Walking status and spinopelvic parameters in young children with achondroplasia: 10-year follow-up.

PURPOSE: Thoracolumbar kyphosis (TLK) is common in children with achondroplasia and resolves in 90% by 10 years of age. Our purpose was to describe the natural progression of TLK in a cohort of pre-walking children with achondroplasia. METHODS: A single-center, retrospective review identified 62 children (32 male, 30 female) with achondroplasia. Clinical information and sagittal spinopelvic parameters were collected. The children were divided into positive pelvic tilt (PT) and negative PT. All parents were routinely counseled about unsupported sitting. RESULTS: Spontaneous resolution rate was 64.5% at 1-year post-walking, 74.2% at 5 years of age, and 88.7% at 10 years of age. None of the children required posterior spinal decompression and fusion for progressive deformity or symptomatic spinal stenosis. At 1-year post-walking, the negative PT group had a higher sacral slope (p = 0.006), higher lumbar lordosis (p < 0.001), and lower pelvic incidence (p < 0.001). This relationship remained constant up to 10 years of age, and there was no association with TLK. CONCLUSION: In this largest series to date, spontaneous resolution of TLK in children with achondroplasia was 64.5% at 1-year post-walking, 74.2% at 5 years of age, and 88.7% in children followed to 10 years of age. With early identification and regular follow-up with patient education, no patient in this series required treatment or developed symptomatic spinal stenosis. While not predictive of resolution of TLK, the dichotomous presentation of PT in young children with achondroplasia persists at 5 and 10 years of age and reliably predicts the spinopelvic parameters. LEVEL OF EVIDENCE: III-retrospective comparative study.

Identification of risk factors for reconstructive hip surgery after intrathecal baclofen therapy in children with cerebral palsy.

OBJECTIVE: This study aimed to determine the risk factors for reconstructive hip surgery after intrathecal baclofen pump application in children with cerebral palsy. METHODS: Inclusion criteria were children with hypertonic (spastic or mixed spastic/dystonic motor type) cerebral palsy, intrathecal baclofen implantation <8 years of age, no reconstructive osteotomies prior to or concomitant with intrathecal baclofen implantation and at least a 5-year follow-up. Exclusion criteria included reconstructive osteotomies prior to or concurrent with intrathecal baclofen implantation, lack of at least 1 hip surveillance radiograph before intrathecal baclofen, lack of a 5-year follow-up, or having selective dorsal rhizotomy. In addition, patients with bony surgery plus last follow-up migration percentage ≥50% were labeled as required reconstruction hips. RESULTS: We identified 34 patients (68 hips). The mean follow-up was 9.2 ± 2.8 years. The mean age for intrathecal baclofen application was 6.4 ± 1.2 years. Seven patients were Gross Motor Function Classification System IV, and 27 were V. Eighteen patients (52.9%) with 31 hips (45.6%) were requiring reconstruction at the final follow-up. In multivariate analysis, male sex (odds ratio 12.8, P=.012), pre-intrathecal baclofen migration percentage (odds ratio 1.1, P=0.003), age at intrathecal baclofen implantation (odds ratio 0.24, P=.002), and delta migration percentage (odds ratio 1.1, P=.002) were significant risk factors for requiring reconstruction. Patients with intrathecal baclofen <6.2 years of age had a significantly higher rate of requiring reconstruction. A pre-intrathecal baclofen migration percentage >31% had a greater risk of progression to requiring reconstruction (P=.001). Delta migration percentage higher than 15% was significantly associated with progression to requiring reconstruction (P=.043). CONCLUSION: The risk of requiring reconstruction osteotomies after intrathecal baclofen was significantly increased in males, those younger (±migration percentage >31%) at the time of intrathecal baclofen implantation and those with an increased rate of migration percentage progression after intrathecal baclofen implantation. LEVEL OF EVIDENCE: Level IV, Prognostic Study.

Closed displaced femur fractures in children with nonambulatory cerebral palsy.

