Efficacy of corticosteroid in decreasing scoliosis and extending time to loss of ambulation in a single clinic: an effectiveness trial.

PURPOSE: Pharmacologic doses of corticosteroid (CS) have been shown to ameliorate the progression of Duchenne muscular dystrophy (DMD) preserving strength, pulmonary function and ambulation as well as reducing the incidence of scoliosis. However, there are serious side effects of CS, which may impact dose tolerance. The purpose of this study was to compare the magnitude of positive CS effects on patients in our clinic to those reported in the literature. METHODS: We retrospectively reviewed medical records and radiographs of 142 DMD patients who were seen between 1st January 1991 and 31st December 2017. RESULTS: In total, 101 boys met study inclusion criteria. Of these 32 were steroid naïve, 37 took the recommended dose (standard of care, SOC) of Prednisone or Deflazacort, and 32 took a lower dose (LD). Following initiation of CS, both treatment groups showed an increase in weight velocity and decrease in linear growth velocity. Although there was a trend to later loss of ambulation (LOA) in the SOC group relative to the naïve group by one year, this was not significant, however, a small subgroup of boys on Deflazacort showed a 3.4 year later LOA than the naïve group. The incidence of scoliosis was reduced from 69% in the naïve, to 41% in the LD and 47% in the SOC group. CONCLUSIONS: Although there was a reduction in the incidence of scoliosis, it was not as robust as seen elsewhere. Many published studies have inadequate data on scoliosis probably due to the lack of inclusion of orthopaedists in the study group. LEVEL OF EVIDENCE: IV.

Use of Magnetic Spinal Growth Rods (MCGR) With and Without Preoperative Halo-gravity Traction (HGT) for the Treatment of Severe Early-onset Scoliosis (EOS).

BACKGROUND: Correction of severe scoliosis through distraction-based techniques poses a challenge. Magnetically controlled growing rod (MCGR) hardware complications are common with a 27.8% to 46.7% revision rate in under 2 years. Loss of correction and diminishing returns are the norm. Treatment of severe scoliosis with halo-gravity traction (HGT) before MCGR has not been previously reported. The purpose of this study was to assess initial correction, maintenance of correction, and complication rate in patients with severe scoliosis treated with and without HGT before MCGR. METHODS: IRB-approved retrospective single site cohort study of a prospectively collected database. Forty-two patients underwent MCGR between 2014 and 2017 at a single site, 12 with prior growing constructs were excluded, 30 patients were included, 12 patients underwent preoperative HGT. Charts were reviewed for demographic, clinical, and radiographic information. RESULTS: The HGT group had larger major curves averaging 90 (69 to 114) degrees versus 77 (56 to 113) degrees in the non-HGT group P=0.018. Percent correction on preoperative flexibility films were 17% versus 40% for those in the HGT versus non-HGT group, P=0.000. An additional 22% correction of the curve magnitude occurred between the flexibility and in-traction films representing 43% of the total correction achieved, P=0.000, was achieved. EBL, and postoperative major curve and major curve correction were not significant. Thirteen percent of patients experienced complications. Average follow-up was 712 versus 561 days in the HGT versus non-HGT groups. CONCLUSIONS: Large, rigid curves can achieve equivalent correction to flexible curves with HGT. Forty-three percent of the total correction achieved occurred during traction. Thirty percent of the total correction occurred at implantation of the MCGR in the HGT group versus 28% in the non-HGT group. At most recent follow-up HGT patients had statistically maintained their major curve correction better than non-HGT patients. LEVEL OF EVIDENCE: Level III-therapeutic study.

What is the Risk of Developing Proximal Junctional Kyphosis During Growth Friendly Treatments for Early-onset Scoliosis?

