Lampasi Manuele, Greggi Tiziana, Sudanese Alessandra
Department of Paediatric Orthopaedics and Traumatology, Istituto Ortopedico Rizzoli, Via Pupilli 1, Bologna, Italy.
Chir Organi Mov. 2008 Apr;91(3):163-6. doi: 10.1007/s12306-007-0027-y. Epub 2008 May 21.
The Authors report the case of a 28-year-old patient with type 1 neurofibromatosis, who presented a pathological dislocation of the hip. This event was preceded by the development of a neurological pattern of spastic paraparesis and decreased pain sensation in both lower limbs, secondary to the progression of a dystrophic kyphoscoliosis. Pathological dislocation of the hip in neurofibromatosis has been reported in the literature as a consequence of intra-articular growth of neurofibromas. Conversely, in the case described here, clinical and radiographic features and the absence of neurofibromas in the joint, verified by means of radiographic and histological examinations, suggested the diagnosis of neuropathic arthropathy. The treatment of hip dislocation should be differentiated according to the aetiology, given the underlying articular instability in cases of neuropathic arthropathy.
作者报告了一例28岁的1型神经纤维瘤病患者,该患者出现了髋关节病理性脱位。在此之前,由于营养不良性脊柱后凸侧弯的进展,出现了痉挛性截瘫的神经学模式以及双下肢疼痛感觉减退。文献报道神经纤维瘤病中的髋关节病理性脱位是神经纤维瘤关节内生长的结果。相反,在本文所述病例中,通过影像学和组织学检查证实的临床和影像学特征以及关节内无神经纤维瘤,提示为神经性关节病。鉴于神经性关节病病例存在潜在的关节不稳定,髋关节脱位的治疗应根据病因进行区分。