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.

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.

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.

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.

What Factors Influence Union and Refracture of Congenital Pseudarthrosis of the Tibia? A Multicenter Long-term Study.

OBJECTIVE: To identify factors influencing union of congenital pseudarthrosis of the tibia (CPT), refractures, and integrity of the tibia at maturity. METHODS: Data of 119 children operated for Crawford-type IV CPT and followed-up till skeletal maturity were analyzed. Logistic regression and recursive partitioning analyses were used to test associations between several variables and the outcome. RESULTS: Primary union occurred in 86% of children. At maturity, 69% remained soundly united. The odds ratio for failure of primary union was 3.89 (95% confidence interval, 1.05-14.40; P=0.042) when bone morphogenetic protein was used, and children who had a combination of the Ilizarov technique and intramedullary nailing were at risk for unsound union at maturity (odds ratio, 6.19; 95% confidence interval, 1.24-30.83; P=0.026). No other association reached statistical significance. On recursive partitioning, use of the Ilizarov technique, transfixing the ankle and subtalar joints, use of cortical graft and not operating on the fibula were associated with a better outcome; use of bone morphogenetic protein and combining intramedullary nailing with the Ilizarov technique were associated with poor results. CONCLUSIONS: A larger sample is needed to confirm which factors truly influence the outcome of CPT. This may be feasible if data are collected prospectively through a multicenter registry.

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.

The Effect of Growing Rod Treatment on Hemoglobin and Hematocrit Levels in Early-onset Scoliosis.

BACKGROUND: This study examines preoperative hemoglobin (Hgb) and hematocrit (Hct) levels in a group of early-onset scoliosis (EOS) patients and the effect of distraction-based growing rods (GRs) on these levels. Children with EOS are at risk for respiratory insufficiency and chronic hypoxemia. Increased Hgb and Hct levels have been identified as surrogate markers for chronic hypoxemia. A study of patients who underwent VEPTR surgery showed a significant decrease in Hgb levels following surgery. METHODS: Data were retrospectively collected on 66 EOS patients without confounding respiratory issues or oxygen dependence who were treated with GRs at 5 institutions. Average age at initial surgery was 5.5 years. Patients were followed for a minimum of 2 years (average 3.7 y). Preoperative and postoperative Hgb and Hct levels were converted to Z-scores based on age-adjusted mean blood indices and were compared using a paired t test. RESULTS: The prevalence of elevated Hgb and Hct levels (Z-score >2) preoperatively was 15% (10/66) and 19% (12/64), respectively. The average Hgb Z-score decreased from 0.20 to -0.31 (P=0.005) 6 to 24 months following surgery and the Hct Z-score decreased from 0.31 to -0.28 (P=0.002) 6 to 24 months following surgery. CONCLUSIONS: Following distraction-based GR treatment of children with EOS there was a significant decrease in both their Hgb and Hct. This is a physiological marker of decreased hypoxemia and improved pulmonary function. LEVEL OF EVIDENCE: Level III-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.

A New Classification System to Report Complications in Growing Spine Surgery: A Multicenter Consensus Study.

INTRODUCTION: The use of growth-sparing instrumentation in pediatric spinal deformity is associated with a significant incidence of adverse events. However, there is no consistent way to report these complications, allowing for meaningful comparison of different growth-sparing techniques and strategies. The purpose of this study is to develop consensus for a new classification system to report these complications. METHODS: The authors, who represent lead surgeons from 5 major pediatric spine centers, collaborated to develop a classification system to report complications associated with growing spine surgery. Following IRB approval, this system was then tested using a minimum of 10 patients from each center with at least 2-year follow-up after initial implantation of growing instrumentation to assess ease of use and consistency in reporting complications. Inclusion criteria were only patients who had surgical treatment of early onset scoliosis and did not include casting or bracing.Complications are defined as an unplanned medical event in the course of treatment that may or may not affect final outcome. Severity refers to the level of care and urgency required to treat the complication, and can be classified as device related or disease related. Severity grade (SV) I is a complication that does not require unplanned surgery, and can be corrected at the next scheduled surgery. SVII requires an unplanned surgery, with SVIIA requiring a single trip and SVIIB needing multiple trips for resolution. SVIII is a complication that substantially alters the planned course of treatment. Disease-related complications are classified as grade SVI if no hospitalization is required and grade SVII if hospitalization is required. SVIV was defined as death, either disease or device related. RESULTS: A total of 65 patients from 5 institutions met enrollment criteria for the study; 56 patients had at least 1 complication and 9 had no complications. There were 14 growing rods, 47 VEPTRs, ,and 4 hybrid constructs. The average age at implant was 4.7 years. There were an average of 5.4 expansions, 1.6 revisions, and 0.8 exchanges per patient. The minimum follow-up was 2 years. The most common complications were migration (60), infection (31), pneumonia (21), and instrumentation failure (23). When classified, the complications were grade I (57), grade IIA (79), grade IIB (10), and grade III (6). CONCLUSIONS: Well-documented uncertainty in clinical decision making in this area highlights the need for more rigorous clinical research. Reporting complications standardized for severity and impact on the course of treatment in growing spine surgery is a necessary prerequisite for meaningful comparative evaluation of different treatment options. This study shows that although complications were common, only 9% (SVIII) were severe enough to change the planned course of treatment. SIGNIFICANCE: We propose that future studies reporting complications of different methods of growth-sparing spine surgery use this classification moving forward so that meaningful comparisons can be made between different treatment techniques.

Successful management of idiopathic early-onset scoliosis: effect of curve correction and bracing after Mehta casting.

STUDY DESIGN: Patients with idiopathic EOS treated by Mehta casting followed by bracing or observation from a single institution. OBJECTIVES: To determine casting protocol parameters leading to successful management; to determine efficacy of bracing vs. observation after cast discontinuance. BACKGROUND: Previous studies have not precisely defined parameters for cast discontinuance (amount of correction, number of casts), nor have documented the efficacy of brace treatment. METHODS: 73 patients undergoing Mehta casting were braced (n = 56) or observed (n = 17) after casting with follow-up for a mean of 51-58 months. 57 patients had ≥ 4 casts applied; 39 had ≥ 5 casts. Success was defined as no further treatment required. Curve magnitude was measured at time points pre-casting, at cast discontinuance, and last follow up. RESULTS: There was no difference in success rate between braced patients (79% success) and observed (71%). Curve correction to < 30° at cast discontinuance was crucial parameter for success, as 95% (45/47) of patients with this correction achieved success, braced or not, while only 42% (11/26) with residual curves ≥ 30° achieved success (p < .001) and 14 of these required surgery compared to 0/47 successful patients. The number of casts (over/under 4 or 5) made no difference in achieving success. 10/26 patients who had residual curves ≥ 30° and were braced achieved success due to further curve diminution during bracing. CONCLUSIONS: Cast correction to < 30° followed by bracing achieved 100% success in 34 patients compared to 85% (13 patients) who were observed (p = .07). For residual curves ≥ 30° bracing can produce some correction and succeed in delaying further treatment.

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 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.