BACKGROUND: Rib-based and spine-based systems are commonly used distraction-based growth friendly treatments for early-onset scoliosis (EOS). Our primary purpose was to determine the risk of developing postoperative proximal junctional kyphosis (PJK) during distraction-based growth friendly surgery. METHODS: A multicenter, retrospective, radiographic comparison was performed for a group of 40 children with EOS who were treated with posterior distraction-based implants. PJK was defined as proximal junction sagittal angle (PJA)≥10 degrees and PJA at least 10 degrees greater than preoperative. RESULTS: Eight subjects (20%) at immediate postoperative follow-up and 11 subjects (27.5%) at minimum 2-year follow-up had developed PJK. The risk of developing PJK between rib-based and spine-based growing systems was not significantly different at immediate postoperative (17% vs. 25%) or at final (25% vs. 31%) follow-ups.Further analysis combining both treatment groups demonstrated that PJK subjects were significantly older at time of initial surgery (7.1 y PJK vs. 5.0 y no PJK). Radiographic comparisons between PJK versus no PJK: Preoperative scoliosis (69.9 vs. 76.0 degrees), thoracic kyphosis (45.1 vs. 28.7 degrees), lumbar lordosis (53.1 vs. 44.0 degrees), PJA (2.2 vs. 2.8 degrees), sagittal vertical axis (1.5 vs. 2.6 cm), pelvic incidence (52.8 vs. 47.4 degrees), pelvic tilt (14.3 vs. 8.7 degrees), and sacral slope (37.7 vs. 35.9 degrees). At both initial postoperative and at final follow-up visits, a significant difference was found for cervical lordosis 32.2 versus 14.0 degrees and 42.0 versus 16.6 degrees, respectively. Risk ratio for developing PJK at final follow-up was 2.8 for subjects with preoperative thoracic hyperkyphosis and was 3.1 for subjects with high pelvic incidence (P<0.05). CONCLUSIONS: The risk of developing PJK during distraction-based growth friendly treatment for EOS was 20% immediately after implantation and 28% at minimum 2-year follow-up, with no difference observed between rib-based and spine-based treatment groups. As this study identifies a significant risk of developing PJK during the treatment of EOS, it allows clinicians to preoperatively council patients and their families about this possible complication. In addition, several potential risk factors for the development of postoperative PJK were identified, but should be investigated further in future studies. LEVEL OF EVIDENCE: Level III-therapeutic study (retrospective, comparative).

Paper #33: Magnetic Spinal Growth Rods (MCGR) with and without Preoperative Traction for the Treatment of Severe Scoliosis.

Large rigid curves can be treated with MCGR and preop traction with equivalent correction to smaller flexible curves and maintain correction over time.

Ventriculoperitoneal Shunt Fracture Following Application of Halo-Gravity Traction: A Case Report.

BACKGROUND: Ventriculoperitoneal (VP) shunt malfunctions are very common, and shunt fracture is one of the most common causes. Shunt fractures are often a result of calcification and tethering, which predispose the tubing to fracture when mechanical stresses are applied. This case report describes a case of shunt fracture following application of halo-gravity traction for correction of spinal deformity. METHODS: Chart and imaging data for a single case were reviewed and reported in this retrospective case study. RESULTS: A 10-year-old female, being treated for syndromic scoliosis, underwent posterior surgical release and application of halo-gravity traction. Increasing weight of traction was applied over a period of 6 weeks, for gradual deformity correction. It was noted on the 6-week cervical spine radiograph that the VP shunt had fractured at the base of the neck. The patient was taken to the operating room and intraoperative findings confirmed shunt fracture. This was repaired without complications. CONCLUSIONS: This case, to our best knowledge, is the first reported occurrence of shunt fracture following application of halo-gravity traction. It demonstrates the importance of careful monitoring of patients with VP shunts, when they are undergoing traction for correction of spinal deformity. LEVEL OF EVIDENCE: Level IV.

High-grade spondylolisthesis treated using a modified Bohlman technique: results among multiple surgeons.

