title: Lengthening With External Fixation Is Effective in Congenital Femoral Deficiency.
abstract: BACKGROUND
  Treatment of congenital femoral deficiency is a complex, multistage protocol and a variety of strategies have been devised to address joint instability, limb length inequality, and deformities. Despite being an important part of the algorithmic approach to the overall treatment of patients with congenital femoral deficiency, a reproducible, safe, and functional treatment for femoral length discrepancy in patients with mild and moderate congenital femoral deficiency has not been reported.
  
  
  OBJECTIVE
  (1) Does femoral lengthening by means of distraction osteogenesis, using a monolateral external fixator, result in effective lengthening without loss of hip or knee range of motion? (2) Does femoral lengthening cause an inhibition of femoral growth in patients with congenital femoral deficiency? (3) Do patients/families report satisfactory functional and emotional outcomes after undergoing femoral lengthening? (4) What proportion of patients develops complications after femoral lengthening with this technique?
  
  
  METHODS
  Between 2005 and 2009, we evaluated 38 patients for femoral length discrepancy secondary to unilateral congenital femoral deficiency. Thirty-two patients completed treatment with distraction osteogenesis using a monolateral external fixator; general indications for this approach were congenital femoral deficiency Paley Types 1a, 1b, or 2a that had not previously undergone lengthening and had stable hip and knee joints. Of the 32 patients that completed treatment, 30 (94%) were available at a minimum of 2 years (mean, 3 years; range, 2-4.5 years) and were evaluated in this retrospective study. Preoperative and postoperative radiographic analysis, physiotherapy data, patient-based outcomes scores, and complications were reviewed for all eligible patients. Growth inhibition was measured using serial radiographs over the 2-year followup with the unaffected limb considered the norm. Functional and emotional outcomes were reported by adolescent patients or parents of younger children using the Pediatric Orthopaedic Society of North America Pediatric Outcomes Data Collection Instruments (PODCI), a validated patient-based outcomes measure.
  
  
  RESULTS
  The mean distal femoral lengthening was 6 cm (SD ± 2 cm; range, 1.6-9 cm), for a mean percent of femoral length discrepancy correction of 112% (SD ± 55%; range, 15%-215%). Comparison of patient preoperative with postoperative mean hip and knee flexion and extension showed no difference with the numbers available (hip flexion: p = 0.219, mean difference of -5, 95% confidence interval [CI], 10, SD = 20; hip extension: p = 0.423, mean difference of -1, 95% CI, 2, SD = 5; knee flexion: mean difference of -7°, SD ± 29°, CI, 15, p = 0.467; knee extension: mean difference of -1°, SD ± 9°, CI, 4, p = 0.757). A comparison of the mean preoperative inhibition of 41% (range, -38% to 300%; SD ± 72; 95% CI, 29%) with the mean postoperative inhibition of 16% (range, -242% to 100%; SD ± 61%; 95% CI, 25%) for a mean postoperative stimulation of 25% (p = 0.055, SD ± 90%; 95% CI, 36%). In all six PODCI categories surveyed, patients had favorable standardized and normative scores, but patients who underwent femoral lengthening greater than 6 cm had both lower global functioning scores (90, SD ± 10 versus 96, SD ± 3, p = 0.043) and worse pain/comfort scores (79, SD ± 25 versus 96, SD ± 7, p = 0.029), and patients who had mean percent femoral lengthening greater than 25% of initial femur length had worse pain/comfort scores (79, SD ± 23 versus 97, SD ± 4, p = 0.012) with similar global functioning scores (90, SD ± 9 versus 96, SD ± 3, p = 0.058). The total number of postsurgical complications was 30 in 60 planned surgical procedures (50%).
  
  
  CONCLUSIONS
  Our study results support the use of the described surgical technique for femoral lengthening in treating patients with congenital femoral deficiency. Additional studies are needed both to follow long-term patient-reported outcome measures, especially after a second or third lengthening, and to determine the effect of serial lengthening on the stimulation or inhibition of growth and rate of complications.
  
  
  METHODS
  Level IV, therapeutic study.
authors.affiliation: Memorial Sloan Kettering Cancer Center, 1275 York Avenue, Howard 1013, New York, NY, 10065, USA, PrinceD@mskcc.org.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Prince
authors.forename: John E
authors.initials: JE
authors.lastname: Herzenberg
authors.forename: Shawn C
authors.initials: SC
authors.lastname: Standard
authors.forename: Dror
authors.initials: D
authors.lastname: Paley
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D001852:Bone Lengthening / Q000295:instrumentation* / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D016267:External Fixators; D005260:Female; D005269:Femur / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D008297:Male; D010384:Pelvic Bones / Q000002:abnormalities* / Q000601:surgery*; D012189:Retrospective Studies