Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA; Department of Department of Plastic, Hand and Reconstructive Surgery, BG Trauma Center Frankfurt am Main, Academic Hospital of the Goethe University Frankfurt am Main, Frankfurt am Main, Germany.
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2019 Sep;129:e761-e766. doi: 10.1016/j.wneu.2019.06.018. Epub 2019 Jun 13.
The natural history of growth and radiologic progression of neuromuscular choristomas (NMCs) remain unknown. The purpose of this study was to describe the radiologic growth pattern of NMCs and to determine how the pattern of growth relates to clinical progression.
A retrospective review was performed for patients with a confirmed diagnosis of NMC and at least 2 years of radiologic (magnetic resonance imaging [MRI]) follow-up. Medical records, including physical examinations and radiologic studies, were reviewed in detail. The NMC length and transverse dimensions were compared between serial MRI examinations.
Eleven patients with a mean radiologic follow-up time of 5.6 years (range 2-19 years) were identified. Motor deficits occurred in 10 patients (90%), sensory deficits in 5 patients (45%), and neuropathic pain in 4 (36%) patients. Eight patients (73%) presented with manifestations of limb undergrowth, 2 (18%) with congenital hip dysplasia, and 1 with a cavus foot deformity. Progression of motor and sensory deficits was observed in 5 (45%) and 1 (9%) patients, respectively. The maximal length and height of the NMC was significantly (P < 0.05) longer (initial 218 ± 118 mm vs. follow-up 270 ± 135 mm) and larger (20 ± 10 mm vs. 24 ± 14 mm) on the follow-up scan. MRI demonstrated abnormalities that were in continuity along the longitudinal extent of the NMC.
According to this small but relatively long-term follow-up cohort, the growth pattern of this lesion is slow but progressive. We found a longitudinal continuity pattern of growth in all MRI scans, often spanning a great distance.
神经肌肉性错构瘤（NMC）的生长和放射学进展的自然史尚不清楚。本研究的目的是描述 NMC 的放射学生长模式，并确定生长模式与临床进展的关系。
对经证实患有 NMC 且至少有 2 年放射学（磁共振成像[MRI]）随访的患者进行回顾性研究。详细回顾了病历，包括体格检查和放射学研究。比较了连续 MRI 检查中 NMC 的长度和横径。
确定了 11 例患者，平均放射学随访时间为 5.6 年（范围 2-19 年）。10 例患者（90%）出现运动功能缺损，5 例（45%）出现感觉功能缺损，4 例（36%）出现神经性疼痛。8 例（73%）患者表现为肢体生长不足，2 例（18%）患者表现为先天性髋关节发育不良，1 例患者表现为高弓足畸形。5 例（45%）患者的运动和感觉功能缺损进展，1 例（9%）患者进展。NMC 的最大长度和高度在随访扫描时明显（P<0.05）更长（初始 218±118mm 比随访时 270±135mm）和更大（20±10mm 比随访时 24±14mm）。MRI 显示病变沿 NMC 的纵向延伸具有连续性异常。
根据这一小但相对长期的随访队列，该病变的生长模式缓慢但呈进行性。我们发现所有 MRI 扫描中都存在纵向连续性生长模式，常常跨越很大的距离。
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