Femoral fractures in children withcerebral palsy (CP) represent a frequent medical problem, and treatment represents a challenge. The purpose of this study was to review the closed displaced femoral fractures in our population of nonambulatory children with CP to compare the results of nonoperative and operative treatment modalities to improve the care of these children. From 2006 to 2020, children with nonambulatory CP were selected with inclusion criteria of displaced femoral fracture and were divided into nonoperative and operative groups. Forty-four children met the inclusion criteria. The nonoperative group included 23 children and the operative group included 21 children. Mechanism of injury was unknown in 48% of the fractures. Fourteen (25%) fractures occurred after a femoral plate fixation during a reconstructive hip surgery, and 38 (86%) children had osteopenia. Our results reveal a high prevalence of osteopenia, low-energy trauma, malunion in nonoperative treatment, and peri-implant fractures. Suspicion of child abuse should be considered when the fracture has an unclear mechanism of the injury. Removal of proximal femoral implants may be considered to prevent peri-implant fractures. Femoral fractures should preferably be treated nonoperatively. Operative treatment should be considered for diaphyseal fractures in children capable of standing transfers, larger children, children with more severe spasticity or movement disorder or those who have suffered a high-energy fracture. Due to the high prevalence of proximal fractures in the presence of hardware, operative treatment is usually required for these fractures. In contrast, distal fractures are adequately managed nonoperatively.

Does intrathecal baclofen therapy decrease the progression of hip displacement in young patients with cerebral palsy?

AIM: To evaluate the effects of intrathecal baclofen pump (ITBP) therapy on hip dysplasia in young patients with cerebral palsy (CP). METHOD: This was a retrospective cohort series of prospectively collected data. Inclusion criteria were all patients with CP in Gross Motor Function Classification System (GMFCS) levels IV or V who underwent ITBP placement under 8 years old with at least 5 years of follow-up. Thirty-four patients were matched to a control group of 71 patients based on GMFCS level, motor type, medical comorbidities, worst hip migration percentage at ITBP placement, age, and Modified Ashworth scale scores. Patients were followed for at least 5 years or until they had hip reconstructive surgery. The primary outcome was the development of hip displacement as measured by the migration percentage at the latest follow-up or the preoperative migration percentage before hip reconstruction. RESULTS: The migration percentage at last follow-up was not statistically different between groups (ITBP: 36.2%, non-ITBP: 44.4%, p = 0.14). The rates of future preventative, reconstructive, and recurrent hip surgery were not different between groups. INTERPRETATION: The use of ITBP as an early treatment of spasticity did not alter the natural history of progressive hip displacement in non-ambulatory patients with CP and hip displacement is likely multifactorial, not solely due to spasticity.

Hip Displacement Does Not Change After Pelvic Obliquity Correction During Spinal Fusion in Children With Cerebral Palsy.

BACKGROUND: Children with cerebral palsy (CP) frequently develop both neuromuscular hip dysplasia and scoliosis, and occasionally, the timing of the worsening of both of these pathologies is concurrent. The question as to whether the hip or spine should be addressed first in CP remains controversial, with the majority of evidence being "expert opinion." The purpose of this project was to determine the impact of posterior spinal fusion (PSF) on the change in hip displacement for children with CP without previous reconstructive hip surgery. METHODS: This was an Institutional Review Board-approved study that observed 67 patients from 2004 to 2018. Inclusion criteria included children with CP, 18 years of age and younger, Gross Motor Function Classification System IV and V, undergoing PSF at a single tertiary care children's hospital with a minimum 2-year follow-up. The primary outcome was the change in hip displacement as quantified by the migration percentage (MP). The hip with the highest MP (worst hip) at the spine preoperative analysis were included for analysis. Triradiate cartilage (TRC) status and pelvic obliquity correction were analyzed with multivariate analysis. RESULTS: Sixty-seven patients were included for analysis, with a mean age of 12.5±2.3 years. The mean major curve angle of the major curve was 77±23 degrees and the mean preoperative pelvic obliquity was 21±12 degrees. There was no statistically significant change in MP after PSF from a mean preoperative value of 41±27%, to a mean postoperative value of 41±29% at the last follow-up, (P=0.76) The mean follow-up time was 4.1±2.7 years. TRC status (P=0.52) and the severity of pelvic obliquity (P=0.10) did not statistically impact the change in MP after PSF. CONCLUSION: PSF did not influence-either negatively or positively-the progression of hip displacement in children with CP, regardless of pelvic obliquity correction or TRC status. The lack of deterioration in hip displacement post-PSF, however, may suggest a protective effect of spine surgery. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

Hip Displacement After Triradiate Cartilage Closure in Nonambulatory Cerebral Palsy: Who Needs Continued Radiographic Surveillance?

