ORIGINAL ARTICLE

Anterior Femoral Epiphysiodesis for the Treatment of Fixed Knee Flexion Deformity in Spina Bifida Patients
Alexander S. Spiro, MD,*w Kornelia Babin, MD,* Sandra Lipovac, MD,* Martin Rupprecht, MD,w Norbert M. Meenen, MD,* Johannes M. Rueger, MD,w and Ralf Stuecker, MD*

Level of Evidence: Therapeutic Study, Level IV. Background: Fixed knee exion deformity is a common problem in spina bida patients. Owing to interference with ambulation, this deformity may lead to anterior knee pain and progressive crouch gait. If conservative treatment fails, surgical procedures including supracondylar femoral extension osteotomy, joint distraction, and posterior capsulotomy have to be considered. Potential complications of these procedures are fractures, neurovascular lesions, knee instability, and recurrent deformity with continued growth. As xed knee exion deformity in spina bida patients frequently occurs bilaterally, this results in higher perioperative and postoperative risks and prolonged recovery, making these relatively invasive methods less appealing. In the skeletal immature patient, anterior femoral stapling is a feasible method to treat xed knee exion deformity as could be shown in an earlier study. In this study, anterior femoral stapling was performed in a series of patients with diverse etiologies, and the overall success rate was then calculated. The aim of this study was to determine whether anterior femoral epiphysiodesis may improve xed knee exion deformity in a group of spina bida patients. Methods: Ten spina bida patients with bilateral xed knee exion deformity (20 knees) were treated by anterior femoral epiphysiodesis through stapling. None of the patients received further knee procedures. The mean preoperative xed knee exion deformity was 20.3 9.9 degrees (range: 10 to 40 degrees). Clinical and radiographic follow-up examinations were performed every 3 to 6 months after surgery. Results: All patients except 1 experienced signicant improvement with a mean correction rate of 0.9 0.5 degrees per month (range: 0.2 to 1.9 degrees). The residual exion deformity averaged 5.3 5.3 degrees (range: 0 to 15 degrees; P <0.001). Conclusions: Anterior femoral epiphysiodesis through stapling is an eective and safe method for the treatment of xed knee exion deformity in growing children and adolescents with spina bida.
From the *Department of Pediatric Orthopaedic Surgery, Childrens Hospital Hamburg-Altona; and wDepartment of Trauma, Hand, and Reconstructive Surgery, University Medical Center HamburgEppendorf, Hamburg, Germany. None of the authors received nancial support for this study. Reprints: Alexander S. Spiro, MD, Department of Trauma, Hand, and Reconstructive Surgery, University Medical Center HamburgEppendorf, Martinistrabe 52, 20246 Hamburg, Germany. E-mail: aspiro@uke.uni-hamburg.de. Copyright r 2010 by Lippincott Williams & Wilkins

Key Words: spina bida, xed knee exion deformity, anterior femoral epiphysiodesis, stapling, crouch gait (J Pediatr Orthop 2010;30:858862)

ixed knee exion deformity is a common problem in patients with spina bida, cerebral palsy, and arthrogryposis.13 The natural history of this deformity was determined in a prospective study of 850 spina bida patients by Wright and coworkers.4 The authors could demonstrate that the severity and progression of xed knee exion deformity increased as the neurosegmental levels of the patient ascended, but there was no association with muscle spasticity, use of orthosis, or physical therapy. The mean xed exion deformity in adolescents with a lesion above the L3 level was 18 degrees in this study, interfering with ambulation. About 60% of these patients with a xed knee exion contracture of less than 20 degrees were walking in late adolescence compared with only 5% of those with a exion contracture greater than 20 degrees.4 Further studies showed that a xed knee exion deformity exceeding 10 degrees may lead to anterior knee pain, decreased endurance, and progressive crouch gait in ambulatory patients, and with respect to wheelchair users, this deformity may impair standing, transfers, and activities of daily living.1,513 If conservative treatment including stretching by the use of orthosis and physical therapy may not prevent the progression of xed knee exion deformity, surgical options have to be considered. In this regard, dierent procedures are reported including supracondylar femoral extension osteotomy, joint distraction through Ilizarov xator, and posterior capsulotomy combined with muscle lengthening (hamstrings, biceps femoris muscle, and gastrocnemius muscles) and/or division of tendons and ligaments (ilio-tibial band, collateral ligaments, and posterior cruciate ligament).2,913 Potential complications of these relatively invasive methods are fractures, neurovascular lesions, knee instability, pin site infection, and recurrent deformity with continued growth.2,913 As xed knee exion deformity in spina bida patients frequently occurs bilaterally, this results in higher perioperative and postoperative risks and prolonged recovery.1013
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Anterior Femoral Epiphysiodesis

