Fusion In Children Research Articles

The Effect of Two Attending Surgeons on the Outcomes of Posterior Spine Fusion in Children With Cerebral Palsy

BackgroundPosterior spinal fusion (PSF) in children with cerebral palsy (CP) carries a high risk of complications and morbidity. The purpose of this study is to investigate the impact of using two attending surgeons on blood loss, operative time, and complications in this fragile population. MethodsThis was a prospective, matched cohort analysis of patients with CP who underwent PSF with two attending surgeons. These were matched with a control group that had a single-surgeon team, assisted by a senior resident or PA. The groups were compared using paired Student t tests and chi-square tests. Results50 patients were included in the study (25 study and 25 matched controls), determined by our power analysis. There was no statistical difference in the mean age, preoperative major curve angle, major curve angle correction, or use of antifibrinolytics. The two-surgeon group decreased surgical time from 5.25 to 3.3 hours (p = .000002), and estimated blood loss from 1,238 to 865 mL (p = .009). The complication rate decreased from 33% to 8% (p=.034). Length of stay was also decreased from 6.5 days to 5.35 (p = .02). ConclusionsAlthough confounding variables were present, this study demonstrates that the use of a two-surgeon team during spinal surgery for patients with cerebral palsy could have a role in reducing operative time, blood loss, complication rates, and hospital length of stay. Overall, these factors and any improved operating room efficiencies may lead to lasting improved patient outcomes. Level of EvidenceLevel III, retrospective, comparative study.

Risk factors for increased blood loss during spinal fusion for children with neuromuscular scoliosis

Risk factors for increased blood loss during spinal fusion for children with neuromuscular scoliosis

New method of sensory fusion rehabilitation using intermittent occlusion with LCD glasses

Recovery of sensory fusion in children with congenital and early acquired strabismus is very important in the functional rehabilitation following strabismus surgery. This study aims to analyze sensory fusion recovery using method of intermittent occlusion with LCD glasses and compare its efficacy with orthoptic treatment using synoptophore.

Does Spinal Fusion in Children with Cerebral Palsy and Neuromuscular Scoliosis Result in Weight Gain and Improvement of Nutritional Status?

Does Spinal Fusion in Children with Cerebral Palsy and Neuromuscular Scoliosis Result in Weight Gain and Improvement of Nutritional Status?

Risk factors for pancreatitis after posterior spinal fusion in children with cerebral palsy.

This study reports on the prevalence and risk factors of acute pancreatitis after posterior spinal fusion for cerebral palsy scoliosis. Pancreatitis diagnosis was based on elevated amylase or lipase above three times the upper normal limit. Perioperative data were compared between patients with and without pancreatitis. We included 300 patients; 55% developed acute pancreatitis. Gastrostomy dependence was more common in the pancreatitis group (P=0.048). Perioperative data were similar between groups. Patients with pancreatitis had longer duration of hospitalization (19 vs. 13 days, P<0.001). Acute pancreatitis is common after cerebral palsy scoliosis surgery. Gastrostomy dependence increases its risk. Although no mortality was reported, hospital stay was longer.

Splenogonadal fusion in children with cryptorchism

Splenogonadal fusion in children with cryptorchism

C1-C2 Pedicle Screw Fixation for Pediatric Atlantoaxial Dislocation: Initial Results and Long-term Follow-up.