BACKGROUND: Recommendations with regard to the need for continued hip surveillance after skeletal maturity are based on expert opinion rather than evidence. This study aimed to determine the prevalence of and risk factors associated with progressive hip displacement in cerebral palsy (CP) after triradiate cartilage (TRC) closure. METHODS: Patients who had spastic nonambulatory CP (Gross Motor Function Classification System IV to V) and hypertonic (spastic or mixed-type) motor type and follow-up of at least 2 years after TRC closure were included. The primary outcome variable was the hip migration percentage (MP). The secondary outcome variables included patient age at the time of TRC closure, prior preventative or reconstructive surgery, a prior intrathecal baclofen pump, history of scoliosis, history of epilepsy, a prior gastrostomy tube, a previous tracheostomy, and gender. An unsuccessful hip outcome was defined as a hip with an MP of ≥40% and/or requiring a reconstructive surgical procedure after TRC closure. RESULTS: In this study, 163 patients met the inclusion criteria, with a mean follow-up of 4.8 years (95% confidence interval [CI], 4.4 to 5.1 years) after TRC closure at a mean patient age of 14.0 years (95% CI, 13.7 to 14.3 years). Of these hips, 22.1% (36 of 163) had an unsuccessful hip outcome. In multivariate analysis, the first MP at TRC closure (hazard ratio [HR] per degree, 1.04; p < 0.001) and pelvic obliquity (HR per degree, 1.06; p = 0.003) were independent risk factors for an unsuccessful hip outcome, but gender was not found to be significant (HR for male gender, 1.7 [95% CI, 0.8 to 3.58; p = 0.16]; female gender was the reference). However, the mean survival time for progression to an unsuccessful hip outcome was longer for female patients at 9.2 years [95% CI, 8.1 to 10.2 years]) compared with 6.2 years (95% CI, 5.6 to 6.9 years) for male patients (p = 0.02). There was also a significant improvement in survivorship for prior reconstructive surgical procedures (p = 0.002). The survivorship in patients who underwent reconstructive surgery performed at a patient age of ≥6 years was significantly higher compared with those who underwent surgery performed at <6 years of age (p < 0.05). A first MP at TRC closure of ≥35% was associated with an unsuccessful hip outcome, as determined by receiver operating characteristic (ROC) curve analysis (p < 0.001; area under the ROC curve of 0.891, sensitivity of 81%, and specificity of 94%). CONCLUSIONS: The risk factors for the progression of hip displacement after TRC closure in patients with CP included a higher MP and increased pelvic obliquity; there was decreased survivorship for male patients and patients with no prior reconstructive surgery. Patients with these risk factors and/or an MP of ≥35% at TRC closure should have continued radiographic surveillance to detect late hip displacement. LEVEL OF EVIDENCE: Prognostic Level III . See Instructions for Authors for a complete description of levels of evidence.

Three different techniques for pelvic fixation in the management of neuromuscular scoliosis in nonambulatory spastic cerebral palsy: A comparative study of Galveston Rod, iliac screw, and sacroiliac screw.

