Unexpected findings in cervical spine in spondylometaphyseal dysplasia Sutcliff type FN1-related.

The autosomal dominant spondylometaphyseal dysplasia Sutcliff type or corner fracture type FN1-related is characterized by a combination of metaphyseal irregularities simulating fractures ("corner fractures"), developmental coxa vara, and vertebral changes. It is linked to heterozygous mutations in FN1 and COL2A1. Vertebral changes as delayed vertebral ossification, ovoid vertebral bodies, anterior vertebral wedging, and platyspondyly have been observed in this condition, while odontoid abnormalities have not been reported. We report an odontoid anomaly in a girl with SMD-CF FN1-related showing the heterozygous variant c.505T>A; p.(Cys169Ser), presenting at 11.9 years of age with acute quadriparesis. Images showed spinal cord compression and injury associated with os odontoideum and C1-C2 instability. She required decompression and instrumented occipitocervical stabilization, suffering from residual paraparesis. This paper describes the first case of SMD-CF FN1-related accompanied by odontoid anomalies.

Long-term follow-up after surgical treatment of adolescent idiopathic scoliosis using high-density pedicle screw constructs: Is 5-year routine visit required?

PURPOSE: The objective of this study is to determine whether routine follow-up 5 years after adolescent idiopathic scoliosis (AIS) surgery is likely to affect postoperative care for patients treated with high-density pedicle screw constructs, when routine 2-year follow-up has been performed. METHODS: We reviewed 80 patients undergoing surgery for AIS using high-density pedicle screw constructs and followed routinely 2 and 5 years after surgery. Quality of life (QOL) was assessed using the SRS-30 outcome questionnaire. Reoperations occurring between 2 and 5 years after surgery were identified. RESULTS: Curve correction and QOL were similar between 2- and 5-year visits. Two patients required revision surgery after presenting during unplanned visits between the 2- and 5-year follow-ups. One patient presented at the routine 5-year visit with an asymptomatic undisplaced rod fracture without loss of correction, and it was decided to follow-up only as needed. CONCLUSIONS: In AIS patients for whom routine follow-up 2 years after surgery using high-density pedicle screw constructs was uneventful, additional routine 5-year follow-up is not likely to affect postoperative care and revision rate. Patients developing complications and needing reoperation between 2 and 5 years after surgery will most likely present during unplanned visits rather than during routine follow-up appointments. Easy access to emergent visits on an as-needed basis is therefore important for this population if routine 5-year follow-up is not planned. These slides can be retrieved under Electronic Supplementary Material.

Hemivertebra Resection in Small Children. A Literature Review.

STUDY DESIGN: Systematic review. OBJECTIVE: To evaluate the lowest possible age to resect an HV in very young patients with a congenital deformity. METHODS: We sought to retrieve all studies reporting age at HV excision in patients with congenital scoliosis. Studies written in English were included. No publication date restrictions were imposed. A search of the PubMed and LiLacs databases was conducted. Additionally, a hand search was performed to supplement the database search. RESULTS: We found 140 articles. Twenty two studies were included into the final assessment. There was considerable heterogeneity in the included studies, both regarding age and the surgical techniques used. There was also a broad spectrum of recommendations regarding suggested age for treatment. The youngest patient undergoing resection and fusion was 3 months of life at the time of surgery. CONCLUSION: How young a patient could and should be submitted to HV resection surgery is still a matter of debate in the literature. Prophylactic surgery might be a proper treatment for young children with congenital scoliosis before malformation becomes a deformity, adding no additional neurological, vascular, or anesthesia-related complications. The defect can be treated early while the deformity is treated late.

Evaluation of outpatient halo-gravity traction in patients with severe scoliosis: development of a monitoring device.

INTRODUCTION: Patients with severe spinal deformities represent a major clinical and surgical challenge. Halo-gravity traction (HGT) is a traditional method to correct the deformity prior to surgery. Typically, children undergoing HGT remain in the hospital until surgery. Therefore, it has been suggested to treat these children at lower level healthcare centers or even at home. The aim of this study was to develop a tool to assess patient adherence to HGT together with a program to analyze traction results. MATERIALS AND METHODS: An original recording system was designed with an Arduino Nano®. The data extracted from the memory card were compiled into a text file and then analyzed with the MatLab R2018a MathWorks®. RESULTS: Five patients receiving HGT for severe scoliosis were asked to use the device both in the wheelchair and in bed to evaluate its usefulness. CONCLUSIONS: A device was developed to monitor the use of HGT at home. The device provides information on the time of HGT use and the traction weight placed throughout the day, as well as on the correct functioning of the system in bed and in the wheelchair.

