Long-term Outcomes of Surgically Treated Congenital Dislocation of the Knee.

BACKGROUND: Congenital dislocation of the knee (CDK) may be idiopathic or associated with another condition, such as Larsen syndrome or arthrogryposis. Surgical reduction of type-3 dislocation may require quadricepsplasty (QP) or femoral diaphyseal shortening (FS). Because it is unknown which treatment is more effective, we evaluated long-term outcomes using patient-reported questionnaires and gait analysis, comparing results by surgery type and underlying diagnosis. METHODS: Twelve patients (mean age, 19 mo) were treated surgically for CDK from 1985 to 2015 and studied 9 to 30 years postoperatively. Three participants had idiopathic CDK, 5 had Larsen syndrome, and 4 had arthrogryposis. Eleven knees underwent QP and 7 underwent FS. Participants were evaluated in our movement science laboratory and completed patient-reported outcome questionnaires. Data were compared with healthy, age-matched control values at the same visit. RESULTS: Surgically treated knees had less flexion during swing (P<0.01), less overall motion (P<0.01), greater coronal instability (P<0.04), and slower gait (P<0.01) compared with controls. QP knees had more instability in midstance (P=0.03) and less flexion during gait compared with FS knees, less sagittal power generation than controls (P<0.01), and trended toward lower scores on Knee Injury and Osteoarthritis Outcome and Lysholm Knee Questionnaires than FS patients did. The idiopathic group had the gait most similar to that of controls, followed by the Larsen syndrome group and then the arthrogryposis group. The idiopathic group also had a better UCLA Activity Score (P=0.03) than the arthrogryposis group did. CONCLUSIONS: Surgical treatment of type-3 CDK will not likely restore normal knee function, suggesting teratologic joint abnormality. In this small series, FS produced better gait mechanics and patient-reported outcomes compared with QP. Not surprisingly, patients with idiopathic CDK had better outcomes than those with a syndromic diagnosis, likely related to having only a single joint affected. LEVEL OF EVIDENCE: Level III.

Definition of Tweener: Consensus Among Experts in Treating Early-onset Scoliosis.

BACKGROUND: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population. METHODS: A 3-round survey of surgeons in an international EOS study group was conducted. Surgeons were provided with various patient characteristics and asked if each was part of their definition for Tweener patients. Responses were analyzed for consensus (≥70%), near-consensus (60% to 69%), and no consensus (<60%). RESULTS: Consensus was reached (89% of respondents) for including chronological age in the Tweener definition; 8 to 10 years for females and 9 to 11 years for males. Surgeons agreed for inclusion of Sanders score, particularly Sanders 2 (86.0%). Patients who have reached Sanders 4, postmenarche, or have closed triradiate cartilage should not be considered Tweeners. Bone age range of 8 years and 10 months to 10 years and 10 months for females (12 y for males) could be part of the Tweener definition. CONCLUSIONS: This study suggests that the Tweener definition could be the following: patients with open triradiate cartilage who are not postmenarche and have not reached Sanders 4, and if they have one of the following: Sanders 2 or chronological age 8 to 10 years for females (9 to 11 y for males) or bone age 8 years and 10 months to 10 years and 10 months for females (12 y for males). This definition will allow for more focused and comparative research on this population. LEVEL OF EVIDENCE: Level V-expert opinion.

Rebound Deformity After Growth Modulation in Patients With Coronal Plane Angular Deformities About the Knee: Who Gets It and How Much?

BACKGROUND: With observed success and increased popularity of growth modulation techniques, there has been a trend toward use in progressively younger patients. Younger age at growth modulation increases the likelihood of complete deformity correction and need for implant removal before skeletal maturity introducing the risk of rebound deformity. The purpose of this study was to quantify magnitude and identify risk factors for rebound deformity after growth modulation. METHODS: We performed a retrospective review of all patients undergoing growth modulation with a tension band plate for coronal plane deformity about the knee with subsequent implant removal. Exclusion criteria included completion epiphysiodesis or osteotomy at implant removal, ongoing growth modulation, and <1 year radiographic follow-up without rebound deformity. Mechanical lateral distal femoral angle, mechanical medial proximal tibial angle, hip-knee-ankle angle (HKA), and mechanical axis station were measured before growth modulation, before implant removal, and at final follow-up. RESULTS: In total, 67 limbs in 45 patients met the inclusion criteria. Mean age at growth modulation was 9.8 years (range, 3.4 to 15.4 y) and mean age at implant removal was 11.4 years (range, 5.3 to 16.4 y). Mean change in HKA after implant removal was 6.9 degrees (range, 0 to 23 degrees). In total, 52% of patients had >5 degrees rebound and 30% had >10 degrees rebound in HKA after implant removal. Females below 10 years and males below 12 years at time of growth modulation had greater mean change in HKA after implant removal compared with older patients (8.4 vs. 4.7 degrees, P=0.012). Patients with initial deformity >20 degrees had an increased frequency of rebound >10 degrees compared with patients with less severe initial deformity (78% vs. 22%, P=0.002). CONCLUSIONS: Rebound deformity after growth modulation is common. Growth modulation at a young age and large initial deformity increases risk of rebound. However, rebound does not occur in all at risk patients, therefore, we recommend against routine overcorrection. LEVEL OF EVIDENCE: Level IV-retrospective study.

