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• W4200288051 abstract "Abstract Background: Neuromuscular choristomas (NMCs) as exquisitely rare developmental lesions have previously been established associated with recurrent fibromatosis after surgery, led to multiple operations, or even amputation. Yet, the report about ultrasound imaging features and clinical conditions of NMCs was rare. The purpose of this study was to describe the ultrasound features and clinical analysis of NMCs to provide suggestions to the optimal management strategy. Methods: From 2020 September to 2021 September, 7 patients with a confirmed diagnosis of NMC who underwent ultrasound examination in our department were enrolled into our study. Physical examinations were performed to detect motor deficit, sensory deficit, neuropathic pain, limb undergrowth, muscular atrophy, cavus foot and bone dysplasia. Ultrasound imaging were performed and investigated in both involved nerve and neuromuscular choristomas associated desmoid-type fibromatosis (NMC-DTF). All patients had a definite history and regular follow up. The clinical course, physical examinations, ultrasound features and pathologic results of NMC patients were analyzed. Results: Seven patients with an average age of 7.0±7.2 years (range: 2-22 years) were enrolled into our study. Nerves involved included the sciatic nerve (6 cases) and the brachial plexus (1 case). 6 patients (85.7%) presented with manifestations of limb undergrowth, 6 (85.7%) with muscular atrophy, and 5 (71.4%) with cavus foot deformity. Based upon ultrasound findings, all visible involved nerve segments presented with hypoechoic and fusiform enlargement with intraneural skeletal muscle elements. Five patients (71.4%) had NMC-DTFs at the site of the affected nerve. All NMC-DTFs were shown as hypoechoic solid lesion adjacent to the nerve and well-circumscribed. In the subset of surgery group, all 5 patients presented with progression to NMC-DTFs at the site of the NMCs. No fibromatosis was detected in the other two non-surgery patients. Conclusions: Understanding of the typical ultrasound features and clinical associated conditions would help to early diagnose the rare disease. When a potential diagnosis is made, invasive procedure like biopsy or resection might be not a good choice given frequent complication by aggressive recurrence." @default.
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• W4200288051 date "2021-12-20" @default.
• W4200288051 modified "2023-09-23" @default.
• W4200288051 title "Clinical Features and Ultrasound Findings of a Rare Musculoskeletal System Disease--neuromuscular Choristomas" @default.
• W4200288051 doi "https://doi.org/10.21203/rs.3.rs-1098730/v1" @default.
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