Kim Eun Ji, Wang Kyu-Chang, Lee Ji Yeoun, Phi Ji Hoon, Park Sung-Hye, Cheon Jung-Eun, Jang Young Eun, Kim Seung-Ki
Divisions of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
J Neurosurg Pediatr. 2013 Jan;11(1):82-6. doi: 10.3171/2012.9.PEDS12245. Epub 2012 Oct 19.
Infantile myofibromatosis, a rare mesenchymal disorder that develops in early childhood, is classified by the number of lesions that occur: solitary or multicentric. Involvement of the CNS is unusual in either type. Infantile myofibromatosis in the spine is exceptional, and most published cases represent a secondary invasion. Here, the authors report on an 8-month-old girl presenting with weakness below the ankle and an intraspinal mass extending from T-6 to the conus. The patient underwent only partial surgical removal of the lesion, and the pathology was confirmed as infantile myofibromatosis. After the operation, weakness in the lower extremities gradually improved; however, she could not walk at the time of the final follow-up. On follow-up MRI performed 19 months after the operation, the residual lesion remained unchanged with decreased enhancement.
婴儿肌纤维瘤病是一种罕见的间充质疾病，发生于儿童早期，根据出现的病变数量分为：孤立性或多中心性。两种类型中中枢神经系统受累均不常见。脊柱婴儿肌纤维瘤病极为罕见，大多数已发表的病例为继发性侵犯。在此，作者报告一名8个月大的女孩，表现为踝关节以下无力，脊髓内肿块从T-6延伸至圆锥。患者仅接受了病变的部分手术切除，病理确诊为婴儿肌纤维瘤病。术后下肢无力逐渐改善；然而，在最后一次随访时她仍不能行走。术后19个月进行的随访MRI显示，残留病变无变化，强化减弱。