Recurrence After Surgical Intervention for Infantile Tibia Vara: Assessment of a New Modified Classification.

BACKGROUND: To propose a modified classification of infantile tibia vara based on the morphology of the metaphyseal/epiphyseal tibial slope that better correlates with treatment outcomes than the traditional Langenskiold classification. METHODS: We performed a retrospective review of 82 patients and 115 limbs that underwent surgery for infantile tibia vara over a 22-year period (1990 to 2012) at a single institution. A modified Langenskiold classification was applied to all patients preoperatively and the outcomes were assessed. The modified system created a 3-stage classification (types A, B, and C): type A has a partially lucent medial metaphyseal defect, with or without "beaking"; type B deformity has downward-sloping curvature of the lateral and inferior rim of a completely lucent metaphyseal defect, which then has an upslope at the medial rim, resembling a ski-jump, with no epiphyseal downward slope; type C has vertical, downsloping deformity of both the epiphysis and metaphysis, with no upward curvature projecting medially at the inferior extent, while the epiphysis slopes downward into the metaphyseal defect. RESULTS: Sixty-seven limbs did not develop recurrence following corrective osteotomy, whereas 48 limbs required at least 1 repeat surgery for recurrent deformity. Preoperative mechanical axis deviation, medial proximal tibial angle, lateral distal tibial angle, and body mass index did not differ significantly between those with recurrence and those with without. Mean age at surgery was significantly different for those who developed recurrence compared with those who did not. Patients without recurrence were 4.3 years of age (range, 2.4 to 10.3 y) compared with 6.2 years of age (range, 2.9 to 10.1 y) for those who recurred (P<0.01). Of patients who developed recurrent deformity, there were significantly more patients with type C changes (71.7%, P<0.01) then either type A (22.5%) or type B (20.7%). High rates of recurrence were seen for both Langenskiold stage III (50%) and stage IV (69.6%). CONCLUSIONS: Consistent with prior studies, age 5 seems to be a critical transition in the risk for recurrent deformity after tibial osteotomy. Extreme vertical sloping of the medial metaphyseal defect, as in some classic Langenskiold III lesions and more precisely described by type C in a newer, modified classification, carries a poor prognosis for successful correction by high tibial osteotomy alone or in combination with epiphysiolysis. LEVEL OF EVIDENCE: Level II.

Quengel Casting for the Management of Pediatric Knee Flexion Contractures: A 26-Year Single Institution Experience.

BACKGROUND: Quengel casting was introduced in 1922 for nonsurgical treatment of knee flexion contractures (KFC) associated with hemophilic arthropathy. It consists of an extension-desubluxation hinge fixed to a cast allowing for gradual correction of a flexion deformity while preventing posterior tibial subluxation. The purpose of this study is to report 1 center's experience with this technique for the treatment of pediatric KFC. METHODS: A retrospective review was conducted over a 26-year period. All patients with KFC treated with Quengel casting were included. Demographic data, associated medical conditions, adjunctive soft tissue releases, complications, and the need for late surgical intervention were recorded. Tibiofemoral angle measurements in maximal extension were recorded at initiation and termination of casting, 1-year follow-up, and final follow-up. Success was defined as no symptomatic recurrence of KFC or need for subsequent surgery. RESULTS: Eighteen patients (26 knees) were treated for KFC with Quengel casting. Average age at initiation of casting was 8.1 years with average follow-up of 59.9 months. Fifteen knees (58%) underwent soft tissue releases before casting. An average of 1.5 casts per knee were applied over an average of 23.9 days. Average KFC before casting was 50.6 degrees (range, 15 to 100 degrees) which improved to 5.96 degrees (0 to 40 degrees) at cast removal (P<0.00001). Sixteen patients (22 knees) had 1-year follow-up or failed casting before 1 year. Of these, 11 knees (50%) had a successful outcome. Residual KFC of those treated successfully was 6.8 degrees (range, 0 to 30 degrees) at 1 year and 8.2 degrees (range, 0 to 30 degrees) at final follow-up, averaging 71.4 months (P=0.81). Of the 11 knees deemed failures, all had recurrence of deformity within an average of 1 year from cast removal. Surgical release before Quengel casting did not improve the chances for success (P=0.09). CONCLUSIONS: Quengel casting can improve pediatric KFC an average of 44.2 degrees with minimal complications. Although 50% of treated patients will demonstrate significant recurrence or need later surgery, the majority of those treated successfully have durable results at intermediate term follow-up. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Radiographic Assessment of Shoulder Position in 619 Idiopathic Scoliosis Patients: Can T1 Tilt Be Used as an Intraoperative Proxy to Determine Postoperative Shoulder Balance?