There are few studies reporting the use of atlantoaxial pedicle screws and the long-term effects of C1-C2 posterior fusion in children. Our study is to investigate the initial results of C1-C2 pedicle screw fixation for pediatric atlantoaxial dislocation (AAD) and assessed spontaneous change of postoperative radiography after a long-term follow-up period. Posterior pedicle screw fixations were performed in 21 pediatric patients with AAD. All the patients underwent implant removal 1 year after their initial surgery and had regular follow-up with an average duration of 76.4 months (range, 52 to 117 mo). Clinical and radiographic data were then collected and compared. Frankel Grade was significantly improved at 3 months follow-up compared with pretreatment values. All patients had good bony fusion at a mean of 4.2±0.9 months (range, 3 to 6 mo) after treatment. None of the patients experienced worsening neurological symptoms or injury to the vertebral artery. However, 2 cases experienced minor complications. Following removal of the implants, no spinal deformities or subaxial instabilities were found. The mean angle of sagittal curvature increased from 12.1±2.4 degrees (range, 0 to 22 degrees) immediately postoperatively to 19.1±2.7 degrees (range, 6 to 31 degrees) at the final follow-up (P>0.05). The results demonstrated that C1-C2 pedicle screw fixation could achieve satisfactory initial results for the management of the pediatric AAD. Moreover, removal of the metal implant after bony fusion did not increase the risk of spinal deformity or subaxial instability at long-term follow-up.

Instrumented cervical spinal fusions in children: indications and outcomes.

PurposeTo report indications, outcomes and complications of instrumented cervical spinal fusion in a consecutive series of children at major university hospitals.MethodsA retrospective, single surgeon series identified 35 consecutive children with a mean follow-up (FU) of 2.5 years undergoing instrumented cervical spinal fusion between 2005 and 2015.ResultsThe main indications were skeletal dysplasia and trauma associated cervical instability. Surgical complications were observed in 12 (34%) patients with multiple complications in four (11%). Four (11%) children required at least one revision surgery, three for nonunion and one for graft dislodgement. All were fused at FU. Surgical complications were more common in children undergoing occipitocervical (OC) fusion than in those avoiding fusion of the OC junction (60% versus 24%) (p = 0.043). Complications were found significantly more in children operated on under the age of ten years than above (50% versus 18%) (p = 0.004). The risk of complications was not dependent on the indications for surgery (skeletal dysplasia versus trauma) (p = 0.177),ConclusionSkeletal dysplasia associated cervical instability and cervical spine injuries represented the most common indications for instrumented cervical spinal fusion in children. Complications were observed in one-third of these children and 11% required revision surgery for complications. OC spinal fusion and spinal fusion before the age of ten years are associated with higher risk of surgical complications and increased mortality than non-OC fusions and cervical spinal fusions at an older age. We urge surgeons to employ caution to the patient, timing and procedure selection when treating paediatric cervical spine.

Upper Cervical Fusion in Children With Spondyloepiphyseal Dysplasia Congenita.

Paraplegia or death secondary to upper cervical spine instability and spinal cord compression are known consequences of spondyloepiphyseal dysplasia congenita (SEDC). Stabilization and occasionally decompression of the upper cervical spine are indicated to treat upper cervical instability and stenosis. The purpose of this study was to report the results of upper cervical spine fusion in children with SEDC who had upper cervical instability. Twenty children (17 females and 3 males) with SEDC who underwent upper cervical spine fusion at a mean age of 72 months were retrospectively analyzed. Three of these children were under the age of 2. Fifteen children had posterior instrumentation and fusion whereas 5 children had posterior in situ fusion without use of any implant. Thirteen of 20 children had iliac crest autograft. Radiographic and clinical results were reported. The average follow-up period was 8 years and 8 months. All children with instrumentation achieved fusion. Three of 5 children who had no instrumentation had nonunion (1 child had a stable nonunion and did not need revision; 1 had a single noninstrumented revision and ended up with a stable nonunion without further intervention; and the third one had a noninstrumented revision and had to have a second, instrumented, revision to achieve fusion). Six children had thoracolumbar scoliosis or kyphoscoliosis which required surgical management.No postoperative neurological deficits were observed. Two of the 3 children with a preoperative neurological deficit showed full recovery and the third one remained unchanged. Pseudarthrosis is the main complication for the noninstrumented group. Distal junctional instability after successful fusion is seen at long-term follow-up (average=6 y) for 13% of patients in instrumented group. Instrumentation and iliac bone grafting results in 100% upper cervical fusion for SEDC children who demonstrated instability before surgery. Level IV-therapeutic.

