Impact of Spinal Surgery on Intrathecal Nusinersen Injections in Pediatric Spinal Muscular Atrophy.

BACKGROUND: Complex spinal deformities necessitate surgical interventions that may intervene with intrathecal injections in patients with spinal muscular atrophy (SMA). This study aimed to determine the effect of spinal deformity correction surgery on nusinersen administration. METHODS: Pediatric patients with SMA, operated by a single surgeon, either via magnetically controlled growing rod (MCGR) or definitive fusion (DF) with skip instrumentation, were evaluated retrospectively in terms of safety and feasibility of intrathecal injections. Patients' and their parents' perspectives were evaluated through a questionnaire regarding any shift in the setting of injections. RESULTS: Fourteen patients with 15 spinal surgeries (10 MCGR and 5 DF) were included. Eleven patients received intrathecal treatment both before and after the surgery. Preoperative (n=3) and postoperative (n=9) fluoroscopic guidance was required leading to a shift in the application settings in 6 patients. Of 106 preoperative injections, 15% required fluoroscopy and 2% required anesthesia. Postoperatively, of 88 injections, 73% required fluoroscopy and 26% required anesthesia. No patients discontinued intrathecal injections due to technical difficulties associated with the spinal surgery. CONCLUSIONS: This study demonstrates that spinal surgery does not prevent safe and successful intrathecal nusinersen injections. In the DF group, the skip instrumentation technique provided access to interlaminal space for intrathecal injections. In either surgical group, no further auxillary approach was required. Modifications in the injection technique require an institutional approach, and concerns of patients and their families should be addressed accordingly. LEVEL OF EVIDENCE: IV.

Parent-patient Discrepancies in the Quality of Life Assessment of Early-onset Scoliosis: A Comparison Between 2 Questionnaires Completed on 2 Different Time Points - A Preliminary Report.

BACKGROUND: EOSQ-24 is a parent proxy questionnaire designed to assess the health-related quality of life (HRQoL) of early-onset scoliosis (EOS) patients during their childhood years. EOSQ-SELF, a novel self-reported questionnaire, assesses HRQoL in older children (>8 y) and adolescents. So far, the same group of EOS patients has not been evaluated with both EOSQ-24 and EOSQ-SELF. The aim of this study was to evaluate how the same pathology was reflected in the parent and patient at different time points by comparing the answers to the common questions between EOSQ-24 and EOSQ-SELF. METHODS: A group of otherwise healthy EOS patients whose parents filled out EOSQ-24 at the early phase of growth-friendly treatment was re-tested by the EOSQ-SELF questionnaire at the end of treatment. Both EOSQ-24 and EOSQ-SELF are validated in Turkish. Inclusion criteria were patients with EOS, independent ambulation, age of 8 years or older at EOSQ-SELF enrollment, literacy in Turkish, no apparent intellectual impairment, and a minimum of 24 months after graduation. The common questions between the 2 surveys with nearly identical phrasings were extracted. Common items from the 2 tests were compared with a Wilcoxon signed rank test. RESULTS: Twenty-one patients (15 females, 6 males) who previously filled out EOSQ-24 met the inclusion criteria. The mean age of the group was 10 (5 to 16) years at EOSQ-24 participation and 18 (13 to 24) at the final analysis. Fourteen questions were found common in 10 domains. The scores were significantly different in 5 questions of 4 domains. EOSQ-SELF had significantly less favorable scores in the pain/discomfort, pulmonary function, and fatigue/energy level domains. Scores in the parental burden/relationships domain were significantly higher (P<0.05). CONCLUSIONS: The self-reported group had a general trend of worse results. Parents and caregivers may not accurately perceive the problems of EOS patients. Our findings indicate a disconnect between caregivers and the patients, as both parties underreported the other side in some domains. These findings suggest the challenges faced by EOS patients are not adequately reflected on proxy questionnaires that assess the HRQoL of children. LEVEL OF EVIDENCE: Diagnostic Level I.

The Fate of Overcorrection After Hemiepiphysiodesis in Valgus Deformities Around the Knee.

