal infection. Sometimes, this intraspinal infection develops intramedurally abscess with severe neurological deficits. Therefore, a lack of awareness about this problem can result in fatal neurological sequelae.
We report the case of a 1-year-old boy who had a lumbo-sacral dimple at birth without further evaluation. He presented repeated fever and rapidly progressive paraplegia. MRI showed a huge intraspinal and intramedullary abscess with dermoid, which was thought to be the result of a dermal sinus. Subsequently, he was transferred to our hospital and received immediate surgery. The patient underwent irrigation of purulent material in the intraspinal abscess including on intramedullary lesion, removal of dermoid tumor, and the resection of the dermal sinus. After that, he was treated with 8 weeks of antibiotic therapy. The patient remained paraplegia but made improvement.
This patient illustrates the importance of the recognition and evaluation of skin markers. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.
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