In the skeletal immature patient, anterior femoral epiphysiodesis through stapling or 8-plate implantation is a viable option to treat xed knee exion deformity as could be shown in 2 studies so far.14,15 In both reports, this technique was used in a series of patients with dierent conditions including cerebral palsy, spina bida, arthrogryposis, and others. In addition, concomitant hamstring lengthening was performed as indicated. The overall success rate of treatment was then evaluated in this heterogenous patient population. In another study, anterior femoral epiphysiodesis through 8-plate was successfully performed to treat knee exion contractures in children with arthrogryposis.16 The aim of this study was to determine whether anterior femoral epiphysiodesis through stapling may improve xed knee exion deformity in a group of spina bida patients. None of the 10 patients (20 knees) received further knee procedures.

Surgical Technique
Surgery is performed under general anesthesia and tourniquet hemostasis with the patient supine on a radiolucent table. Under uoroscopic localization, 2 longitudinal incisions (3 cm) are made just medial and lateral to the femoral sulcus, centered at the level of the distal femoral physis. The retinaculum and synovium are opened to expose the distal femur and a 20-gauge needle is inserted into the physis. A Blount staple (Blount Epiphysenklammern; Stryker GmbH & Co. KG, Duisburg, Germany) is then impacted into the femur in a 45 degree oblique direction under uoroscopic guidance (anteroposterior and lateral view), removing the needle. The implanted staples are placed about 0.5 cm away from the ridge of the sulcus and therefore do not interfere with patello-femoral articulation. None of the patients received hamstring lengthening/division or posterior capsulotomy, but 2 patients underwent other concomitant procedures, including proximal femoral osteotomies (n = 1) and treatment of foot deformities (n = 1). After surgical treatment, all patients except those who received concomitant procedures were allowed full weight bearing and started with knee movements immediately as tolerated. Clinical and radiographic follow-up examinations were performed every 3 to 6 months after surgery. The staples were left in place until the deformity was corrected or skeletal maturity occurred.

METHODS Patients
We reviewed the medical records of 10 spina bida patients (6 boys and 4 girls) with bilateral xed knee exion deformity (20 knees) who had been treated by anterior femoral epiphysiodesis between 2003 and 2009. The mean patient age at the time of surgery was 12.1 2.0 years (range: 8.8 to 14.5 y). The neurosegmental level of the spina bida lesion was thoracic in 2 patients, lumbar (L1 to L4) in 6, and lumbo-sacral (L5/S1) in 2. Clinical assessment included gait evaluation, straight leg raise, and screening for concomitant deformities such as hyperkyphosis (n = 2), scoliosis (n = 2), hydrocephalus (n = 6), Arnold-Chiari malformation (n = 2), Tethered spinal cord syndrome (n = 2), angular or rotational deformities of the hips (n = 5), genu valgum (n = 2), and foot deformities (n = 2). The degree of xed knee exion deformity was measured with a goniometer. Owing to bilateral knee deformity, a crouch gait and impaired stance was present in 7 patients. Three patients were wheelchair bound and surgery was indicated to improve assisted standing and transfers. Anteroposterior and lateral radiographs of the knee joints were taken from all patients to document open femoral physes and look for patella alta (n = 0), patella baja (n = 2), or tibial tuberosity avulsion (n = 0). In addition, a hand bone lm can be helpful to calculate bone age, as early skeletal maturity is often observed in spina bida patients.17,18 The indication for surgical treatment was a xed knee exion deformity exceeding 10 degrees, and unsuccessful conservative management including physical therapy and stretching by the use of orthoses. It is our policy to provide night braces for spina bida patients to prevent knee exion deformity. Thus, all our patients were treated accordingly. Contraindications for surgery included less than 12 months of predicted growth remaining and exion deformities caused by a dynamic etiology such as hamstring contracture.
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Statistical Analyses
Statistical analyses were performed using SPSS (Statistical Package for the Social Sciences, SPSS Inc., an IBM Company) 17.0. Descriptive statistics were given as mean SD and range (minimum to maximum). Student t tests were applied to compare pretreatment and posttreatment values and to detect dierences between young and old patients. P values less than 0.05 denote statistical signicance.

