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.

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.

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.

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.

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.

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.

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 Effect of Proximal Anchor Choice During Distraction-based Surgeries for Patients With Nonidiopathic Early-onset Scoliosis: A Retrospective Multicenter Study.

BACKGROUND: It is unclear whether the type of proximal anchor affects the spine length achieved with distraction-based surgeries in patients with nonidiopathic early-onset scoliosis (EOS). Since distraction may produce kyphosis, spine length should be assessed in the sagittal plane using the sagittal spine length (SSL-curved arc length of the spine in the sagittal plane). Our purpose was to determine if the type of proximal anchor in distraction-based surgeries will affect final spine length. METHODS: Patients with nonidiopathic EOS treated with distraction-based systems (minimum 5 y follow-up, 5 lengthenings) were identified from 2 EOS registries. Radiographic analysis preoperative, postimplant (L1), and after each lengthening (L2-L5, L6-L10, L11-L15) was performed with the primary outcome of T1-S1 SSL. RESULTS: We identified 126 patients-70 had rib-based implants (52 congenital, 9 syndromic, 9 neuromuscular) and 56 had spine-based implants (15 congenital, 29 syndromic, 12 neuromuscular) with preoperative age 4.6 years, scoliosis 75 degrees, and kyphosis 48 degrees. After initial correction (P<0.05), scoliosis remained constant [58 degrees (13 to 104 degrees) at L11-L15] and kyphosis increased over time [38 degrees (9 to 108 degrees) at L1 to 60 degrees (17 to 134 degrees) at L11-L15] (P<0.05). Preoperative SSL was higher in the spine-based group (29.6 cm) when compared with the rib-based group (25.2 cm) (P<0.05). This difference was maintained after initial implantation (spine-based: 32.2 cm vs. rib-based: 26.7 cm, P<0.05) and at final follow-up (spine-based: 37.0 cm vs. rib-based: 34.4 cm, P<0.05). As preoperative SSL differed between groups, maximum SSL gains per interval were also normalized to preoperative SSL. There was no statistically significant difference between groups at L1, L2-L5, and L6-L10. However, at L11-L15, the rib-based group achieved a more relative increase in spine length compared with the spine-based group (45% vs. 31%, P<0.05). CONCLUSION: At minimum 5 year follow-up, distraction-based surgeries increased spine length for patients with nonidiopathic EOS; regardless of proximal anchor type.

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.

Continued deterioration in pulmonary function at average 23-year follow-up from early thoracic fusion in non-neuromuscular scoliosis.

PURPOSE: Historically, early-onset scoliosis was treated with early fusion to prevent further deformity at the expense of thoracic growth. This has proven to have a detrimental effect on pulmonary function. The purpose of this study is to evaluate patients' pulmonary and functional status at long-term follow-up after undergoing thoracic fusion at a young age. METHODS: All patients at a single institution who had undergone thoracic spinal fusion prior to age nine with minimum 13-year follow-up were eligible. Patients underwent pulmonary function testing, radiographic analysis, and functional testing. Results were compared to the patients' previous pulmonary function data at average of 11 years post-surgery. RESULTS: Fifteen out of twenty-eight eligible patients returned for testing. The average age at the time of surgery was 3.3 years (range 0.9-8.4 years) with follow-up of 23.6 years (range 13.2-33.2 years). There was a statistically significant interval decline in predicted forced vital capacity (42.8% versus 54.7% of normal predicted values, p = 0.0001) and predicted forced expiratory volume in one second (42.2% versus 55.2% of normal predicted values, p = 0.0001) when compared to previous follow-up. There was a strong positive correlation between thoracic height and forced vital capacity (r = 0.925, p = 0.002). CONCLUSIONS: Pulmonary function in patients who had undergone thoracic spinal fusion for scoliosis prior to the age of six continues to decline into adulthood at a rate that is faster than that of their peers. The majority of these patients have clinically important restrictive lung disease, which can be fatal. Alternative treatment strategies should be considered. LEVEL OF EVIDENCE: III.

