Publications by authors named "Paul D Sponseller"

304 Publications

Surgical Evaluation and Management of Spinal Pathology in Patients with Connective Tissue Disorders.

Neurosurg Clin N Am 2022 Jan;33(1):49-59

Pediatric Orthopaedics, Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA. Electronic address:

Connective tissue disorders represent a varied spectrum of syndromes that have important implications for the spine deformity surgeon. Spine surgeons must be aware of these diverse and global manifestations of disease because they have significant impact on perioperative and postoperative outcomes.
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http://dx.doi.org/10.1016/j.nec.2021.09.005DOI Listing
January 2022

The Effect of Surgeon Experience on Outcomes Following Growth Friendly Instrumentation for Early Onset Scoliosis.

J Pediatr Orthop 2021 Nov 11. Epub 2021 Nov 11.

Children's Hospital New Orleans School of Public Health, LSU Health Sciences Center, New Orleans, LA Department of Orthopaedics, University Hospitals Cleveland Medical Center Division of Orthopaedic Surgery, Cincinnati Children's Hospital, Cincinnati, OH Children's Hospital Colorado Orthopaedics Institute, Aurora, CO Shriner's for Children Medical Center, Pasadena, CA Children's of Mississippi, University of Mississippi Medical Center, Jackson, MS Division of Pediatric Orthopaedics, All Children's Hospital at Johns Hopkins, Baltimore, MD Department of Orthopaedic Surgery, Columbia University Medical Center, New York, NY Department of Orthopaedic Surgery, Boston Children's Hospital, Boston, MA.

Background: The purpose of this study was to utilize a multicenter, multisurgeon cohort to assess the effect of surgeon experience on outcomes of growth friendly instrumentation (GFI) in early onset scoliosis (EOS). We hypothesized that unplanned return to the operating room (UPROR), estimated blood loss (EBL), and surgical time would be greater amongst early career surgeons (ECSs) when compared with advanced career surgeons (ACSs).

Methods: An international pediatric spine database was queried for patients ages 2 to 10 years treated by posterior distraction-based GFI with at least of 2-year follow up. Two groups were created for analysis based on surgeon experience: ECSs (with ≤10 y of experience) and ACSs (with >10 y of experience). The primary outcome was UPROR. Additional outcomes included: operating room time, EBL, neurological deficits, infection rate, hardware failure, and the Early Onset Scoliosis Questionnaire (EOSQ-24). Subgroup analysis was performed for further assessment based on procedure type, superior anchor type, etiology, and curve severity.

Results: A total of 960 patients met inclusion criteria including 243 (25.3%) treated by ECS. Etiology, sex, superior anchor, and EOSQ-24 scores were similar between groups (P>0.05). There were no clinically significant differences in patient age or preoperative major coronal curve. UPROR (35.8% vs. 32.7%, P=0.532), infection (17.0% vs. 15.6%, P=0.698), operating room time (235 vs. 231 min, P=0.755), and EBL (151 vs. 155 mL, P=0.833) were comparable between ECS and ACS groups. The frequency of having at least 1 complication was relatively high but comparable among groups (60.7% vs. 62.6%, P=0.709). EOSQ-24 subdomain scores were similar between groups at 2-year follow-up (P>0.05). Subgroup analysis revealed that ECS had increased surgical time compared with ACS in severe curves >90 degrees (270 vs. 229 min, P=0.05).

Conclusions: This study represents the first multicenter assessment of surgeon experience on outcomes in EOS. Overall, surgeon experience did not significantly influence UPROR, complication rates, EBL, or surgical time associated with GFI in this cohort of EOS patients.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000002000DOI Listing
November 2021

Treatment of Early-onset Scoliosis: Similar Outcomes Despite Different Etiologic Subtypes in Traditional Growing Rod Graduates.

J Pediatr Orthop 2021 Nov 4. Epub 2021 Nov 4.

San Diego Spine Foundation, San Diego Department of Orthopaedic Surgery, University of California San Diego School of Medicine, La Jolla Children's Orthopedic Center, Children's Hospital Los Angeles, Los Angeles, CA Birmingham Children's Hospital, Birmingham, UK Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE Division of Spine Surgery, Department of Orthopedic Surgery, Harvard Medical School, Boston, MA Department of Orthopaedic Surgery, Division of Pediatric Orthopaedic Surgery, Rainbow Babies and Children's Hospital, Cleveland, OH Department of Orthopaedic Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD.

Background: It is unclear whether traditional growing rod (TGR) treatment outcomes vary by early-onset scoliosis (EOS) subtype. The goal of this study was to compare radiographic outcomes and complications of TGR treatment by EOS subtype.

Methods: We queried an international database of EOS patients from 20 centers to identify "graduates" who had (1) undergone primary TGR treatment from 1993 to 2014; (2) completed TGR treatment; and (3) had an uneventful clinical examination within 6 months after completion of TGR treatment with no anticipated further intervention. We included 202 patients in 4 etiologic subgroups: neuromuscular (n=65), syndromic (n=57), idiopathic (n=52), and congenital (n=28). Mean age at surgery was 7.1 years (range, 1.6 to 14.9 y); mean duration of follow-up was 8 years (range, 2 to 18.6 y). The groups did not differ by mean age, body mass index, sex, number of lengthenings, or duration of follow-up. The following preoperative differences were significant: (1) greater mean major curve in the neuromuscular versus idiopathic subgroup; (2) shorter spinal height (T1-S1) in the congenital versus idiopathic subgroup; and (3) smaller proportion of ambulatory patients in the neuromuscular subgroup versus all other subgroups.

Results: We found no significant differences among subgroups in mean major curve correction or changes in thoracic height (T1-T12), spinal height, or global kyphosis at any point. Rates of deep surgical site infection, implant-related complications, and neurological complications were not different among subgroups. The medical complication rate was significantly lower in the idiopathic group compared with the other groups.

Conclusions: Major curve correction and spinal and thoracic height increases did not differ significantly at any point by EOS subtype. Rates of deep surgical site infection, implant-related complications, and neurological complications did not differ by subtype. Except for the lower rate of medical complications in the idiopathic group, our findings suggest that, after TGR treatment, patients can expect similar outcomes regardless of their EOS subtype.

Level Of Evidence: Level III, therapeutic.
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http://dx.doi.org/10.1097/BPO.0000000000001985DOI Listing
November 2021

Spinal Fusion with Sacral Alar Iliac Pelvic Fixation in Severe Neuromuscular Scoliosis.

JBJS Essent Surg Tech 2021 Jul-Sep;11(3). Epub 2021 Aug 16.

Department of Pediatric Orthopaedics, Johns Hopkins University, Baltimore, Maryland.

Neuromuscular scoliosis is characterized by rapid progression of curvature during growth and may continue to progress following skeletal maturity. Posterior spinal fusion in patients with cerebral palsy and severe scoliosis results in substantial improvements in health-related quality of life. Correction of pelvic obliquity can greatly improve sitting balance, reduce pain, and decrease skin breakdown. The sacral alar iliac (SAI) technique has key advantages over prior techniques, including the Galveston and iliac-screw techniques. The SAI technique eliminates the need for subcutaneous muscle dissection over the iliac crest, does not require the use of connectors from the rod to the iliac screw, and decreases the risk of implant prominence.

