García-González Oscar, Mireles-Cano J Nicolás, Sánchez-Zavala Natalia, Chagolla-Santillan Miguel A, Orozco-Ramirez Segio M, Silva-Cerecedo Pedro, Murguia-Perez Mario, Rueda-Franco Fernando
Hospital Regional de Alta especialidad del Bajío, León, Guanajuato, Mexico.
Department of Pediatric Neurosurgery, Hospital Regional de Alta Especialidad del Bajío, Blvd. Milenio 130, 37660, León, Guanajuato, Mexico.
Childs Nerv Syst. 2018 Mar;34(3):565-569. doi: 10.1007/s00381-017-3645-1. Epub 2017 Nov 11.
The purpose of the report is to describe a patient with hereditary osteochondromatosis and spinal cord compression at the thoracic level.
An 8-year-old patient with hereditary osteochondromatosis inherited from his father presented paraparesis in the left foot, leading to complete paralysis in both legs.
In a CT scan, a bony tumor rising from the posterior wall of the T3 body narrowing the spinal canal, and the MRI spinal cord compression at the same level and the hydrosyringomyelic cavity extended to the conus medullaris; with an anterior thoracic approach to T2-T4, the fibro-cartilaginous tumor was removed, and the stabilization was completed with bone graft and a plate. Two months after surgery, the patient recovered strength in both legs.
A detailed family history through examination-guided advanced imaging and biopsy provides useful information for diagnosis and appropriate management of occupative lesions in patients affected with multiple hereditary exostosis.
本报告旨在描述一名患有遗传性骨软骨瘤病并伴有胸段脊髓受压的患者。
一名8岁患者，从父亲那里遗传了遗传性骨软骨瘤病，出现左脚轻瘫，最终双腿完全瘫痪。
在CT扫描中，一个从T3椎体后壁长出的骨肿瘤使椎管变窄，MRI显示同一水平脊髓受压，并且积水性脊髓空洞症延伸至圆锥；采用经胸前路T2 - T4手术，切除纤维软骨瘤，并用骨移植和钢板完成固定。术后两个月，患者双腿恢复力量。
通过检查引导下的高级影像学检查和活检获取详细家族史，可为诊断和妥善处理患有多发性遗传性骨软骨瘤病患者的占位性病变提供有用信息。