Can distraction-based surgeries achieve minimum 18 cm thoracic height for patients with early onset scoliosis?

PURPOSE: Karol et al. introduced the concept that 18 cm thoracic height is the critical point where a patient with early onset scoliosis (EOS) can maintain adequate pulmonary function. Our purpose was to determine if distraction-based surgeries will increase thoracic spine height to at least 18 cm in patients with EOS. METHODS: Patients with EOS treated with distraction-based systems (minimum 5 years follow up, minimum five lengthenings). Radiographic analysis of thoracic spine height (T1-T12) at the last lengthening procedure. RESULTS: One hundred and fifty-three patients (67 congenital, 21 neuromuscular, 38 syndromic, 27 idiopathic) with pre-operative mean age 4.6 years, scoliosis 75°, kyphosis 47° were evaluated. Their mean age at final lengthening procedure was 11 years (6-16), average number of lengthening procedures was 10.5 (4-21), mean final scoliosis was 53°, and mean final kyphosis was 58°. Final thoracic height was > 18 cm in 65% and was > 22 cm in 31% of patients. Based on etiology, only 48% of the congenital patients reached 18 cm compared to 81% neuromuscular, 84% syndromic and 67% idiopathic. This height gain was closely related to the percentage of scoliosis correction achieved for each etiology. Comparing congenital etiology to other etiologies, there was a lower percentage of patients in the congenital group that passed the 18 cm threshold (48% vs. 78%) (p < 0.05). CONCLUSION: At minimum 5 years follow up, distraction-based surgeries increased thoracic height for patients with EOS to greater than 18 cm in 65% of patients; however, only 48% of congenital patients reached this thoracic height threshold. DESIGN: Retrospective review of prospectively collected registry data. LOI III.

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.

Minimum 5-Year Follow-up on Graduates of Growing Spine Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth friendly (GF) surgery for early onset scoliosis, patients undergo spinal fusion or continued observation. This last planned treatment is colloquially called "definitive" treatment, conferring these patients as "graduates" of a growing program. The 5-year radiographic and clinical outcomes of this cohort are unknown. METHODS: An international pediatric spine database was queried for patients from a GF program (spine or rib-based) with minimum 5-year follow-up from last planned surgery (GF or spinal fusion). Radiographs and charts were reviewed for main coronal curve angle and maximum kyphosis as well as occurrence of secondary surgery. RESULTS: Of 580 graduates, 170 (29%) had minimum 5-year follow-up (37% male). Scoliosis etiology was congenital in 41 (24%), idiopathic 36 (21%), neuromuscular 51 (30%), and syndromic 42 (25%). Index surgery consisted of spine-based growing rods in 122 (71%) and rib-based distraction in 48 (29%). Mean age at index surgery was 6.8 years, and patients underwent an average of 5.4 lengthenings over an average of 4.9 years (range, 6 mo to 11 y). Last planned treatment was at an average age of 11.8 years (range, 7 to 17 years). Last planned treatment consisted of spinal fusion in 114 patients, 47 had growing implants maintained, 9 had implants removed. Average follow-up was 7.3 years (range, 5 to 13 y).When compared from postdefinitive treatment to 2-year follow-up, there was noted progression of the coronal curve angle (46±19 to 51±21 degrees, P=0.046) and kyphosis (48±20 to 57±25 degrees, P=0.03). However, between 2 and 5 years, no further progression occurred in the coronal (51±21 to 53±21 degrees, P=0.26) or sagittal (57±25 to 54±28 degrees, P=0.93) planes. When stratified based on etiology, there was no significant coronal curve progression between 2- and 5-year follow-up. When comparing spinal fusion patients to those who had maintenance of their growing construct, there was also no significant curve progression.Thirty-seven (21%) underwent at least 1 (average, 1.7; range, 1 to 7) revision surgery following graduation, and 15 of 37 (41%) underwent 2 or more revision surgeries. Reason for revision was implant revision (either GF or spinal fusion) in 34 patients, and implant removal in 3. On an average, the first revision was 2.5 years after the definitive treatment plan (range, 0.02 to 7.4 y). In total, 15 of 37 (41%) revisions occurred over 2 years following the final decision for treatment plan, and 7 of 37 (19%) occurred 5 or more years after the definitive treatment.Patients who underwent spinal fusion as a definitive treatment strategy were more likely to undergo revision surgery (27%) than patients who had their GF implants maintained (11%) (P=0.04). CONCLUSIONS: Five years following "graduation" from growing surgery for early onset scoliosis, there is progression of curve magnitude in both the coronal and sagittal planes up to 2 years, with no further progression at 5 years. A total of 21% of patients undergo at least 1 revision surgery, and average time to revision surgery is over 2 years from last planned surgery. Risk of revision surgery was higher in patients who underwent a spinal fusion as their definitive treatment strategy. LEVEL EVIDENCE: Level III-retrospective comparative. TYPE OF EVIDENCE: Therapeutic.