RESULTS
Anterior femoral epiphysiodesis was performed on 10 spina bida patients (20 knees) through stapling (demographics in Table 1). No complications occurred during surgery and although the staples are intracapsular, none of the patients developed reactive synovitis, eusion, deep wound infection, or neurovascular injury. The mean duration of treatment was 20.4 5.3 months (range: 12 to 29 mo), and 9 of 10 patients have had follow-up for more than 18 months (average 27.2 13.8 mo; range: 7 to 60 mo). The mean preoperative xed knee exion deformity was 20.3 9.9 degrees (range: 10 to 40 degrees). All patients except 1 experienced signicant improvement with a residual exion deformity averaging 5.3 5.3 degrees (range: 0 to 15 degrees) at the most recent followup (P <0.001) (Fig. 1). Excluding the patient achieving no correction, the mean improvement was 0.9 0.5 degrees per month (range: 0.2 to 1.9 degrees). Young patients (<12 y, n = 10 knees) showed superior results in
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TABLE 1. Patients Demographics

Patients No. (Side) 1 (R) 1 (L) 2 (R) 2 (L) 3 (R) 3 (L) 4 (R) 4 (L) 5 (R) 5 (L) 6 (R) 6 (L) 7 (R) 7 (L) 8 (R) 8 (L) 9 (R) 9 (L) 10 (R) 10 (L) Average Minimum Maximum Age (y) 11.7 13.8 8.8 14.2 11.5 13.0 9.5 14.5 13.6 10.8 12.1 8.8 14.5 Deformity (Degrees) 20 30 10 10 30 40 10 15 15 15 15 15 40 40 10 15 20 15 20 20 20.3 10.0 40.0 Duration of Treatment (mo) 22 22 25 25 18 18 12 12 29 29 24 24 23 23 18 18 24 16 13 13 20.4 12.0 29.0 Change (Degrees) 15 25 5 5 15 35 5 15 15 15 0 0 40 35 10 15 10 15 15 10 15.0 0.0 40.0 Residual Deformity (Degrees) 5 5 5 5 15 5 5 0 0 0 15 15 0 5 0 0 10 0 5 10 5.3 0.0 15.0 Change/Month (Degrees) 0.7 1.1 0.2 0.2 0.8 1.9 0.4 1.3 0.5 0.5 0.0 0.0 1.7 1.5 0.6 0.8 0.4 0.9 1.2 0.8 0.9 0.0 1.9

L indicates left; R, right.

comparison with older children (>12 y, n = 10 knees). The preoperative xed knee exion deformity improved from 27 10.3 to 5.0 4.7 degrees in the young group, but only from 13.5 3.4 to 5.5 6.0 degrees in the older one. Furthermore, signicant dierences could be detected in the mean correction rate between both groups (young patients: 1.1 0.5 degrees/mo; old patients: 0.5 0.4 degrees/mo; P <0.01). None of the patients showed impairment of knee exion or knee stability at the most recent follow-up. Five patients (10 knees) have completed treatment and have had all their staples removed. Four of these patients achieved full extension (8 knees). Another patient had full correction of her left knee but there was still a 10 degree of exion deformity on the right side. Thus, the staples within the left femur were removed. None of the patients showed overcorrection into genu recurvatum or changes in the relative position of the patella. Staple loosening (1 staple) occurred in 2 patients after 20 and 24 months of treatment, respectively, necessitating staple removal and restapling in one of these patients. The other patient had full correction of deformity thus all staples were removed. Only 1 nonambulatory patient achieved any correction of xed knee exion deformity for unknown reasons.

