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.

A prospective comparison of computer-navigated and fluoroscopic-guided in situ fixation of slipped capital femoral epiphysis.

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is usually treated with percutaneous in situ screw fixation to prevent further progression of deformity. The purpose of this investigation is to compare computer navigation (CN) techniques with traditional fluoroscopic (fluoro) techniques for in situ fixation of SCFE. METHODS: This study was an IRB-approved prospective study of 39 hip pinnings in 33 children. CN techniques were used in 22 cases and fluoro in 17. The CN and fluoro groups were statistically similar in terms of grade and acuity of the slip. Children were assigned to the groups based on the intraoperative imaging technique used by the attending on call, with 3 surgeons in each group taking equal amounts of call. The "approach-withdraw" technique was used in all cases. Postoperative limited-cut, reduced-dose computed tomography (CT) scans were obtained to evaluate screw placement. This included blinded analysis for screw penetration of the joint, screw tip-to-apex distance, the distance the screw passed to the center of the physis, and attainment of center-center position. The number of pin passes, intraoperative radiation exposure, and operating room (OR) time were also analyzed. Statistics used included ANOVA, the χ and median tests. RESULTS: Compared with the fluoro group, CN resulted in more accurate screw placement. There was 1 case of joint penetration in the fluoro group not appreciated intraoperatively but detected on postoperative CT. CN also resulted in statistically significant (P < 0.05) reduced screw tip-to-apex distance and distance to the center of the physis. There was no statistically significant difference between the 2 groups in attainment of the center-center position, number of pin passes, or intraoperative radiation exposure. OR time averaged 19 minutes longer in the CN group. There was no case of avascular necrosis or chondrolysis in either of the groups. CONCLUSIONS: Compared with traditional fluoro techniques, CN in situ fixation of SCFE results in more accurate screw placement, comparable number of pin passes and intraoperative radiation exposure, and increased OR time. The cost-benefit ratio of this technology requires careful consideration at each individual institution. LEVEL OF EVIDENCE: II.

Modelling competition and hybridization between native cutthroat trout and nonnative rainbow and hybrid trout.

Native salmonid fish have been displaced worldwide by nonnatives through hybridization, competition, and predation, but the dynamics of these factors are poorly understood. We apply stochastic Lotka-Volterra models to the displacement of cutthroat trout by rainbow/hybrid trout in the Snake River, Idaho, USA. Cutthroat trout are susceptible to hybridization in the river but are reproductively isolated in tributaries via removal of migratory rainbow/hybrid spawners at weirs. Based on information-theoretic analysis, population data provide evidence that hybridization was the primary mechanism for cutthroat trout displacement in the first 17 years of the invasion. However, under some parameter values, the data provide evidence for a model in which interaction occurs among fish from both river and tributary subpopulations. This situation is likely to occur when tributary-spawned cutthroat trout out-migrate to the river as fry. The resulting competition with rainbow/hybrid trout can result in the extinction of cutthroat trout even when reproductive segregation is maintained.