BACKGROUND: Tension band plating is widely used in the surgical treatment of coronal plane deformities around the knee. The rebound phenomenon after implant removal is a common complication of this technique. Overcorrection of joint orientation angles is a method to minimize the effect of the rebound phenomenon. This study aims to investigate the natural course of overcorrected joint orientation angles after plate removal in patients with genu valgum deformity. METHODS: Patients who underwent hemiepiphysiodesis with tension band plating due to genu valgum deformity between 2010 and 2019 were retrospectively analyzed. Mechanical lateral distal femoral angles (mLDFA) and mechanical medial proximal tibial angles were calculated before plate application, before implant removal, and at the last follow-up. At the implant removal, mLDFA>90 degrees and mechanical medial proximal tibial angles <85 degrees were accepted as overcorrected. RESULTS: Seventy-two segments from 45 patients were included. For femoral valgus deformities (n=59), the mean mLDFAs at index surgery, implant removal, and the last follow-up were 79.8±3.9 degrees, 95.5±3.7 degrees, and 87.3±5.1 degrees, respectively. In the more and less than 10 degrees rebound groups, the median age of patients at index surgery were 66 and 101 months ( P =0.04), the mLDFA during implant removal were 97.8 degrees and 94.4 degrees ( P =0.005), and the mean amount of correction in mLDFA was 17 degrees and 13 degrees ( P =0.001), respectively. At the last follow-up, joint orientation angles were found to be still overcorrected in 16 (22%), within normal limits in 36 (50%), and undercorrected in 20 (28%) segments. Ten (13%) segments required additional surgery due to residual deformity. CONCLUSIONS: Overcorrection with tension band plating is an effective modality in the treatment of genu valgum deformity. Rebound after plate removal increases as the age at index surgery decreases and the amount of conscious overcorrection increases. Most segments return to normal joint orientation angle limits after overcorrection. We recommend a mean of 5 degrees routine overcorrection in patients with genu valgum deformity to overcome the rebound phenomenon and to make future interventions easier if ever needed. LEVEL OF EVIDENCE: Level III.

The Effect of Apical Vertebra Position on Growing Rod Treatment: A Clinical and Finite Element Study.

BACKGROUND: Growing rods (GRs) is a commonly utilized technique for the management of early-onset scoliosis. The significance of the position of the apical vertebrae relative to the rods is not known. The purpose of this study is to analyze the potential effects of the position of the apical vertebrae in relation to the GRs on deformity control through plain radiographs and finite element analysis (FEA) modeling. METHODS: We identified 140 patients treated with GR between 2000 and 2018. Patients who had a congenital vertebral anomaly or <2-year follow-up were excluded. Curve magnitude, traction radiograph under general anesthesia (TRUGA) flexibility, apical rotation, the lengths of T1-12, T1-S1, and the instrumented segments were recorded. Patients were divided into 3 groups according to the apical position on the postoperative radiographs: group 1 (both pedicles are between the rods), group 2 (convex rod is between the apical vertebra pedicles), group 3 (both pedicles are lateral to the convex rod). FEA models were created simulating the 3 groups. Both radiographic and FEA data were analyzed to compare the deformity control and growth in each group. RESULTS: Fifty-eight patients were included in the final analyses (mean age 84 mo; range: 38 to 148). Ten patients (17%) were in group 1, 34 (59%) in group 2, and 14 (24%) in group 3. Difference between TRUGA flexibilities was statistically insignificant. Group 3 was the least successful in terms of both height gain and rotational control. FEA showed a decrease in rotation and displacement for every group, however, the residual rotation and displacement was highest in group 3. CONCLUSIONS: Bringing the apex in line with the GR increases the capacity of growth preservation as it results in largest height gain and better deformity control. FEA model demonstrated that distraction alone is inadequate for controlling rotation, and with increasing apical translation, residual rotation after distraction also increases. LEVEL OF EVIDENCE: Level III.

Posterior Instrumentation and Fusion in Multiple-segment Adolescent Congenital Scoliosis: When Are Posterior Column and Concave Rib Osteotomies Needed?

