Kulkarni Ruta M, Arora Nitish, Saxena Sagar, Kulkarni Sujay M, Saini Yadwinder, Negandhi Rajiv
Department of Orthopaedics, Post Graduate Institute of Swasthiyog Pratishthan, Maharashtra, India.
J Pediatr Orthop. 2019 Oct;39(9):e708-e717. doi: 10.1097/BPO.0000000000001012.
Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities.
We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014. Among the 29 patients, 27 were treated with reconstructive procedures. Type 1 (8 patients) cases were treated with only limb lengthening, and correction of tibial deformities. Type 2 (7 patients) cases were treated by distal tibial medial hemiepiphysiodesis or supramalleolar varus osteotomy. In type 3 (10 patients) cases, the foot deformity was corrected using the SUPERankle procedure. Type 4 (2 patients) cases were treated with supramalleolar osteotomy along with posteromedial release and lateral column shortening. In a second stage, limb lengthening was performed, using the Ilizarov technique. In the remaining 2 patients (type 3A and type 3C), amputation was performed using Syme technique as a first choice of treatment.
The results were evaluated using Association for the Study and Application of Methods of Ilizarov scoring. Excellent results were obtained in 15 of 27 (55%) patients. Six (22%) patients had good results, 4 (14.8%) had fair results, and 2 (7%) had poor results. Mean limb length discrepancy at initial presentation was 3.55 cm (range: 2 to 5.5 cm) which significantly improved to 1.01 cm (range: 0 to 3 cm) after treatment (P=0.015).
Our results and a review of the literature clearly suggest that limb reconstruction according to Paley classification, is an excellent option in the management of fibular hemimelia. Our 2-staged procedure (SUPERankle procedure followed by limb lengthening) helps in reducing the complications of limb lengthening and incidence of ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities.
Level IV.
腓骨半侧发育不全是最常见的长骨发育缺陷。帕利分类法基于踝关节形态，确定基本病理情况，并有助于制定手术治疗方案。重建手术包括矫正足部畸形和均衡肢体长度。SUPERankle手术是一种骨与软组织联合手术，可稳定足部并矫正所有畸形。
我们回顾性分析了2000年12月至2014年12月期间接受手术治疗的29例连续患者（29个肢体节段）。在这29例患者中，27例接受了重建手术。1型（8例）仅行肢体延长及胫骨畸形矫正。2型（7例）采用胫骨远端内侧半骨骺阻滞术或内踝上内翻截骨术治疗。3型（10例）采用SUPERankle手术矫正足部畸形。4型（2例）采用内踝上截骨术联合后内侧松解及外侧柱缩短术治疗。在第二阶段，采用伊里扎洛夫技术进行肢体延长。其余2例（3A型和3C型）患者首选赛姆截肢术。
采用伊里扎洛夫方法研究与应用协会评分法评估结果。27例患者中有15例（55%）获得了优异的结果。6例（22%）患者结果良好，4例（14.8%）结果一般，2例（7%）结果较差。初次就诊时平均肢体长度差异为3.55厘米（范围：2至5.5厘米），治疗后显著改善至1.01厘米（范围：0至3厘米）（P = 0.015）。
我们的结果以及文献回顾清楚地表明，根据帕利分类法进行肢体重建是治疗腓骨半侧发育不全的极佳选择。我们的两阶段手术（先进行SUPERankle手术，然后进行肢体延长）有助于减少肢体延长的并发症和踝关节僵硬的发生率。在较早年龄（5岁以下）进行首次手术对预防复发性足部畸形起着重要作用。
四级。