BACKGROUND: The purpose of this study is to assess radiographic shoulder measures from the preoperative to the postoperative time period, specifically to determine whether T1 tilt could be used as an intraoperative proxy for shoulder balance determination. This study focused on radiographic shoulder measures of 619 adolescent idiopathic scoliosis patients who underwent spinal deformity surgery. METHODS: A prospective, multicenter database of adolescent idiopathic scoliosis was queried to identify all patients who had undergone spinal deformity surgery with >2 years of follow-up postoperatively. Radiographic analysis focused on measures of shoulder balance: T1 tilt, clavicle angle, and radiographic shoulder height. RESULTS: A total of 619 patients were included in this analysis. Mean age at surgery was 14.8 years with 83% female. Mean preoperative curve size was 58.0 degrees. Mean T1 tilt preoperatively was -0.10 degrees and postoperatively 2.42 degrees. Mean clavicle angle preoperatively was -1.39 degrees and postoperatively 0.79 degrees. Mean radiographic shoulder height preoperatively was -7.04 mm and postoperatively 1.63 mm. All 3 radiographic parameters demonstrated reasonable correlation preoperatively and postoperatively to each other. To assess the viability of T1 tilt as an intraoperative proxy for shoulder balance, standardized ratios between the variables were created. Analysis of these ratios demonstrated little or no relationship preoperatively to postoperatively, hence the relationship of T1 tilt to radiographic shoulder height does not remain constant. CONCLUSIONS: Analysis of the relationship of T1 tilt to radiographic shoulder height from preoperative to postoperative did not demonstrate consistency. Lenke 3 and 6 curve patterns demonstrated preoperative to postoperative correlation, both with nonstructural proximal thoracic curves; however, for the remaining curve patterns T1 tilt cannot be used as an intraoperative proxy for shoulder balance. LEVEL OF EVIDENCE: Level IV.

Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

BACKGROUND: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history. METHODS: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed. RESULTS: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points). CONCLUSIONS: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Upper thoracic pedicle screw loss of fixation causing spinal cord injury: a review of the literature and multicenter case series.

STUDY DESIGN: Case Series and Review of the Literature. OBJECTIVE: To report on cases of spinal cord injury from loss of fixation of upper thoracic pedicle screws. SUMMARY OF BACKGROUND DATA: Despite generally low rates of intraoperative neurological injury from pedicle screws, there is 1 reported case of T2 pedicle screw pullout causing spinal cord injury. METHODS: A review of the literature and an informal poll of 2 professional societies searching for cases in which thoracic pedicle screws migrated postoperatively into the spinal canal was performed. RESULTS: Three patients had failure of spinal instrumentation with the most cephalad pedicle screws (T2, T4 and T4) plowing into the spinal canal, causing direct trauma to the spinal cord with resulting clinical and neurological injury. Failure of fixation occurred at 1 month, 1 year, and 2 years after index procedure. In 2 patients, neurological injury was severe enough that they became nonambulatory; the third patient had rapidly progressive leg weakness. In each case, there were only 1 or 2 pedicle screws at the top of the construct, and a span of 6 to 7 vertebrae without rigid fixation below this. One similar case was found in the literature. CONCLUSIONS: Spinal instrumentation with only 1 to 2 pedicle screws at the top of the construct, and a span of >5 vertebrae below these screws without rigid fixation may be at risk for implant failure and catastrophic spinal cord injury. In the rare instance in which only 1 to 2 pedicle screws can be placed at the cephalad half of long spinal constructs, one may consider using hooks that would fail posteriorly and may present less risk to the spinal cord.

Comparison of treatment outcomes between growth-sparing instrumentation and definitive one-stage fusion for EOS patients ages 6-10 years.