DISCUSSION
The development of xed knee exion deformity is a common problem in patients with dierent neuromuscular disorders and skeletal dysplasias.13,14,15 Deformities of less than 10 degrees may be treated conservatively by physical therapy and the use of orthoses, but should be

monitored closely because of frequent progression. If a xed knee exion deformity exceeds 10 degrees, this may interfere with ambulation, standing, and activities of daily living.1,513 Rueter and Pierre5 demonstrated that progressive increases in xed knee exion during gait caused progressive increases in oxygen cost. In another study, Moen and coworkers6 provided evidence that the degree of knee exion during gait (median: 24.5 degrees) was signicantly greater than the degree of knee exion contracture measured clinically (median: 15.0 degrees) in patients with spina bida. Thus, the authors concluded that there would be a greater oxygen cost in patients with xed knee exion deformity than expected from the clinical examination. This should be considered when evaluating patients for possible surgical treatment, particularly patients with borderline-level contractures. In this regard, several surgical procedures are reported for the treatment of xed knee exion deformity, some of which are fraught with potential complications and recurrent deformity with continued growth.2,913 Patients with a xed knee exion deformity of 40 degrees or greater (10 knees) were treated by posterior knee releases and gradual contracture distraction with an Ilizarov external xator in an earlier study (average residual exion deformity: 20.5 degress).2 The mean total xator time was 102 days, and complications included 3 fractures, 2 posterior subluxations, and 3 contracture recurrences. In our study, anterior femoral stapling was successfully performed in 2 patients (3 knees) with a xed knee exion deformity of 40 degrees. The mean residual exion deformity was solely 3.3 2.9 degrees (range: 0 to 5 degrees), and no complications occurred. These data indicate that anterior femoral epiphysiodesis may be
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FIGURE 1. A and B, Anteroposterior and lateral radiographs of the left knee 1 day after anterior femoral stapling was performed in this 11.7-year-old boy. The patient started with a fixed knee flexion deformity of 30 degrees at the time of surgery. C and D, Twenty-two months after stapling, the patient presented with a residual fixed knee flexion deformity of only 5 degrees. The divergent staple prongs 22 months after surgery indicate changes of implant shape because of growth forces.