Distraction-based surgeries increase thoracic sagittal spine length after ten lengthening surgeries for patients with idiopathic early-onset scoliosis.

STUDY DESIGN: Retrospective, comparative, multicenter. OBJECTIVES: To determine if the choice of proximal anchor affects thoracic sagittal spine length (SSL) for children with idiopathic early-onset scoliosis (EOS). Debate exists as to whether spine growth is maintained during treatment for EOS. As rib- (RB) and spine-based (SB) distraction procedures may be kyphogenic, the traditional measurement of spine growth on coronal radiographs may not identify out-of-plane increase in spine length. A measure of SSL, along the spine's sagittal arc of curvature, has been validated to reliably assess the length of the thoracic spine. METHODS: Patients with idiopathic EOS treated with distraction-based systems (minimum 5-year follow-up, five lengthening surgeries) with radiographic analysis preoperatively, postimplant (L1), and during lengthening periods (L2-L5, L6-L10) were evaluated with primary outcome of T1-T12 SSL. RESULTS: We identified 34 patients (14 RB, 20 SB) with preoperative age 4.9 years (4.2 RB vs. 5.4 SB), scoliosis 72° (60° RB vs. 77° SB; p < 0.05), kyphosis 39° (50° RB vs. 34° SB; p < 0.05), and SSL 17.8 cm (15.5 RB vs. 18.5 SB; p < 0.05). After initial scoliosis correction from implantation, scoliosis remained constant over time. RB patients had greater kyphosis than SB patients: L1, 46° RB vs. 19° SB (p < 0.05); L2-L5, 50° RB vs. 27° SB (p < 0.05); L6-L10, 56° RB vs. 26° SB (p < 0.05). SSL increased for both groups from preoperative to the tenth lengthening (p < 0.05). As compared with RB patients, SB patients had higher SSL preoperatively and maintained this difference to the tenth lengthening (p < 0.05). After ten lengthening surgeries, when normalized to preoperative SSL, relative thoracic growth was greater for RB (27%) than for SB patients (19%) (p < 0.05). CONCLUSION: Regardless of proximal anchor choice, thoracic length continued to increase during the distraction phase of treatment for idiopathic EOS. LEVEL OF EVIDENCE: Level III.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

AIMS: The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. PATIENTS AND METHODS: A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. RESULTS: Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). CONCLUSION: Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.

Distraction-Based Surgeries Increase Spine Length for Patients With Nonidiopathic Early-Onset Scoliosis-5-Year Follow-up.