Irreversible growth plate fusions in children with medulloblastoma treated with a targeted hedgehog pathway inhibitor.

The permanent defects in bone growth observed in preclinical studies of hedgehog (Hh) pathway inhibitors were not substantiated in early phase clinical studies of vismodegib in children. Consequently, vismodegib advanced into pediatric trials for malignancies suspected of being driven by aberrant activation of the Hh pathway. In one multicenter phase II trial, vismodegib was added to the therapy regimen for newly diagnosed Hh pathway activated medulloblastoma. Herein, we report on 3 children (2 on trial and one off trial) treated with vismodegib who developed widespread growth plate fusions that persist long after cessation of therapy. Currently, all 3 patients exhibit profound short stature and disproportionate growth, and 2 subsequently developed precocious puberty. Notably, the growth plate fusions only developed after a prolonged exposure to the drug (> 140 days). These findings resulted in a major trial amendment to restrict the agent to skeletally mature patients as well as a product label warning and update. Moreover, these findings alter the risk-benefit ratio of Hh inhibitors and underscore the importance of careful study of targeted agents in children.

A Modified Technique for Occipitocervical Fusion Using Compressed Iliac Crest Allograft Results in a High Rate of Fusion in the Pediatric Population.

In children, high rates of occipitocervical (OC) fusion have been demonstrated with the use of rigid instrumentation in combination with harvested autograft, with or without bone morphogenetic protein (BMP). Historically, the use of allograft materials demonstrated inferior OC fusion outcomes compared with autograft. However, autograft harvest harbors an increased risk of patient morbidity, and the use of BMP is costly and controversial in children. Thus, there remains a need for safer, less costly, yet efficacious techniques for OC fusion in the pediatric population. We retrospectively reviewed the charts of patients younger than 21 years of age who underwent OC fusion with structural allograft placement at our institution from 2010 to 2015. Data collected included age, sex, follow-up duration, fusion outcomes, and postoperative complications. A total of 19 patients (8 female and 11 male) underwent OC fusion with our surgical technique. Mean age was 8.5 ± 4.3 years. Radiographic follow up data were available for 18 of 19 patients. One patient was lost to clinical follow up but had radiographic confirmation of fusion. Thus, 18 of 18 (100%) of patients with radiographic follow-up achieved successful arthrodesis as determined by computed tomography. Median duration to documented fusion was 4.5 months. Clinical follow-up was available for 17 of 19 patients and was on average 18.8 ± 13.5 months. One patient required reoperation for graft fracture 8 months after radiographic confirmation of successful fusion. There were no vertebral artery injuries or other postoperative complications. We demonstrate a modified technique for OC fusion in children with unique structural allograft shaping and affixation, leading to excellent fusion outcomes at follow up. This technique obviates the need for autograft harvest or BMP, and may decrease postoperative morbidity.

Health-Related Quality of Life and Care Giver Burden Following Spinal Fusion in Children With Cerebral Palsy.