an eective and save option for the treatment of distinct xed knee exion deformities of 40 degrees or greater. The results of joint distraction through Ilizarov external xator were reviewed in another study as well (mean knee contracture: 53 degrees; 9 knees).9 Fixed knee exion deformity could be reduced to 10 degrees but several complications occurred, including pin site infection, transient peroneal nerve neuropraxia (2 knees), distal femoral epiphyseal separation (1 knee), plastic deformation of the femur (1 knee), fracture (1 knee), and recurrence of deformity (2 knees). Supracondylar femoral extension osteotomy was performed in 20 patients (32 knees) for the correction of xed knee exion deformity (mean deformity: 31 degrees for the meningomyelocele group) by Zimmerman et al.10 In the early postoperative period, the corrections obtained were within 10 degrees of neutral position. However, a total of 6 fractures and 6 recurrences of deformity occurred in the spina bida group (18 treated knees).
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In our study, 6 patients (11 knees) have completed treatment and have had all their staples removed. Owing to the fact that in some instances (6 knees) staple removal was performed recently (<4 mo), no reliable conclusion can be drawn with respect to a possible rebound phenomenon. In 2 patients (3 knees), no recurrence of exion deformity could be observed 8 and 48 months after staple removal, respectively. The patient achieving no correction also had staple removal after growth plate closure (2 knees). Concomitant tenotomy of the biceps femoris and hamstrings showed unsatisfactory results with minimal correction of xed knee exion deformity in spina bida patients.11 Thus, several authors preferred radical soft tissue releases of this deformity with division of the semitendinosus and gracilis tendons, lengthening or division of the biceps femoris and semimembranosus muscles, partial division of the ilio-tibial band, extensive posterior capsulotomy, partial division of the collateral ligaments, and if necessary division of the posterior cruciate ligament.1113 Neurovascular lesions, destabilization of the knee, and recurrent deformity are potential complications of these procedures.1113 Interestingly, none of our patients showed impairment of knee exion or knee stability at the most recent follow-up, indicating a possible advantage of anterior femoral stapling over the above mentioned surgical procedures for the treatment of xed knee exion deformity. The treatment of xed knee exion deformity by anterior femoral stapling was described in only 1 study so far.14 In a series of 28 skeletal immature patients (47 knees) with dierent disorders, including cerebral palsy, arthrogryposis, spina bida, and others, the authors were able to demonstrate an improvement of xed knee exion deformity from 10 to 25 degrees preoperatively to 0 to 11 degrees at the most recent follow-up. Several years later another study was published by the same working group, using 8 plates instead of staples for anterior femoral epiphysiodesis.15 The authors revealed signicant improvement of xed knee exion deformity in a group of 23 patients (40 knees) with dierent conditions (cerebral palsy, spina bida, stickler syndrome, and others). The mean exion contracture improved from 23.4 to 8 degrees. Thus the results of both studies are in line with our ndings. However, there are some dierences compared with our study which have to be considered. Concomitant hamstring lengthening was performed as indicated in the above-mentioned studies, but none of our patients received further knee procedures. In those studies, a mixed patient population with varying underlying conditions were included.14,15 The success rate of anterior femoral epiphysiodesis relating to the etiology was not calculated.14,15 In our study, evaluation of anterior femoral epiphysiodesis was limited to patients with spina bida only. The mean improvement of xed knee exion deformity was 1.4 degrees per month using 8-plates compared with 0.9 degrees per month in our study.15 None of the 8-plates broke, and there was no screw migration or loosening.15 In our study, staple loosening (1 staple) occurred in 2 patients. These data
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indicate a possible advantage of 8-plates over staples for anterior femoral epiphysiodesis.15 Advanced skeletal maturity is frequently observed in spina bida patients.17,18 In this study, authors could demonstrate that young patients (<12 y) revealed superior improvement of knee exion deformity compared with older patients (>12 y) after anterior femoral stapling. In addition, young patients had more rapid correction in our study. On account of the incalculable growth potential of spina bida patients and based on the results of our study, we recommend early treatment (children around 10 y of age) of xed knee exion deformity by anterior femoral epiphysiodesis to achieve adequate improvement. Limitations of this study include the small number of patients, the diculties of accurately documenting deformities, and the relatively short follow-up. Nevertheless, the results of this study show that anterior femoral epiphysiodesis through stapling is an eective and save method for the treatment of xed knee exion deformity in growing children and adolescents with spina bida. REFERENCES
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5. Rueter K, Pierre M. Energy cost and gait characteristics of exed knee ambulation. In: Bunch WE, Keagy R, Knitter AE, et al, eds. Atlas of Orthotics. St Louis: CV Mosby; 1985:154155. 6. Moen T, Gryfakis N, Dias L, et al. Crouched gait in myelomeningocele: a comparison between the degree of knee exion contracture in the clinical examination and during gait. J Pediatr Orthop. 2005;25:657660. 7. Delp SL, Arnold AS, Speers RA, et al. Hamstrings and psoas lengths during normal and crouch gait: implications for muscletendon surgery. J Orthop Res. 1996;14:144151. 8. Duy CM, Hill AE, Cosgrove AP, et al. Three-dimensional gait analysis in spina bida. J Pediatr Orthop. 1996;16:786791. 9. Devalia KL, Fernandes JA, Moras P, et al. Joint distraction and reconstruction in complex knee contractures. J Pediatr Orthop. 2007;27:402407. 10. Zimmerman MH, Smith CF, Oppenheim WL. Supracondylar femoral extension osteotomies in the treatment of xed exion deformity of the knee. Clin Orthop Relat Res. 1982;171:8793. 11. Dias LS. Surgical management of knee contractures in myelomeningocele. J Pediatr Orthop. 1982;2:127131. 12. Marshall PD, Broughton NS, Menelaus MB, et al. Surgical release of knee exion contractures in myelomeningocele. J Bone Joint Surg Br. 1996;78:912916. 13. Abraham E, Verinder DG, Sharrard WJ. The treatment of exion contracture of the knee in myelomeningocele. J Bone Joint Surg Br. 1977;59:433438. 14. Kramer A, Stevens PM. Anterior femoral stapling. J Pediatr Orthop. 2001;21:804807. 15. Klatt J, Stevens PM. Guided growth for xed knee exion deformity. J Pediatr Orthop. 2008;28:626631. 16. Palocaren T, Thabet AM, Rogers K, et al. Anterior distal femoral stapling for correcting knee exion contracture in children with arthrogryposisFpreliminary results. J Pediatr Orthop. 2010;30:169173. 17. Kalen V, Harding CR. Skeletal maturity in myelodysplasia. Dev Med Child Neurol. 1994;36:528532. 18. Greene SA, Frank M, Zachmann M, et al. Growth and sexual development in children with meningomyelocele. Eur J Pediatr. 1985;144:146148.

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