PURPOSE: To compare objective outcomes for EOS patients age 6-10 years treated by growth-sparing (GS) surgery or definitive one-stage correction and fusion (DF). METHODS: We reviewed surgical, radiographic, PFT's, and EOSQ-24 outcomes for EOS patients > age 6 at index surgery treated at a single institution, minimum 2-year follow-up. Neuromuscular diagnoses were excluded. RESULTS: 47 patients underwent index surgery between age 6 and 10.9 years. Twenty-one had DF, 26 had GS surgery (13 MCGR, 13 TGR). Diagnoses included 15 congenital, 15 idiopathic, 17 syndromic. Age at index was 9.1 years DF, 7.8 GS (p < .001). Follow-up was 63-78 months. 18/26 GS cases converted to DF, 13 due to complications, which occurred in 8/21 DF cases vs 19/26 GS (p = .016). DF patients had fewer post-index surgeries (0.6 vs 3.7, p < .001). At follow-up there were no differences in curve magnitudes, %correction, T1-12/T1-S1 segment lengths, EOSQ-24 scores or PFTs. 18 patients converting to DF after initial GS had equal outcomes as DF initially. 31 patients > age 8 at index ("tweeners") were studied separately. 13 had GS surgery (7 MCGR), 18 had DF. At > 60 months follow-up, curve magnitudes, spine lengths, PFT's, or EOSQ scores were equivalent. DF patients had fewer procedures and complications. CONCLUSION: For patients age 6-10.9 years, outcomes were no different at > 5 year follow-up between DF and GS groups. DF patients had fewer total surgeries and complications. Equal outcomes also occurred for tweeners. As a result, GS treatment does not appear to benefit patients > age 8.

Is spinal height gain associated with rod orientation and the use of cross-links in magnetically controlled growing rods in early-onset scoliosis?

Optimal orientation for magnetically controlled growing rods (MCGRs) is unclear. The objective of this study was to investigate associations of rod orientation with implant-related complications (IRCs) and spinal height gains. Using an international early-onset scoliosis (EOS) database, we retrospectively reviewed 57 patients treated with dual MCGRs from May 2013 to July 2015 with minimum 2-year follow-up. Outcomes of interest were IRCs and left/right rod length gains and thoracic (T1-T12) and spinal (T1-S1) heights. We compared patients with two rods lengthened in the cephalad ( standard; n  = 18) versus opposite ( offset; n  = 39) directions. Groups did not differ in age, sex, BMI, duration of follow-up, EOS cause, ambulatory status, primary curve magnitude, baseline thoracic height, or number of distractions/year. We compared patients whose constructs used ≥1 cross-link (CL group; n  = 22) versus no CLs (NCL group; n  = 35), analyzing thoracic height gains per distraction ( α  = 0.05). Offset and standard groups did not differ in left or right rod length gains overall or per year or in thoracic or spinal height gain. Per distraction, the CL and NCL groups did not differ significantly in left or right rod length or thoracic or spinal height gain. Complications did not differ significantly between rod orientation groups or between CL groups. MCGR orientation and presence of cross-links were not associated with differences in rod length gain, thoracic height, spinal height, or IRCs at 2-year follow-up. Surgeons should feel comfortable using either MCGR orientation. Level of evidence: 3, retrospective.

Serial casting as a delay tactic in the treatment of moderate-to-severe early-onset scoliosis.

BACKGROUND: Serial casting can cure mild infantile idiopathic scoliosis. Its use in delaying surgery in older children and those with larger curves or syndromes is poorly defined. METHODS: A review of a single center's experience with casting was performed. Patients were included if they had a syndromic, neuromuscular, or congenital scoliosis or were older than 2.5 years with an idiopathic scoliosis measuring >50 degrees. RESULTS: A retrospective review was performed on 29 patients meeting all inclusion criteria. Of these, 12 were idiopathic and 17 were nonidiopathic curves. Average age at first cast was 4.4 ± 2.1 years, and 3.0 ± 1.8 cast changes were performed over 1.4 ± 1.1 years. Patients were transitioned to a brace and followed up for 5.5 years (range, 2.2 to 11.4 y). The main thoracic Cobb angle before casting was 68.8 ± 12.3 degrees, which corrected to 39.1 ± 16.4 degrees in a cast. Cobb angle after cast removal was 60.9 ± 18.4 degrees, which increased to 76.3 ± 24.0 degrees at final follow-up. T1-T12 height increased to 1.1 ± 2.6 cm during the treatment period (P=0.05). There were 5 minor complications. Fifteen patients (51.7%) required surgical treatment for their scoliosis at most recent follow-up and an additional 7 patients (24.1%) were delayed until a definitive anterior/posterior spinal fusion could be performed. Surgery was delayed 39 ± 25 months from the first cast. Growing rods were required in 8 patients (27.6%). The patients who ultimately underwent surgical intervention (SG) were more likely to have a larger postcasting residual main thoracic Cobb angle than those who did not require surgery [NS; 69.5 ± 14.6 degrees (SG) vs. 51.6 ± 17.9 degrees (NS), P=0.007] and had a greater progression of their curves after cast removal [20.9 ± 13.5 degrees (SG) vs. 9.4 ± 11.0 degrees (NS), P=0.02]. CONCLUSIONS: Serial casting is a viable alternative to surgical growth sparing techniques in moderate-to-severe early-onset scoliosis and may help delay eventual surgical intervention. Although a cure cannot be expected, an average of 39 months of delay was achieved in this patient cohort and 72.4% have avoided growing spine surgery. LEVEL OF EVIDENCE: Level IV, case series.