Description: We demonstrate how to perform posterior spinal fusion with SAI pelvic fixation in a patient with cerebral palsy. In correcting the scoliosis, we utilize the segmental 3-dimensional technique, which includes compression, distraction, transverse approximation to 1 rod at a time, and derotation around 2 rods. We also demonstrate SAI pelvic fixation with identification of the screw starting point on the lateral-caudal border of the first sacral foramen and trajectory toward the anterior inferior iliac spine.

Alternatives: Nonoperative alternatives include bracing, trunk support, contouring of sitting surfaces (such as wheelchairs), and physical therapy to slow curve progression during growth periods and delay the need for surgical treatment. Decision-making is shared with the family following education about the risks and benefits. Families who are satisfied with the function of the child at baseline should not be persuaded into pursuing surgical treatment.

Rationale: Neuromuscular scoliosis can include difficulty sitting secondary to increased pelvic obliquity, along with poor trunk control and balance. Surgical intervention is considered in patients with curves exceeding approximately 50°, as these curves will often continue to progress even after maturity. In patients with neuromuscular scoliosis, indications for pelvic fixation include pelvic obliquity of >15°, poor control of the trunk as indicated by lack of independent sitting or standing, and location of the apex of the curve in the lumbar spine. SAI screws are utilized as a low-profile option for pelvic fixation to avoid implant prominence and an increased risk of skin breakdown and infection, which are associated with traditional sacroiliac screws.

Expected Outcomes: Miyanji et al. reported quality outcomes in patients with cerebral palsy and Gross Motor Function Classification Scores of ≥4. In that study, caregivers completed a validated disease-specific questionnaire grading the health-related quality of life of the patient preoperatively and at 1, 2, and 5 years postoperatively. Complication data were prospectively collected for each patient and preoperative outcome scores were compared at each of the postoperative time points. Survey scores at 1, 2, and 5 years postoperatively were significantly higher compared with baseline preoperative values.Sponseller et al. compared the 2-year postoperative radiographic parameters of 32 pediatric patients who underwent SAI fixation and 27 patients who underwent pelvic fixation with the sacroiliac technique. Among patients who underwent SAI fixation, the mean correction of pelvic obliquity was 20° ± 11° (70% correction) and the mean Cobb angle 42° ± 25° (67%). Among patients who underwent pelvic fixation with the sacroiliac technique, those values were 10° ± 9° (50%) and 46° ± 16° (60%), respectively. SAI screws provided significantly better pelvic obliquity correction (p = 0.002) but no difference in Cobb correction or complications compared with other traditional techniques.

Important Tips: Family discussion prior to surgical treatment is paramount.Perform preoperative neurologic examination.Examine the cranium carefully for a ventriculoperitoneal shunt or prior cranial reconstruction prior to cranial traction.Transcranial neuromonitoring may be useful. Use descending neural motor evoked potentials when no signals from transcranial monitoring are obtained.Sink the SAI screw until it lines up with the S1 screw. Bury the SAI screw so it is not prominent.Measure rods longer in order to ensure adequate length for compression and distraction in correction of the pelvic obliquity.Use a T-square to verify adequate spinopelvic alignment.Postoperatively, the use of incisional vacuum-assisted closure can decrease soiling in these patients.

Acronyms And Abbreviations: SAI = Sacral alar iliacCP = Cerebral palsyAIS = Adolescent idiopathic scoliosisSMA = Spinal muscular atrophyIONM = Intraoperative neuromonitoringGMFCS = Gross Motor Functional Classification SystemDNMEP = Descending neural motor evoked potentialTXA = Tranexamic acidFFP = Fresh frozen plasmaASIS = Anterior superior iliac spineAIIS = Anterior inferior iliac spinePJK = Proximal junctional kyphosis.
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http://dx.doi.org/10.2106/JBJS.ST.20.00060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8505341PMC
August 2021

Matched Comparison of Magnetically Controlled Growing Rods with Traditional Growing Rods in Severe Early-Onset Scoliosis of ≥90°: An Interim Report on Outcomes 2 Years After Treatment.

J Bone Joint Surg Am 2021 Oct 13. Epub 2021 Oct 13.

Department of Orthopaedics and Traumatology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Background: Severe early-onset scoliosis (EOS) is managed surgically but represents a challenge due to limited implant fixation points, large curve size, and fragile patients with comorbidities. Magnetically controlled growing rods (MCGRs) have the advantage of avoiding surgical intervention for routine lengthening, but their ability to address severe EOS has not been studied, to our knowledge.

Methods: A retrospective review of a prospectively collected international database identified 44 children with severe (≥90°) EOS treated with MCGRs who met our study criteria. Etiology, age, and sex-matched patients treated with traditional growing rods (TGRs) were identified from the same database. Patients were evaluated at a 2-year follow-up. No patients with vertically expandable prosthetic titanium ribs (VEPTRs) were included. The health-related quality of life was evaluated with the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24).

Results: The mean preoperative major coronal curve was 104° in the MCGR group and 104° in the TGR group. At the 2-year follow-up, the mean major coronal curves were 52° and 66° (p = 0.001), respectively. The mean T1-T12 heights were 155 mm and 152 mm preoperatively and 202 mm and 192 mm at the 2-year follow-up (p = 0.088). According to Kaplan-Meier analysis, the 2-year unplanned-revision-free survival was 91% in the MCGR group and 71% in the TGR group (p < 0.005). The 2-year score in the EOSQ-24 pulmonary function domain was better in the MCGR group. There were no other significant differences in the EOSQ-24 scores between the groups.

Conclusions: MCGRs for severe EOS provided significantly better major curve correction with significantly fewer unplanned revisions than TGRs at a 2-year follow-up.

Level Of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.
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http://dx.doi.org/10.2106/JBJS.20.02108DOI Listing
October 2021

No Difference in the Rates of Unplanned Return to the Operating Room Between Magnetically Controlled Growing Rods and Traditional Growth Friendly Surgery for Children With Cerebral Palsy.

J Pediatr Orthop 2021 Oct 7. Epub 2021 Oct 7.

Department of Medicine, Dalhousie University Department of Orthopaedics, IWK Health Centre, Halifax, NS, Canada Department of Orthopaedics, A.I. Dupont Institute in Wilmington, Delaware Department of Orthopaedics, Pediatric Spine Foundation, Valley Forge, PA Department of Orthopaedics, Johns Hopkins Hospital, Baltimore, MD Department of Orthopaedics, University of Utah, Salt Lake City, UT Department of Orthopaedics, Rainbow Babies and Children's Hospital, Cleveland, OH.

Background: Early-onset scoliosis (EOS) is common in children with cerebral palsy (CP). The effectiveness of magnetically controlled growing rods (MCGR) and the risk for unplanned return to the operating room (UPROR) remain to be studied in this patient population. The primary outcome of this study was to examine the frequency of UPROR between MCGRs as compared with traditional growth friendly (TGF) surgeries for children with EOS secondary to CP.

Methods: Patients with EOS secondary to CP were prospectively identified from an international database, with data retrospectively analyzed. Scoliosis, kyphosis, T1-S1, and T1-T12 height were measured preoperation, immediate postoperation, and at minimum 2-year follow-up. The risk and etiology of UPRORs were compared between MCGR and TGF.

