Sing Quillan Young, Pai Ashwin Alke, Geeroms Maxim, Cha Soo-Min, Lin Chih-Hung
Swanseabay University Hospital, Swansea, United Kingdom.
Chang Gung Memorial Hospital, Linkou, Taiwan.
JPRAS Open. 2024 Feb 21;40:305-319. doi: 10.1016/j.jpra.2024.02.007. eCollection 2024 Jun.
Congenital pseudoarthrosis of the tibia (CPT) is a rare pathological disease associated with neurofibromatosis type 1 (NF1). It presents with tibial bowing and can progress into a nonhealing fracture. Treatment options include conservative approaches such as serial bracing or various surgical options.Surgically, the aims are to achieve long-term bone union, prevent limb length discrepancies (LLDs), and avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture.The purpose of our study is to highlight our experience with both the conservative approach and the use of vascularized free fibula reconstruction of these deformities, including the challenges encountered with a long-term follow-up until skeletal maturity.
We present a retrospective analysis of a total of nine (9) patients consisting of three (3) girls and six (6) boys. Six (6) children were treated with a vascularized fibula flap, and the other three (3) were treated conservatively. Outcomes measured included fractures, LLD, ankle valgus deformity, donor site morbidity, and number of surgical corrections.
All patients had flap survival. Three (3) of six children had a previous failed surgery with intramedullary nail and bone graft prior to performing a vascularized free fibula reconstruction. The follow-up period ranged from 8 months to 200 months. The complications included stress fractures (50%), LLD (66.6%), and ankle valgus (33.3%). During growth phases, these children required multiple corrective surgeries.
Fibula free flap is a good treatment option for CPT even in patients with prior surgical failures with variable results. - Level 4 - Case series Therapeutic Studies-Investigating the Results of Treatment.
先天性胫骨假关节(CPT)是一种与1型神经纤维瘤病(NF1)相关的罕见病理性疾病。其表现为胫骨弯曲,并可发展为不愈合骨折。治疗选择包括保守方法,如系列支具治疗或各种手术选择。在手术方面,目标是实现长期骨愈合,预防肢体长度差异(LLD),并避免机械轴偏差、软组织损伤、附近关节僵硬和病理性骨折。我们研究的目的是突出我们在这些畸形的保守治疗方法以及使用带血管游离腓骨重建方面的经验,包括直至骨骼成熟的长期随访中遇到的挑战。
我们对总共9例患者进行了回顾性分析,其中包括3名女孩和6名男孩。6名儿童接受了带血管腓骨瓣治疗,另外3名接受了保守治疗。测量的结果包括骨折、LLD、踝关节外翻畸形、供区并发症和手术矫正次数。
所有患者的皮瓣均存活。6名儿童中有3名在进行带血管游离腓骨重建之前曾接受过髓内钉和植骨手术但失败。随访期为8个月至200个月。并发症包括应力性骨折(50%)、LLD(66.6%)和踝关节外翻(33.3%)。在生长阶段,这些儿童需要多次矫正手术。
即使对于先前手术失败且结果各异的CPT患者,游离腓骨瓣也是一种很好的治疗选择。- 4级 - 病例系列治疗研究 - 调查治疗结果。