Contraindications to magnetically controlled growing rods: consensus among experts in treating early onset scoliosis.

PURPOSE: The purpose of this study was to describe contraindications to the magnetically controlled growing rod (MCGR) in patients with early onset scoliosis (EOS) by establishing consensus amongst expert surgeons who treat these patients frequently. METHODS: Nine pediatric spine surgeons from an international EOS study group participated in semi-structured interviews via email to identify factors that influence decision making in the use of MCGR. A 39-question survey was then developed to specify these factors as contraindications for MCGR-these included patient age and size, etiology, medical comorbidities, coronal and sagittal curve profiles, and skin and soft tissue characteristics. Pediatric spine surgeons from the EOS international study group were invited to complete the survey. A second 29-item survey was created to determine details and clarify results from the first survey. Responses were analyzed for consensus (> 70%), near consensus (60-69%), and no consensus/variability (< 60%) for MCGR contraindication. RESULTS: 56 surgeons of 173 invited (32%) completed the first survey, and 64 (37%) completed the second survey. Responders had a mean of over 15 years in practice (range 1-45) with over 6 years of experience with using MCGR (range 2-12). 71.4% of respondents agreed that patient size characteristics should be considered as contraindications, including BMI (81.3%) and spinal height (84.4%), although a specific BMI range or a specific minimum spinal height were not agreed upon. Among surgeons who agreed that skin and soft tissue problems were contraindications (78.6%), insufficient soft tissue (98%) and skin (89%) to cover MCGR were specified. Among surgeons who reported curve stiffness as a contraindication (85.9%), there was agreement that this curve stiffness should be defined by clinical evaluation (78.2%) and by traction films (72.3%). Among surgeons who reported sagittal curve characteristics as contraindications, hyperkyphosis (95.3%) and sagittal curve apex above T3 (70%) were specified. Surgeons who indicated the need for repetitive MRI as a contraindication (79.7%) agreed that image quality (72.9%) and not patient safety (13.6%) was the concern. In the entire cohort, consensus was not achieved on the following factors: patient age (57.4%), medical comorbidities (46.4%), etiology (53.6%), and coronal curve characteristics (58.9%). CONCLUSION: Surgeon consensus suggests that MCGR should be avoided in patients who have insufficient spinal height to accommodate the MCGR, have potential skin and soft tissue inadequacy, have too stiff a spinal curve, have too much kyphosis, and require repetitive MRI, particularly of the spine. Future data-driven studies using this framework are warranted to generate more specific criteria (e.g. specific degrees of kyphosis) to facilitate clinical decision making for EOS patients. LEVEL OF EVIDENCE: Level V-expert opinion.

Simultaneous open reduction of ipsilateral congenital dislocation of the hip and knee assisted by femoral diaphyseal shortening.

BACKGROUND: Congenital dislocation of the hip (CDH) and congenital dislocation of the knee (CDK) occurring in the same extremity is a rare condition. Traditional treatment usually involves staged procedures, obtaining knee reduction first, and then addressing the hip once the knee can be flexed adequately. Simultaneous surgical reduction of both dislocations assisted by femoral shortening is a logical treatment option not previously reported. METHODS: A retrospective radiographic and clinical review of surgically treated patients was performed, with a minimum 5-year follow-up. Outcome of CDK treatment was graded according to a scale consisting of radiographic congruity, clinical stability, function, and gait. Outcome of CDH treatment was graded according to Severin classification. RESULTS: Eight patients (11 limbs) were identified, with follow-up of 5 to 16 years. Four patients (5 limbs) underwent simultaneous open reduction of both dislocations stabilized by capsulorrhaphy and femoral shortening, with 3 patients operated at age 1 year. Subsequent procedures included 2 physeal-sparing anterior cruciate ligament reconstructions, and 1 high tibial osteotomy to correct angular deformity. Additional procedures for hip dysplasia included 2 pelvic osteotomies in the same patient. Four patients (6 limbs) underwent staged treatment: open reduction of the knee using quadricepsplasty at age 4 to 11 months, followed by open reduction of the hip by either medial approach 3 months later, or anterior approach 12 to 24 months later. No additional knee procedures were performed in this group. Additional hip procedures included 1 repeat open reduction and 1 pelvic/femoral osteotomy. At follow-up in the simultaneous group, knees were 3 excellent, 1 good, and 1 fair, whereas hips had 3 Severin grade 1 to 2 results, 1 grade 3, and 1 grade 4. Outcomes for the staged group knees were 2 fair and 4 poor results, whereas hips had 5 grade 1 to 2 results and 1 grade 3. CONCLUSIONS: Outcome for CDK treated simultaneously seems better than staged treatment due to improved retained quadriceps function and attention to teratologic instability. Outcomes for CDH were essentially equal regardless of simultaneous or staged treatment. LEVEL OF EVIDENCE: : III.