OBJECTIVE: This study aimed to compare the clinical and radiographic results of three different pelvic fixation techniques, i.e., Galveston Rod, Iliac Screw, and Sacroiliac Screw, in managing neuromuscular scoliosis in nonambulatory children with spastic cerebral palsy (CP). METHODS: This retrospective study included nonambulatory children aged < 18 years with neuromuscular scoliosis secondary to CP, undergoing a spinal fusion and pelvic fixation by either Galveston rod, iliac screw, or sacroiliac screw techniques. The primary outcome variable was to determine the stability of the major curve angle and pelvic obliquity over timeline intervals for each pelvic fixation tech nique. The two radiographic parameters were measured at five timeline intervals and were compared to define stability among the groups. RESULTS: One hundred and one patients (54 females [53%]) with spastic nonambulatory CP met the inclusion criteria; the mean age at sur gery was 13.5±3.1 years. Mean follow-up intervals were first-year (12.9±1.5) and second-year (25.8±2.5). Forty-one patients had minimum five-year (81.5±23 months) postoperative follow-up. Groups were based on pelvic fixation techniques: 25 patients with the Galveston rod, 24 with the iliac screw, and 52 with the sacroiliac screw. Of the 41 patients with a minimum five-year follow-up, 10 had the Galveston rod, 11 had an iliac screw, and 20 had sacroiliac screw fixation. Gross Motor Function Classification System level, medical comorbidities, intra thecal baclofen pump, and vitamin D level were compared with each pelvic fixation technique (P > .05). Major curve angle parameters were measured at the five timelines as 70.5°±21.1°, 15.7°±13°, 15.7°±12°, 17.5°±12.7°, and 15.1°±9.6°, and pelvic obliquity as 14.8°±10.4°, 4.9°±4.2°, 5.7°±4.6°, 5°±4.4°, and 7.2°±4.4°, respectively. After the surgery, corrected major curve angle and pelvic obliquity showed no sta tistically significant difference between pelvic fixation techniques (P > .05) and remained stable over timeline intervals (P > .05). Fifteen patients had complications requiring additional surgery. The iliac screw group (nine patients) had the highest rate of complications. CONCLUSION: All three pelvic fixation techniques can provide equivalent correction for major curve angle and pelvic obliquity in managing neuromuscular scoliosis in nonambulatory CP children. Pelvic obliquity after surgery may remain stable regardless of pelvic fixation type. A higher rate of postoperative complication can be encountered with the iliac screw. LEVEL OF EVIDENCE: Level III, Retrospective Study.

Does the Addition of Intrathecal Baclofen Along with or After Soft-Tissue Hip Adductor Surgery Decrease the Need for Hip Reconstruction Compared with Soft-Tissue Surgery Alone for Children with Nonambulatory Cerebral Palsy?

Purpose: Intrathecal baclofen (ITB) is a well-known treatment option for cerebral palsy (CP) spasticity. The combination of soft-tissue release and ITB for spasticity is common. This study compared patients who had soft-tissue release before ITB (PRE-ITB), soft-tissue release at the same time as ITB (ST-ITB), and no ITB (NON-ITB) but had soft-tissue release at a similar age as PRE-ITB. Methods: Inclusion criteria were a spastic or mixed nonambulatory CP diagnosis, prior hip adductor surgery, no prior reconstructive surgery, and at least a five-year post-operative follow-up. Thirty hips were identified as PRE-ITB, 20 hips as ST-ITB, and 43 hips as NON-ITB. The primary outcome variables were the subsequent hip surgery during the study period and/or a migration percentage ≥ 50% at the final follow-up defined as "unsuccessful hip." Results: The mean follow-up duration was 9.0 years (SD 2.4) for PRE-ITB, 9.4 (SD 3.6) for ST-ITB, and 9.3 (SD 3) for NON-ITB. The odds of unsuccessful outcomes were not different between NON-ITB and PRE-ITB but were lower for the ST-ITB group. The need for subsequent osteotomies or revision adductor surgery was significantly higher in ST-ITB compared with PRE-ITB (p = 0.02) or NON-ITB (p = 0.015). The incidence of surgical site infection over the whole follow-up period was higher in ST-ITB (40%) compared with PRE-ITB (13.3%, p = 0.035) and NON-ITB (0, p < 0.001). Conclusion: The addition of tone management with ITB did not reduce the need for later hip surgery but did increase the risk for surgical site infection.

No difference in postoperative complication rates or cardiopulmonary function for early versus late scoliosis correction in Duchenne muscular dystrophy.

