Publications by authors named "Kenneth J Rogers"

68 Publications

Neuromonitoring for Proximal Fibular Osteochondroma Excision.

J Pediatr Orthop 2022 Mar 29. Epub 2022 Mar 29.

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: The peroneal nerve is at risk when excising tumors in the proximal fibula. The rate of nerve injuries during proximal fibular tumor resection varies from 3% to 20%. Our goal was to report our experience with resection of osteochondromas in the proximal fibula and describe the technique and utility of neuromonitoring during excision of proximal fibular osteochondromas (PFO).

Methods: Patients with a diagnosis of symptomatic PFO who had undergone excision at one institution from 1994 to 2018 were included. An institutional review board-approved retrospective review was performed. Intraoperative neuromonitoring was provided from 2006 on by a single group utilizing a multimodality protocol.

Results: This study contains 29 patients who had excision of osteochondromas in the proximal fibula. Of these 29 consecutively monitored patients, there were 34 involved extremities. Intraoperative neuromonitoring alerts occurred in 10/29 (34.5%) procedures, which included 3 electromyography (EMG) (30%), 2 motor-evoked potential (20%), 1 somatosensory-evoked potential (10%), and 4 alerts with a combination of EMG/motor-evoked potential/somatosensory-evoked potential changes (40%). The interventions that were taken resulted in resolution of the neuromonitoring changes in all procedures. Postoperatively, we noted 2 (6.9%) new mild sensory deficits, which resolved during follow up. There were 3 patients in whom pre-existing sensory-motor deficits improved but not completely after surgery, 1 motor weakness, and 2 with residual paresthesia. In those initially presenting with paresis, there was improvement in 8 of the 8 extremities by the last follow-up visit. Pain as a symptom was resolved in all cases. There were no iatrogenic foot drop injuries. The average follow up was 32.2 months.

Conclusions: Neuromonitoring during PFO excision demonstrated a high number of alerts, all of which resolved following timely corrective action. The use of neuromonitoring may help decrease the risk of iatrogenic postoperative neurological deficits following fibular osteochondroma surgery.

Level Of Evidence: Level IV.
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http://dx.doi.org/10.1097/BPO.0000000000002149DOI Listing
March 2022

Multicenter Series of Deformity Correction Using Guided Growth in the Setting of Osteogenesis Imperfecta.

J Pediatr Orthop 2022 Mar 14. Epub 2022 Mar 14.

Department of Orthopaedics, Nemours Childrens Hospital, Wilmington, DE Department of Orthopaedic Surgery, University of Nebraska Medical Center, Children's Hospital and Medical Center, Omaha, Nebraska Division of Orthopedic Surgery, Specialty Hospital "St. Catherine", Zagreb, Croatia.

Background: The mainstay of deformity correction and fracture prevention for patients with osteogenesis imperfecta (OI) includes osteotomies and intramedullary rodding. Guided growth, described in the setting of skeletal dysplasias, offers a less invasive means of deformity correction. We report a multicenter case series of guided growth procedures in the setting of OI.

Methods: We retrospectively reviewed patients with OI at three institutions from April 2012 to April 2019: 18 patients underwent guided growth for angular deformity correction with minimum 1-year follow-up or full deformity correction and removal of guided growth hardware. Clinical characteristics, deformity measurements, and complications were collected. Distal femoral and proximal tibial hemiepiphysiodesis was performed using figure-of-eight plates and screws, and distal tibial medial hemiepiphysiodesis with cannulated screws. Preoperative and postoperative lateral distal femoral angle, medial proximal tibial angle, and lateral distal tibial angle were measured. Frequency and descriptive statistics were completed.

Results: Eighteen patients with OI (five-I, four-III, six-IV, three-V) underwent 33 guided growth procedures with mean follow-up of 3.09 years; all received routine bisphosphonate treatment. Preoperative and postoperative mean joint angles were measured. The location for hemiepiphysiodesis included 8 distal femoral medial, 2 distal femoral lateral, 8 proximal tibial medial, 3 proximal tibial lateral, and 12 distal tibial medial. Twelve of the 33 procedures were in patients who had an intramedullary rod; 1 demonstrated backout of the epiphyseal and metaphyseal screws of a distal femoral medial figure-of-eight plate. It was revised to a larger plate with longer screws and removed upon completion of deformity correction.

Conclusion: Guided growth may be used as an effective means of angular deformity correction with dysplastic OI bone. Having an intramedullary rod did not preclude the use of a guided growth technique. One procedure demonstrated screw backout. Given the short stature associated with OI, performing a guided growth procedure at an early enough age to allow time for correction should be considered.

Level Of Evidence: Level IV-case series.
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http://dx.doi.org/10.1097/BPO.0000000000002140DOI Listing
March 2022

Incidence of Deep Vein Thrombosis in Cerebral Palsy Following an Orthopaedic Surgical Event.

J Pediatr Orthop 2022 May-Jun 01;42(5):285-288

Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Purpose: The purpose of this study was to identify the incidence of venous thromboembolism (VTE) and characterize the demographics, comorbidities, and risk factors for patients with cerebral palsy (CP) having orthopaedic surgery.

Methods: All patients diagnosed with CP who underwent an orthopaedic surgical procedure at one institution between 2008 and 2017 were identified. Diagnosis codes and associated patient events were recovered from the electronic medical record. Each VTE event was reviewed to ascertain an actual VTE episode related to a surgical event.

Results: The review included 2583 orthopaedic surgical events in 1371 patients. Of the initial 88 cases identified, 28 cases had a deep thrombosis documented. Six cases of VTE occurred within 3 months following the surgical event. Three of these cases had thigh thrombosis, and 2 patients had upper arm thrombosis, and 1 patient had a superior vena cava thrombosis. On further workup, 5 of these 6 patients were identified as having a congenital hypercoagulable condition.

Conclusions: VTE is a relatively rare occurrence after orthopaedic surgery in pediatric patients with CP, but when it occurs, a full hematologic workup for a congenital hypercoagulable condition is indicated. Based on the low incidence of thigh thrombosis, routine pharmacological or intermittent mechanical calf compression is not recommended. A careful clinical and family history should be performed to identify patients with possible genetic hypercoagulable conditions who would merit prophylaxis.

Level Of Evidence: Level IV.
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http://dx.doi.org/10.1097/BPO.0000000000002113DOI Listing
April 2022

Outcomes of medial patellofemoral ligament reconstruction and tibial tubercle osteotomy in syndromic adolescents with patellar dislocation.

J Clin Orthop Trauma 2022 Feb 14;25:101770. Epub 2022 Jan 14.

Department of Orthopaedic Surgery, Nemours/Alfred I. DuPont Hospital for Children, 1600 Rockland Rd., Wilmington, DE, 19803, USA.

Background: Treatment of congenital and habitual dislocation of the patella in syndromic adolescents can be difficult due to accompanying soft-tissue and/or osseous abnormalities often present in the knee. The aim of this study was to report the results of surgical treatment of congenital and habitual patellar dislocation with medial patellofemoral ligament (MPFL) reconstruction and tibial tubercle osteotomy (TTO) in adolescents with an underlying syndrome.

Methods: Syndromic adolescent patients with congenital or habitual patellar dislocation treated with MPFL reconstruction and TTO between 2005 and 2019 with a minimum of one year of follow-up were identified. Demographic, clinical, radiographic, and surgical data were recorded, and any complications were noted. Kujala and Lysholm scores were used to quantitate knee function.