Results: Of the 120 patients that met inclusion criteria, 86 received TGF (age 7.5±0. 1.8 y; mean follow-up 7.0±2.9 y) and 34 received MCGR (age 7.1±2.2 y, mean follow-up 2.8±0.0.5 y). Compared with TGF, MCGR resulted in significant improvements in maintenance of scoliosis (P=0.007). At final follow-up, UPRORs were 8 of 34 patients (24%) for MCGR and 37 of 86 patients (43%) for TGF (P=0.05). To minimize the influence of follow-up period, UPRORs within the first 2 years postoperation were evaluated: MCGR (7 of 34 patients, 21%) versus TGF (20 of 86 patients, 23%; P=0.75). Within the first 2 years, etiology of UPROR as a percentage of all patients per group were deep infection (13% TGF, 6% MCGR), implant failure/migration (12% TGF, 9% MCGR), dehiscence (4% TGF, 3% MCGR), and superficial infection (4% TGF, 3% MCGR). The most common etiology of UPROR for TGF was deep infection and for MCGR was implant failure/migration.

Conclusion: For patients with EOS secondary to CP, there was no difference in the risk of UPROR within the first 2 years postoperatively whether treated with TGF surgery or with MCGRs (23% TGF, 21% MCGR).

Level Of Evidence: Level III-retrospective cohort, therapeutic study.
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http://dx.doi.org/10.1097/BPO.0000000000001892DOI Listing
October 2021

Tranexamic acid use is associated with reduced intraoperative blood loss during spine surgery for Marfan syndrome.

Spine Deform 2021 Oct 5. Epub 2021 Oct 5.

Department of Orthopaedic Surgery, The Johns Hopkins University, 601 N. Caroline Street, JHOC 5223, Baltimore, MD, 21287, USA.

Purpose: The utility of tranexamic acid (TXA) in patients with Marfan syndrome (MFS) is uncertain given associated aberrations within the vasculature and clotting cascade. Therefore, this study aimed to assess the association of TXA use with intraoperative blood loss and allogeneic blood transfusions in patients with MFS who underwent spinal arthrodesis.

Methods: We queried our institutional database for MFS patients who underwent spinal arthrodesis for scoliosis between 2000 and 2020 by one surgeon. We excluded procedures spanning < 4 vertebral levels, those using anterior or combined anterior/posterior approaches, and those involving growing rods, postoperative infection, or spondylolisthesis. Fifty-two patients met our criteria, of whom 22 were treated with TXA and 30 were not. Mean differences in blood loss, transfusion volume, and proportions receiving transfusion were compared between TXA and the control groups using Student t, chi-squared, or Fisher exact tests. Alpha = 0.05.

Results: MFS patients treated with TXA experienced less mean (± standard deviation) intraoperative blood loss (1023 ± 534 mL) compared to the control group (1436 ± 1022 mL) (p = 0.01). The TXA group had estimated blood volume loss of 27% ± 16% compared to 36% ± 21% for controls (p = 0.05). No differences were found in allogeneic transfusion rate (p = 0.66) or transfusion volume (p = 0.15).

Conclusions: We found an association between TXA use and reduced blood loss during surgical treatment of MFS-associated scoliosis, suggesting that the connective tissue deficiency in MFS does not interfere with TXA's mechanism of action.

Level Of Evidence: III.
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http://dx.doi.org/10.1007/s43390-021-00416-1DOI Listing
October 2021

Interlocking Fixation in Fassier-Duval Rods: Performance and Success Factors.

J Pediatr Orthop 2021 Sep;41(8):525-529

Department of Orthopaedics, Johns Hopkins University Hospital, Baltimore, MD.

Introduction: Pediatric patients with osteogenesis imperfecta (OI) can be treated with intramedullary Fassier-Duval rod (FDR) systems for limb deformity or recurrent fractures. Single-interlocking pins can improve epiphyseal fixation, but there is a paucity of literature examining incidence of rod migration or pin backout long-term. The purpose of this study is to quantify rates of rod migration and pin backout in OI patients treated with single-interlocking FDRs.

Methods: A retrospective chart review was performed on pediatric patients treated at a tertiary care center across a 15-year period. Inclusion criteria to select patients was: (1) Pediatric patients (below 18 y of age); (2) Patients with confirmed OI; and (3) Patients with lower extremity fractures or deformity treated with FDRs with distal interlocking pins. Age at time of surgery, rates of obturator migration and pin backout and prominence were collected. We recorded if pin tips were bent by the surgeon during the procedure. Bivariate statistics were used to analyze risk factors for pin backout and prominence.

Results: Twenty-four single-interlocking pin FDRs (21 tibia, 3 femur) were identified. The mean age at index surgery was 5.7±3.4 years, with the mean follow-up time of 7.2±4.7 years. Fourteen (58%) rods underwent revision surgery. Obturator proximal migration was observed in 3/24 rods (13%). No cases of obturator distal migration were observed (0/24, 0%). Mean proximal obturator migration was 2.16±1.8 cm. Revision for pin backout was observed in 10 (42%) rods and pin prominence in 11 (46%) extremities. Bending interlocking pins on at least 1 end was associated with decreased pin backout (P=0.01) and prominence (P=0.04).

Conclusions: Even with distal interlocking pins, the obturator of FDRs can still migrate over time. Pin backout is a common indication for revision surgery. Bending interlocking pins can decrease rates of pin backout and prominence.

Level Of Evidence: Level III.
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http://dx.doi.org/10.1097/BPO.0000000000001883DOI Listing
September 2021

Early-Onset Spinal Deformity in Neurofibromatosis Type 1: Natural History, Treatment, and Imaging Surveillance.

JBJS Rev 2021 07 23;9(7). Epub 2021 Jul 23.

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland.

»: Early-onset scoliosis (EOS) or kyphosis is common in patients with neurofibromatosis (NF) and is characterized by rapid progression of deformity.

»: Traditional growing rods provide good functional and deformity outcomes in patients with NF and EOS; magnetically controlled growing rods (MCGRs) also provide good deformity correction, although high rates of revision have been reported after their use.

»: Among patients with NF type 1 (NF1), morphologic characteristics of the spinal deformity are different in those with paraspinal neurofibromas than in those without paraspinal tumors.

»: Patients with NF1 are at low risk for developing malignant peripheral nerve sheath tumors during childhood (<1%) and their lifetime (8% to 12%), and routine imaging surveillance for malignancy in the absence of symptoms should be clinically directed.

»: Further investigation is needed to standardize screening for EOS in children with NF1 and to develop guidelines for ideal imaging modalities, including their frequency and a timeline.
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http://dx.doi.org/10.2106/JBJS.RVW.20.00285DOI Listing
July 2021

Scoliosis Research Society Annual Meeting 2019 Abstracts.

J Bone Joint Surg Am 2020 08;102(16):e96

Department of Orthopaedic Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

The Scoliosis Research Society (SRS) aims to foster optimal care of pediatric and adult patients with spinal deformity of all etiologies. Founded 53 years ago, the SRS has maintained a strong commitment to research and education. At the 2019 SRS Annual Meeting in Montreal, Québec, Canada, >170 papers were presented on an array of spinal deformity topics. This article represents the top abstracts that were presented at the SRS 54th Annual Meeting. Two of the abstracts were nominees for the Hibbs Basic Research Award, and 8 were nominees for the Hibbs Clinical Research Award.
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http://dx.doi.org/10.2106/JBJS.20.00401DOI Listing
August 2020

Factors associated with increased back pain in primary thoracic adolescent idiopathic scoliosis 10 years after surgery.

Spine Deform 2021 Jul 12. Epub 2021 Jul 12.

Orthopedics and Scoliosis Division, Rady Children's Hospital, San Diego, CA, USA.