STUDY DESIGN: Retrospective, comparative. OBJECTIVES: To determine if distraction-based surgeries will increase spine length in patients with nonidiopathic EOS and whether etiology affects final spine length. SUMMARY OF BACKGROUND DATA: As early-onset scoliosis (EOS) has many etiologies, it is unclear whether etiology affects the spine length achieved with distraction-based surgeries. Since distraction may produce kyphosis, sagittal spine length (SSL; curved arc length of the spine in the sagittal plane) should be utilized. METHODS: Patients with nonidiopathic EOS treated with distraction-based systems (minimum 5-year follow-up, 5 lengthenings) were identified from two EOS registries. Radiographic analysis preoperation, postimplant (L1), and after each lengthening (L2-L5, L6-L10, L11-L15) was performed with primary outcome of T1-S1 SSL. RESULTS: We identified 126 patients (67 congenital, 38 syndromic, 21 neuromuscular) with a mean preoperative age of 4.6 years, scoliosis 75°, kyphosis 48°, and a mean of 12 lengthenings. After initial correction (p < .05), scoliosis remained constant (58° at L11-L15) and kyphosis increased (38° at L1 to 60° at L11-L15) (p < .05). SSL increased for the entire group from 27.1 cm preoperation to 35.3 cm at L11-L15 (p < .05) and during the distraction phase (29.2 cm at L1 to 35.3 cm at L11-L15) (p < .05). Preoperative SSL was higher in neuromuscular compared with congenital patients and maintained that difference until the 10th lengthening. Preoperative SSL did not differ between syndromic and congenital patients (28.0 cm vs. 25.6 cm); however, syndromic patients had greater SSL after implantation (L1: 30.5 cm vs. 26.8 cm) (p < .05) and maintained that difference until the 15th lengthening (37.1 cm vs. 34.3 cm) (p < .05). CONCLUSION: At minimum 5-year follow-up, distraction-based surgeries increased spine length for all patients with nonidiopathic EOS; however, neuromusculars had higher preoperative spine length compared with congenital patients and maintained that difference until the 10th lengthening. Although congenital and syndromic patients had similar preoperative spine length, syndromic patients had greater SSL after implantation (L1) and maintained that difference until the 15th lengthening. LEVEL OF EVIDENCE: Level III.

Congenital Pseudarthrosis of the Tibia: Results, at Skeletal Maturity, of the Charnley-Williams Procedure.

BACKGROUND: This study assessed the outcomes, at skeletal maturity, for 34 patients in whom congenital pseudarthrosis of the tibia (CPT) had been treated with intramedullary (IM) rod fixation. METHODS: The results in skeletally mature patients in whom type-4 CPT had been treated with an IM rod at an average of 11.9 years earlier were reviewed. The rod procedures varied according to whether both the tibia and the fibula were resected and both bones (type A) or just the tibia (type B) were fixed with an IM rod or whether only the tibia was resected and the fibula received no surgery (type C). Outcome grading ranged from unequivocal union with brace-free function (grade 1) to a functional limb with residual angulation or cortical defects (grade 2) to a severely impaired extremity with insufficient union or refracture (grade 3). RESULTS: Thirty-four patients were evaluated at a mean age of 16.9 years, a mean of 11.9 years after their initial surgical procedure. Seventeen patients had a grade-1 result; 11 patients, grade 2; and 6, grade 3. Thus, 82% (28) of the 34 patients had a functional extremity at maturity. All patients with a final grade-3 outcome eventually requested amputation. The final outcomes were not affected by the age at the initial fracture or surgery, the presence of neurofibromatosis-1, or cross-ankle fixation. A total of 58 IM rod procedures were performed in the 34 patients. Twenty-four (73%) of the 33 type-A procedures produced grade-1 or 2 outcomes, as did 14 (88%) of the 16 type-B procedures. Of the 9 type-C procedures, none produced a grade-1 result and 4 produced a grade-2 outcome. The results of types A and B combined were superior to those of type-C procedures (p = 0.03). Refracture occurred in 13 of 33 patients with initial stability/union after rod fixation, with 3 of those fractures remaining ununited at the latest follow-up. A dystrophic fibula had no effect on the eventual achievement of a grade-1 or 2 outcome. CONCLUSIONS: This review, in which all patients had reached skeletal maturity, documents functional (grade-1 or 2) outcomes in 82% of cases of IM rod fixation for CPT. This finding was almost identical to the result in our earlier report and confirms the long-term value of permanent IM rod fixation in maintaining union and function in patients with CPT. Procedures not addressing the fibula produced inferior results. Although 13 patients had a refracture following initial union, only 3 of these fractures failed to heal with additional treatment. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.