BACKGROUND: No clear guidelines exist for appropriate surgical treatment of congenital scoliosis. The spectrum varies from isolated posterior instrumentation and fusion (PIF-only) to vertebral column resections. Multiple posterior column osteotomies (PCOs) with and without concave rib osteotomies have previously been suggested as an alternative to invasive 3-column osteotomies. The aim of the study is to analyze outcomes of spinal surgery in congenital scoliosis patients for a new treatment algorithm based on a traction radiograph under general anesthesia (TruGA). METHODS: Surgical intervention was determined according to flexibility with TruGA: patients with >40% correction underwent PIF-only; patients with <40% of correction underwent PCO. Patients, who had <30% of correction in TruGA and/or more than 5 vertebral levels with failure of segmentation, underwent additional concave rib osteotomies. The radiologic and clinical results were compared. RESULTS: Forty-three patients met inclusion criteria (14M). The mean age was 13.8 (10 to 17) years, average follow-up 67 (24 to 107) months. Fourteen patients were in the PIF-only group, 29 in the PCO group. PCO patients were significantly older (14.5 vs. 12.3, P=0.001). Mean operative time and blood loss of PCO group were significantly greater than those of PIF-only group. Nine patients required concave rib osteotomies. While the curve magnitude of patients in the PIF-only and PCO groups did not differ significantly (54.6 vs. 63 degrees, P=0.067), curve rigidity was significantly higher in the patients of PCO group (51.2% vs. 32%, P=0.001). Despite this, postoperative correction rates for 2 groups were similar (51.1% vs. 47.8%, P=0.545). CONCLUSIONS: The number of anomalous segments and the curve flexibility on TruGA play important roles in the decision-making process in congenital scoliosis and <40% flexibility of the major curve could be an important indicator of the need for PCO. Curves with more than 5 anomalous vertebral segments might be more likely to need additional concave rib osteotomies for adequate correction. LEVEL OF EVIDENCE: Level III.

Clinical Outcomes of 3-column Osteotomy at Growing Rod Graduation.

BACKGROUND: Several different strategies were described on how to graduate a patient when skeletal maturity is reached after growing rod treatment. A final instrumentation and fusion is commonly performed, but the stiffness of the spine after numerous lenghthenings can be a major problem. When correction is needed in a severe and rigid curve, 3-column osteotomies (3CO) can be used as a method of bringing the spine back to a well-balanced position. This study aims to evaluate the clinical outcomes of 3CO osteotomies at growing rod graduation. METHODS: A retrospective search of the database was performed between 1996 and 2018. Inclusion criteria were: diagnosis of early-onset scoliosis, underwent 3CO at the final fusion procedure, and ≥1 year of follow-up. Indications for this osteotomy were: significant sagittal and/or coronal off-balance or severe crankshaft deformity or significant distal and/or proximal adding-on. Clinical, radiographic, and operative data were analyzed. Complications were classified according to the modified Clavien-Dindo-Sink system. RESULTS: Eight patients were included in the study with a mean age of 13.5 at graduation (male/female: 2/6). The mean scoliosis angle significantly reduced from 65.6 to 39 degrees (P=0.012). Mean T1-T12 height increased from 19 to 20.5 (P=0.084) and T1-S1 from 29.6 to 31.6 (P=0.144). Five patients had a staged surgery. Average operative time was 338 minutes and blood loss was 1321 mL. Four grade 1, 1 grade 2, and 1 grade 3 complications occurred. CONCLUSIONS: This is the first case-series in the literature reporting on the use of 3CO at growing rod graduation. These procedures can be safely and effectively used to overcome significant multiplanar deformity and/or off-balance in the graduation of patients with early-onset scoliosis and significant correction can be achieved on these severe and rigid curves. LEVEL OF EVIDENCE: Level IV-case series.

Correction of coronal plane deformities around knee in children with two-hole tension band plates.

OBJECTIVES: This study aims to present the 10-year results and complications of two-hole tension band plate hemiepiphysiodesis for coronal deformities around knee in a large population from a single center. PATIENTS AND METHODS: Seventy-seven patients (46 boys, 31 girls; mean age 93±36 months; range, 22 to 181 months) who underwent temporary hemiepiphysiodesis around knee between January 2009 and January 2019 with two-hole tension band plates were retrospectively evaluated. Improvement of joint orientation angles and mechanical axis deviations, deformity correction rates, etiology groups, and complications were noted. RESULTS: A total of 166 bone segments (93 femurs, 73 tibias) were included in the study. Mean follow-up duration after the implantation was 36±17 (range, 12 to 88) months. Plates were removed at mean 18±8 (range, 7 to 47) months of implantation. Of the bone segments, 95.2% (n=158) responded successfully to the plates. Mean correction rate of mechanical lateral distal femoral angle in femoral valgum deformity was 0.94±0.43° (range, 0.17 to 2.22)/month and mean correction rate of mechanical medial proximal tibial angle in tibial valgum deformity was 0.62±0.36° (range, 0.11 to 1.55)/month. Mean correction rate of mechanical lateral distal femoral angle in femoral varum deformity was 1.3±0.8° (range, 0.48 to 2.92)/month and mean correction rate of mechanical medial proximal tibial angle in tibial varum deformity was 0.94±0.49° (range, 0.26 to 1.67)/month. The most common complication observed was rebound deformity seen in 41.2% (n=70) of the bone segments. Nine bone segments had persistent hemiepiphysiodesis despite plate removal. Four screw breakages (three metaphysial and one epiphysial) were observed. CONCLUSION: Two-hole tension band plate hemiepiphysiodesis appears to be an effective and safe method for the correction of coronal deformities around knee.

