An operative approach to address severe genu valgum deformity in the Ellis-van Creveld syndrome

Background

The genu valgum deformity seen in the Ellis-van Creveld syndrome is one of the most severe angular deformities seen in any orthopaedic condition. It is likely a combination of a primary genetic-based dysplasia of the lateral portion of the tibial plateau combined with severe soft-tissue contractures that tether the tibia into valgus deformations. Progressive weight-bearing induces changes, accumulating with growth, acting on the initially distorted and valgus-angulated proximal tibia, worsening the deformity with skeletal maturation. The purpose of this study is to present a relatively large case series of a very rare condition that describes a surgical technique to correct the severe valgus deformity in the Ellis-van Creveld syndrome by combining extensive soft-tissue release with bony realignment.

Methods

A retrospective review examined 23 limbs in 13 patients with Ellis-van Creveld syndrome that were surgically corrected by two different surgeons from 1982 to 2011. Seven additional patients were identified, but excluded due to insufficient chart or radiographic data. A successful correction was defined as 10° or less of genu valgum at the time of surgical correction. Although not an outcomes study, maintenance of 20° or less of genu valgum was considered desirable. Average age at surgery was 14.7 years (range 7–25 years). Clinical follow-up is still ongoing, but averages 5.0 years (range 2 months to 18 years). Charts and radiographs were reviewed for complications, radiographic alignment, and surgical technique. The surgical procedure was customized to each patient’s deformity, consisting of the following steps:

1. Complete proximal to distal surgical decompression of the peroneal nerve
2. Radical release and mobilization of the severe quadriceps contracture and iliotibial band contracture
3. Distal lateral hamstring lengthening/tenotomy and lateral collateral ligament release
4. Proximal and distal realignment of the subluxed/dislocated patella, medial and lateral retinacular release, vastus medialis advancement, patellar chondroplasty, medial patellofemoral ligament plication, and distal patellar realignment by Roux-Goldthwait technique or patellar tendon transfer with tibial tubercle relocation
5. Proximal tibial varus osteotomy with partial fibulectomy and anterior compartment release
6. Occasionally, distal femoral osteotomy

Results

In all cases, the combination of radical soft-tissue release, patellar realignment and bony osteotomy resulted in 10° or less of genu valgum at the time of surgical correction. Complications of surgery included three patients (five limbs) with knee stiffness that was successfully manipulated, one peroneal nerve palsy, one wound slough and hematoma requiring a skin graft, and one pseudoarthrosis requiring removal of hardware and repeat fixation. At last follow-up, radiographic correction of no more than 20° of genu valgum was maintained in all but four patients (four limbs). Two patients (three limbs) had or currently require revision surgery due to recurrence of the deformity.

Conclusion

The operative approach presented in this study has resulted in correction of the severe genu valgum deformity in Ellis-van Creveld syndrome to 10° or less of genu valgum at the time of surgery. Although not an outcomes study, a correction of no more than 20° genu valgum has been maintained in many of the cases included in the study. Further clinical follow-up is still warranted.

Level of evidence

IV.     

Clinical outcome and correction following V osteotomy for genu valgum in adolescents and young adults: A systematic review

BackgroundGenu Valgum usually originates from distal femur and many open and closed wedge osteotomies of distal femur have been reported but none has proved to be the best. Distal femur V osteotomy is a wedgeless osteotomy and is a rarely reported osteotomy with only a few case series and 167 cases of isolated distal femur V osteotomy for genus valgum in young patients (<25 years) in literature but with a tendency to have an excellent outcomes.PurposeTo determine correction and clinical outcome following V osteotomy for genu valgum in adolescents and young adults (10–25 years).Patient and methodsA systematic research was conducted of PubMed, MEDLINE and Google Scholar to identify studies reporting the correction and clinical outcome following V osteotomy in adolescents and young adults (10–25 years) for genu valgum by 2 different authors according to PRISMA guidelines.Results5 studies with 167 patients and 263 knees were identified meeting the inclusion criteria for review. The follow-up period ranged from 3 months to 36 months. Overall complication rate was 12.3%, the most common being plaster sore, 4.2% (11/263) followed by superficial infection, 2.7% (7/263). The mean correction was 19.1° and a mean post-operative valgus was 5.7°. In those compared with Bostman score (157/167), 94.3% had excellent (148/157) and remaining 5.7% (9/157) had good scores (20–27/30) with none showing poor score (<20/30).ConclusionV osteotomy is a reliable method of distal femoral osteotomy to attain a good correction with an excellent clinical outcome even with variations of fixation however longer follow-up period are required for better evaluation of recurrence.     

强化分离运动配合等长收缩训练对脑卒中偏瘫患者膝反张的影响

目的 探讨强化分离运动配合等长收缩训练对脑卒中偏瘫患者膝反张治疗效果。方法对90例脑卒中后并发膝反张患者随机分成3组,对照1组30例,对照2组30例,观察组30例,三组均行常规康复治疗,对照2组还给予等长收缩训练,观察组除常规康复治疗外也给予强化分离运动配合等长收缩训练。治疗1个月后评定疗效。结果观察组患者膝反张角度、下肢肌力、分离运动和步行能力与治疗前比较有明显改善,与对照组1、2比较有显著差异（P〈0．05）。结论强化分离运动配合等长收缩训练能增强脑卒中偏瘫患者膝关节稳定性,改善膝反张,提高其步行能力。     