Results: Seventeen knees in 11 patients met the criteria for inclusion. The mean age at operation was 14.8 years (range, 13.3-18.3 years). Patients were identified as having Ehlers-Danlos (four), Down (two), trichorhinophalangeal (one), McCune-Albright (one), Klippel-Feil (one), and generalized joint hypermobility (two) syndromes. The mean follow-up was 2.2 years for each individual knee (range, 1-5.9 years). The mean Kujala score increased from 56 ± 10 preoperatively to 86 ± 6 at the most recent postoperative visit (p < 0.001). The mean Lysholm score increased from 53 ± 10 preoperatively to 85 ± 7 at the most recent postoperative visit (p < 0.001). Knee flexion increased significantly from 117° ± 15° preoperatively to 154° ± 13° postoperatively (p < 0.001). However, knee extension was no different pre- and postoperatively (4° ± 8° vs. 1° ± 4°, respectively, p = 0.2).

Conclusions: Congenital and habitual patellar dislocation in adolescent-aged patients with an underlying syndromic diagnosis can be successfully treated with MPFL reconstruction combined with TTO.
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http://dx.doi.org/10.1016/j.jcot.2022.101770DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8803613PMC
February 2022

Increased medial pedicle angulation of the lumbar spine as a function of the posterior osseous defect in patients with myelomeningocele.

Spine Deform 2022 05 30;10(3):709-716. Epub 2022 Jan 30.

Department of Orthopedics, Division of Spine and Scoliosis Surgery, Nemours Children's Health/Alfred I.duPont Hospital for Children, Delaware, 1600, Rockland Road, Wilmington, DE, 19899, USA.

Purpose: Bony anatomy in patients with immature spines has been previously characterized. Alterations in lumbosacral morphology in patients with myelodysplasia, demonstrating increasing medial pedicle angulation (MPA) from L1-S1, have been examined; however, MPA related to size of the defect in myelomeningocele patients has not been defined. We aimed to establish that magnitude of posterior vertebral arch defects determines the extent of MPA in the lumbar spine, with larger posterior defects associated with increased MPA.

Methods: This retrospective case-control study compared lumbar morphometric measurements of patients with and without myelomeningocele. Eighteen patients with myelomeningocele underwent computed tomography with three-dimensional reconstruction; there was no concurrent pathology. Advanced imaging software was utilized to manipulate the axial, sagittal, and coronal axes at each lumbar level, and obtain accurate measurements of pedicle width, height, length, MPA, and posterior laminar defect.

Results: In lumbar vertebrae with a posterior arch defect (PAD), increased magnitude was associated with a concurrent increase in MPA between 16.2° (43.4%) and 28.1° (299.4%) depending on lumbar level. At levels without a PAD, increases in MPA were between 0.8° (4.3%) and 5.7° (60.6%) depending on lumbar level. Although the actual degree of medial angulation increases from cephalad to caudal levels, the percentage of deviation from normal is higher at the more cephalad levels compared with controls.

Conclusion: In patients with myelomeningocele, larger posterior vertebral arch defects were associated with increased MPA in the lumbar spine. Lumbar levels without a PAD also demonstrated increases in MPA compared with normal values.

Level Of Evidence: 4:
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http://dx.doi.org/10.1007/s43390-021-00459-4DOI Listing
May 2022

Risk Factors for Rebound After Correction of Genu Valgum in Skeletal Dysplasia Patients Treated by Tension Band Plates.

J Pediatr Orthop 2022 Apr;42(4):190-194

Department of Orthopaedics, Nemours Childrens Hospital, Delaware, Wilmington, DE.

Background: Growth modulation using tension band plates (TBPs) is increasingly important for lower limb deformity correction in patients with skeletal dysplasia (SKD). Development of rebound deformity is a concern after TBP removal. Data regarding this complication are rare; therefore, we evaluated the prevalence and risk factors for rebound deformity in children with SKD undergoing correction of genu valgum using TBP.

Methods: All patients with SKD with genu valgum treated by TBP at the distal femur or/and proximal tibia at a single center were reviewed. Inclusion criteria were: (1) minimum 2-year follow-up after TBP removal or having revision surgery for rebound deformity and (2) implant removal age for girls 14 years and below and boys 16 years and below. Exclusion criteria were any femoral/tibial osteotomies during TBP treatment or follow-up. A change of ≥3 degrees of mechanical lateral distal femoral and/or medial proximal tibial angle was accepted as rebound deformity and analyzed statistically.

Results: Thirty-three patients (59 limbs; 52 femur and 29 tibia physes) met our criteria. Mean follow-up after implant removal was 43.7 months. Rebound deformities were seen in 43 limbs (39 femurs and 13 tibias). Boys had more rebound than girls; however, this was not influenced by body mass index. Femurs had more rebound than tibias. Patients in the rebound group were younger than the nonrebound group. Time from application to removal of TBP was shorter in the rebound versus nonrebound group. Overcorrected limbs had more rebound deformity than not overcorrected. The difference in growth velocity of lower limbs in the rebound versus nonrebound group was statistically significant. Patients with epiphyseal dysplasia had more rebound than metaphyseal dysplasia, but this was not statistically significant.

Conclusion: Risk factors for developing a rebound deformity after correction of genu valgum using TBP in SKD included male sex, TBP surgery at a young age, short duration of TBP implantation, overcorrected extremity (mechanical axis deviation ≤1), and high percent growth velocity after TBP removal.

Level Of Evidence: Level IV-retrospective study.
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http://dx.doi.org/10.1097/BPO.0000000000002053DOI Listing
April 2022

Elongation-Derotation-Flexion Casting Treatment of Early-Onset Progressive Scoliosis in Skeletal Dysplasia.

J Pediatr Orthop 2022 Mar;42(3):e229-e233

Nemours Children's Hospital, Delaware, Wilmington, DE.

Background: Early-onset scoliosis in children with skeletal dysplasia is progressive, contributing to cardiopulmonary restrictive disease. Serial elongation-derotation-flexion (EDF) casting, used in other etiologies of scoliosis to delay curve progression, may be beneficial in maximizing spine growth. Our hypothesis is serial EDF casting can be safely used as a temporary alternative to surgery, delaying progression and preserving growth, to treat scoliosis in skeletal dysplasia.

Methods: All patients with skeletal dysplasia treated at a single institution with serial EDF casting for scoliosis were reviewed retrospectively. Radiographic parameters: Cobb angle of major and minor curves, curve location, thoracic height, thoracolumbar height, space available for lung, and rib vertebra angle difference were measured before casting (C1), in first casting (C2), in last casting (C3), and out of last casting (C4). Peak inspiratory pressure (PIP) values were monitored and recorded during the casting application.

Results: Eleven patients met the inclusion criteria (mean 9.7 castings). The mean duration of EDF serial casting was 35 months. The mean major Cobb angles were 54 degrees° (C1), 30 degrees (C2), 37 degrees (C3), and 49 degrees (C4) with no statistically significant differences. The mean minor Cobb angles were 35 degrees (C1), 25 degrees (C2), 33 degrees (C3), and 51 degrees (C4) with no statistically significant differences. The mean thoracic heights were 130 mm (C1), 155 mm (C2), 173 mm (C3), and 160 mm (C4). The 19-mm mean difference between C2 and C3 represents spinal growth. The PIP-1, PIP-2, and PIP-3 mean values were 15, 27, and 18 cmH2O, respectively. Changes in PIP-1 and PIP-2 and PIP-2 and PIP-3 were statistically significant.