OBJECT: The ideal surgical management of high-grade spondylolisthesis remains unclear. Concerns regarding the original Bohlman transsacral interbody fusion technique with stand-alone autologous fibular strut include late graft fracture and incomplete reduction of lumbosacral kyphosis. The authors' goal was to evaluate the radiographic and surgical outcomes of patients treated for high-grade spondylolisthesis with either transsacral S-1 screws or standard pedicle screw fixation augmenting the Bohlman posterior transsacral interbody fusion technique. METHODS: A retrospective review of patients who underwent fusion for high-grade spondylolisthesis in which a Bohlman oblique posterior interbody fusion augmented with either transsacral or standard pedicle screw fixation was performed by 4 spine surgeons was completed. Estimated blood loss, operating time, perioperative complications, and need for revision surgery were evaluated. Upright pre- and postsurgical lumbar spine radiographs were compared for slip percent and slip angle. RESULTS: Sixteen patients (12 female and 4 male) with an average age of 29 years (range 9-66 years) were evaluated. The average clinical follow-up was 78 months (range 5-137 months) and the average radiographic follow-up was 48 months (range 5-108 months). Ten L4-S1 and 6 L5-S1 fusions were performed. Five fibular struts and 11 titanium mesh cages were used for interbody fusion. Six patients had isolated transsacral screws placed, with 2 (33%) of the 6 requiring revision surgery for nonunion. No nonunions were observed in patients undergoing spanning pedicle screw fixation augmenting the interbody graft. Six patients experienced perioperative complications including 3 iliac crest site infections, 1 L-5 radiculopathy without motor involvement, 1 deep vein thrombosis, and 1 epidural hematoma requiring irrigation and debridement. The average estimated blood loss and operating times were 763 ml and 360 minutes, respectively. Slip percent improved from an average of 62% to 37% (n = 16; p < 0.01) and slip angle improved from an average of 18° to 8° (n = 16; p < 0.01). No patient experienced L-5 or other motor deficit postoperatively. CONCLUSIONS: The modified Bohlman technique for treatment of high-grade spondylolisthesis has reproducible outcomes among multiple surgeons and results in significant improvements in slip percent and slip angle. Fusion rates were high (14 of 16; 88%), especially with spanning instrumentation augmenting the oblique interbody fusion. Rates of L-5 motor deficit were low in comparison with techniques involving reduction of the anterolisthesis.

The efficacy of rib-based distraction with VEPTR in the treatment of early-onset scoliosis in patients with arthrogryposis.

BACKGROUND: Although arthrogryposis multiplex congenital (AMC) is a rare condition, rapidly progressive scoliosis is common in children with AMC. Only a limited number of studies characterize the nature of these curves, and even fewer describe surgical outcomes. To determine efficacy or rib-based distraction in these patients, we reviewed the outcomes of the use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device in what we believe is the first study to report this. METHODS: Search of the Chest Wall Spinal Deformity Study Group database identified 10 children with AMC and early-onset scoliosis who were treated with the VEPTR device at 6 different pediatric health centers. The 7 female and 3 male patients had their initial surgery at an average age of 5 years. Mean follow-up was 4.2 years. RESULTS: The most common curve was from T5 to L2. After initial VEPTR insertion, the scoliosis decreased from a mean of 67 to 43 degrees (37% correction) and kyphosis from 65 to 48 degrees (29% correction). The mean proximal junctional kyphosis after initial insertion was 33 degrees. At final follow-up, scoliosis and kyphosis were 55 degrees (17% correction) and 62 degrees (8% correction), respectively. Spinal growth during the treatment period showed a mean T1-S1 increase of 4.2 cm or approximately 1 cm/y. In the 62 procedures performed over the course of the study period, 6 complications occurred in 4 patients: 3 infections, 2 rib failures, and 1 implant failure. Six patients had proximal junctional kyphosis of ≥45 degrees at the last follow-up. CONCLUSIONS: In children with AMC, rib-based distraction using the VEPTR is an effective treatment method for controlling scoliosis and kyphosis and maintaining thoracic growth, but proximal junctional kyphosis remains a problem.

Sagittal Spinopelvic Parameters of Young Children With Scoliosis.