正常人前后交叉韧带与腓骨长肌腱的解剖学形态、力学特性对比

为对比腓骨长肌腱与前后交叉韧带的解剖学形态和生物力学特性,将8例18～64岁因不同原因行大腿中下段以上截肢的标本,正中矢状面锯开股骨至髁间窝,标记出前、后交叉韧带中线,用精度0.02的游标卡尺进行测量,测出ACL、PCL中心线在膝关节屈曲90°时的长度和中点的宽度、厚度。测量腓骨长肌从出现腱性部分到下胫腓关节平面肌腱的长度和其上、下1/2段中点的宽度、厚度。分别切取前、后交叉韧带(两端各带一骨块),放入拉力试验机测试。切取腓骨长肌键长20cm,将切取的肌腱分成两段,各长10cm,标记为上段、下段。肌腱两端用艾西康10号丝线锁边式缝合,每端用3根丝线,缝合长度为3cm,放入拉力试验机测试。结果显示,腓骨长肌腱从出现腱性组织到下胫腓关节平面长(224.60±14.41)mm,前交叉韧带长(41.21±3.86)mm,后交叉韧带长(38.00±2.20)mm。腓骨长肌腱上段的最大形变、极限拉伸强度与后交叉韧带比较无显著性差异,腓骨长肌腱下段的最大形变、极限拉伸强度与前交叉韧带比较,均无显著性差异。表明腓骨长肌腱可以作为前、后交叉韧带同时重建的理想供体。     

What Happens at the Adjacent Knee Joint After Total Hip Arthroplasty of Crowe Type III and IV Dysplastic Hips?

We prospectively evaluated 30 hips of 22 patients who had normal knees with a mean age of 53.4 years (range, 38-72 years). In the early postoperative period, genu valgum deformity was observed in all knees. Of 22 patients, 17 complained of severe pain owing to strain in the medial collateral ligament and iliotibial tract. Postoperatively, the ipsilateral extremities of the patients were extended by a mean of 16.5 mm (8-25 mm). Q angles of the patients increased by a mean of 4.4° ± 2.5° (P < .001). Although the Harris hip scores were improved (40.7-87.8 points), postoperative Lysholm-Gillquist knee scores were significantly reduced (92-76 points, P < .001). Reduction of displaced hips into the anatomical hip center and lengthening the extremity despite shortening procedure may lead to strain at the knee joint iatrogenically, particularly with the mechanical effect of tensor fascia lata, which results with changes in the knee biomechanics.     

Early experience with medial femoral tension band plating in idiopathic genu valgum

Purpose  

For correction of angular deformity, tension band plating has been proposed as a safe and minimally invasive technique. The purpose of this study was to assess the experiences and report the rate of correction obtained with this procedure in patients with idiopathic genu valgum.     

Ⅰ期双侧全膝置换治疗骨关节病14例临床分析

目的：探讨Ⅰ期双侧全膝置换术治疗骨关节病的Ⅰ临床效果。方法：对14例Ⅰ期双侧全膝置换术进行临床分析和总结,并用HSS评分系统分析。结果：手术优良率92．86％。患者术后疼痛明显缓解,畸形矫正,功能改善。结论：选择合适病例,确保安全的前题下,Ⅰ期双侧全膝关节置换术可以获得满意的效果。     

重度膝关节外翻畸形的全膝关节置换术

 目的 探讨重度膝关节外翻畸形全膝关节置换术的手术方法及临床效果。方法 对2007年1月至2012年12月采用全膝关节置换治疗的重度膝关节外翻畸形患者22例（23膝）进行回顾性分析。男7例,女15例;年龄41~78岁,平均65岁。股胫角（股骨和胫骨解剖轴线的夹角）22°~50°,平均为34.6°。骨关节炎17例,类风湿关节炎5例。髌骨完全脱位3例3膝,内侧不稳定1例1膝,屈曲挛缩畸形3例4膝。21例22膝采用后稳定型假体,1例1膝采用限制型假体。髌旁内侧入路、常规截骨及单纯外侧软组织松解,术中行髌骨置换5例。以膝关节活动度、X线股胫角及美国特种外科医院（Hospital for Special Surgery,HSS）膝关节评分评价术后疗效。结果 全部病例随访时间1~5年,平均2.5年。膝关节活动度由术前平均43.7°±5.8°（0°~80°）提高至末次随访时110.6°±7.5°（80°~130°）,HSS膝关节评分由术前平均（19.6±4.7）分（6~34分）提高至末次随访时（89.7±3.6）分（84~96分）,手术前后的差异有统计学意义。外翻畸形基本得到矫正,末次随访时股胫角平均为8.6°±0.8°（0°~12°）,较术前34.6°±2.4°（22°~50°）明显改善,手术前后的差异有统计学意义。术后2例2膝有膝关节内侧不稳症状,给予膝关节支具保护;1例1膝术前严重髌骨脱位患者术后存在半脱位,未予特殊处理;2例2膝术后出现腓总神经麻痹,未予特殊处理。随访期间未发生感染、松动及深静脉血栓形成等并发症。结论 对重度膝关节外翻畸形患者可采用常规截骨、单纯外侧软组织松解及后稳定型假体植入,能较好地矫正外翻畸形,近期疗效满意。     

“U”形截骨孟氏架外固定矫治膝内外翻畸形33例

本文通过对３３例膝内、外翻畸行“Ｕ”形截骨孟氏架外固定治疗及疗效观察，认灯一法矫形确切、固定牢靠、调整灵活、组织损小、截骨愈合早、功能恢复快、是膝内，外翻畸形手术治疗的好方法。