Conclusion: Serial EDF casting can delay surgical scoliosis correction in children younger than 7 years with a diagnosis of skeletal dysplasia. Our study showed that serial casting controls progression of the major curve and allows longitudinal growth of the spine with possible expansion of lung volume for nearly 3 years. During cast application, PIP increased with molding and traction, and improved until windowing and trimming of the cast.

Level Of Evidence: Level IV-retrospective study.
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http://dx.doi.org/10.1097/BPO.0000000000002037DOI Listing
March 2022

The impact of asymmetry on the radiographical outcomes following hip reconstruction in patients with cerebral palsy.

J Child Orthop 2021 Oct;15(5):510-514

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, United States.

Purpose: The purpose of this study was to evaluate the impact of asymmetric hip dysplasia on the outcome of hip reconstruction in patients with cerebral palsy according to preoperative migration percentage (MP).

Methods: This study was institutional review board-approved for retrospective cohort review. From 2008 to 2018, 65 patients met inclusion criteria: Gross Motor Function Scale Classification (GMFSC) III to V with spastic hips (MP > 30%) who underwent bilateral hip reconstruction, with a follow-up > 24 months. Main exclusion criteria: children with associated syndromes or chromosomal disorders. The cohort was subdivided into three groups according to preoperative MP difference between hips: Group A > 50%, group B 20% to 50% and Group C < 20%. Subsequently, the groups were analyzed individually and then compared. The asymmetry of extended abduction of the hip was also evaluated and separated into three groups: no asymmetry (< 20° difference), mild asymmetry (20° to 50° difference) and severe (> 50° difference).

Results: In total, 65 patients underwent bilateral bony reconstructive surgery (130 hips). Mean age at surgery was 10.1 years (sd 3.6; 3.6 to 18.4). Mean age at follow-up was 14.7 years (sd 3.8; 8 to 21). Preoperative GMFSC distribution was grade III (four, 6%), IV (15, 23%) and V (46, 71%). In all, 21 symmetric hips (< 20% MP difference) had a preoperative MP difference of 9% and a follow-up MP difference of 18% (p > 0.05); 32 had a preoperative MP difference of 34% and a follow-up MP difference of 16% (p < 0.0001); 12 had a preoperative MP difference of 80% and a follow-up difference of 6% (p < 0.0001). According to pre- and postoperative abduction values, the mean high hip abduction preoperatively was 34° (sd 17°), whereas low hip abduction was 23° (sd 17°).

Conclusion: Hips with asymmetrical dysplasia and/or abduction undergoing bilateral reconstructive surgery focused on symmetric abduction, and corrected dysplasia in patients with cerebral palsy has improved symmetry in hip abduction and MP. Obtaining this goal immediately postoperatively is maintained to medium-term follow-up.

Level Of Evidence: IV.
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http://dx.doi.org/10.1302/1863-2548.15.210056DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8582612PMC
October 2021

Recurrent hip instability after hip reconstruction in cerebral palsy children with spastic hip disease.

J Orthop Sci 2021 Nov 25. Epub 2021 Nov 25.

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Background: Migration percentage (MP) is widely used to evaluate hip stability in children with spastic cerebral palsy (CP). Orthopedic surgeons need more objective information to make a proper hip reconstruction surgical plan and predict the outcome.

Methods: Medical records and plain radiographs of children with CP who underwent the hip reconstruction procedure for dysplasia were reviewed retrospectively.

Results: In total, 253 operated hips (140 patients; 11.7 ± 3.3 years old) were included in this study. MP at pre-operative (T) was 35.3 ± 22.5%; at immediate follow-up (T) was 5.9 ± 9.5%; at last follow-up (T) was 9.8 ± 10.8% (4.5 ± 2.3 years post-operative at age 16.3 ± 2.8 years). In hips with Melbourne Cerebral Palsy Hip Classification Scale (MCPHCS) grade 3 (n = 78), around 30-45% had an unsatisfactory outcome at T and T. However, hips categorized as other grades showed only 2.1-9.1% of unsatisfactory outcome. In less affected hips (pre-operative MP<30%, n = 122), 109 hips (89.3%) had varus derotation osteotomy only, the other 13 hips (10.7%) were combined with a pelvic osteotomy. In more severely affected hips (pre-operative MP ≥ 30%, n = 131), 26 hips (19.8%) had varus derotation osteotomy only, the other 105 hips (80.2%) were combined with a pelvic osteotomy.

Conclusions: Hips with pre-operative MP between 15 and 29% (MCPHCS grades 3) can be a higher risk group of recurrent hip instability after hip reconstruction surgery. Multiple indications beyond MP should be considered when indicating pelvic osteotomy or hip muscle release as combined procedures with varus femoral osteotomy for hip reconstruction in this milder group to achieve a consistent long-term satisfactory outcome.
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http://dx.doi.org/10.1016/j.jos.2021.10.017DOI Listing
November 2021

Applications and Error Ratios of Calibration Techniques in EOS, Orthoroentgenogram, and Teleoroentgenogram for Length Measurement: A Comparative Study.

J Pediatr Orthop 2022 Jan;42(1):e21-e26

Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Accurate length measurements of extremity bones are essential in treating limb deformities and length discrepancies in children.

Objective: This study aimed to determine errors in common techniques used to measure lower limb lengths in children.

Methods: Precision and instrument errors in length measurements were studied utilizing electro-optical system (EOS), orthoroentgenogram, and teleoroentgenogram The goal was to measure a 70-cm metallic rod phantom (average length of the lower extremity of a 10-year-old boy in the 50th percentile) in 3 phases. In Phase 1, the length measurements were performed in an EOS unit with internal calibrations, a magball/magstrip in various scan positions, and measurement with TraumaCAD software. In Phase 2, the measurements were repeated utilizing a single radiation "shot" teleoroentgenogram. In Phase 3, an orthoroentgenogram was utilized with a radiopaque ruler reference. The reliability and validity of measurements were calibrated by 4 physicians (a radiologist, senior orthopaedic attending, and 2 orthopaedic fellows).

Results: EOS measurements utilizing internal references had excellent accuracy (for a 700-mm real length, magnification error (ME)] of 0.09%. Teleoroentgenogram with a magball reference and measurements performed with automatic calibration by TraumaCAD program results in ME of 1.83% with insignificant intraobserver/interobserver difference. Teleoroentgenogram with a magball or magstrip reference measured manually showed that the magball has higher intraobserver/interobserver variance than magstrip, with a 6.60 and 0.33-mm SD, respectively. The length by manual measurement utilizing the magstrip has ME of 2.21%. Orthoroentgenogram is accurate with ME of 0.26%, but does not allow anatomical analysis and is also radiation-costly.

Conclusion: EOS and orthoroentgenogram are very accurate for length measurements. Teleoroentgenogram is less accurate in measuring length; however, addition of an external reference (magball, magstrip) placed lateral to the target improves accuracy. Automatic calibration with computer-based analysis of the external reference improves the accuracy more than manual calibration. If manual calibration is utilized, the length measurement is less accurate with the magball than the magstrip.

Level Of Evidence: Level II-comparative in vitro study.
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http://dx.doi.org/10.1097/BPO.0000000000001931DOI Listing
January 2022

The change in sagittal plane gait patterns from childhood to maturity in bilateral cerebral palsy.

