Outcome of distraction-based growing rods at graduation: a comparison of traditional growing rods and magnetically controlled growing rods.

INTRODUCTION: Distraction-based growing rods have been considered as an alternative surgical option for the operative treatment of EOS. TGR has been challenged by MCGR, which is reported to have the advantage of non-invasive lengthening with fewer planned returns to theatre. This study explores the radiographic outcomes, Unplanned Returns to the Operating Room (UPROR) and complication profile of both the procedures at the end of the planned growing rod treatment with either TGR or MCGR. METHODS: We included all the EOS cases from the PSSG database that underwent either TGR or MCGR with spine-based proximal anchors, followed up to the time of graduation. Any crossover or hybrid procedures were excluded. 549 patients (409 TGR and 140 MCGR) were eligible for review. We measured the coronal curve magnitude, Kyphosis, T1-T12, T1-S1 and L1-S1 lengths at 4 time points (before and after the index surgery and before and after the definitive surgery). RESULTS: The TGR group were slightly younger at the time of the index procedure (7 years for TGR vs. 8.5 years for MCGR, p < 0.001). We noted an improvement in all radiological parameters after the growing rod implantation. The spinal lengths increased through the lengthening period, while the coronal curve magnitude and the kyphosis increased. The kyphosis normalized following the final fusion, the coronal curve magnitude reduced further with a further increase in spinal lengths. The final follow-up from the time of the index implantation to the definitive surgery was 5.1 years (IQR 3.8) in TGR and 3.5 years (IQR 1.65) in the MCGR groups. The total number of complications was fewer in the MCGR group. The overall risk of UPROR was lower in the MCGR group and implant breakage was less in the MCGR group by 4.7 times. CONCLUSIONS: This study confirms the equivalence of both the distraction-based growing rods systems from the radiological stand-point, during the lengthening phase and at the time of the definitive surgery. The TGR was more kyphogenic during the lengthening period. The complications and UPROR were fewer in the MCGR groups.

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.

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.

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.

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.

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.

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.

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.

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.

Comparison of implant density in the management of lenke 1b and 1c adolescent idiopathic scoliosis / Comparação da densidade do implante no tratamento da escoliose idiopática do adolescente lenke 1b e 1c

ABSTRACT Objective: To compare radiographic and surgical outcomes of Lenke 1B and 1C patterns. Methods: One hundred twenty patients with Lenke 1B and 1C scoliosis were grouped according to implant density as follows: low density (LD) of ≤1.4 and high density (HD) of >1.4. Matched subgroups (30 patients each) based on age, curve magnitude, and body mass index (BMI) were analyzed. Radiographic parameters were evaluated before operation, immediately after operation (ipo), and at 2 years' follow-up. SRS-30 was administered before operation and at 2 years' follow-up. Results: The major curves of the LD (n = 82) and HD groups (n=38) were respectively 59.1° and 65.6° before operation (p <.001), 26.3° and 22.9° ipo (p =.05), and 29.9° and 19.8° at 2 years' follow-up (p <.001). No significant differences in postoperative trunk shift and coronal balance were found (p =.69 and p =.74, respectively). The HD group had higher blood loss (p =.02), number of implants (p <.001), levels fused (p =.002), and surgical time (p <.001). The HD group had a higher prevalence of hypokyphosis from before operation to follow-up (p <.001). No significant differences were observed in the SRS-30 scores before operation and at 2 years' follow-up. The matched groups had similar preoperative major curves (p =.56), ages (p =.75), and BMIs (p =.61). Significantly longer surgical time (p =.009), higher density (p <.001), and better correction (p =.0001) were found in the HD group at 2 years' follow-up. No significant differences were found in the SRS-30 scores before operation and at 2 years' follow-up. Conclusion: LD constructs included fewer segments fused, lower intraoperative estimated surgical blood loss, and shorter operation time, and potentially decreasing complication risks due to fewer implants. Level of evidence III, Retrospective Cohort Study.

