Department of Plastic, Reconstructive and Hand Surgery, Inselspital, University of Bern, Bern, Switzerland.
J Plast Reconstr Aesthet Surg. 2010 Oct;63(10):1699-704. doi: 10.1016/j.bjps.2009.09.016. Epub 2009 Nov 12.
Congenital pseudarthrosis of the tibia (CPT) is caused by an ill-defined, segmental disturbance of periosteal bone formation leading to spontaneous bowing, followed by fracture and subsequent pseudarthrosis in the first 2 years of life. The results of conventional treatment modalities (e.g., bracing, internal and external fixation and bone grafting) are associated with high failure rates in terms of persisting pseudarthrosis, malunion and impaired growth. As a more promising alternative, a more aggressive approach, including wide resection of the affected bone, reconstruction with free vascularised fibula grafts from the healthy contralateral leg and stable external fixation at a very early stage has been suggested. Between 1995 and 2007, 10 children (age 12-31 months, median 20 months) suffering from CPT were treated at our institutions according to this principle. Two patients were treated before a fracture had occurred. The length of the fibula graft was 7-9cm. End-to-end anastomoses were performed at the level of the distal tibia stump. The follow-up was 80 months (median, range 12 months to 12 years). Radiologic examination at 6 weeks postoperatively showed normal bone density and structure of the transplanted fibula in all cases and osseous consolidation at 19 of the 20 graft/tibia junctions. One nonunion was sucessfully treated with bone grafting and plate osteosynthesis. Pin-tract infection occurred in three patients. Five children sustained graft fractures that were successfully treated with internal or external fixation. Two patients developed diminished growth of the affected limb or foot; all others had equal limb length and shoe size. At long-term follow-up, tibialisation of the transplant had occurred, and normal gait and physical activities were possible in all children. We conclude that in spite of a relatively high complication rate and the reluctance to perform free flap surgery in infants at this young age, the present concept may successfully prevent the imminent severe sequelae associated with CPT.
先天性胫骨假关节（CPT）是由一种定义不明确的、节段性的骨膜骨形成障碍引起的，导致自发性弯曲，随后在生命的头 2 年内发生骨折和随后的假关节。传统治疗方法（如支具、内外固定和植骨）的结果与持续的假关节、愈合不良和生长受损的高失败率有关。作为一种更有前途的替代方法，一种更激进的方法，包括受影响骨的广泛切除、来自健康对侧腿的游离带血管腓骨移植重建和非常早期的稳定外固定，已经被提出。1995 年至 2007 年间，我们机构根据这一原则治疗了 10 名患有 CPT 的儿童（年龄 12-31 个月，中位数 20 个月）。两名患者在骨折发生前接受了治疗。腓骨移植的长度为 7-9cm。在胫骨残端的远端进行端端吻合。随访时间为 80 个月（中位数，范围 12 个月至 12 年）。术后 6 周的放射学检查显示所有病例移植腓骨的骨密度和结构正常，20 个移植/胫骨连接处中有 19 个有骨融合。1 例非愈合患者成功接受植骨和钢板内固定治疗。3 例患者出现针道感染。5 例儿童发生移植骨骨折，均成功采用内固定或外固定治疗。2 例患儿出现受累肢体或足部生长发育不良；所有其他患者的肢体长度和鞋码均相等。长期随访时，移植腓骨发生胫骨化，所有儿童均能正常行走和进行体育活动。我们的结论是，尽管并发症发生率相对较高，而且在这么小的年龄不愿意对婴儿进行游离皮瓣手术，但目前的概念可能成功地预防了与 CPT 相关的严重后遗症。