Gait Posture 2021 10 28;90:154-160. Epub 2021 Aug 28.

Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Rd., Wilmington, DE 19803, United States. Electronic address:

Background: The longitudinal stability of sagittal gait patterns in diplegic cerebral palsy (CP), stratified using the Rodda classification, is currently unknown.

Research Question: What is the trajectory of sagittal plane gait deformities as defined by the Rodda classification in a large cohort treated with orthopedic surgery guided by gait analysis?

Methods: A retrospective study utilized gait analysis to evaluate sagittal gait parameters before age 8 and after age 15 years. Individual limbs were categorized at each time point according to the Rodda classification based on mean sagittal plane knee and ankle angle during stance. Welch's t-tests compared gait variables from early childhood with maturity and examined changes associated with plantarflexor lengthening surgery.

Results: 100 youth with CP were evaluated twice: at a mean age of 5.49 ± 1.18 and 19.09 ± 4.32 years, respectively. Gross Motor Function Classification System distribution at maturity was I (10.5 %), II (55.2 %), III (28.6 %), and IV (5.7 %). At the initial visit, most limbs were in either true equinus (30 %) or jump-knee gait (26.5 %). At maturity, crouch gait (52.5 %) was the most common classification, of which 47.6 % were mild (1-3 standard deviations from age-matched norm; 21°-30°) and 52.4 % moderate or severe. For the entire cohort, at initial and final visits, respectively, mean knee flexion in stance was 26.8°±14.8° and 25.9°±11.4° (p = 0.320), ankle dorsiflexion in stance increased from -0.3°±11.5° to 9.0°±6.0° (p < 0.001), and passive knee flexion contracture was -2.3°±7.0° and -3.9°±8.0° (p = 0.043). In children who started in true equinus, apparent equinus, and crouch, there was no difference in stance phase knee flexion at maturity between those who underwent plantarflexor lengthenings versus those who did not (p > 0.18).

Significance: The trend in this cohort was toward crouch with increased stance phase ankle dorsiflexion from early childhood to maturity. Plantarflexor lengthenings were not a significant factor in the progression of stance phase knee flexion.
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http://dx.doi.org/10.1016/j.gaitpost.2021.08.022DOI Listing
October 2021

Hip Displacement in MECP2 Disorders: Prevalence and Risk Factors.

J Pediatr Orthop 2021 Oct;41(9):e800-e803

Department of Orthopedic Surgery, Division of Cerebral Palsy, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Methyl-CpG binding protein 2 (MECP2) disorders, including Rett syndrome and MECP2 duplication syndrome, are typified by profound intellectual disability, spasticity, and decline in gross motor function. Unlike scoliosis, linked to disease severity, little has been reported regarding the hip. The aim of this study was to report the prevalence and risk factors of hip displacement (HD) in MECP2 disorders.

Methods: This was a retrospective, comparative study. Children with a genetically confirmed MECP2 disorder were included. The primary outcome measure was the prevalence of HD (migration percentage>30%). Secondary outcomes included age at HD onset, ambulatory status, presence of clinically relevant scoliosis, genetic severity, presence of seizures, and associated comorbidities. Analysis of proportions of categorical variables was performed using χ2 testing (P=0.05).

Results: Fifty-six patients (54 Rett syndrome and 2 MECP2 duplication syndrome), diagnosed at 6.6 (SD: 4.7) years, met the inclusion criteria. The prevalence of HD was 36% [onset, 7.7 (SD: 3.8) y]. Risk factors for HD were nonwalker status (P=0.04), scoliosis (P=0.001), and refractory epilepsy (P=0.04).

Conclusions: The prevalence of HD in MECP2 disorders is comparable to cerebral palsy, associated with proxy measures of disease severity. These results can be used to develop hip surveillance programs for MECP2 disorders, allowing for timely management.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000001898DOI Listing
October 2021

Single-event multilevel surgery in cerebral palsy: Value added by a co-surgeon.

Medicine (Baltimore) 2021 Jun;100(24):e26294

National Academy of Medicine Fellowship, American Osteopathic Association, Chicago, IL, USA.

Abstract: The aim of this study was to compare outcomes for single-event multilevel surgery (SEMLS) in cerebral palsy (CP) performed by 1 or 2 attending surgeons.A retrospective review of patients with CP undergoing SEMLS was performed. Patients undergoing SEMLS performed by a single senior surgeon were compared with patients undergoing SEMLS by the same senior surgeon and a consistent second attending surgeon. Due to heterogeneity of the type and quantity of SEMLS procedures included in this study, a scoring system was utilized to stratify patients to low and high surgical burden. The SEMLS events scoring less than 18 points were categorized as low burden surgery and SEMLS scoring 18 or more points were categorized as high burden surgery. Operative time, estimated blood loss, hospital length of stay, and operating room (OR) utilization costs were compared.In low burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 8 patients had SEMLS performed by 2 surgeons. In high burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 12 patients had SEMLS performed by 2 surgeons. For high burden SEMLS, operative time was decreased by a mean of 69 minutes in cases performed by 2 co-surgeons (P = 0.03). Decreased operative time was associated with an estimated savings of $2484 per SEMLS case. In low burden SEMLS, a trend toward decreased operative time was associated for cases performed by 2 co-surgeons (182 vs 221 minutes, P = 0.11). Decreased operative time was associated with an estimated savings of $1404 per low burden SEMLS case. No difference was found for estimated blood loss or hospital length of stay between groups in high and low burden SEMLS.Employing 2 attending surgeons in SEMLS decreased operative time and OR utilization cost, particularly in patients with a high surgical burden. These findings support the practice of utilizing 2 attending surgeons for SEMLS in patients with CP.Level of Evidence: Level III.
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http://dx.doi.org/10.1097/MD.0000000000026294DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8213317PMC
June 2021

Risk Factors for Failure of Pavlik Harness Treatment in Infants With Dislocated Hips That Are Evaluated by Dynamic Sonography.

J Pediatr Orthop 2021 Jul;41(6):e386-e391

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Frankly dislocated hips occur in ∼1% to 3% of infants with developmental dysplasia of the hip and are often difficult to treat. In the most severely dislocated hips, the femoral head is positioned outside the posterior/lateral rim of the acetabulum and is irreducible, that is, the femoral head will not reduce by positioning the leg. The purpose of this study was to determine risk factors, using univariate and multivariate analyses, for Pavlik harness failure in infants who initially presented with irreducible/dislocated hips (confirmed by dynamic sonography).

Methods: Following institutional review board approval, 124 infants (170 hips) with frankly dislocated hips treated using a Pavlik harness between 2000 and 2018 were evaluated. Patients' demographic characteristics, clinical findings, dynamic sonographic findings (dislocated-fixed vs. dislocated-mobile), age at onset of Pavlik harness treatment, duration of harness usage, and follow-up treatments were recorded. Univariate analyses were used to determine risk factors for treatment failure.

Results: In frankly dislocated hips (confirmed by dynamic sonography to be positioned outside the posterior/lateral rim of the acetabulum), Pavlik harness treatment was successful in 104 of 170 hips (61%) while it failed in 66 hips. Mean follow-up was 4.86±4.20 years. Univariate analysis determined the risk factors to be onset of treatment after the seventh week of age (P=0.049) and initial mobility (dislocated-fixed group) (P<0.001) by dynamic sonography. In addition, multivariate analysis (P=0.007) showed infants of multigravida mothers (non-firstborn) to be another risk factor for failure. Six percent of hips with no risk factors failed Pavlik harness treatment, those with 1 risk factor had 42% failure, 2 risk factors had 69% failure, and all 3 risk factors had 100% failure.