Purpose: To identify the prevalence and predictors of nonspecific back pain in primary thoracic adolescent idiopathic scoliosis (AIS) patients at 10 years after surgery.

Methods: This was a case-control multi-center study. A query of patients who underwent surgical correction of major thoracic AIS between 1997 and 2007 with 10-year follow-up was reviewed. SRS-22 pain scores at 10 years were classified as below normal (≤ 2 standard deviations below average for controls of similar age/sex from published literature) or within/above the control range.

Results: One hundred and seventy-one patients with an average of 10.5 ± 0.8-years follow-up were included. Average age at surgery was 14 ± 2 years. The rate of pain was 23% for males and 11% for females (p = 0.08). Differences in age, 10-year SRS mental health score, and radiographic measures were noted. Of 12 patients who underwent revision surgery, 42% reported below normal pain scores versus 11% in cases without revision (p = 0.012). Classification and regression tree (CART) analysis identified 10-year thoracic curve magnitude and 10-year mental health scores as significant predictors. Thoracic Cobb of ≤ 26° at 10 years was associated with a 7% rate of below normal pain scores compared to 27.5% when the curve was > 26° (OR = 4.8, p < 0.05). Of those with a curve ≤ 26°, no patients had abnormal pain if the SRS mental health score was > 4.2 and 15% had more pain than normal if mental health score was ≤ 4.2 (OR 23, p < 0.05).

Conclusion: Increased primary thoracic curve magnitude (> 26°) at 10 years was the primary predictor of increased pain. For patients with less coronal deformity (< 26°), a poor mental health score was associated with an increased rate of pain. Male gender and revision surgery may also play a role in increased pain, however, the overall frequency of these variables were low.

Level Of Evidence: Level 3.
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http://dx.doi.org/10.1007/s43390-021-00384-6DOI Listing
July 2021

Case of the Missing Vertebra: A Report of a Radiographic Stitching Error in a Scoliosis Patient.

JBJS Case Connect 2021 07 6;11(3). Epub 2021 Jul 6.

Department of Orthopaedic Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Case: A 14-year-old girl with adolescent idiopathic scoliosis underwent imaging in preparation for scoliosis surgery. Posteroanterior traction radiographs showed 4 lumbar vertebrae, while the standing film showed 5. Reconciliation with the component radiographs used for the traction showed the discrepancy was caused by a software error. She underwent surgical correction, and her recovery has been uncomplicated.

Conclusion: Image stitching errors can lead to false depiction of structural abnormalities. Radiology technicians and clinicians should be cautious when reviewing digitally stitched images. We recommend that technicians label stitched images and indicate the overlapping region to assist with radiographic assessment.
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http://dx.doi.org/10.2106/JBJS.CC.21.00295DOI Listing
July 2021

How low can you go? Implant density in posterior spinal fusion converted from growing constructs for early onset scoliosis.

Spine Deform 2021 09 6;9(5):1479-1488. Epub 2021 Jul 6.

Children's Orthopaedic Center, Children's Hospital Los Angeles, 4650 Sunset Blvd, MS#69, Los Angeles, CA, 90027, USA.

Study Design: Retrospective, multicenter comparative.

Objectives: Our purpose was to compare early onset scoliosis (EOS) patients treated with ultra-low, low, and high implant density constructs when undergoing conversion to definitive fusion. Larson et al. demonstrated that implant density (ID) at fusion does not correlate with outcomes in the treatment of adolescent idiopathic scoliosis, but did not address growth-friendly graduates.

Methods: EOS patients treated with growth-friendly constructs converted to fusion between 2000 and 2017 were reviewed from a multicenter database. ID was defined as number of pedicle screws, hooks, and sublaminar/bands per level fused. Patients were divided into ultra-low ID (< 1.3), low (≥ 1.3 and < 1.6), and high ID (≥ 1.6).

Exclusion Criteria: < 2 years follow-up from fusion or inadequate radiographs.

Results: A total of 152 patients met inclusion criteria with 39 (26%) patients in the high ID group, 33 (22%) patients in the low ID group, and 80 (52%) in the ultra-low ID group. Groups were similar in operative time (p = 0.61), pre-fusion major curve (p = 0.71), mean number of levels fused (p = 0.58), clinical follow-up (p = 0.30), and radiographic follow-up (p = 0.90). Patients in the low ID group (11.6 ± 1.5 years) were slightly younger at the time of definitive fusion than patients in the ultra-low ID group (12.9 ± 2.2 years) and high ID group (12.5 ± 1.7 years) (p = 0.009). There was significantly more blood loss in the high ID group than the other two groups (high ID: 946.8 ± 606.0 mL vs. low ID: 733.9 ± 434.5 mL and ultra-low ID: 617.4 ± 517.2 mL; p = 0.01), but there was no significant difference with regard to percent of total blood volume lost (high ID: 59.3 ± 48.7% vs. low ID: 54.5 ± 37.5% vs. ultra-low ID: 51.7 ± 54.9%; p = 0.78). There was a difference in initial improvement in major curve between the groups (high ID: 21.6° vs. low ID: 18.0° vs. ultra-low ID: 12.6°; p = 0.01). However, during post-fusion follow-up, correction decreased 7.1° in the high ID group, 2.6 in the low ID group, and 2.8 in the ultra-low ID group (p = 0.19). At final follow-up, major curve correction from pre-fusion was similar between groups (high ID: 14.5° vs. low ID: 15.5° vs. ultra-low ID: 9.7°, p = 0.14). At final follow-up, there was no difference in T1-T12 length gain (p = 0.85), T1-S1 length gain (p = 0.68), coronal balance (p = 0.56), or sagittal balance (p = 0.71). The revision rate was significantly higher in the ultra-low ID group (13.8%; 11/80) versus the high ID group (2/39; 5.1%) and low ID group (0/33; 0%) (p = 0.04).

Conclusions: Although an ID < 1.3 in growth-friendly graduates produces similar outcomes with regard to curve correction and spinal length gain as low and high ID, this study suggests that an ID < 1.3 is associated with an increased revision rate.

Level Of Evidence: III.
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http://dx.doi.org/10.1007/s43390-021-00321-7DOI Listing
September 2021

Surgical Treatment of Unstable Pelvic Ring Injury in a Young Child: A Case Report.

JBJS Case Connect 2021 06 10;11(2). Epub 2021 Jun 10.

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, MD.

Case: We describe an anterior and posterior pelvic ring construct, with emphasis on the posterior construct, to treat a vertical displacement fracture in a 2-year-old girl who was struck by a motor vehicle. Eighteen months after her injury, radiographs showed intact sacroiliac joints and symmetrical pubic symphysis.

Conclusion: Although commonly performed in adults, pelvic fixation is challenging in children because of the small size of the child's pelvis and osseous fixation pathways. However, this approach enabled successful vertical stabilization of the pelvis, complete resolution of symphyseal diastasis, and recovery of function and mobility.
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http://dx.doi.org/10.2106/JBJS.CC.20.00942DOI Listing
June 2021

Orthopaedic Problems in 35 Patients With Organic Acid Disorders.

J Pediatr Orthop 2021 Jul;41(6):e457-e463

Departments of Orthopaedic Surgery.