Current treatment preferences for early onset scoliosis: a survey of POSNA members.

BACKGROUND: Surgical options for the management of idiopathic early onset scoliosis (EOS) have increased over the past decade, perhaps surpassing traditional nonoperative methods. We sought to assess current treatment preferences in the management of EOS among pediatric orthopaedic surgeons. We hypothesized that practitioner access to casting tables and halo traction would be significantly associated with treatment choices. METHODS: A web-based survey was distributed to the members of the Pediatric Orthopaedic Society of North America. Information with regard to practice type, access to casting tables and halo traction, and management of patients with EOS was obtained. Clinical vignettes were used to assess current physician preferences in the treatment of EOS, including the use of bracing, casting, halo-gravity traction, fusionless spine techniques, definitive fusion, and chest wall devices. RESULTS: Members of Pediatric Orthopaedic Society of North America (19.8%) completed the survey with the vast majority of respondents (93.8%) treating children with EOS. Sixty-six percent of respondents had access to a casting table and 77% reported access to halo-gravity traction. Access to casting tables and access to halo-gravity traction was associated with the use of casting and traction (P<0.0001). Equal numbers of surgeons currently use casting (62%) and growing spine techniques (64.1%). Chest wall expansion was offered as a treatment option by 39.1% of surgeons, and 27% of surgeons reported the use of halo-gravity traction. Ninety-three percent of respondents chose nonoperative management of a 2-year-old child with a 50° progressive scoliosis. In contrast, 63% of surgeons would offer surgery as the initial management to a 5-year-old child with a progressive 70° idiopathic scoliosis. CONCLUSIONS: The majority of respondents had access to halo traction and casting tables at their hospitals. There was a statistically significant association between access to equipment and use of casting and halo traction. Nonoperative management was the preferred treatment option in the very young (2 year-old). Two-thirds of surgeons report initial surgical management of the 5-year-old child with a large idiopathic curve.

Reliability and validity of a kyphosis-specific spinal appearance questionnaire.

PURPOSE: Hyperkyphosis, including Scheuermann and postural kyphosis, is associated with decreased perceived cosmesis and well being. No patient reported outcome questionnaire specific to kyphosis exists. We sought to assess the internal consistency, test-retest reliability and concurrent validity of a new Kyphosis-specific Spinal Appearance Questionnaire (K-SAQ). METHODS: A K-SAQ was developed from a modified SAQ to measure kyphosis-specific aspects of appearance. Patients with hyperkyphosis (ages 10-20 years) curves ≥ 50° completed the K-SAQ and SRS-22R at baseline and the K-SAQ 2 weeks later. RESULTS: 55 patients completed the K-SAQ and SRS-22R. 28 patients completed the K-SAQ 2 weeks later. The K-SAQ total averages showed excellent internal consistency (Cronbach's α = 0.91) and test-retest reliability (ICC = 0.84). Moderate associations were seen between the SRS-22R subtotal average score and K-SAQ total average score and (r = - 0.62, p < 0.001) and the SRS-22R self-image domain with the K-SAQ total average score (r = - 0.57, p < 0.001). Higher BMI and increased age scored worse on the K-SAQ total average, whereas only higher BMI scored worse on the SRS-22R subtotal average. CONCLUSION: The K-SAQ is a reliable patient reported outcome measure of kyphosis-specific aspects of appearance.

Comparison of Outcomes by Reconstructive Strategy in Patients with Prostheses for Proximal Femoral Focal Deficiency.

