先天性对疼痛和无汗不敏感:一个无痛儿童的痛苦经历

Sia Ung, Z. Osman, Nur Syahida Termizi, Mohd Firdaus Hafni Bin Ahmad
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引用次数: 0

摘要

一个三岁的男孩先天性疼痛不敏感和无汗症(CIPA)首次提出了经骨骺分离左肱骨远端。肱骨远端椎弓根分离闭合复位和k-钢丝治疗导致手术部位严重感染和骨髓炎。多次手术清创和应用外固定架消除感染无效。四岁时,由于左髋关节脱臼,他拒绝负重。闭合复位和spica石膏不成功,导致复发性左髋关节脱位。从那以后,他就不能走路了。5岁时,右髋关节脱位,随后发生同侧股骨粗隆下骨折。由于右大腿的大面积瘀伤和皮肤溃疡,不可能进行适当的治疗,如髋关节钉和钛弹性钉。这名儿童的左上肢功能丧失,双侧髋关节脱位,右股骨骨折不愈合。保守治疗和谨慎忽视可能是CIPA患者的理想治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Congenital Insensitivity to Pain and Anhidrosis: A painful experience by a painless child
A three-year-old boy with congenital insensitivity to pain and anhidrosis (CIPA) was first presented with transphyseal separation of distal left humerus. Surgical treatment with closed reduction and k-wiring of transphyseal separation of distal humerus resulted in devastating surgical site infection and osteomyelitis. Multiple surgical debridement and application of external fixator to eliminate infection were futile. At the age of four years, he refused to bear weight as a result of left hip dislocation. Closed reduction and spica cast was unsuccessful, resulting in recurrent left hip dislocation. He has since not been able to walk. At the age of five years, the right hip was dislocated followed by ipsilateral subtrochanteric femur fracture. Appropriate treatments such as hip spica and titanium elastic nail were not possible due to extensive bruising and skin ulceration over the right thigh. This child ended up with a functionless left upper limb, bilateral hip dislocation and malunited right femur fracture. Conservative treatment and watchful neglect might be the ideal treatment for patients with CIPA.
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