BACKGROUND: Given reduced rates of both pulmonary function decline and scoliosis progression with steroid treatment in Duchenne muscular dystrophy (DMD), the role of early scoliosis surgery has been questioned. The purpose of this study was to compare the postoperative complication rates of early versus late scoliosis correction in DMD. METHODS: This study was a retrospective cohort, conducted at an academic tertiary level children's hospital. Patients with DMD who underwent posterior scoliosis correction, with preoperative pulmonary function testing [forced vital capacity (FVC)] were included and divided into two groups by preoperative curve angles: ≤ 45° and > 45°. The primary outcome variable was postoperative complications by Clavien-Dindo classification grading. Secondary outcome variables included postoperative complications occurring after the first 90 days, age at surgery, duration of wheelchair dependency preoperatively, pulmonary function, steroid utilization, shortening fraction by echocardiogram, surgery duration, intensive care unit/hospital length of stay, days intubated, infection, and percent curve correction. Two-tailed t-test and Chi-square testing were used for analysis of patient factors and Clavien-Dindo complication grade, respectively. RESULTS: Thirty-one patients were included with a total follow-up of 8.3 ± 3.2 years, 4.8 ± 2.2 years post-spinal fusion. Steroid treatment (prednisone, deflazacort) was utilized for 21 (67.7%) patients. Primary curve correction was not different between groups (65.0% vs 71.4% [p = 0.37]). There were no significant differences in Clavien-Dindo classification grades between groups (p > 0.05). For the entire cohort, the overall complication rate was higher for patients with steroid treatment (61.9% vs 10.0% [p = 0.008]). Neither forced vital capacity nor fractional shortening on echocardiogram was different between groups at final follow-up (p = 0.6 and p = 0.4, respectively). CONCLUSION: The comparable risk of perioperative complications for early and late scoliosis correction supports a "watchful waiting" approach, whereby curves less than 45° can be carefully followed while cardiopulmonary function is maintained. Patients undergoing steroid treatment should be counseled regarding the higher risk of postoperative blood transfusion and deep wound infection. LEVEL OF EVIDENCE: III Retrospective cohort.

The influence of tone on proximal femoral and acetabular geometry in neuromuscular hip displacement: A comparison of cerebral palsy and spinal muscular atrophy.

Purpose: The aim of this article was to compare longitudinal changes in hip morphology in cerebral palsy (hypertonic) and spinal muscular atrophy (hypotonic) to examine the influence of muscle tone on development of hip displacement. Methods: Children with spinal muscular atrophy (Types I and II) and cerebral palsy (Gross Motor Function Classification System IV and V) with hip displacement (migration percentage >30%) were included. Head shaft angle, migration percentage, and acetabular index were measured at T1 (1-2.5 years), T2 (3-5 years), and T3 (6-8 years). Analysis of variance testing and linear regression were utilized. Results: Sixty patients (cerebral palsy, N = 41; spinal muscular atrophy, N = 19) were included. Hip displacement occurred earlier in spinal muscular atrophy (34 months) than cerebral palsy (49 months) (p = 0.003). Head shaft angle was high and did not change between T1, T2, and T3, but significant changes in migration percentage were found (cerebral palsy: 23%, 36%, 45% (p < 0.01) and spinal muscular atrophy: 37%, 57%, 61% (p = 0.02)). Migration percentage increased by age in cerebral palsy (r = 0.41, p < 0.001), but not in spinal muscular atrophy (r = 0.18, p = 0.09). Acetabular index increased with migration percentage (cerebral palsy: r = 0.41, p < 0.001; spinal muscular atrophy: r = 0.48, p < 0.001). Conclusion: Persistent lateral physeal tilt by head shaft angle was found for both spinal muscular atrophy and cerebral palsy. Abnormal physeal alignment may be causally related to weakness of the hip abductor muscles rather than spasticity or muscle imbalance, resulting in coxa valga and secondary acetabular dysplasia. Level of evidence: III (case-control study).

Evaluation of titanium release from titanium alloy implants in patients with spinal instrumentation.