BACKGROUND: The present study compares prosthetic treatment options for proximal femoral focal deficiency in terms of gait analysis, oxygen consumption, and patient-reported outcomes. METHODS: Twenty-three patients who had been managed with a prosthesis for unilateral proximal femoral focal deficiency underwent gait analysis; this group included 7 patients who had received an equinus prosthesis, 6 who had received a rotationplasty prosthesis, and 10 who had undergone Syme amputation and had received an above-the-knee prosthesis. Cadence parameters, kinematic and kinetic data, and oxygen consumption were measured, and the Gait Deviation Index (GDI) was calculated. Medical records and radiographs were reviewed. The Pediatric Outcomes Data Collection Instrument (PODCI) was completed by the child's parent. RESULTS: Patients underwent gait analysis at a mean age of 11.6 years (range, 4 to 19 years). Proximal femoral focal deficiency classification was not predictive of the chosen treatment. Patients in the rotationplasty group had undergone more procedures than those in the Syme amputation and equinus groups (mean, 3.3, 1.8, and 0.7 procedures, respectively) (p = 0.001). Oxygen cost did not differ between groups; however, all required greater energy expenditure than normal (170%, 144%, and 159%, in the equinus, rotationplasty, and Syme amputation groups, respectively) (p = 0.427). Likewise, hip power, abductor impulse, and GDI did not differ, but all groups had GDI scores >3 standard deviations below normative values. Patients in the equinus group walked faster (97% of normal for age) than those in the rotationplasty (84%) and Syme amputation groups (83%) (p = 0.018), whereas those in the Syme amputation group had superior knee range of motion (55° from the prosthetic knee) than those in the equinus (20°) and rotationplasty groups (15° generated from the ankle) (p = 0.003). There were no differences in terms of the PODCI subscales for pain, sport/physical function, happiness, or global function. Transfer/basic mobility improved with age (r = 0.516, p = 0.017), but no other associations were found between gait variables and PODCI scores. CONCLUSIONS: Rotationplasty provided no patient-reported benefit and no functional benefit in terms of gait parameters or oxygen consumption, despite requiring more surgical procedures compared with other prosthetic options. Patients with an equinus prosthesis walked the fastest, whereas treatment with a Syme amputation and prosthetic knee yielded equivalent gait parameters and oxygen consumption as compared with those for patients using an equinus prosthesis. These findings contradict those of previous reports that rotationplasty provides superior function over other proximal femoral focal deficiency prosthetic treatment options. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

The 18-cm Thoracic-Height Threshold and Pulmonary Function in Non-Neuromuscular Early-Onset Scoliosis: A Reassessment.

BACKGROUND: Thoracic spine height is cited as a crucial outcome measure in the treatment of early-onset scoliosis (EOS) because of its reported relationship to pulmonary function tests (PFTs). An 18-cm threshold has been proposed, although this single parameter might be overly simplistic for cases of different etiologies and deformity magnitude. We aimed to reevaluate pulmonary function in patients undergoing corrective surgery, assessing the role of residual scoliosis as well as spine elongation. METHODS: Patients undergoing EOS correction with a minimum of 5 years of follow-up since initial treatment were evaluated. Standard spirometry (forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1]) was correlated to deformity magnitude and T1-T12 height. Patients were compared by age at first surgery (<5 or ≥5 years), final thoracic height (≤18 or >18 cm), and percentage of predicted pulmonary function (<60% or ≥60%). RESULTS: Twenty-nine patients (15 congenital, 11 syndromic, and 3 idiopathic cases) were tested at a mean of 8.5 years following initial surgery. Twenty-two patients (mean initial age, 4.8 years) had growth-sparing instrumentation, and 7 patients (age, 5.1 years) had definitive fusion performed. Age at initial surgery was not associated with a difference in PFT results at the time of follow-up, and both age groups had ominously low percentages of predicted pulmonary-function volumes (50% to 55%). Only 18 of the 29 patients achieved a T1-T12 height of >18 cm. Those with a thoracic height of ≤18 cm had similar percentage-of-predicted spirometry results at the time of follow-up as those with greater thoracic height, possibly because of increased deformity correction. Only 14 of 29 patients had spirometry of ≥60% of predicted volume at the time of follow-up. These 14 had slightly smaller curves and slightly greater T1-T12 heights but significantly better spirometry results than the 15 subjects with <60% of predicted volume. For those with a T1-T12 height of ≤18 cm, the residual Cobb angle negatively correlated with spirometry results. In those with a final T1-T12 height of >18 cm, spirometry did correlate with thoracic height, especially when residual deformity was ≥60°. CONCLUSIONS: Regardless of thoracic height of ≤18 or >18 cm, with residual curves of >50o, pulmonary function was ominously low in fully half of the patients, raising doubt about the value of this threshold as an EOS outcome parameter. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

The growth-friendly surgical treatment of scoliosis in children with osteogenesis imperfecta using distraction-based instrumentation.