A prospective longitudinal cohort. The objective of this study was to evaluate changes in caregivers' perceptions of health-related quality of life (HRQOL) and caregiver burden in children with severe cerebral palsy (CP) following spinal fusion. Progressive scoliosis is common in nonambulatory children with CP; the utility of spine fusion has been long debated and prospective evaluations of patient reported outcomes are limited. Children 3 to 21 years old, gross motor classification system (GMFCS) IV-V CP, scheduled for spine fusion were enrolled consecutively from September 2011 to March 2014. Caregivers completed the CPCHILD and ACEND pre-operatively and at 6 weeks, 3, 6, 12, and 24 months postoperatively. Changes in CPCHILD and ACEND scores from preoperative to 1 and 2 years after surgery were assessed using paired t tests. Correlations between preoperative Cobb angle and CPCHILD and ACEND scores were evaluated using Pearson's correlation analysis. Twenty-six GMFCS IV-V CP patients with severe scoliosis treated with spine fusion were included. Mean age was 14 years, 50% male, and 46% had instrumentation to the pelvis. Average preoperative Cobb angle was 68.9° (SD 25.68) with an average improvement of 76%. The CPCHILD score increased by 9.8 points above baseline [95% confidence interval (95% CI): 3.4-16.2] at 1 year postoperatively (P = 0.005). However, at 2 years, the CPCHILD score regressed to baseline (P = 0.40). ACEND scores did not change from baseline scores at 1-year (P = 0.09) and 2-year (P = 0.72) follow-up, reflecting that caregiver burden is little changed by spine fusion. There was no correlation between preoperative Cobb angle and CPCHILD score (P = 0.52) or ACEND score (P = 0.56) at 1-year or 2-year follow-up (P = 0.69, P = 0.90). Children with Cobb angle ≤75° experienced more improvement 1 year after surgery than children with Cobb angle >75°. HRQOL improves 1 year following spine fusion but regresses to baseline after 2 years. Caregiver burden was unchanged following spine fusion. 2.

Proximal Junctional Kyphosis Following Posterior Hemivertebra Resection and Short Fusion in Children Younger Than 10 Years.

A retrospective study. To investigate whether proximal junctional kyphosis (PJK) or obvious proximal junctional angle (PJA) changes in the sagittal plane develops following short fusion in children younger than 10 years of age with congenital scoliosis, and to investigate the possible risk factors. PJK following long spinal fusion in adolescents and adults is a serious postoperative complication. Although the same problem may occur in patients with early-onset scoliosis who have undergone spine fusion, few studies have been reported any relationship between PJK and spinal fusion in young children with congenital scoliosis. Thirty-seven children treated in a single institution between 1998 and 2010 were reviewed retrospectively. The inclusion criteria included (1) younger than 10 years of age at the time of operation; (2) simple congenital deformity; (3) hemivertebra treated by posterior hemivertebrectomy with short fusion at a maximum of 5 motion segments; and (4) minimum follow-up for 2 years. The PJA from the caudal endplate of the upper instrumented vertebra (UIV) to the cephalad endplate of the vertebra adjacent to the UIV, thoracic kyphosis (T5-T12), lumbar lordosis (T12-S1), global sagittal balance, and magnitude of scoliosis of the major curves and upper compensated curves were measured on lateral radiographs. PJK was defined by a PJA>10 degrees during the follow-up and at least 10 degrees greater than the preoperative or early postoperative measurement. Wilcoxon tests were performed for statistical analysis. PJK occurred in 7 of 37 patients (18.9%), during an average of 4.5±3.2 years of follow-up (2-12 y). The UIV level of children with PJK was on T9 in 4 patients, and T11, T12, and L1 in 1. Screw malposition at UIV was confirmed by postoperative computed tomography images in 6 patients. Only 1 patient with a screw deviation did not develop PJK during the follow-up period. None of the patients with PJK was symptomatic, and no patients required revision surgery because of PJK. PJK occurred and progressed during the first 6 months after surgery followed by almost no progression or slight improvement in patients that could be followed up beyond 6 months postoperatively; in association with an increase of the lumbar lordosis. PJK occurred in pediatric patients with simple congenital deformities following hemivertebrectomy and short fusion. PJK was more common in patients with (1) greater immediately postoperative segmental kyphosis and PJA; (2) screw malposition on the UIV; and (3) hemivertebra located on the lower thoracic or the thoracolumbar region.