Conclusions: In our patients with frankly dislocated irreducible hips, 39% of hip failed Pavlik harness treatment. Independent multivariate, logistic regression analysis, and multivariate analysis determining the risk factors for failure of Pavlik harness treatment were onset of treatment after the seventh week of age, infants of multigravida mothers, and initial hip mobility (fixed-dislocated hips) by dynamic sonography.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000001799DOI Listing
July 2021

Orthopaedic Manifestations of Transverse Myelitis in Children.

J Pediatr Orthop 2021 May 13. Epub 2021 May 13.

Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE Seton Hall Orthopaedics, St. Joseph's University Medical Center, Paterson, NJ Icahn School of Medicine at Mount Sinai, New York, NY Department of Orthopaedic Surgery, Nemours Children's Specialty Care, Jacksonville, FL.

Background: Transverse myelitis (TM) is a rare inflammatory disorder of the spinal cord. It can have a heterogeneous presentation with sensory, motor, and autonomic dysfunction. Neurological sequelae of TM include autonomic dysfunction, motor weakness, and/or spasticity. Studies describing orthopaedic deformities and treatments associated with TM are nonexistent. This purpose of this study was to describe the orthopaedic manifestations of TM in children.

Methods: A multicenter retrospective review was conducted of patients, 0 to 21 years of age, with TM presenting over a 15-year period at 4 academic children's hospitals. Those with confirmed diagnosis of TM and referred to an orthopaedic surgeon were included. Demographics, orthopaedic manifestations, operative/nonoperative treatments, and complications were recorded. Descriptive statistics were used for data reporting.

Results: Of 119 patients identified with TM, 37 saw an orthopaedic surgeon. By etiology, 23 were idiopathic (62%), 10 infectious (27%), 3 (8%) inflammatory/autoimmune, and 1 (3%) vascular. The mean age at diagnosis was 6.7 (SD: 5.5) years and at orthopaedic presentation was 8.4 (SD: 5.2) years. Orthopaedic manifestations included scoliosis in 13 (35%), gait abnormalities in 7 (19%), foot deformities in 7 (19%), upper extremity issues in 7 (19%), symptomatic spasticity in 6 (16%), lower extremity muscle contractures in 6 (16%), fractures in 6 (16%), hip displacement in 3 (8%), pain in 2 (5%), and limb length discrepancy in 2 (5%) patients. Seven children (19%) were seen for establishment of care. In all, 14 (38%) underwent operative intervention, mainly for soft-tissue and scoliosis management. Four patients had baclofen pump placement for spasticity management. Postoperative complications occurred in 36% of cases, most commonly because of infection. Neither topographic pattern nor location of lesion had a significant relationship with need for hip or spine surgery.

Conclusions: This report describes the orthopaedic manifestations associated with TM in children, nearly 40% of whom required operative intervention(s). Understanding the breadth of musculoskeletal burden incurred in TM can help develop surveillance programs to identify and treat these deformities in a timely manner.

Level Of Evidence: Level IV.
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http://dx.doi.org/10.1097/BPO.0000000000001845DOI Listing
May 2021

Intraoperative Neurological Monitoring in Lower Limb Surgery for Patients With Mucopolysaccharidoses.

J Pediatr Orthop 2021 Mar;41(3):182-189

Gillette Children's Specialty Healthcare, Saint Paul.

Background: There are reports of spinal cord injury (SCI) occurring after lower extremity (LE) surgery in children with mucopolysaccharidoses (MPS). Intraoperative neurological monitoring (IONM) has been adopted in some centers to assess real-time spinal cord function during these procedures. The aim of this investigation was to review 3 specialty centers' experiences with MPS patients undergoing LE surgery. We report how IONM affected care and the details of spinal cord injuries in these patients.

Methods: All pediatric MPS patients who underwent LE surgery between 2001 and 2018 were reviewed at 3 children's orthopaedic specialty centers. Demographic and surgical details were reviewed. Estimated blood loss (EBL), surgical time, positioning, use of IONM, and changes in management as a result of IONM were recorded. Details of any spinal cord injuries were examined in detail.

Results: During the study period, 92 patients with MPS underwent 252 LE surgeries. IONM was used in 83 of 252 (32.9%) surgeries, and intraoperative care was altered in 17 of 83 (20.5%) cases, including serial repositioning (n=7), aggressive blood pressure management (n=6), and abortion of procedures (n=8). IONM was utilized in cases with larger EBL (279 vs. 130 mL) and longer operative time (274 vs. 175 min) compared with procedures without IONM. Three patients without IONM sustained complete thoracic SCI postoperatively, all from cord infarction in the upper thoracic region. These 3 cases were characterized by long surgical time (328±41 min) and substantial EBL (533±416 mL or 30.5% of total blood volume; range, 11% to 50%). No LE surgeries accompanied by IONM experienced SCI.

Conclusions: Patients with MPS undergoing LE orthopaedic surgery may be at risk for SCI, particularly if the procedures are long or are expected to have large EBL. One hypothesis for the etiology of SCI in this setting is hypoperfusion of the upper thoracic spinal cord due to prolonged intraoperative or postoperative hypotension. IONM during these procedures may mitigate the risk of SCI by identifying real-time changes in spinal cord function during surgery, inciting a change in the surgical plan.

Level Of Evidence: Level III-retrospective comparative series.
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http://dx.doi.org/10.1097/BPO.0000000000001720DOI Listing
March 2021

Cervical kyphosis: A predominant feature of patients with osteogenesis imperfecta type 5.

Bone Rep 2020 Dec 21;13:100735. Epub 2020 Nov 21.

Division of Orthogenetics, Department of Pediatrics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Objectives: Osteogenesis imperfecta (OI) is a heterogeneous group of genetic disorders of connective tissue that cause skeletal fragility and extra-skeletal manifestations. Classically, four different types of OI were distinguished. Type 5 OI was added due to its distinct clinical and radiographic features. In 2012, two independent groups identified a recurrent heterozygous c.-14C>T mutation in as the responsible genetic change for this type of OI. To our knowledge, cervical kyphosis has not been identified in the literature as a finding in type 5 OI patients. This is a retrospective review of a cohort of patients with type 5 OI and a description of associated cervical spine deformity.

Methods: After institutional review board approval, a retrospective review identified 13 patients with type 5 OI. Clinical, radiologic, and genetic data from 2002 to 2020 were reviewed.

Results: We identified 13 patients with clinical diagnosis of type 5 OI. Twelve had molecular confirmation and the classic , c.14C>T gene mutation was identified. The remaining individual did not undergo genetic testing. Dentinogenesis imperfecta was observed in one patient, while blue sclerae or hearing loss were not present. All patients had at least one fracture and four underwent intramedullary rodding. Radiologic features included subphyseal metaphyseal radiodense line in 12/13 patients (92%), interosseous membrane calcification in seven of 13 patients (54%) (more commonly noted in the upper extremities), and hypertrophic callus in six of 13 patients (46%). Thoracolumbar spinal deformities were seen in six of 13 patients (46%) with two of these individuals requiring surgery. Cervical kyphosis was noted in nine of 13 individuals (69%) ranging in age from 3 months to 22 years. Anterior wedging of the cervical vertebral bodies was noted in the absence of any fractures. Six of nine individuals demonstrated listhesis of C2-C3 or C3-C4 segment. Magnetic resonance imaging studies were performed and reviewed in patients with cervical kyphosis and subluxation; three patients showed narrowing of spinal canal without cervical cord compression and one asymptomatic patient showed impingement of the spinal cord.