OBJECTIVE: This study was performed to investigate the baseline serum titanium levels in patients with short-segment titanium alloy posterior instrumentation and to assess patient-, implant-, and surgery-related factors that might affect the serum titanium level. METHOD: Two groups of patients were included in the study. The study group comprised 39 patients who had undergone short-segment posterior instrumentation from January 2013 to June 2016. The control group comprised 11 randomly selected patients who presented to the outpatient clinic with no history of orthopedic surgery. The serum titanium levels and inter-group differences were analyzed. RESULTS: The mean serum titanium level was significantly higher in the study group than in the control group. No significant difference was observed between patients with different etiologies, implants used for fusion, numbers of instrumented segments, or postoperative durations. CONCLUSION: The serum titanium levels of patients with posterior lumbar spinal instrumentation are significantly higher than those of the normal population even after achievement of solid fusion. These levels are not affected by the use of transverse connectors, the use of cages, the operated segments, or the duration of implants.

Magnetically controlled growing rod in 13 patients with early-onset scoliosis and spinal improvement.

OBJECTIVE: The aim of this study was to examine the use of magnetically controlled growing rods as a method of providing spinal improvement while preventing thoracic insufficiency in patients with early-onset scoliosis (EOS). METHODS: Of a total of 13 patients, 4 patients underwent a dual magnetic rod implantation, while 9 patients had a single magnetic rod procedure. The study group comprised 12 (93%) female and 1 (7%) male patients. Six patients (46%) had an idiopathic form of scoliosis, in 4 (30%) it was congenital, and in 3 (23%) it was neuromuscular scoliosis. The patients' Cobb angles, thoracic kyphosis, T1-T12 and T1-S1 distance prior to and following the treatment were compared. RESULTS: The mean Cobb angle before surgery was 53.780, whereas it decreased to 39.290 postoperatively (p < 0.001). The mean thoracic kyphosis angle was 400 before and 29.790 after surgery (p < 0.001). The mean T1-S1 distance was 32.14 cm before and 36.36 cm after surgery (p < 0.001). The mean T1-T12 distance was 18.69 cm before and 20.64 cm after surgery (p < 0.001). CONCLUSION: The use of magnetic rods is an effective method of EOS treatment. It allows for spinal growth while managing the progression of the scoliosis. LEVEL OF EVIDENCE: Level IV, therapeutic study.

The comparison of the results after spinal fusion with or without iliac screw insertion in the treatment of neuromuscular scoliosis.

INTRODUCTION: Neuromuscular scoliosis leads to a wide range of spinal disorders which disturb the musculoskeletal system. The aim of this study is to compare the clinical and radiological results of posterior spinal fusion with and without extending the instrumentation to iliac bones in treatment of neuromuscular scoliosis. METHODS: Medical records and radiographies of 36 patients with neuromuscular scoliosis who underwent posterior instrumentation between 2011 and 2015 were reviewed. Age and body mass index at time of surgery, underlying diagnosis, gender, postoperative infection rates, perioperative and postoperative blood transfusion, duration of surgery, complication rates were identified for each patient retrospectively. SF-36 physical questionnaire was applied to all patients. Surgery was performed in each patient and included posterior spinal fusion with pedicle screws from the proximal thoracic spine (T2 or T3) to S1 (Group A) or extension of distal instrumentation to pelvis by bilateral iliac screws (Group B). RESULTS: A total of 23 patients in group A were compared with 13 patients in group B. Median age was 14 (9-38) years for group A and 16 (12-25) years for group B. Median follow-up period was 20 (12-66) months. Preoperative median Cobb angles were 66° and 60° and postoperative Cobb values were 33° and 31° in group A and B respectively. Median Cobb angle reduction was 40° and 34° for group A and B. We are able to see in this study that the usage of illiac screws do not increase implant failure and help achieve better functional results. CONCLUSION: This study shows that the extention of instrumentation to the pelvis with illiac screws can be beneficial in terms of functional and complicational incidences. LEVEL OF EVIDENCE: Level III, therapeutic study.