Introduction: Organic acid disorders (OADs) are a subset of inborn errors of metabolism that result in a toxic accumulation of organic acids in the body, which can lead to metabolic derangements and encephalopathy. Patients with these disorders are managed by a team of biochemical geneticists and metabolic nutritionists. However, subspecialists such as neurologists and orthopaedic surgeons are often needed to help manage the sequelae of the metabolic derangements. The breadth of orthopaedic sequelae of these disease states is poorly understood. Herein, we describe orthopaedic problems associated with 5 types of OAD most commonly seen at our institution: maple syrup urine disease, methylmalonic aciduria, propionic aciduria, pyruvate dehydrogenase deficiency, and glutaric aciduria type 1.

Methods: We retrospectively reviewed medical records of 35 patients with an OAD who were seen at our academic tertiary care center from May 1999 to May 2020. Patients were grouped into cohorts according to OAD type and analyzed for orthopaedic presentations of hip, knee, or foot disorders, presence and severity of scoliosis, history of fracture, movement disorders, and osteopenia/osteoporosis.

Results: Of the 35 patients, 13 had maple syrup urine disease, 12 had methylmalonic aciduria, 4 had propionic aciduria, 4 had pyruvate dehydrogenase deficiency, and 2 had glutaric aciduria type 1. Associated orthopaedic problems included spasticity causing neuromuscular scoliosis and/or hip subluxation or dislocation (10 patients), fractures (7 patients), and osteopenia/osteoporosis (7 patients). Overall, 22 of 35 patients had some orthopaedic condition.

Conclusions: Most in this cohort of patients with OAD also had an orthopaedic abnormality. It is important for physicians treating these patients to understand their propensity for musculoskeletal problems. When treating patients with OAD, it is important to initiate and maintain communication with specialists in several disciplines and to develop collaborative treatments for this unique population.

Level Of Evidence: Level IV-prognostic study.
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http://dx.doi.org/10.1097/BPO.0000000000001812DOI Listing
July 2021

Osteotomy in the newborn classic bladder exstrophy patient: A comparative study.

J Pediatr Urol 2021 08 22;17(4):482.e1-482.e6. Epub 2021 Apr 22.

Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA. Electronic address:

Introduction: Pelvic osteotomy is indicated in classic bladder exstrophy (CBE) patients with a wide pubic diastasis or non-malleable pelvis. While the safety of pelvic osteotomy in delayed and failed closures is established, there remains less clarity on their safety in newborns. The authors herein sought to present their experience with CBE patients who underwent pelvic osteotomy for assistance with bladder closure during both the newborn and delayed time periods.

Objective: The authors hypothesize that pelvic osteotomy during exstrophy closure may be performed safely in newborns with few perioperative or post-operative negative sequelae.

Study Design: A prospectively maintained IRB-approved database was reviewed for CBE patients who underwent osteotomy during primary closure. Patient demographics, performing institution (authors' or outside), closure outcome, diastasis width, and post-operative complications were noted. Patient subgroups included newborn and delayed (>28 days of life) closures. Failure was defined as bladder dehiscence, prolapse, outlet obstruction, or vesicocutaneous fistula requiring reoperation. Orthopedic complications included nerve palsies, superficial pin-site infection, and bladder neck erosion by orthopedic hardware. Analyses were performed using a Chi-square test.

Results: 286 patients were included: 186 newborn and 100 delayed closures. The authors' institution performed 109 cases (44 newborn and 65 delayed). Within the overall newborn closure cohort, no significant differences were found in outcomes among the osteotomy types with success rates of 80%, 60.8%, and 71.4% in the combined, posterior iliac, and anterior innominate groups, respectively (p = 0.24). In the delayed group, success rates were significantly different with rates of 100%, 72.4%, and 93.8% in the combined, posterior iliac, and anterior innominate groups, respectively (p < 0.001). Febrile urinary tract infection (UTI) was the most common complication at 8% (23/286). Only 1.7% (5/286) of patients had orthopedic complications with 3 patients in the newborn cohort, 2 patients in the delayed cohort, and only one patient requiring reoperation.

Discussion: Orthopedic complications are rare in CBE patients who undergo osteotomies regardless of the closure period. No clinically significant difference in orthopedic complication rate was found between newborn and delayed closure periods.

Conclusions: While current trends have moved toward delayed primary closures, there remains a role for osteotomy during exstrophy closure in select newborn patients and can be performed safely with few complications.
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http://dx.doi.org/10.1016/j.jpurol.2021.04.009DOI Listing
August 2021

What happens to the unfused upper thoracic curve after posterior spinal fusion for adolescent idiopathic scoliosis?

J Neurosurg Pediatr 2021 Apr 23:1-7. Epub 2021 Apr 23.

2Department of Orthopaedic Surgery and Neurosurgery, Shriners Hospitals for Children-Philadelphia, Pennsylvania.

Objective: Spontaneous lumbar curve correction after selective thoracic fusion in surgery for adolescent idiopathic scoliosis (AIS) is well described. However, only a few articles have described the course of the uninstrumented upper thoracic (UT) curve after fusion, and the majority involve a hybrid construct. In this study, the authors sought to determine the outcomes and associated factors of uninstrumented UT curves in patients with AIS.

Methods: The authors retrospectively reviewed a prospectively collected multicenter AIS registry for all consecutive patients with Lenke type 1-4 curves with a 2-year minimum follow-up. UT curves were considered uninstrumented if the upper instrumented vertebra (UIV) did not extend above 1 level from the lower end vertebra of the UT curve. The authors defined progression as > 5°, and divided patients into two cohorts: those with improvement in the UT curve (IMP) and those without improvement in the UT curve (NO IMP). Radiographic, demographic, and Scoliosis Research Society (SRS)-22 survey outcome measures were compared using univariate analysis, and significant factors were compared using a multivariate regression model.

Results: The study included 450 patients (370 females and 80 males). The UT curve self-corrected in 86% of patients (n = 385), there was no change in 14% (n = 65), and no patients worsened. Preoperatively, patients were similar with respect to Lenke classification (p = 0.44), age (p = 0.31), sex (p = 0.85), and Risser score (p = 0.14). The UT curves in the IMP group self-corrected from 24.7° ± 6.5° to 12.6° ± 5.9°, whereas in the NO IMP group UT curves remained the same, from 20.3° ± 5.8° to 18.5° ± 5.7°. In a multivariate analysis, preoperative main thoracic (MT) curve size (p = 0.004) and MT curve correction (p = 0.001) remained significant predictors of UT curve improvement. Greater correction of the MT curve and larger initial MT curve size were associated with greater likelihood of UT curve improvement.

Conclusions: Spontaneous UT curve correction occurred in the majority (86%) of unfused UT curves after MT curve correction in Lenke 1-4 curve types. The magnitude of preoperative MT curve size and postoperative MT curve correction were independent predictors of spontaneous UT curve correction.
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http://dx.doi.org/10.3171/2020.10.PEDS20671DOI Listing
April 2021

Hip-Spine Relationship: Thoracolumbar Deformation in a Patient with Limited Hip Flexion: A Case Report.

JBJS Case Connect 2021 Apr 21;11(2):e20.00548. Epub 2021 Apr 21.

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland.

Case: We describe thoracolumbar kyphosis with severe vertebral deformation in a 13-year-old boy with cerebral palsy, hip extension contractures, and history of hip flexion-adduction releases.

Conclusion: Patients with cerebral palsy and hip extension contractures may develop thoracolumbar kyphosis to maintain sitting balance. It is important to recognize hip extension contractures as the underlying cause of the compensatory kyphosis and to be familiar with treatment options that address the hips and the spine.
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http://dx.doi.org/10.2106/JBJS.CC.20.00548DOI Listing
April 2021

Why the Hips Remain Stable When the Spine Strays: A Deeper Analysis of the Relationship Between Hip Displacement and Severe Scoliosis in Patients With Cerebral Palsy.