Conclusions: Cervical kyphosis appears to be a common feature of type 5 OI. It can be a presenting and apparently life-long association and does not appear to be caused by vertebral body fractures. Evaluation for cervical kyphosis should be performed in patients with a suspected or confirmed diagnosis of type 5 OI. Furthermore, if cervical kyphosis is noted in an individual with OI, type 5 OI should be considered.Level of evidence: IV.
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http://dx.doi.org/10.1016/j.bonr.2020.100735DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7718132PMC
December 2020

Evaluation of Risk Factors for Cerebrospinal Leakage in Pediatric Patients With Cerebral Palsy Treated With Intrathecal Baclofen.

J Pediatr Orthop 2020 Jul;40(6):e522-e526

Department of Orthopaedic Surgery, Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Insertion of an intrathecal baclofen (ITB) pump can provide significant benefits in patients with cerebral palsy (CP). However, there are little data describing the risk of complications. Specifically, there is a lack of data describing the incidence of cerebrospinal fluid (CSF) leakage and risk factors following ITB placement. The purpose of our study was to describe risk factors for developing CSF leak in pediatric patients with CP treated with ITB and to report the treatment and outcome of CSF leaks.

Methods: Following institutional review board approval, 720 ITB procedures in 341 children with CP were identified retrospectively over a 15-year study period. Patients' demographic characteristics, medical comorbidities, muscle tone patterns, feeding tube status, seizure history, inpatient events, ITB-related CSF leak and headache complaints and their management, and other complications were evaluated.

Results: Eighty-five (24.9%) patients experienced 90 CSF leak episodes over a follow-up time of 6.3±3.9 years. There were 72 episodes of headache as a result of CSF leakage in 61 (71.7%) of these 85 patients. There was a positive correlation between the risk of CSF leak and preoperative comorbidities such as epilepsy/seizure history, feeding tube, mixed type CP, and dystonic type CP. The risk of CSF leak after primary ITB administration was 5.8% (20/341), and the risk after secondary ITB procedures due to complications was 24.2% (32/132). There was no significant relationship between CSF leak and primary ITB (P=0.21), but the risk of CSF leak was positively correlated to the secondary ITB due to complications (P<0.05).

Conclusions: CSF leak was fairly common (25% incidence), and it correlated with epilepsy/seizure history, feeding tube, mixed type CP, and dystonic type CP. Recurrent ITB procedures were a risk factor for CSF leak. Half of these patients had self-limited symptoms that improved with conservative medical treatment, and the epidural blood patch was successful in resistant cases. Successful treatment of CSF leakage complications allows patients to continue ITB.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000001472DOI Listing
July 2020

Sprengel Deformity in Biological Sisters.

J Am Acad Orthop Surg Glob Res Rev 2020 04 2;4(4). Epub 2020 Apr 2.

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Sprengel deformity is the main congenital shoulder deformity seen in children. First described by Otto Sprengel in 1891, it consists of a high-riding, dysplastic, and malrotated scapula leading to functional and cosmetic impairments. It has never been reported in siblings.

Case Presentation: Two sisters, 8 and 9 years old, presented for an evaluation of atraumatic limitation in the shoulder range of motion and neck webbing with an unknown family history. Physical examination revealed a small high-riding scapula, webbed neck, and painless limitation in shoulder abduction (<70°) and flexion (<80°). The 9-year-old sibling had a bilateral shoulder involvement, and the younger had unilateral. Imaging revealed bony and fibrous omovertebral connections between the dysplastic scapulas and cervical spine along with Klippel-Feil deformities. Both sisters underwent scapula repositioning via a modified Woodward procedure. The omovertebral connection was resected followed by scapula derotation and inferior migration. Both had a dramatic improvement in cosmesis and near-complete restoration of shoulder function at follow-up.

Conclusions: Although uncommon, Sprengel deformity results in notable derangement of shoulder function. If untreated, children experience difficulty with most overhead activities and often have cosmetic reports. Although no previous genetic link has been identified, its presence in biological sisters suggests that more research is needed.
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http://dx.doi.org/10.5435/JAAOSGlobal-D-19-00120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188265PMC
April 2020

Evaluation of Fracture and Osteotomy Union in the Setting of Osteogenesis Imperfecta: Reliability of the Modified Radiographic Union Score for Tibial Fractures (RUST).

J Pediatr Orthop 2020 Jan;40(1):48-52

Department of Orthopaedics.

Background: Evaluation of the union of osteotomies and fractures in patients with osteogenesis imperfecta (OI) is a critical component of patient care. Studies of the OI patient population have so far used varied criteria to evaluate bony union. The radiographic union score for tibial fractures (RUST), which was subsequently revised to the modified RUST, is an objective standardized method of evaluating fracture healing. We sought to evaluate the reliability of the modified RUST in the setting of the tibias of patients with OI.

Methods: Tibial radiographs of 30 patients with OI fractures, or osteotomies were scored by 3 observers on 2 separate occasions. Each of the 4 cortices was given a score (1=no callus, 2=callus present, 3=bridging callus, and 4=remodeled, fracture not visible) and the modified RUST is the sum of these scores (range, 4 to 16). The interobserver and intraobserver reliabilities were evaluated using intraclass coefficients (ICC) with 95% confidence intervals.

Results: The ICC representing the interobserver reliability for the first iteration of scores was 0.926 (0.864 to 0.962) and for the second series was 0.915 (0.845 to 0.957). The ICCs representing the intraobserver reliability for each of the 3 reviewers for the measurements in series 1 and 2 were 0.860 (0.707 to 0.934), 0.994 (0.986 to 0.997), and 0.974 (0.946 to 0.988).

Conclusions: The modified RUST has excellent interobserver and intraobserver reliability in the setting of OI despite challenges related to the poor quality of the bone and its dysplastic nature. The application and routine use of the modified RUST in the OI population will help standardize our evaluation of osteotomy and fracture healing.

Level Of Evidence: Level III-retrospective study of nonconsecutive patients.
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http://dx.doi.org/10.1097/BPO.0000000000001068DOI Listing
January 2020

Cervical Spine Deformities in Children With Rhizomelic Chondrodysplasia Punctata.

J Pediatr Orthop 2019 Oct;39(9):e680-e686

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Cervical spine deformity in rhizomelic chondrodysplasia punctata (RCDP) has been described with different findings reported in the literature. However, available literature provides limited data from a few cases with magnetic resonance imaging (MRI) of the cervical spine. Our report describes the MRI findings in a group of children with RCDP, aiming to reach a better understanding of this pathology.

Methods: An Institutional Review Board-approved RCDP Registry was created at our institution with the goal of identifying pertinent medical issues over the lifespan of individuals with RCDP. Records of children within the registry were evaluated, and magnetic resonance images obtained between 2004 and 2015, were available for review. The levels of spinal canal stenosis were recorded and the severity of the stenosis was decided based on adults' parameters. Cord compression and myelomalacia were confirmed on the axial images. Sagittal lumbar spine magnetic resonance images were also evaluated when available, and the presence of tethered cord and fatty filum was recorded.

