Miraj Faisal, Aprilya Dina
Department of Orthopaedic and Traumatology, Faculty of Medicine Universitas Indonesia, Fatmawati Hospital, Indonesia.
Ann Med Surg (Lond). 2020 Aug 29;58:112-116. doi: 10.1016/j.amsu.2020.08.030. eCollection 2020 Oct.
Congenital pseudoarthrosis of the tibia is a rare congenital disease. Late presentation in adult, makes the diagnostic far more challenging and often misdiagnosed as a common non-union fracture with high re-operation rate. In long courses of repeated surgery, non-union persisted along with severe leg length discrepancy.
A 19-year-old male presented with history of left tibia fracture with repeated surgery. Current problems were progressed bowing of the left lower leg and length discrepancy without recent injury. There was a sign of neurofibroma and pseudoarthrosis at distal third of the tibia shaft with fibula involvement. A radical resection was performed followed by staged deformity correction with Ilizarov's method which was consisted of bone transport procedure in 4 months and lengthening procedure in another 4 months, without grafts.
After one-year, patient achieved union at docking site, equal lower limb length, good alignment and consolidation in bone transport and lengthening site. Three months after frame removal patient has achieved functional bipedal gait with no sign of recurrence.
Diagnostic and therapeutic challenge in the late onset of congenital pseudoarthrosis of tibia is in differentiating it with another cause of non-union and in dealing with deformities. Although none of surgical methods have proven their superiority, reconstruction using Ilizarov method is proved to be safe, practical, and effective to solve both problems. However, the patient still needs to be closely observed and protected weight bearing due to the refractory nature of the disease.
先天性胫骨假关节是一种罕见的先天性疾病。成年后才出现症状,这使得诊断极具挑战性,且常被误诊为常见的骨不连骨折,再次手术率很高。在漫长的反复手术过程中,骨不连持续存在,同时伴有严重的下肢长度差异。
一名19岁男性,有左胫骨骨折并反复手术史。目前的问题是左小腿逐渐出现弓形畸形和长度差异,近期无外伤史。在胫骨干远端三分之一处有神经纤维瘤和假关节迹象,腓骨也受累。进行了根治性切除,随后采用伊里扎洛夫方法分阶段进行畸形矫正,包括4个月的骨搬运过程和另外4个月的延长过程,未进行植骨。
一年后,患者在对接部位实现骨愈合,下肢长度相等,骨搬运和延长部位对线良好且骨痂形成。拆除外固定架三个月后,患者实现了功能性双足步态,无复发迹象。
先天性胫骨假关节晚期的诊断和治疗挑战在于将其与其他骨不连原因相鉴别以及处理畸形。虽然没有一种手术方法已证明其优越性,但使用伊里扎洛夫方法进行重建被证明是安全、实用且有效的,可解决这两个问题。然而,由于该疾病的难治性,患者仍需密切观察并避免负重。