Factors associated with broad binaural pitch fusion in children and adults with hearing aids and cochlear implants

Many hearing-impaired adults who use hearing aids (HAs) and/or cochlear implants (CIs) have broad binaural pitch fusion, such that sounds with large pitch differences are fused across ears (Reiss et al., JARO 2014; JASA). The goal was to determine which subject factors are associated with broad binaural pitch fusion. Binaural pitch fusion was measured in bilateral HA users, bimodal CI users who use a HA in the contralateral ear, and bilateral CI users. Fusion ranges were measured by simultaneous, dichotic presentation of reference and comparison stimuli in opposite ears, and varying the comparison stimulus to find the range that fused with the reference stimulus. Both children (ages 6-9) and adults were tested. Children in the HA and bilateral CI groups had broader fusion than the bimodal CI group. In addition, broad fusion was positively correlated with long durations of HA use and early onset of hearing loss in adult HA users, and negatively correlated with duration of CI use in pediatric bimodal CI users. No correlations with subject factors were seen in bilateral CI users. The findings suggest that type of hearing device experience may influence binaural pitch fusion in hearing-impaired individuals, especially children. [Work supported by NIH-NIDCD grant R01 DC013307.]

Hallux valgus deformity correction without fusion in children with cerebral palsy.

Level IV Therapeutic Studies.

Tyrosine kinase fusion genes in pediatric BCR-ABL1-like acute lymphoblastic leukemia.

Approximately 15% of pediatric B cell precursor acute lymphoblastic leukemia (BCP-ALL) is characterized by gene expression similar to that of BCR-ABL1-positive disease and unfavorable prognosis. This BCR-ABL1-like subtype shows a high frequency of B-cell development gene aberrations and tyrosine kinase-activating lesions. To evaluate the clinical significance of tyrosine kinase gene fusions in children with BCP-ALL, we studied the frequency of recently identified tyrosine kinase fusions, associated genetic features, and prognosis in a representative Dutch/German cohort. We identified 14 tyrosine kinase fusions among 77 BCR-ABL1-like cases (18%) and none among 76 non-BCR-ABL1-like B-other cases. Novel exon fusions were identified for RCSD1-ABL2 and TERF2-JAK2. JAK2 mutation was mutually exclusive with tyrosine kinase fusions and only occurred in cases with high CRLF2 expression. The non/late response rate and levels of minimal residual disease in the fusion-positive BCR-ABL1-like group were higher than in the non-BCR-ABL1-like B-others (p<0.01), and also higher, albeit not statistically significant, compared with the fusion-negative BCR-ABL1-like group. The 8-year cumulative incidence of relapse in the fusion-positive BCR-ABL1-like group (35%) was comparable with that in the fusion-negative BCR-ABL1-like group (35%), and worse than in the non-BCR-ABL1-like B-other group (17%, p=0.07). IKZF1 deletions, predominantly other than the dominant-negative isoform and full deletion, co-occurred with tyrosine kinase fusions. This study shows that tyrosine kinase fusion-positive cases are a high-risk subtype of BCP-ALL, which warrants further studies with specific kinase inhibitors to improve outcome.

Outcomes of Spinal Fusion for Cervical Kyphosis in Children with Neurofibromatosis.