J Pediatr Orthop 2021 May-Jun 01;41(5):261-266

Department of Orthopaedic Surgery, Division of Pediatric Orthopaedic Surgery, The Johns Hopkins Hospital.

Background: Many patients with spastic quadriplegic cerebral palsy (CP) and severe scoliosis develop hip displacement, whereas others do not. We investigated demographic characteristics, risk factors for CP, and imaging findings associated with nondisplaced hips in patients with CP and severe scoliosis.

Methods: We retrospectively analyzed records of 229 patients with spastic quadriplegic CP and severe scoliosis who presented for treatment at our US academic tertiary care hospital between August 2005 and September 2015. Demographic characteristics, risk factors for CP, and brain magnetic resonance imaging (MRI) findings were documented. Patients were classified as Gross Motor Function Classification System (GMFCS) level 4 or higher, with 58% at GMFCS level 5.3. Displaced hips (n=181 patients) were defined as a migration percentage of ≥30% or previous surgery for hip displacement/adductor contractures. Patients who did not meet these criteria were classified as nondisplaced (n=48 patients). We used univariate analysis and multivariate logistic regression to determine associations between patient factors and hip displacement (alpha=0.05).

Results: Patients born at term (≥37 wk) had 2.5 times the odds [95% confidence interval (CI): 1.3-5.0] of having nondisplaced hips compared with patients born prematurely. Females had 2.0 times the odds (95% CI: 1.0-3.9) of having nondisplaced hips compared with males. Patients with normal brain MRI findings had 9.6 times the odds (95% CI: 2.3-41) of having nondisplaced hips compared with patients with abnormal findings. Hip displacement was not associated with race (P>0.05).

Conclusions: Gestational age 37 weeks or above, female sex, and normal brain MRI findings are independently associated with nondisplaced hips in patients with spastic quadriplegic CP and severe scoliosis. These findings direct attention to characteristics that may place patients at greater risk of displacement. Future work may influence preventative screening practices and improve patient counseling regarding the risk of hip displacement.

Level Of Evidence: Level III-retrospective comparative study.
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June 2021

Spine deformity care: a team effort.

Spine Deform 2021 03;9(2):311-313

John Hopkins Hospital, Baltimore, MD, USA.

This address highlights the Society's progress during the past year, with special initiatives in inter-disciplinary education, research, and increased engagement.
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http://dx.doi.org/10.1007/s43390-021-00298-3DOI Listing
March 2021

Growth-friendly surgery results in more growth but a higher complication rate and unplanned returns to the operating room compared to single fusion in neuromuscular early-onset scoliosis: a multicenter retrospective cohort study.

Spine Deform 2021 05 8;9(3):851-858. Epub 2021 Feb 8.

Department of Orthopaedic Surgery, Texas Scottish Rite Hospital for Children and University of Texas Southwestern, Dallas, TX, USA.

Purpose: Compare radiographic outcomes, complications, and QoL in neuromuscular early-onset scoliosis (EOS) patients treated with single posterior spinal fusion (PSF) versus growth-friendly surgery and definitive fusion (GFDF).

Methods: In a retrospective cohort study, children with neuromuscular EOS, age 8-11 years at index surgery with PSF or GF devices, with minimum 2-year follow-up after final fusion were identified from a multicenter database.

Results: 16 PSF and 43 GFDF patients were analyzed. Demographics were similar except PSF patients were older at index surgery and had shorter follow-up. PSF patients had greater percentage major curve correction (62% vs 38%, p = 0.001) and smaller major curve at final follow-up (23° vs 40°, p = 0.005). The GFDF group underwent over five times more surgeries (8.7 vs 1.6, p = 0.0001). Four PSF patients (25%) experienced ten complications, resulting in five unplanned returns to the operating room (UPROR) in three patients (19%). 36 GFDF patients (84%) experienced 83 complications, resulting in 45 UPRORs in 24 patients (56%). Poisson regression adjusted for age showed that the GFDF group had more complications (p = 0.001) and UPRORs (p = 0.01). Although the GFDF patients had smaller preoperative T1-T12 and T1-S1 lengths, these were similar to the PSF patients at final follow-up, indicating that the GFDF patients had greater spinal growth. PSF patients had better postoperative EOSQ-24 Financial Impact and Family Burden scores.

Conclusion: While there was a difference in age at index surgery, PSF may be more effective than GFDF at controlling neuromuscular EOS. GFDF patients achieved more spinal growth but eight times more complications and nine times more UPRORs.
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http://dx.doi.org/10.1007/s43390-020-00270-7DOI Listing
May 2021

Residual lumbar hyperlordosis is associated with worsened hip status 5 years after scoliosis correction in non-ambulant patients with cerebral palsy.

Spine Deform 2021 07 1;9(4):1125-1136. Epub 2021 Feb 1.

Department of Orthopedic Surgery, The Johns Hopkins University, Baltimore, MD, USA.

Background: Cerebral palsy (CP) is a static encephalopathy with progressive musculoskeletal pathology. Non-ambulant children (GMFCS IV and V) with CP have high rates of spastic hip disease and neuromuscular scoliosis. The effect of spinal fusion and spinal deformity on hip dislocation following total hip arthroplasty has been well studied, however in CP this remains largely unknown. This study aimed to identify factors associated with worsening postoperative hip status (WHS) following corrective spinal fusion in children with GMFCS IV and V CP.

Methods: Retrospective review of GMFSC IV and V CP patients in a prospective multicenter database undergoing spinal fusion, with 5 years follow-up. WHS was determined by permutations of baseline (BL), 1 year, 2 years, and 5 years hip status and defined by a change from an enlocated hip at BL that became subluxated, dislocated or resected post-op, or a subluxated hip that became dislocated or resected. Hip status was analyzed against patient demographics, hip position, surgical variables, and coronal and sagittal spinal alignment parameters. Cutoff values for parameters at which the relationship with hip status was significant was determined using receiver operating characteristic curves. Logistic regression determined odds ratios for predictors of WHS.

Results: Eighty four patients were included. 37 (44%) had WHS postoperatively. ROC analysis and logistic regression demonstrated that the only spinopelvic alignment parameter that significantly correlated with WHS was lumbar hyperlordosis (T12-L5) > 60° (p = 0.028), OR = 2.77 (CI 1.10-6.94). All patients showed an increase in pre-to-postop LL. Change in LL pre-to-postop was no different between groups (p = 0.318), however the WHS group was more lordotic at BL and postop (pre44°/post58° vs pre32°/post51° in the no change group). Age, sex, Risser, hip position, levels fused, coronal parameters, global sagittal alignment (SVA), thoracic kyphosis, and reoperation were not associated with WHS.

Conclusion: Postoperative hyperlordosis(> 60°) is a risk factor for WHS at 5 years after spinal fusion in non-ambulant CP patients. WHS likely relates to anterior pelvic tilt and functional acetabular retroversion due to hyperlordosis, as well as loss of protective lumbopelvic motion causing anterior femoracetabular impingement.

Level Of Evidence: III.
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July 2021

Results of Conservative and Surgical Management in Children with Idiopathic and Nonidiopathic Os Odontoideum.

World Neurosurg 2021 03 15;147:e324-e333. Epub 2020 Dec 15.