RESUMO Objetivo: Comparar os desfechos radiográficos e cirúrgicos da escoliose Lenke 1B e 1C. Métodos: Cento e vinte pacientes com escoliose Lenke 1B e 1C foram agrupados de acordo com a densidade do implante, como segue: baixa densidade (BD) de ≤ 1,4 e alta densidade (AD) de > 1,4. Foram analisados os grupos pareados (30 pacientes cada) com base na idade, magnitude da curva e índice de massa corporal (IMC). Os parâmetros radiográficos foram avaliados antes da cirurgia, no pós-operatório imediato (POI) e no acompanhamento de dois anos. O questionário SRS-30 foi administrado antes da cirurgia e no acompanhamento de dois anos. Resultados: As principais curvas dos grupos BD (n = 82) e AD (n = 38) foram respectivamente 59,1° e 65,6° antes da operação (p < 0,001), 26,3° e 22,9° no POI (p = 0,05) e 29,9° e 19,8° aos 2 anos de acompanhamento (p < 0,001). Não foram encontradas diferenças significantes no desvio do tronco e no balanço coronal no pós-operatório (p = 0,69 e p = 0,74, respectivamente). O grupo AD teve mais perda sanguínea (p = 0,02), número de implantes (p < 0,001), níveis de fusão (p = 0,002) e tempo de cirurgia (p < 0,001). O grupo AD teve maior prevalência de hipocifose do período anterior à cirurgia até o acompanhamento (p < 0,001). Não houve diferenças significantes nas pontuações do SRS-30 antes da operação e aos 2 anos de acompanhamento. No pré-operatório, os grupos pareados tinham curvas principais (p = 0,56), idade (p = 0,75) e IMC (p = 0,61) semelhantes. Constatou-se tempo cirúrgico expressivamente maior (p = 0,009), maior densidade (p < 0,001) e melhor correção (p = 0,0001) no grupo AD aos 2 anos de acompanhamento. Não foram encontradas diferenças significantes nas pontuações do SRS-30 antes da cirurgia e no acompanhamento de 2 anos. Conclusão: As estruturas de BD incluíram menos segmentos fundidos, menor perda de sangue intraoperatória estimada, menor tempo de cirurgia e menos risco de complicações, com possibilidade de redução, por causa do menor número de implantes. Nível de evidência III, Estudo retrospectivo de coorte.

Surgical and Health-related Quality-of-Life Outcomes of Growing Rod "Graduates" With Severe versus Moderate Early-onset Scoliosis.

STUDY DESIGN: A retrospective review of a prospective, multicenter database. OBJECTIVE: The aim of this study was to compare surgical and quality-of-life outcomes at the end of growing rod treatment in patients with severe versus moderate early-onset scoliosis (EOS). SUMMARY OF BACKGROUND DATA: Knowledge of the outcomes of severe EOS after growth-friendly treatment is limited because this condition is uncommon. METHODS: We identified 40 children with severe EOS (major curve ≥90°) treated with growing rods before age 10 with minimum 2-year follow-up after last lengthening or final fusion. From the same registry, we matched 40 patients with moderate EOS (major curve < 90°). Twenty-seven patients in the severe group and 12 in the moderate group underwent final fusion (P < 0.001). RESULTS: Mean preoperative curves were 102° (range, 90°-139°) in the severe group and 63° (range, 33°-88°) in the moderate group (P < 0.001). At final follow-up, mean curves were 56° (range, 10°-91°) and 36° (range, 12°-89°), respectively (P < 0.001). Fourteen (35%) children in the severe group and 32 (80%) in the moderate group had scoliosis of < 45° at final follow-up [risk ratio (RR), 0.44; 95% confidence interval (95% CI), 0.20-0.57]. At final follow-up, 30 (75%) children in the severe group and 35 (88%) in the moderate group had achieved T1-T12 length of ≥18 cm (RR, 0.86; 95% CI, 0.70-1.09). Thirty-five children in the severe group and 26 in the moderate group had at least one complication (RR, 1.35; 95% CI, 1.05-1.73). Mean 24-Item Early-Onset Scoliosis Questionnaire scores were similar between groups at final follow-up. CONCLUSION: Delaying surgery until the major curve has progressed beyond 90° is associated with larger residual deformity and more complications than treating at a lesser curve magnitude. Quality-of-life outcomes were similar between those with severe and moderate EOS. LEVEL OF EVIDENCE: 3.