STUDY DESIGN: Retrospective, multicenter review of the spinopelvic parameters in young children with scoliosis. OBJECTIVES: To describe sagittal alignment of the spine and pelvis in young children with scoliosis. SUMMARY OF BACKGROUND DATA: The natural history of spinopelvic parameters has been defined for the first 10 years of life in normal children; however, they have not been described for children with scoliosis. Such information is important because these values can be used as a baseline for the assessment of radiographic outcomes after surgical intervention. METHODS: Seven measures of sagittal alignment were taken from standing lateral radiographs of 80 children with scoliosis (coronal Cobb angle greater than 50°) and compared with age-matched normal children described in the literature. Statistical analysis was performed using 2-tailed Student t tests (level of significance = .05) and Pearson correlation coefficient. RESULTS: Patients had a mean age of 4.8 years (range, 1-10 years) and a mean Cobb angle of 72.0° ± 16°. Mean sagittal spine parameters were sagittal balance (2.2 ± 4 cm), thoracic kyphosis (38.0° ± 20.8°), and lumbar lordosis (49.0° ± 16.6°). These values were similar to those of children without scoliosis. Mean sagittal pelvic parameters were: pelvic incidence (46.5° ± 15.8°), pelvic tilt (10.7° ± 13.6°), sacral slope (35.5° ± 12.1°), and pelvic radius (55.7° ± 21.3°). Pelvic incidence was not significantly different from that of age-matched normal children; however, pelvic tilt was significantly higher and sacral slope was significantly lower in children with scoliosis. CONCLUSIONS: Sagittal plane spine parameters and some pelvic parameters were similar for young children with scoliosis versus age-matched normal children; however, children with scoliosis showed signs of increased pelvic tilt and decreased sacral slope. These values can be used as a baseline for both the natural history and the assessment of radiographic outcomes after surgical intervention.

Dorsal arthrodesis in prepubertal New Zealand white rabbits followed to skeletal maturity: Effect on thoracic dimensions, spine growth and neural elements.

BACKGROUND: Several studies have shown that severe spinal deformity and early arthrodesis can adversely affect the development of the spine and thorax by changing their shape and reducing their normal function. This article analyzes the consequences of posterior fusion on the growth of spine, thorax and neural elements in New Zealand white rabbits and compares with similar human data. MATERIALS AND METHODS: The first section of the article analyzes the consequences of T1-T6 dorsal arthrodesis on the growth of the spine, sternum, thorax volume and neural elements in 12 prepubertal female New Zealand white rabbits, through a study of CT scans and histology specimens. The second part, evaluates thoracic dimensions in 21 children with spinal arthrodesis for treatment of deformity performed prior to nine years of age. RESULTS: Dorsal arthrodesis in prepubertal rabbits changes thoracic growth patterns. In operated rabbits thoracic depth grows more slowly than thoracic width. The sternum as well as length of thoracic vertebral bodies in the spinal segment T1-T6 show reduced growth. Children undergoing spinal arthrodesis before nine years of age were noted to have shortened height, short trunk and disproportionate body habitus at skeletal maturity. Observed spine height and chest dimension values were reduced compared to the expected norms. The ratio between chest width and chest depth was below normal values. CONCLUSIONS: The first part of the study shows that thoracic dorsal arthrodesis in prepubertal New Zealand white rabbit influences thoracic, spine growth and affects the shape of pseudo unipolar neurons of the dorsal root ganglia. The second part demonstrates that children treated before nine years of age have significantly reduced spine height and thoracic dimensions. The thorax becomes elliptical as chest depth grows less than chest width. Both experimental and clinical findings contribute to explain reduced chest growth and subsequent thoracic growth disturbance in patients treated with early arthrodesis.

Acute celiac artery compression syndrome after surgical correction of Scheuermann kyphosis.

STUDY DESIGN: A case of acute celiac artery compression syndrome after spinal fusion in a patient with Scheuermann kyphosis is reported. OBJECTIVE: To describe the unusual complication of acute celiac artery compression after surgical kyphosis correction, to outline diagnostic methods, and to review the pertinent literature. SUMMARY OF BACKGROUND DATA: Chronic celiac artery compression syndrome is well described, yet there is only 1 reported case of acute celiac artery compression after surgical correction of kyphosis. There have been no previous reports of this complication leading to foregut ischemic necrosis after correction of Scheuermann kyphosis. METHODS: Case report and literature review. RESULTS: After an anterior release and posterior spinal fusion for a 106 degrees kyphotic deformity performed under 1 anesthetic, our patient developed a perforated gastric antrum on postoperative day 5, evolving to ischemic necrosis of the stomach, gallbladder, and spleen discovered on postoperative day 7. Abdominal angiography indicated that his celiac artery had been occluded at its origin. After this event, the patient required a prolonged intensive care hospital stay and required a Roux-en-Y gastro-jejeunostomy reconstruction. He is now doing well at 1-year follow-up with independent ambulation and a regular diet. CONCLUSION: Acute celiac artery compression after surgical kyphosis correction is a rare but potentially serious adverse event. Spinal deformity surgeons and intensivists should be aware of this entity, and should have a high index of suspicion for it if sepsis of unknown origin, an acute abdomen, or elevated liver enzymes are encountered after surgery after correction of a kyphotic deformity.