Preliminary results of rigid fixation (locking plate/screw) after triple pelvic osteotomy without a hip spica cast: A modified fixation method in 21 patients.

OBJECTIVES: The aim of this study is to investigate whether rigid fixation after triple pelvic osteotomy (TPO) utilizing a 3.5-mm locking plate and screws without hip spica cast can provide enough stability and prevent correction loss in pediatric patients with developmental dysplasia of the hip (DDH) and Legg-Calvé-Perthes disease (LCPD). PATIENTS AND METHODS: A total of 21 hips of 21 pediatric patients (9 males, 12 females; mean age: 9.3±2.0 years; range, 6 to 14 years) who underwent rigid fixation with locking plate/screws after TPO for DDH and LCPD between June 2015 and October 2018 were retrospectively analyzed. Preoperative, immediate postoperative, and six-month follow-up anteroposterior radiographs were compared for the Wiberg's center-edge angle (CE), Sharp angle, acetabular coverage of the femoral head (ACFH), and center-head distance discrepancy (CHDD). The patient demographics, surgery time, perioperative complications were evaluated. RESULTS: Underlying diagnosis were DDH in 14 patients and LCPD in seven patients. In patients with DDH, postoperative evaluation showed significant increase in the mean CE angle (5.6±16.1° vs. 30.5±9.3°, respectively) and ACFH (46.4±16.8% vs. 84.5±12.1%, respectively), and a significant decrease in the mean Sharp angle (55.3±6.2° vs. 35.6±7.8°, respectively) and CHDD (14.6±10.7% vs. 6.2±5.6%, respectively). The final follow-up revealed that there was no correction loss in these parameters. In the patients with LCPD, postoperative evaluation showed a significant increase in the mean CE (20.1±11.1° vs. 38.3±9.6°, respectively) and ACFH (62.9±18% vs. 91.4±10.1%, respectively), and a significant decrease in the mean Sharp angle (46±3.6° vs. 25.2±5.5°, respectively). The final follow-up revealed that there was no correction loss in radiological parameters. No perioperative complications were noted. CONCLUSION: Our study results suggest that rigid fixation construct with a 3.5-mm locking plate and screws without hip spica cast can provide adequate stability to allow early mobilization following TPO in children without any loss of correction, until bony healing at the osteotomy sites.

Rebound phenomenon after hemiepiphysiodesis: Determination of risk factors after tension band plate removal in coronal plane deformities of lower extremities.

Risk factors for rebound phenomenon (ReP) have not been investigated in detail. Aim of this study is to investigate risk factors of ReP in lower extremity deformities after two-hole plate removal. Patients who underwent hemiepiphysiodes were retrospectively reviewed. Joint orientation angles of tibia and femur, deformity type, age of patient at initial surgery and plate removal, deformity correction rate, bone growth rate were noted. An increase of 5° or more in joint orientation angles in the direction of the initial deformity was considered ReP. A relationship between the patient parameters and ReP was evaluated. Ninety-four segments (55 femurs and 39 tibias) of 43 patients (15 males and 28 females) were included. ReP was observed in 56.2% of all valgus deformities (n = 73), and in 23.8% of varus deformities (n = 21). The ReP rate was 60% (n = 27) and 20% (n = 2) in femoral segments with preoperative valgus and varus deformities, respectively (P = 0.03). When gender, etiology, sides and age of implant application and removal were compared, there was no difference between ReP(+) and ReP(-) groups in femur and tibia. The correction rate of mechanical lateral distal femoral angle in ReP(-) and ReP(+) femurs was 0.7 ± 0.4°/month and 1.1 ± 0.3°/month, respectively (P = 0.01). Age at implant application and removal in the femoral valgus group was significantly lower in the ReP(+) group (P = 0.008 and P = 0.005, respectively). The ReP rate was higher in femoral segments with overcorrection (P = 0.008). The ReP rate was higher in femoral valgus deformities. Younger age at plate application and removal, higher correction rate in deformity and intentional overcorrection increased risk for ReP.