Department of Orthopaedics and Traumatology, University of Helsinki, Helsinki, Finland.

Objective: The outcomes of conservative and operative treatment of os odontoideum in children remain unclear. Our objective was to study the outcomes of conservative and surgical treatment of idiopathic os odontoideum in children and compare these outcomes in age- and treatment-matched nonidiopathic children with os odontoideum.

Methods: A retrospective multicenter review identified 102 children with os odontoideum, of whom 44 were idiopathic with minimum 2-year follow-up. Ten patients were treated conservatively, and 34 underwent spinal arthrodesis. Both groups were matched with nonidiopathic patients by age and type of treatment. Cervical arthrodesis was recommended for patients with increased atlantoaxial distance or reduced space available for the cord in flexion-extension radiographs.

Results: All 20 children undergoing conservative treatment remained asymptomatic during follow-up, but 1 nonidiopathic patient developed cervical instability. The idiopathic group had significantly less severe radiographic cervical instability and less neurologic complications than the nonidiopathic group (P < 0.05 for all comparisons). Thirty-three (97%) patients in the idiopathic group and 32 (94%) patients in the nonidiopathic group (94%) had spinal fusion at final follow-up (P = 0.55). The risk of complications (15% vs. 41%; odds ratio 0.234, 95% confidence interval 0.072-0.757, P = 0.015) and nonunion (6% vs. 24%; odds ratio 0.203, 95% confidence interval 0.040-0.99, P = 0.040) were significantly lower in the idiopathic than in the nonidiopathic group. Idiopathic children undergoing rigid fixation achieved spinal fusion.

Conclusions: Idiopathic patients with stable atlantoaxial joint at presentation remained asymptomatic and intact during conservative treatment. Idiopathic children with os odontoideum undergoing spinal arthrodesis had significantly fewer complications and nonunion than nonidiopathic children.

Level Of Evidence: III.
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http://dx.doi.org/10.1016/j.wneu.2020.12.043DOI Listing
March 2021

Risk factors for gastrointestinal complications after spinal fusion in children with cerebral palsy.

Spine Deform 2021 03 17;9(2):567-578. Epub 2020 Nov 17.

Department of Orthopaedic Surgery, University Hospital Cleveland Medical Center, Rainbow Babies and Children's Hospital, 201 Adelbert Road, Cleveland, OH, 44106, USA.

Design: Prospective cerebral palsy (CP) registry review.

Objectives: (1) Evaluate the incidence/risk factors of gastrointestinal (GI) complications in CP patients after spinal fusion (SF); and (2) investigate the validity of the modified Clavien-Dindo-Sink classification.

Background: Perioperative GI complications result in increased length of stay (LOS) and patient morbidity/mortality. However, none have analyzed the outcomes of GI complications using an objective classification system.

Methods: A prospective/multicenter CP database identified 425 children (mean, 14.4 ± 2.9 years; range, 7.9-21 years) who underwent SF. GI complications were categorized using the modified Clavien-Dindo-Sink classification. Grades I-II were minor complications and grades III-V major. Patients with and without GI complications were compared.

Results: 87 GI complications developed in 69 patients (16.2%): 39 minor (57%) and 30 major (43%). Most common were pancreatitis (n = 45) and ileus (n = 22). Patients with preoperative G-tubes had 2.2 × odds of developing a GI complication compared to oral-only feeders (OR 2.2; 95% CI 0.98-4.78; p = 0.006). Similarly, combined G-tube/oral feeders had 6.7 × odds compared to oral-only (OR 6.7; 95% CI 3.10-14.66; p < 0.001). The likelihood of developing a GI complication was 3.4 × with normalized estimated blood loss (nEBL) ≥ 3 ml/kg/level fused (OR 3.41; 95% CI 1.95-5.95; p < 0.001). Patients with GI complications had more fundoplications (29% vs. 17%; p = 0.03) and longer G-tube fasting periods (3 days vs. 2 days; p < 0.001), oral fasting periods (5 days vs. 2 days; p < 0.001), ICU admissions (6 days vs. 3 days; p = 0.002), and LOS (15 days vs. 8 days; p < 0.001). LOS correlated with the Clavien-Dino-Sink classification.

Conclusion: Gastrointestinal complications such as pancreatitis and ileus are not uncommon after SF in children with CP. This is the first study to investigate the validity of the modified Clavien-Dindo-Sink classification in GI complications after SF. Our results suggest a correlation between complication severity grade and LOS. The complexity of perioperative enteral nutritional supplementation requires prospective studies dedicated to enteral feeding protocols.

Level Of Evidence: Therapeutic-level III.
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http://dx.doi.org/10.1007/s43390-020-00233-yDOI Listing
March 2021

Role of the Pubic Symphysis in Osseous Pelvic Development: A Novel Model of Bladder Exstrophy in Rabbits.

J Pediatr Orthop 2021 Feb;41(2):e181-e187

Departments of Orthopaedic Surgery.

Background: It has been posited that the osseous pelvic anomalies seen in patients with classic bladder exstrophy (CBE) result from disruption of the pubic symphysis. This hypothesis, however, has not been tested. In the present animal study, our objective was to determine whether the tension of the pubic symphysis helps maintain the shape of the pelvic ring, or whether the growing bones maintain a ring shape even without the tension of the symphysis.

Methods: In total, 12 neonatal New Zealand White rabbits underwent pubic symphysiotomy (experimental group, n=9) or sham surgery (control group, n=3) on days 3 or 4 of life. Rabbits were scanned with cone-beam computed tomography at 1, 4, 12, and 20 weeks postoperatively to monitor changes in the following pelvic parameters, which are known to be altered in CBE: anterior segment angle, anterior segment length, intertriradiate distance, interpubic distance, and posterior segment angle. Changes within the experimental and control groups were evaluated using repeated-measures analysis of variance and post hoc Tukey honest significant difference testing. Two-tailed t tests were used to compare treatment groups at each time point.

Results: Both groups showed increases in anterior segment length and intertriradiate distance during the study period; rabbits in the experimental group also showed a steady increase in interpubic distance (F=43.9; P<0.001). Experimental rabbits had significantly larger mean values for anterior segment angle, intertriradiate distance, interpubic distance, and posterior segment angle than did control rabbits at all time points. We found no difference in mean anterior segment length between control and experimental groups at any time point. The difference in interpubic distance was particularly pronounced by 20 weeks (experimental group, 13±2.7 mm; control group, 1.1±0.1 mm; P<0.001).

Conclusions: The pubic symphysis is essential for normal pelvic development. Its absence led to early pelvic angulation and progressive pubic separation in a rabbit model. However, we found no significant difference in the mean anterior segment length, and it is likely that other factors are also implicated in the growth disturbance seen in CBE.

Level Of Evidence: Level V.
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http://dx.doi.org/10.1097/BPO.0000000000001698DOI Listing
February 2021

New neurologic deficit and recovery rates in the treatment of complex pediatric spine deformities exceeding 100 degrees or treated by vertebral column resection (VCR).

Spine Deform 2021 03 9;9(2):427-433. Epub 2020 Oct 9.

Department of Orthopedics, Washington University School of Medicine, St. Louis, MO, USA.

Study Design: Prospective multicenter international observational study.

Objective: To investigate incidence of new neurologic deficit (NND) and the long-term recovery patterns following complex pediatric spine deformity surgery. The SRS M&M reports identify pediatric patients as having higher rate of new neurologic deficit compared with adults, while congenital and neuromuscular deformities are associated with higher new neurologic risks. Very few studies have had the large numbers of pediatric patients with curves exceeding 100 deg to ascertain the new neurologic deficit (NND) rates and recovery patterns as it relates to curve laterality and diagnosis.

Method: The FOX pediatric database from 17 international sites was queried for New Neurologic Deficit (NND) as characterized by change in American Spinal Injury Association (ASIA) Lower or Upper Extremity Motor Score. Recovery rates at specific intervals were recorded and related to the curve type and etiology.

Results: Data of 286 consecutive patients with normal pre-operative neurologic exams were reviewed. There were 160 females vs 125 males with an average age of 14.6 years. NND occurred in 27 patients (9.4%) in the immediate post-operative period. Diagnostic categories included idiopathic scoliosis (3 patients); idiopathic kyphoscoliosis(5 patients); congenital scoliosis (7 patients); congenital kyphoscoliosis (4 patients); congenital kyphosis (6 patients), other kyphosis (1 patient) and syndromic (1 patient). 1 patient was lost to follow-up (f/u) after discharge; 1 had chronic deficits at the first post-operative erect visit (from discharge to 9 months f/u) and was subsequently lost to follow-up; 2 patients were improving at 1-year f/u but lost to subsequent f/u. 16 patients had normal neurologic function by the time of the first post-operative erect visit, 21 patients at 1-year f/u and 21 patients at the 2-year f/u. 2 patients (0.69%) had improved NND at 2-year mark.

Conclusion: A significant proportion of patients with complex spine deformity experience NND. However, significant improvement in neurologic function can be expected over time as seen in this study without additional surgical intervention in most cases. Congenital deformities accounted for 63% of the patients experiencing NND.
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http://dx.doi.org/10.1007/s43390-020-00211-4DOI Listing
March 2021

Radiographic assessments of pediatric supracondylar fractures and mid-term patient-reported outcomes.

Medicine (Baltimore) 2020 Oct;99(41):e22543

Department of Pediatric Orthopaedic Surgery, The Johns Hopkins Hospital, Baltimore, MD.

Radiographic parameters are commonly used to determine the need for surgical supracondylar humeral (SCH) fracture reduction and the postoperative quality of reduction. We studied whether such parameters are correlated with mid-term patient-reported outcome (PRO) scores in pediatric patients.We retrospectively reviewed data from 213 patients (104 girls) treated surgically for Gartland type-II (n = 84) or type-III (n = 129) SCH fractures from 2008-2016. Mean (± standard deviation) age at surgery was 5.1 ± 2.1 years. Mean time from initial treatment to outcome survey completion was 5.0 ± 2.1 years (range, 2.0-10 years). We evaluated preoperative radiographs for coronal/sagittal fracture displacement, presence of impaction/comminution, Gartland classification, and rotation. Patients, parents were asked via telephone to complete the QuickDASH (Quick Disability of the Arm, Shoulder, and Hand) and PROMIS (Patient-Reported Outcomes Measurement Information System) Strength Impact, Upper Extremity, and Pain Interference questionnaires. Parents were also asked whether the previously fractured arm appeared normal or abnormal. We evaluated postoperative radiographs for coronal/sagittal deformity, Baumann angle, and rotation and classified reductions as near complete/complete or incomplete. Anterior humeral line through the capitellum, Baumann angle in the 7.5th to 92.5th percentile of the sample, or rotation ratio between 0.85 and 1.15 were considered near complete/complete reductions; all others were considered incomplete. Bivariate analysis was used to determine whether radiographic parameters and arm appearance were associated with QuickDASH and PROMIS scores.Patients with Gartland type-III fractures had significantly greater disability on the QuickDASH at follow-up compared with those with Gartland type-II fractures (P < .01). It is unknown if this statistical difference translates to clinical relevance. No other preoperative or postoperative radiographic parameter was significantly associated with PRO scores. There was no association between fractured arm appearance at follow-up and PRO scores.Radiographic parameters that are used to evaluate the need for and quality of pediatric SCH fracture reduction are not significantly associated with mid-term PROMIS and QuickDASH scores.LOE: Prognostic Level III.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7544399PMC
October 2020

Orthopaedic Conditions Associated with Aneurysms.

JBJS Rev 2020 06;8(6):e0122

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland.

Orthopaedic surgeons are sometimes the first specialists encountered by patients with inherited conditions that predispose them to aneurysms. The skeletal features are evident, but the aneurysm is silent. Early recognition of the conditions associated with aneurysms can lead to effective treatment and minimize risks of morbidity and death. Marfan syndrome is characterized by abnormal fibrillin-1 protein and has a broad range of skeletal manifestations, including scoliosis, hindfoot deformity, arachnodactyly, pectus excavatum or carinatum deformity, dural ectasia, and acetabular protrusio. Aneurysm-associated complications are the leading cause of early morbidity and death in patients with Marfan syndrome. Ehlers-Danlos syndrome is caused by a disturbance in collagen biosynthesis most commonly resulting in joint hypermobility and skin abnormalities. Among the types of Ehlers-Danlos syndrome, vascular Ehlers-Danlos syndrome presents the highest risk of vascular complications. Clubfoot and joint dislocations are common presenting symptoms in vascular Ehlers-Danlos syndrome. Loeys-Dietz syndrome is a connective tissue disorder resulting in aortic root dilation and several skeletal manifestations, including scoliosis, cervical malformations, joint contractures, and foot deformities.
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http://dx.doi.org/10.2106/JBJS.RVW.19.00122DOI Listing
June 2020

A report of two conservative approaches to early onset scoliosis: serial casting and bracing.

Spine Deform 2021 03 28;9(2):595-602. Epub 2020 Sep 28.

Department of Orthopaedic Surgery, Rainbow Babies and Children's Hospital, Cleveland, OH, USA.

Purpose: Previous reports have demonstrated the effectiveness of casting for EOS. Brace treatment for EOS has not been studied. The purpose of this multicenter retrospective study was to compare radiographic outcomes, complications, and rates of conversion to surgery in children with EOS treated with casting or bracing.

Methods: Children aged 2-6 years with idiopathic or neuromuscular EOS treated with casting or bracing with minimum follow-up of 2 years were identified.

Results: 68 patients (36 cast, 32 brace) were analyzed. Diagnosis, age at start of treatment, and duration of follow-up were similar. Although the cast patients had a larger pre-treatment major curve magnitude (50° vs 31°, p < 0.001), both groups had a similar major curve magnitude at most recent follow-up (36° vs 32°, p = 0.456). T1-T12 and T1-S1 length increased in both groups. The cast and brace patients had similar complications and conversions to surgery. Sub-analysis showed that while casting resulted in curve improvement regardless of etiology, bracing was able to prevent curve progression in patients with idiopathic EOS but not in patients with non-idiopathic EOS (Δ- 15° vs 27°, p = 0.006). Regression analysis (significance p = 0.10) controlling for baseline age, major curve magnitude, and T1-T12 and T1-S1 length showed that treatment method was associated with difference in major curve magnitude (p = 0.090) and T1-T12 length (p = 0.024).

Conclusion: In our study, serial casting led to curve improvement in children with idiopathic and neuromuscular EOS, whereas brace treatment appeared to prevent curve progression in patients with idiopathic EOS but did not appear to control the curve in neuromuscular EOS.
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http://dx.doi.org/10.1007/s43390-020-00213-2DOI Listing
March 2021
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