Standardization of criteria for adolescent idiopathic scoliosis brace studies: SRS Committee on Bracing and Nonoperative Management.

STUDY DESIGN: Literature review. OBJECTIVE: To establish consistent parameters for future adolescent idiopathic scoliosis bracing studies so that valid and reliable comparisons can be made. SUMMARY OF BACKGROUND DATA: Current bracing literature lacks consistency for both inclusion criteria and the definitions of brace effectiveness. METHODS: A total of 32 brace treatment studies and the current bracing in adolescent idiopathic scoliosis proposal were analyzed to: (1) determine inclusion criteria that will best identify those patients most at risk for progression, (2) determine the most appropriate definitions for bracing effectiveness, and (3) identify additional variables that would provide valuable information. RESULTS: Early brace studies lacked clarity in their inclusion criteria. In more recent studies, inclusion criteria have narrowed considerably to include primarily those patients most at risk for curve progression who may benefit from the use of a brace. Brace effectiveness was usually defined by various degrees of curve progression at maturity. Less frequently, it was defined by the resultant curve magnitude at maturity, whether or not surgical intervention was needed, or if there was change to another brace. CONCLUSIONS: Optimal inclusion criteria for future adolescent idiopathic scoliosis brace studies consist of: age is 10 years or older when brace is prescribed, Risser 0-2, primary curve angles 25 degrees -40 degrees , no prior treatment, and, if female, either premenarchal or less than 1 year postmenarchal. Assessment of brace effectiveness should include: (1) the percentage of patients who have < or =5 degrees curve progression and the percentage of patients who have > or =6 degrees progression at maturity, (2) the percentage of patients with curves exceeding 45 degrees at maturity and the percentage who have had surgery recommended/undertaken, and (3) 2-year follow-up beyond maturity to determine the percentage of patients who subsequently undergo surgery. All patients, regardless of subjective reports on compliance, should be included in the results (intent to treat). Every study should provide results stratified by curve type and size grouping.

Pediatric spinal trauma: injuries in very young children.

Injuries to the spine in very young children are comparatively rare. The prevalence of upper cervical injuries and spinal cord injuries is greater. Spinal cord injury is more common in young children and fracture is less common than in older children and adolescents. This is because of the anatomic and biomechanical differences in the growing spine including a more horizontal facet orientation, greater elasticity of the soft tissues, less muscular development, and relatively greater head size compared to the trunk. These features are more pronounced in the very young child. The clinical and radiographic evaluation of small children can be difficult. Unossified bone and physeal cartilage can be confused with fractures. The evaluation, safe transportation, and spinal clearance of the unconscious multiply injured child suspected of having spinal injury present special challenges.

Social and economic benefits of flexible intramedullary nails in the treatment of pediatric femoral shaft fractures.

All patients between the ages of 5 and 15 years with isolated femoral shaft fractures treated at our institution with flexible intramedullary nails between 1996 and 1998 were examined and compared to an age-matched group of patients treated with spica casting. All fractures healed well with no significant complications. Patients treated with flexible intramedullary nails achieved earlier independent ambulation, at an average of 19 days, compared to 106 in the control group (P<.0001). They also attained earlier independent bathroom use (21 versus 79 days, P<.0001). Hospital stays were significantly shorter as well (6 versus 29 days, P<.0001). These patients also returned to school earlier, at 28 days postinjury, compared to 120 days for patients in spica casts (P<.0001). The use of flexible intramedullaty nails allowed patients and their families to achieve independence months earlier than the spica cast patients. Earlier return to school, independent ambulation, and independent bathroom use are advantages of this treatment modality.