Results: Twenty-six children (15 boys and 11 girls) were identified in the RCDP Registry. Eleven children (6 boys and 5 girls) had sagittal MRI of the cervical spine available for review. Age at the time of MRI study was variable (1 wk to 32 mo). All patients except 1 had stenosis of the cervical spinal canal. Myelomalacia of the cord was noted only in this patient.

Conclusions: This study suggests that, in children with RCDP, cervical spinal stenosis and cord compression are a real risk, and children with this diagnosis should have monitoring for these issues. Tethered cord is also a possible finding that needs to be evaluated. Full sagittal spine MRI is necessary to detect the possible deformities at the cervical and lumbar levels.
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http://dx.doi.org/10.1097/BPO.0000000000001014DOI Listing
October 2019

Developmental dysplasia of the hip and clubfoot treated by Pavlik and Ponseti methods.

J Pediatr Orthop B 2019 Sep;28(5):446-451

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

Children having both typical developmental dysplasia of the hip (DDH) and clubfoot are rare, and early treatments of both conditions are recommended. The aim of this study was to evaluate the effects of the Ponseti method of clubfoot treatment on hips with DDH. After institutional review board approval, we identified children treated by the Ponseti and Pavlik methods between 2003 and 2016. During the Ponseti method treatment, the duration of manipulations, number of casts, tenotomies performed, and days in clubfoot orthosis were recorded. During DDH treatment, we registered duration for Pavlik and hip brace usage. Hips had dynamic sonography and radiographic evaluations. The cases were grouped according to the combination of DDH and clubfoot treatments: (a) concurrent, (b) sequential, and (c) hip observation. Seven cases of DDH and clubfoot were identified. The average number of Ponseti casts was 5.8 (range: 4-8 casts). The average number of days following the post-Achilles-tenotomy casting to the end of clubfoot bracing was 870 days (range: 90-1605 days). Eleven (four bilateral cases and three unilateral) clubfeet were corrected initially by the Ponseti method. The average number of days for Pavlik harness treatment was 74 (range: 10-126 days). Additionally, a hip orthosis was utilized in three children for an average of 131 days. At follow-up, all children had a high femoral neck-shaft angle averaging 152° (range: 144°-164°). One child (case 5) developed avascular necrosis of the femoral head, Kalamchi type I. Children with typical and nonsyndromic DDH and clubfoot treated by Pavlik harness and Ponseti methods are associated with abnormal hip development (coxa valga).
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http://dx.doi.org/10.1097/BPB.0000000000000618DOI Listing
September 2019

Valgus Hip Osteotomy in Children With Spondyloepiphyseal Dysplasia Congenita: Midterm Results.

J Pediatr Orthop 2019 Jul;39(6):282-288

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.

Background: Coxa vara has been frequently reported in spondyloepiphyseal dysplasia congenita (SEDC), and proximal femoral osteotomy has been described as a useful treatment. The aim of this study was to discuss the clinical, radiographic, and gait outcomes after valgus extension osteotomy of the proximal femur. Changes of lumbar lordosis, associated with coxa vara correction, are reported as well as the outcome differences between different ages.

Methods: Records of children with SEDC, who were followed at our institution between 2004 and 2014, were reviewed; and children had hip surgery were identified. Hip pain and passive range of motion, radiographic neck shaft angle (NSA), and Hilgenreiner trochanteric (H-T) angle, sagittal spinopelvic parameters, and gait data were recorded. Preoperative and last follow-up data were compared. Outcomes were also compared between 3 age groups.

Results: Of the 79 children with SEDC, 26 children (12 boys and 14 girls) had hip osteotomy in 48 hips. Mean age at surgery was 9.6 years and the mean follow-up was 5 years. Preoperative hip pain was noted in 30 hips. At the last follow-up, 3 hips were painful at the extreme range of motion. Passive range of motion, NSA, and H-T improved postoperatively. Although NSA was maintained over the follow-up, H-T deterioration was noted. Spinopelvic measurements changed significantly and gait data remained stable except pelvic tilt that reduced significantly after surgery. The changes of radiographic measurements in each age group were similar to the total group of patients.

Conclusions: In children with SEDC, surgical correction of coxa vara, by proximal femoral valgus osteotomy, is an effective treatment that improves hip pain and range of motion in addition to the radiographic alignment of the proximal femur and the sagittal spinopelvic alignment. Children are expected to maintain their level of function after surgery and to have good results over the midterm regardless of their age at surgery.

Level Of Evidence: Level IV-therapeutic study.
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July 2019

Altered brain tissue viscoelasticity in pediatric cerebral palsy measured by magnetic resonance elastography.

Neuroimage Clin 2019 7;22:101750. Epub 2019 Mar 7.

Department of Biomedical Engineering, University of Delaware, Newark, DE, United States; Department of Psychological and Brain Sciences, University of Delaware, Newark, DE, United States. Electronic address:

Cerebral palsy (CP) is a neurodevelopmental disorder that results in functional motor impairment and disability in children. CP is characterized by neural injury though many children do not exhibit brain lesions or damage. Advanced structural MRI measures may be more sensitively related to clinical outcomes in this population. Magnetic resonance elastography (MRE) measures the viscoelastic mechanical properties of brain tissue, which vary extensively between normal and disease states, and we hypothesized that the viscoelasticity of brain tissue is reduced in children with CP. Using a global region-of-interest-based analysis, we found that the stiffness of the cerebral gray matter in children with CP is significantly lower than in typically developing (TD) children, while the damping ratio of gray matter is significantly higher in CP. A voxel-wise analysis confirmed this finding, and additionally found stiffness and damping ratio differences between groups in regions of white matter. These results indicate that there is a difference in brain tissue health in children with CP that is quantifiable through stiffness and damping ratio measured with MRE. Understanding brain tissue mechanics in the pediatric CP population may aid in the diagnosis and evaluation of CP.
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http://dx.doi.org/10.1016/j.nicl.2019.101750DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416970PMC
January 2020

Categorization of the Usage of Adjunctive Structural Allograft Bone Graft in Extremity Surgery for Patients with Osteogenesis Imperfecta.

J Long Term Eff Med Implants 2018 ;28(3):205-208

Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Structural allograft bone plays a role in orthopedic surgery. Our purpose is to describe the methods of using structural allograft in extremity reconstruction surgery in patients with osteogenesis imperfecta (OI) and create a classification of usage with a single-center review of OI extremity cases from January 2002 to February 2017. Structural allograft was used in 19 bone segments in 15 patients with type III OI. Four categories of usage were defined: (1) interpositional, (2) onlay, (3) inlay, and (4) blocking to prevent rod migration. The mean time to incorporation was 5.4 months (range 1-16). Structural allograft bone incorporates into OI bone and may be a supplement to intramedullary fixation.
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http://dx.doi.org/10.1615/JLongTermEffMedImplants.2018028895DOI Listing
November 2019

Prevalence of mental health conditions and pain in adults with skeletal dysplasia.

Qual Life Res 2019 Jun 14;28(6):1457-1464. Epub 2019 Jan 14.

Division of Orthogenetics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Purpose: We sought to examine the prevalence of depression and anxiety in adults with skeletal dysplasias, and to assess any correlations with pain.

Methods: Participation was via an anonymous REDCap survey, which consisted of sociodemographic questions followed by the brief pain inventory-short form (BPI-SF), patient health questionnaire-8 (PHQ-8), and generalized anxiety disorder-7 (GAD-7) questionnaires. These assessed pain, depression, and anxiety respectively.