Cervical kyphosis may occur with neurofibromatosis type I (NF1) and is often associated with vertebral dysplasia. Outcomes of cervical spinal fusion in patients with NF1 are not well described because of the rarity of the condition. We aimed to (1) characterize the clinical presentation of cervical kyphosis and (2) report the outcomes of posterior and anteroposterior cervical fusion for the condition in these children. The medical records and imaging studies of 22 children with NF1 who had undergone spinal fusion for cervical kyphosis (mean, 67°) at a mean age of 11 years and who had been followed for a minimum of 2 years were reviewed. Thirteen children presented with neck pain; 10, with head tilt; 9, with a previous cervical laminectomy or fusion; and 5, with a neurologic deficit. Two patients had spontaneous dislocation of the mid-cervical spine without a neurologic deficit. Eleven had scoliosis, with the major curve measuring a mean of 61°. Nine patients underwent posterior and 13 underwent anteroposterior surgery. Twenty-one received spinal instrumentation, and 1 was not treated with instrumentation. Preoperative halo traction was used for 9 patients, and it reduced the mean preoperative kyphosis by 34% (p = 0.0059). At the time of final follow-up, all spinal fusion sites had healed and the cervical kyphosis averaged 21° (mean correction, 69%; p < 0.001). The cervical kyphosis correction was significantly better after the anteroposterior procedures (83%) than after the posterior-only procedures (58%) (p = 0.031). Vertebral dysplasia and erosion continued in all 17 patients who had presented with dysplasia preoperatively. Thirteen patients had complications, including 5 new neurologic deficits and 8 cases of junctional kyphosis. Nine patients required revision surgery. Junctional kyphosis was more common in children in whom ≤5 levels had been fused (p = 0.054). Anteroposterior surgery provided better correction of cervical kyphosis than posterior spinal fusion in children with NF1. Erosion of vertebral bodies continued during the postoperative follow-up period in all patients who had presented with dysplastic changes preoperatively. The cervical spine should be screened in all children with NF1. Fusion should include at least 6 levels to prevent junctional kyphosis. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Patient-reported outcomes of occipitocervical and atlantoaxial fusions in children

OBJECTIVE There is limited literature on patient-reported outcomes (PROs) and health-related quality of life (HRQOL) outcomes in pediatric patients undergoing surgery for craniovertebral junction pathology. The aim of the present study was to assess surgical and quality of life outcomes in children who had undergone occipitocervical or atlantoaxial fusion. METHODS The authors retrospectively reviewed the demographics, procedural data, and outcomes of 77 consecutive pediatric patients who underwent posterior occipitocervical or atlantoaxial fusion between 2008 and 2015 at Texas Children's Hospital. Outcome measures (collected at last follow-up) included mortality, neurological improvement, complications, Scoliosis Research Society Outcomes Measure-22 (SRS-22) score, SF-36 score, Neck Disability Index (NDI), and Pediatric Quality of Life Inventory (PedsQL). Multivariate linear regression analysis was performed to identify factors affecting PROs and HRQOL scores at follow-up. RESULTS The average age in this series was 10.6 ± 4.5 years. The median follow-up was 13.9 months (range 0.5-121.5 months). Sixty-three patients (81.8%) were treated with occipitocervical fusion, and 14 patients (18.1%) were treated with atlantoaxial fusion. The American Spinal Injury Association (ASIA) grade at discharge was unchanged in 73 patients (94.8%). The average PRO metrics at the time of last follow-up were as follows: SRS-22 score, 4.2 ± 0.8; NDI, 3.0 ± 2.6; the parent's PedsQL (ParentPedsQL) score, 69.6 ± 22.7, and child's PedsQL score, 75.5 ± 18.7. Multivariate linear regression analysis revealed that older age at surgery was significantly associated with lower SRS-22 scores at follow-up (B = -0.06, p = 0.03), and the presence of comorbidities was associated with poorer ParentPedsQL scores at follow-up (B = -19.68, p = 0.03). CONCLUSIONS This study indicates that occipitocervical and atlantoaxial fusions in children preserve neurological function and are associated with acceptable PROs and ParentPedsQL scores, considering the serious nature and potential for morbidity in this patient population. However, longer follow-up and disease-specific scales are necessary to fully elucidate the impact of occipitocervical and atlantoaxial fusions on children.

Paper #11 Hemivertebra Resection and Transpedicular Short Fusion in Children Younger than 5 Years. A Mid-Term Follow-Up Analysis

Restrospective analysis of early hemivertebra resection and short fusion for children under the age of 5, with a 7.6-year follow-up. Early surgery allowed for a good coronal correction initially which was difficult to maintain at final follow-up, especially in the lumbosacral junction. Sagittal plane correction was excellent in thoracolumbar deformities and was maintained over time. Early surgery prevented the development of severe deformities and secondary structural curves, however results were more challenging in the lumbosacral group compared with thoracolumbar deformities.

Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis

Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis