Spinal Manifestations of Skeletal Dysplasia: A Practical Guide for Clinical Diagnosis.

Skeletal dysplasias are a group of genetic conditions defined by atypical bone or cartilage growth and development. Skeletal abnormalities include short stature, limb deformity, joint contracture, and spinal deformity. Over 90% of disorders have a known genetic mutation that can definitively determine the diagnosis. As patients may present with a primary spinal concern, a careful clinical and radiographic evaluation can allow the physician to develop a working diagnosis to guide additional evaluation. Spinal manifestations include scoliosis and kyphoscoliosis, cervical instability, cervical kyphosis, thoracolumbar kyphosis, spinal stenosis, and atypical vertebral body morphology. An understanding of the affected conditions, prevalence, and natural history of these radiographic findings aids the orthopaedic surgeon in establishing a diagnosis and guides appropriate orthopaedic care.

Use of Surgical Adjuvants Does Not Decrease Recurrence of Aneurysmal Bone Cysts in Surgical Intervention With Pediatric Patients.

BACKGROUND: Aneurysmal bone cysts (ABCs) are benign active tumors often requiring intralesional curettage with or without adjuvants. The primary aim of this study was to analyze whether recurrence is influenced by the use of surgical adjuvants in pediatric patients with ABCs. Secondary aims examined recurrence rates based on age, sex, and physeal contact. METHODS: A retrospective review was performed at a tertiary pediatric hospital from 2004 to 2020. Inclusion criteria consisted of patients treated surgically for histologically confirmed ABCs with a minimum of 6 months follow-up. Patients with treatment for a recurrent tumor or incomplete records were excluded. Patient demographics, location of the lesion, treatment technique, and incidence of recurrence were collected. Statistical analyses were performed using STATA. RESULTS: There were 129 patients (74 males and 55 females) with a mean age of 11.5 ± 4.1 years and an average follow-up of 29.0 ± 25.4 months. The most common locations for ABCs were the femur and tibia. Of the patients, 53.5% had tumors abutting the physis, 28.7% had no physeal contact, and 17.8% had insufficient imaging to evaluate physeal contact. Surgical adjuvants (high-speed burr, coagulation, liquid nitrogen, and/or hydrogen peroxide) were used in 91 of the 129 cases (70.5%). There was no significant difference in recurrence when comparing those who received an adjuvant and those who did not (25.3% vs 23.7%, P = 1.000). Physeal contact was also not significantly associated with recurrence ( P = 0.146). Finally, patients younger than 6 years old were significantly more likely to have recurrence compared with those 6 years old or older (66.7% vs 21.7%, P = 0.007). CONCLUSION: Our study found no association between the use of surgical adjuvants and the risk of recurrence after intralesional curettage for ABCs. Although our study did demonstrate that patients 6 years old or younger had an increased rate of recurrence, no significant association was found regarding physeal contact or sex. These data indicate that surgical adjuvant may not affect the recurrence rates of pediatric patients with ABCs. LEVEL OF EVIDENCE: Level III. This retrospective review compares rates of recurrence based on the choice of surgical adjuvant.

Kyphectomy in myelomeningocele revisited: risk factors for failure.

PURPOSE: Lumbar kyphosis occurs in approximately 8-20% of patients with myelomeningocele (MMC). The purpose of this article is to analyze the risks and benefits of vertebrectomy and spinal stabilization in MMC children with severe lumbar kyphosis and to establish treatment guidelines. METHODS: This is an IRB-approved retrospective analysis of 59 patients with MMC who underwent kyphectomy and posterior instrumentation in three centers. Average age at surgery was 7.9 years (2 weeks-17 years). Sitting trunk position, skin status, kyphosis angle, and thoracic lordosis were analyzed preoperatively, postoperatively, and at an average follow-up of 8.2 years (range 2.5-16). The correction was maintained by applying a short posterior instrumentation in 6 patients, and extending to the pelvis in 53 cases. Pelvic fixation was achieved using the Warner and Fackler technique in 24 patients, the Dunn-McCarthy in 8, Luque-Galveston in 8, sacral screws in 2, and ilio-sacral screws in 11. RESULTS: Sitting position improved postoperatively in 47 of the 53 patients who underwent pelvic fixation and only in one patient with short instrumentation. All 6 patients with long instrumentation and poor postoperative sitting balance were in the Dunn-McCarthy fixation group. Skin sores at the apex of the deformity disappeared postoperatively in all patients but recurred in two patients with short instrumentations. Kyphosis angle improved from 109° (45°-170°) preoperatively to 10° (0°-45°) postoperatively and 21° (0°-55°) at last follow-up. The best results were seen in cases where a cross-k-wire fixation of the kyphectomy site was used, augmented with a long thoraco-pelvic instrumentation consisting of Luque sublaminar wires in the thoracic region and a Warner-Fackler type of pelvic fixation. Good results were also found with the bipolar technique and ilio-sacral screw fixation. Six over 24 patients with the Warner and Fackler technique showed gradual dislodgment or hardware failure, with subsequent nonunion of the kyphectomy site in four. Infection, with or without wound dehiscence and/or hardware exposure, occurred in 17 cases, necessitating hardware removal in 9 patients. CONCLUSION: Lumbar kyphosis in MMC children is best managed by resection of enough vertebrae from the apex to produce a flat lumbar spine, with perfect bone-to-bone contact and long thoraco-pelvic instrumentation using the Warner and Fackler technique through the S1 foramina or the bipolar technique with ilio-sacral screw fixation. Additional local fixation of the osteotomy site using cross-wires with or without cerclage increases the stability of the construct. The majority of complications occurred in patients with short instrumentations or where residual kyphosis persisted postoperatively regardless of the type of pelvic fixation or hardware density. The Dunn-McCarthy technique for pelvic fixation following kyphectomy in MMC was less successful in producing stable pelvic fixation and should not be considered in this patient category.

Treatments and Sequelae of Pediatric Pathologic Proximal Femur Fractures Due to Benign Bone Cyst.

BACKGROUND: The proximal femur is a common location for pathologic fractures in children, yet there is little published information regarding this injury. The purpose of this study was to investigate the outcomes of pediatric pathologic proximal femur fractures due to benign bone tumors. METHODS: A retrospective review of patients treated for pathologic proximal femur fractures from 2004 to 2018 was conducted. Inclusion criteria were age below 18 years and pathologic proximal femur fracture secondary to a benign bone tumor. Patients were excluded if they had <1 year of follow-up. Medical charts and serial radiographs were reviewed for fracture classification, underlying pathology, treatment, complications, and time to fracture healing. RESULTS: A total of 14 patients were included. Mean age was 6±3 (3 to 11) years, and mean follow-up was 44±21 (22 to 86) months. Index treatment was spica casting in 9/14 (68%) patients, while 5/14 (32%) were treated with internal fixation. Of the 9 patients initially treated with casting, 22% (2/9) required repeat spica casting at a mean of 0.6 months after index treatment, 67% (6/9) required internal fixation at a mean of 20.3 months after index treatment, and 11% (1/9) did not require revision treatment. Eighty-eight percent (8/9) of patients treated with casting required revision treatment compared with 40% (2/5) of those treated with internal fixation (P=0.05). Nonunion occurred after 1 refracture, malunion with coxa vara occurred in 2 fractures, and the remaining 11/14 (84%) fractures had a union at a mean of 4.9±3.0 months All cases of malunion occurred in patients initially treated nonoperatively. There were 19 distinct complications in 10/14 (71%) patients. The incidence of any revision surgery was 64% (9/14). CONCLUSIONS: In this series, pediatric pathologic proximal femur fractures demonstrated prolonged time to union, high incidence of revision surgery (64%), and substantial complication rate (71%). In children with pathologic proximal femur fractures, treatment with internal fixation is recommended as this series showed a 78% failure rate of initial conservative management. LEVEL OF EVIDENCE: Level IV.

Using a dedicated spine radiology technologist is associated with reduced fluoroscopy time, radiation dose, and surgical time in pediatric spinal deformity surgery.

STUDY DESIGN: Retrospective comparative study OBJECTIVES: The goal of this study was to investigate fluoroscopy time and radiation exposure during pediatric spine surgery using a dedicated radiology technologist with extensive experience in spine operating rooms. Repetitive use of intraoperative fluoroscopy during posterior spinal fusion (PSF) exposes the patient, surgeon, and staff to radiation. METHODS: Retrospective review was conducted on patients with posterior spinal fusion (PSF) of ≥ 7 levels for adolescent idiopathic scoliosis (AIS) at a pediatric hospital from 2015 to 2019. Cases covered by the dedicated radiology technologist (dedicated group) were compared to all other cases (non-dedicated group). Surgical and radiologic variables were compared between groups. RESULTS: 230 patients were included. 112/230 (49%) were in the dedicated group and 118/230 (51%) were in the non-dedicated group. Total fluoroscopy time was significantly reduced in cases with the dedicated technologist (46 s) compared to those without (69 s) (p = 0.001). Radiation dose area product (DAP) and air kerma (AK) were reduced by 43% (p < 0.001) and 42% (p < 0.001) in the dedicated group, respectively. The dedicated group also had reduced total surgical time (4.1 vs. 3.5 h; p < 0.001) and estimated blood loss (447 vs. 378 cc (; p = 0.02). Multivariate regression revealed that using a dedicated radiology technologist was independently associated with decreased fluoroscopy time (p = 0.001), DAP (p < 0.001), AK (p < 0.001), surgical time (p < 0.001), and EBL (p = 0.02). CONCLUSIONS: In AIS patients undergoing PSF, using a dedicated radiology technologist was independently associated with significant reductions in fluoroscopy time, radiation exposure, surgical time, and EBL. This adds to the growing body of research demonstrating that the experience level of the team-not just that of the surgeon-is necessary for optimal outcomes. LEVEL OF EVIDENCE: III.

High Parental Anxiety Increases Narcotic Use in Adolescent Patients Following Spinal Fusion.

BACKGROUND: Parental mental status and behavior may influence postoperative recovery and the use of pain medication. The purpose of this study is to identify if parents with high anxiety are associated with prolonged narcotic use in adolescent patients following posterior spinal fusion surgery. Prolonged narcotic use in this study was defined as opioid use at their first postoperative visit. METHODS: AIS patients age 11 to 20 years undergoing posterior spinal fusion and a parent were prospectively enrolled. At the preoperative appointment, patients completed the Spence Children's Anxiety Scale and parents completed the State-Trait Anxiety Inventory. High parental anxiety was defined as 1 SD above the normative mean. At the first postoperative visit, patients were asked about medication use. RESULTS: A total of 58 patients (49 females and 9 males) were enrolled. Overall, 29% (17/58) of parents had a high general anxiety trait on the State-Trait Anxiety Inventory and 71% (41/58) had normal general anxiety. Of the patients whose parents had high general anxiety, 47% (8/17) were still taking narcotics at their first postoperative visit compared with 20% (8/41) of patients with normal anxiety parents (P=0.03). CONCLUSIONS: Patients with high general anxiety parents were more than twice as likely to still be on narcotics at their first postoperative visit. This information can be used to counsel families on the impact of anxiety on narcotic usage. LEVEL OF EVIDENCE: Level II-prognostic studies-investigating the effect of a patient characteristic on the outcome of the disease.

Surgical Outcomes, Complications, and Long-Term Functionality for Free Vascularized Fibula Grafts in the Pediatric Population: A 17-Year Experience and Systematic Review of the Literature.

BACKGROUND: In the pediatric population, bony defects of the extremities pose a significant challenge for which free vascularized fibular grafts (FVFGs) represent a valuable reconstructive option. The purpose of this study was to explore surgical outcomes, complications, and long-term functionality of FVFG for this patient group. METHODS: Using MEDLINE and PubMed databases, studies were identified of pediatric extremity reconstruction using FVFG which reported functional outcomes and/or complications. The operative logs at a tertiary pediatric center were then reviewed for cases of FVFG between January 2000 and 2017. Demographic characteristics, surgical indications, operative details, graft survival, bony union, complications, and functionality of the reconstruction were recorded. RESULTS: A total of 366 studies were identified with 23 ultimately meeting inclusion criteria in the systematic review. In the institutional series, 29 patients were included with mean age of 9.7 years (1-17 years). Indications for reconstruction included malignant bone tumor (n = 11), osteomyelitis (n = 9), congenital pseudoarthrosis (n = 6), and osteofibrous dysplasia (n = 3). Major postoperative complications included graft nonunion (24.1%), fracture (17.2%), and hardware failure (17.2%). Thirteen patients (44.8%) experienced delayed complications, while two (6.8%) experienced an immediate complication. Complications occurred in the donor site in 2 cases and the recipient site in all 13 cases. Long-term graft survival was achieved in 27 patients (93.1%), and 23 (79.3%) had full functional recovery, with an average Musculoskeletal Tumor Society score of 90% (60-100%). Mean follow-up was 5.17 years (2-12 years). CONCLUSION: This review and institutional series demonstrate the versatility of FVFG to facilitate limb reconstruction in large defects or serve as a salvage option in complex cases. While immediate and donor-site complications are uncommon, delayed recipient-site complications are frequent, and patients and families should be counseled regarding this expectation. Though technically challenging, excellent long-term graft viability and functional recovery can be achieved in the large majority of patients.

High Satisfaction in Adolescent Idiopathic Scoliosis Patients on Enhanced Discharge Pathway.

BACKGROUND: Recently, there have been several reports of using an enhanced discharge pathway following posterior spinal fusion (PSF) in patients with adolescent idiopathic scoliosis (AIS). No previous studies have prospectively examined patient satisfaction of patients with AIS using an enhanced discharge pathway. The purpose of this study was to evaluate patient satisfaction with an enhanced discharge pathway for PSF and whether patients felt that their length of stay was appropriate. METHODS: Patients with AIS undergoing PSF were prospectively enrolled. At their first postoperative clinic visit, patients were administered a survey regarding their experience. RESULTS: Of the 46 patients enrolled (mean age, 14 y), 1 was discharged on postoperative day (POD) 2, 33 were discharged on POD 3, 9 were discharged on POD 4, and 3 were discharged on POD 5. Eighty (37/46) of patients felt that they were discharged at an appropriate time, whereas 20% (9/46) felt they were discharged too early. Patients who felt they were discharged at an appropriate time (mean, 3.2 d) had a trend toward shorter stays than those who felt they were discharged too early (mean, 3.7 d). Overall patient satisfaction of hospital stay was high with a mean of 9 on a 10-point scale (range, 1 to 10). There was no correlation between length of stay and patient satisfaction (P=0.723). Patients who felt they were discharged early had a significantly higher mean FACES pain scores than those who felt they were discharged about right both as inpatients (mean, 4.8 vs. 3.4; P=0.0319) and at their first postoperative clinic visit (5.4 vs. 2.9; P=0.004). CONCLUSIONS: Eighty percent of patients with AIS who underwent PSF felt that the time of discharge was appropriate with an enhanced discharge pathway. There was no correlation between patient satisfaction and length of stay. LEVEL OF EVIDENCE: Level II.

Restricted Range of Motion and a Cold Upper Extremity in a Two-Year-Old Boy: Kaposiform Hemangioendothelioma of the Bone and the Brachial Plexus: A Case Report.

CASE: We report a case of kaposiform hemangioendothelioma (KHE) of the scapula in a 2-year-old boy with motor and sensory abnormalities of the left upper extremity, suggesting brachial plexus involvement. The locally invasive nature prevented resection; sirolimus therapy resulted in improvement of the motor and sensory impairment, as well as decreased tumor size on imaging. CONCLUSION: Osseous infiltration of KHE is known to occur, but its primary presentation in bone without skin involvement is rare and diagnostically challenging. Awareness of rare presentations of KHE, along with accurate histopathologic interpretation, is important to achieve a diagnosis and to differentiate KHE from more common vascular lesions (e.g., infantile hemangioma). Sirolimus therapy is emerging as a promising treatment for unresectable KHE.

Pain is the Greatest Preoperative Concern for Patients and Parents Before Posterior Spinal Fusion for Adolescent Idiopathic Scoliosis.

STUDY DESIGN: Prospective cross-sectional study. OBJECTIVE: To evaluate patients' and parents' concerns so they can be addressed with appropriate preoperative counseling. SUMMARY OF BACKGROUND DATA: Despite much research on outcomes for posterior spinal fusion (PSF) in adolescent idiopathic scoliosis (AIS), little is available about preoperative fears or concerns. METHODS: Patients with AIS undergoing PSF, their parents, and surgeons were prospectively enrolled and asked to complete a survey on their fears and concerns about surgery at their preoperative appointment. RESULTS: Forty-eight patients and parents completed surveys. Four attending pediatric spine surgeons participated and submitted 48 responses. Mean age of patients was 14.2 years. On a scale of 0 to 10, mean level of concern reported by parents (6.9) was higher than that reported by patients (4.6). Surgeons rated the procedure's complexity on a scale of 0 to 10 and reported a mean of 5.2. Neither patients' nor parents' level of concern correlated with the surgeons' assessment of the procedure's complexity level (R = 0.19 and 0.12, P = 0.20 and P = 0.42, respectively). Top three concerns for patients were pain (25%), ability to return to activities (21%), and neurologic injury (17%). Top three concerns for parents were pain (35%), neurologic injury (21%), and amount of correction (17%). Top three concerns for surgeons were postoperative shoulder balance (44%), neurologic injury (27%), and lowest instrumented vertebrae selection (27%). Patients reported the same concerns 23% of the time as parents, and 17% of the time as surgeons. Parents and surgeons reported the same concerns 21% of the time. CONCLUSION: Pain was the greatest concern for both patients and parents but was rarely listed as a concern by surgeons. Parent and patient level of concern did not correlate to the surgeon's assessment of the procedure's complexity. Neurologic injury was a top concern for all groups, but otherwise there was little overlap between physician, patient, and parent concerns. LEVEL OF EVIDENCE: 3.

Sacral Alar Iliac (SAI) Screws Fail 75% Less Frequently Than Iliac Screws in Neuromuscular Scoliosis.

BACKGROUND: Despite recent popularity of sacral alar iliac (SAI) screws for fusion to the pelvis for neuromuscular scoliosis, there are little data regarding the failure rate of this technique compared with traditional modes of iliac fixation. Theoretical advantages of the SAI screws are obviating the need for a rod to iliac screw connector and a lower implant profile. The purpose of this study is to determine whether SAI screws have fewer failures than iliac screws in neuromuscular scoliosis. METHODS: Review of neuromuscular patients treated with posterior spinal fusion with pelvic fixation from 2004 to 2012 with minimum 2-year follow-up was conducted. Medical records and imaging studies were reviewed. Patients were divided into 2 groups based on the type of pelvic fixation (SAI or iliac screws), and implant failures were compared between the groups. RESULTS: A total of 101 patients were reviewed, including 55 patients with iliac screws and 46 patients with SAI screws. Implant failures included: disengagement of the rod to iliac screw connector (10%, 10/101), separation of screw head from screw shaft (4%, 4/101), and set screw disengagement (2%, 2/101). The SAI group had a lower implant failure rate (7%, 3/46) compared with the iliac screw group (24%, 13/55) (P=0.031). Rod to iliac screw connectors failed in 18% (10/55) of patients. There were significantly less surgical revisions in the SAI group (2%, 1/46) for pelvic screw prominence compared with the iliac screw group (11%, 6/55) (P=0.027). CONCLUSIONS: SAI screws had a lower rate of implant failure and revision surgery compared with iliac screws. If rod to screw connector failures are excluded, the failure rate of SAI screws of 6.5% (3/46) is similar to that of iliac screws 5.5% (3/55); therefore, the most important advantage of the SAI technique may be obviating the need for a screw to rod connector. LEVEL OF EVIDENCE: Level III.

How do you know it is true? Integrity in research and publications: AOA critical issues.

High-quality medical care is the result of clinical decisions based upon scientific principles garnered from basic, translational, and clinical research. Information regarding the natural history of diseases and their responses to various treatments is introduced into the medical literature through the approximately one million PubMed journal articles published each year. Pharmaceutical and device companies, universities, departments, and researchers all stand to gain from research publication. Basic and translational research is highly competitive. Success in obtaining research funding and career advancement requires scientific publication in the medical literature. Clinical research findings can lead to changes in the pattern of orthopaedic practice and have implications for the utilization of pharmaceuticals and orthopaedic devices. Research findings can be biased by ownership of patents and materials, funding sources, and consulting arrangements. The current high-stakes research environment has been characterized by an increase in plagiarism, falsification or manipulation of data, selected presentation of results, research bias, and inappropriate statistical analyses. It is the responsibility of the orthopaedic community to work collaboratively with industry, universities, departments, and medical researchers and educators to ensure the integrity of the content of the orthopaedic literature and to enable the incorporation of best practices in the care of orthopaedic patients.

Simple steps to minimize spine infections in adolescent idiopathic scoliosis.

BACKGROUND: To examine the surgical site infection (SSI) rates in patients undergoing posterior spinal fusion surgery for adolescent idiopathic scoliosis (AIS) after implementation of a change in antibiotic prophylaxis and intraoperative irrigation. METHODS: A retrospective review of all consecutive spinal fusions for AIS from 1996 to 2008 was performed. In 2003, 2 changes in our protocol were implemented: (1) routine antibiotic prophylaxis was changed from cefazolin alone to vancomycin and ceftazidime; (2) intraoperative irrigation technique was changed from bulb syringe to pulse lavage irrigation. We compared the rates of deep SSI requiring irrigation and debridement before institution of these changes (1996 to 2002) to the rates after these changes (2003 to 2008). RESULTS: Before the change in the antibiotic and lavage regimen, 261 spinal fusions were performed. Of these, 28/261 (11%) patients underwent irrigation and debridement for SSI. The most common infecting pathogen was coagulase-negative Staphylococcus aureus (47%). Between the years 2003 and 2008, 263 spinal fusions were performed. Only 2/263 (0.7%) patients underwent irrigation and debridement for SSI. This decrease in infection rate is highly significant (P<0.001). CONCLUSIONS: Routine use of vancomycin and ceftazidime and pulsatile lavage for posterior spinal fusion in AIS patients decreased the rates of postoperative infection by 10 fold. As 2 variables were changed, it is impossible to know the relative effect of each. However, as spine infections can be so devastating, and the potential risks of these changes are small, we recommend both the new antibiotic and irrigation protocol. LEVEL OF EVIDENCE: Level III.