Results: Of the 336 usable responses, 16.1% scored 10 or greater on the PHQ-8 consistent with current depression while 17.3% scored 10 or greater on the GAD-7 consistent with current anxiety. The majority of participants (76.2%) experienced pain, which was significantly associated with prior mental health diagnoses (p < 0.05). A total of 34% reported either a prior diagnosis of depression or scored 10 or greater on the PHQ-8, and 31% reported either a prior diagnosis of anxiety or scored 10 or greater on the GAD-7.

Conclusions: This study identified a substantial percentage of individuals with mental health concerns as well as pain in the adult skeletal dysplasia population. Further research is warranted to investigate barriers to service or treatment of mental health disorders as well as pain management.
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http://dx.doi.org/10.1007/s11136-019-02102-2DOI Listing
June 2019

Sagittal Spinopelvic Parameters in Children With Achondroplasia.

Spine Deform 2019 01;7(1):163-170

Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, PO Box 269, Wilmington, DE 19899, USA. Electronic address:

Study Design: Retrospective cross-sectional, longitudinal radiographic analysis.

Objective: To report the natural history of spinopelvic parameters in achondroplasia.

Summary Of Background Data: Sagittal spinal deformity is common in children with achondroplasia. However, few data exist on their normative spinal parameters.

Methods: Lateral standing spine radiographs of children with achondroplasia were reviewed. Measurements included thoracic kyphosis, lumbar lordosis (LL), thoracolumbar kyphosis (TLK), pelvic incidence (PI), T1 pelvic angle (TPA), and sagittal balance (SVA). Comparison between age groups and longitudinal analysis of children with minimum five-year radiographic evaluation was performed; evolution of radiographic measurements was assessed. Children who underwent surgical correction of TLK were studied separately to describe changes of sagittal spinal parameters associated with TLK surgical correction.

Results: In cross-sectional analysis, 745 radiographs (282 children) were measured. During the first three years, TLK decreased and LL and sacral slope increased significantly. After age 3 years, TLK decreased gradually until age 10. Afterwards, TLK decrease became non-significant. PI increased gradually after age 10. In the longitudinal group, 81 children were followed an average of 8.7 (5-19) years between age 4.4 and 13.1 years. TLK decreased; LL and PI increased significantly. TPA and SVA remained within the normal range although changes with growth were statistically significant. In the surgical group, 19 children underwent surgical TLK correction. Apart from TLK correction, no sagittal parameters changed significantly after surgery. These 19 children had higher TLK and lower LL compared with a nonsurgical group at similar average age.

Conclusion: In children with achondroplasia, TLK improvement occurs primarily before age 3 years; hyperlordosis at the lumbosacral level is the compensatory mechanism. Significant changes in the sagittal spinal parameters occur early in life, suggesting the importance of attention to sagittal malalignment to prevent any possible clinical sequelae of severe hyperlordosis.

Level Of Evidence: Level III, prognostic study.
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http://dx.doi.org/10.1016/j.jspd.2018.06.001DOI Listing
January 2019

Cryptorchidism in Boys With Cerebral Palsy Is Associated With the Severity of Disease and With Co-Occurrence of Other Congenital Anomalies.

Front Endocrinol (Lausanne) 2018 16;9:151. Epub 2018 Apr 16.

Nemours Biomedical Research/Division of Urology, Department of Orthopedics and Biostatistics Core, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.

Background: Cryptorchidism is reported in 40-50% of small case series of cerebral palsy (CP) and attributed to hypothalamic-pituitary-gonadal axis abnormalities, intellectual disability (ID), or cremaster spasticity. We collected demographic and clinical data to define the frequency of cryptorchidism and clinical comorbidities in a large CP population.

Methods: Electronic health record data were collected for all male patients ≥7 years of age seen in a large, multidisciplinary CP clinic between 2000 and 2016. Variables including age, testicular position, surgical findings, CP severity, birth history, and comorbidities were tested for association using univariable and stepwise backward logistic regression analyses.

Results: Of 839 established patients, testis position was scrotal in 553, undescended in 185 (24%), retractile in 38 (5%), and undocumented in 63 cases. Cryptorchidism were diagnosed at a mean age of 5.8 years, with 20% documented as acquired, and testes were most commonly in the superficial inguinal pouch (41%) and associated with an inguinal hernia (56%). Severity was bilateral in 114/166 (69%) undescended and 24/36 (66%) retractile cases, respectively. Mean birth weight and the frequency of prematurity (55, 58, and 54%) and multiple birth (14, 13, and 9%) were not significantly different among the three groups. We observed a strong ordinal trend in the frequency of comorbidities, including quadriplegia, syndromic features/known genetic disease, intrauterine growth restriction (IUGR), death, brain malformations, seizures, gastrostomy, absent continence, ID and hearing, speech or visual impairment, with the retractile group holding the intermediate position for the majority. The stepwise multivariable analysis showed independent positive associations of cryptorchidism with quadriplegia, syndromic features/known genetic disease, hearing loss, and absent continence, and inverse associations with gestational age and multiple birth.

Conclusion: These data suggest that cryptorchidism is less common than previously reported in CP cases, but most strongly associated with quadriplegia. Delayed diagnosis may be related to an acquired condition or to the multiple additional functional deficits that occur in this population. Our data suggest that UDT and CP may both be components of malformation syndromes occurring in singleton births whose clinical features are more likely to include earlier delivery, IUGR, hearing loss, and/or global spasticity.
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http://dx.doi.org/10.3389/fendo.2018.00151DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5911456PMC
April 2018

Paper #40: Is There an Improvement in Quality of Life with Early Onset Scoliosis Managed with Traditional Growing Rods Converted to Magnetically Controlled Growing Rods?

Spine Deform 2017 Nov;5(6):460-461

Introduction of magnetically controlled growing rods (MCGR) for early onset scoliosis treatment was anticipated to improve quality of life for patients and their families. A cohort of patients converted from traditional growing rods to MCGR may be best suited to detect this improvement and have not previously been examined. Using the validated EOSQ-24, no HRQoL differences were detected between TGR, MCGR, or converted patient cohorts.
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http://dx.doi.org/10.1016/j.jspd.2017.09.043DOI Listing
November 2017

Infections of Intrathecal Baclofen Delivery Systems and Ventriculoperitoneal Shunting Systems: Clinical Series Discussion.

Pediatr Neurosurg 2018 2;53(1):1-6. Epub 2017 Sep 2.

Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

Background/aims: The physiological interaction between the intrathecal baclofen (ITB) delivery system and the ventriculoperitoneal (VP) shunting system in a patient who had both systems implanted has not been reported previously. The aim of our report is to evaluate the effect that one system's infection might have on the other.

Methods: Records of children who were followed at our institution between 2004 and 2015 for management of their ITB systems were reviewed. In this group, children who had VP shunts were identified, and those who had any of their ITB or VP systems infected were included.

Results: Out of 313 children managed with ITB therapy at our institution, 31 (24%) children had VP shunts. Two patients had infection in both systems, and 3 patients had infection in 1 system.

Conclusion: This report suggests that if aspiration from both systems showed positive cultures, the treatment would be removal of both systems. If the primarily not infected system does not show positive cultures, it does not need to be removed. Close follow-up is recommended, and any sign of infection or malfunction of the primarily not infected device should be approached with a high level of suspicion.
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http://dx.doi.org/10.1159/000475468DOI Listing
July 2018
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