PURPOSE: The study was undertaken to determine the feasibility of growth-friendly distraction-based surgery in children with OI. METHODS: Two multi-center databases were queried for children with OI who had undergone GR or VEPTR surgery. Inclusion criteria were a minimum 2-year follow-up and three lengthening procedures following the initial implantation. Details of the surgical techniques, surgical complications, and radiographic measurements of deformity correction, T1-T12 and T1-S1 elongation and growth were recorded. RESULTS: Five patients were identified. There was one patient with type I OI and two patients each with type III and type IV. Four patients had GR constructs and one a VEPTR construct. The initial scoliosis deformity averaged 80° (70°-103°), and the subsequent corrections averaged 32% for initial correction, 48% at last follow-up, and 54% for the two patients that had a final fusion. The T1-T12 and T1-S1 growth averaged 31 mm and 44 mm respectively, and yearly growth averaged 4 mm and 6 mm, respectively. Growth was notably much less in those with more severe disease. There were 13 complications in 4 patients. Nine of the 10 surgical complications were anchor failures which were corrected in 7 planned and 2 un-planned procedures. Significant migration occurred in one patient with severe OI type III. CONCLUSION: The results varied in this heterogeneous population. In general, satisfactory deformity corrections were obtained and maintained, modest growth was obtained, and complications were similar to those reported in other series of growth-friendly surgery. Limited growth and significant anchor migration are to be anticipated in this population. LEVEL OF EVIDENCE: IV.

An initial effort to define an early onset scoliosis "graduate"-The Pediatric Spine Study Group experience.

PURPOSE: Increasingly, patients with early onset scoliosis (EOS) are completing a growth friendly surgical program followed by observation, removal of implants or a definitive spinal fusion. These patients are colloquially referred to as "graduates". A standardized definition of a graduate is needed for research and comparing the outcomes, family counseling, and a better understanding of the population. METHODS: A 15-question electronic survey was completed by 39 experienced pediatric spine surgeons to identify factors salient to the definition of a graduate of EOS surgical programs. A Delphi/Nominal group technique session with nine questions was then performed face-to-face with 21 members of the Pediatric Spine Study Group to discuss and refine the definition. A follow-up electronic survey was then distributed to these same 21 members to gain consensus on the final definition. RESULTS: From the initial survey, it was identified that a graduate did not require definitive spinal fusion after a growing program. From the Delphi session, it was determined that skeletal maturity was the most important factor in defining a graduate. A strictly defined minimum length of follow-up was not felt to be a prerequisite for qualification of graduation. After the final electronic version was distributed, > 80% of respondents agreed upon the final definition, thereby achieving consensus. CONCLUSION: The Pediatric Spine Study Group recommends adoption of the following definition: a "graduate" is a patient who has undergone any surgical program to treat early onset scoliosis, and has reached skeletal maturity and does not have a planned surgical intervention for EOS in the future. LEVEL OF EVIDENCE: V.

Functional results after surgical treatment for congenital knee dislocation.

BACKGROUND: Congenital knee dislocation (CDK) is a rare congenital deformity, which often requires surgery for treatment. Little objective data exist characterizing the outcome of patients who require operative treatment for this condition. The purposes of this study were to objectively evaluate the functional, clinical, and gait outcomes of patients who underwent surgical treatment of CDK; and compare the results of outcome between 2 surgical approaches for this condition: quadricepsplasty and femoral shortening. METHODS: We performed a retrospective review of all patients (7) treated surgically for CDK. Patients were evaluated at an average follow-up of 12+6 years. Each patient underwent a clinical examination, functional evaluation using the Lysholm Knee Questionnaire and Pediatric Outcomes Data Collection Instrument, and a 3-dimensional gait evaluation. The results of the total group were compared with normal controls. Additionally, results of the patients treated with quadricepsplasty were compared with patients treated with femoral shortening. RESULTS: Total knee range of motion for the entire group averaged 112 degrees, with 8 of the 9 knees having flexion>90 degrees. Seven of the 9 knees were found to have some degree of instability on examination, yet none of the patients reported using any form of brace for ambulation. Functional evaluation showed good knee specific and overall function, comparable to normal controls. There were no differences in clinical or functional outcomes between the 2 surgical approaches. Gait analysis revealed a stiff-knee gait pattern to the congenital knee dislocation group, as compared with normal controls, and subtle differences in knee function between the surgical approaches. CONCLUSIONS: The function of patients after surgical treatment for CDK seems to be quite good compared with normal controls. Good knee specific and overall function scores are reported with limitations seen only in higher demand activities. Despite instability of the knee noticed on clinical examination, patients ambulate without braces and have a functional knee range of motion. Little difference in outcome was seen between the 2 surgical approaches used to treat this condition. LEVEL OF EVIDENCE: Therapeutic Study, Level III.