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.

Surgeon Survey Shows No Adverse Events With MRI in Patients With Magnetically Controlled Growing Rods (MCGRs).

STUDY DESIGN: Surgeon survey. OBJECTIVES: To determine if magnetic resonance imaging (MRI) following implantation of magnetically controlled growing rods (MCGRs) is associated with any adverse events. SUMMARY OF BACKGROUND DATA: Magnetically controlled growing rods have been shown to reduce the need for repeated surgical procedures and improve costs when compared to traditional growing rods, but concerns about MRI compatibility exist. MRIs are often clinically indicated in the EOS population. METHODS: Pediatric spine surgeons who are members of the Growing Spine Study Group, Children's Spine Study Group, and early international users of this technology were surveyed regarding MRI use after performing MCGR surgery. RESULTS: A total of 118 surgeons were surveyed. Four surgeons reported that 10 patients had an MRI with an implanted MCGR. Loss of fixation (0%, 0/10), movement of implants (0%, 0/10), unintended lengthening/shortening (0%, 0/10), or noticeable heating of MCGR (0%, 0/10) were not observed. No problems were observed with function of the MCGR following MRI, and a mean of 2.1 mm was obtained at the next lengthening (range, 0.5-3.0 mm). Two patients had brain MRIs, both of which could be interpreted. All cervical spine MRIs could be interpreted without excessive artifact (100%, 7/7). Six patients had MRIs of the thoracic or lumbar spine, but these were considered uninterpretable as a result of artifact from the MCGR device (0%, 0/6). CONCLUSION: These are the first reported cases of MRI use in humans with MCGR. There were no adverse events observed. MCGR rods lengthened as expected following MRI. MRIs of the brain and cervical spine were able to be interpreted, but MRIs of the thoracolumbar spine could not be interpreted because of MCGR artifact. MRIs can be safely performed in patients with MCGRs; however, MRIs of thoracic and thoracolumbar spine may be of limited clinical benefit because of artifact. LEVEL OF EVIDENCE: Level IV, case series.

Comparison of Percentile Weight Gain of Growth-Friendly Constructs in Early-Onset Scoliosis.

STUDY DESIGN: Multicenter retrospective cohort. OBJECTIVE: To compare improvement in nutritional status seen in early-onset scoliosis (EOS) patients following treatment with various growth-friendly techniques, especially in underweight patients (<20th weight percentile). BACKGROUND: Thoracic insufficiency resulting from EOS can lead to severe cardiopulmonary disease. In this age group, pulmonary function tests are often difficult or impossible to perform. Weight gain has been used in prior studies as a proxy for improvement and has been demonstrated following VEPTR and growing rod implantation. In this study, we aim to analyze weight gain of EOS patients treated with four different spinal implants to evaluate if significant differences in weight percentile change exist between them. METHODS: Retrospective review of patients treated surgically for EOS was performed from a multicenter database. Exclusion criteria were index instrumentation at >10 years old and <2 years' follow-up. RESULTS: 287 patients met the inclusion criteria and etiologies were as follows: congenital = 85; syndromic = 79; neuromuscular = 69; and idiopathic = 52. Average patient age at surgery was 5.41 years, with an average follow-up of 5.8 years. Preoperatively, 55.4% (162/287) fell below the 20th weight percentile. There was no significant difference in preoperative weight between implants (p = .77), or diagnoses (p = .25). Among this group, the mean change in weight percentile was 10.5% (range: -16.7% to 88.7%) and all implant groups increased in mean weight percentile at final follow-up. There were no significant differences in weight percentile change between the groups when divided by implant type (p = .17). CONCLUSIONS: Treatment of EOS with growth-friendly constructs resulted in an increase in weight percentile for underweight patients (<20th percentile), with no significant difference between constructs. LEVEL OF EVIDENCE: Level III.

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.