Time to surgery for adolescent idiopathic scoliosis: How long does it take? A multicenter study.

Study design: Retrospective review of multicentric data. Objectives: To estimate the time from initial visit to surgery in adolescent idiopathic scoliosis (AIS) patients and the main reasons for the time to surgery in a multicenter study. Methods: This retrospective study evaluated 509 patients with AIS from 16 hospitals across six Latin American countries. From each hospital's deformity registry, the following patient data were extracted: demographics, main curve Cobb angle, Lenke Classification at the initial visit and time of surgery, time from indication-for-surgery to surgery, curve progression, Risser skeletal-maturity score and causes for surgical cancelation or delay. Surgeons were asked if they needed to change the original surgical plan due to curve progression. Data also were collected on each hospital's waiting list numbers and mean delay to AIS surgery. Results: 66.8% of the patients waited over six months and 33.9% over a year. Waiting time was not impacted by the patient's age when surgery first became indicated (p = 0.22) but waiting time did differ between countries (p < 0.001) and hospitals (p < 0.001). Longer time to surgery was significantly associated with increasing magnitude of the Cobb angle through the second year of waiting (p < 0.001). Reported causes for delay were hospital-related (48.4%), economic (47.3%), and logistic (4.2%). Oddly, waiting time for surgery did not correlate with the hospital's reported waiting-list lengths (p = 0.57). Conclusion: Prolonged waits for AIS surgery are common in Latin America, with rare exceptions. At most centers, patients wait over six months, most commonly for economic and hospital-related reasons. Whether this directly impacts surgical outcomes in Latin America still must be studied.

Neuromuscular lordoscoliosis: an unusual response to post-operative halo-gravity traction.

PURPOSE: To report the results of prolonged post-operative halo-gravity traction in a patient in whom the surgery had to be interrupted unexpectedly and for whom subsequently specific clinical circumstances contraindicated completion of the surgical procedure. METHODS: The patient was a 15-year-old male with severe cervico-dorsolumbar lordoscoliosis who was being studied for associated diffuse axonal injury. He performed halo-gravity traction for 12 weeks. Subsequent surgical management consisted of occipito-lumbar posterior instrumented fusion. During the surgical approach, electrocardiographic changes with hemodynamic decompensation were detected that did not improve with anesthetic reanimation. The intervention was stopped, the surgical wound was closed, and the patient was transferred to the intensive care unit (ICU). It was decided that a revision surgery with the aim to continue with the previous strategy would imply a high risk of perioperative morbidity and mortality. RESULTS: Orthopedic management was decided upon consisting of continued halo-gravity traction with wheelchair modification at home, which was extended to a period of 12 months because of the good results obtained in terms of cervicothoracic realignment. Two years after halo-gravity discontinuation, clinical and radiographic occipito-cervical alignment was good and the patient conserved certain occipito-cervical range of motion and had the capacity of maintaining a horizontal gaze. CONCLUSION: We considered the outcome extraordinary and relevant in this complex and unusual patient. A longer follow-up will provide more data regarding the final outcome of this treatment.

Infection Recurrence in Instrumented Spinal Fusion in Children.

STUDY DESIGN: Level 4 retrospective case series. OBJECTIVES: Surgical site infection (SSI) is one of the main complications of instrumented spinal fusion. The aim of our study was to evaluate infection recurrence (same bacteria) or reinfection (different bacteria) in posterior spinal fusion in children. METHODS: A retrospective study was conducted to evaluate patients who were successfully treated for SSI after instrumented spinal fusion due to deformity, with irrigation and debridement (I&D) procedures, followed by antibiotic therapy, with a follow-up of at least 2 years. RESULTS: Overall, 29 patients with a mean age of 14 + 3 years were evaluated. Preoperative diagnosis was nonidiopathic scoliosis in 23, idiopathic scoliosis in 5, and Scheuermann's disease in 1 patient. The etiology was Gram-positive cocci (40.9%), Gram-negative bacilli (27.2%), and polymicrobial infection (31.8%). A mean of 1.5 (1-3) I&D procedures were performed. Intravenous antibiotic treatment was given for a mean of 15.8 (4-86) days, followed by oral treatment for a mean of 335.1 (0-1095) days. Mean follow-up was 5 + 2 years (2 to 14 + 7 years) during which 28 patients were cured (96.6%) and 1 patient developed reinfection (3.4%). This reinfection was treated with oral clindamycin for 6 months. After the infection persisted, the decision was to remove the implants. CONCLUSIONS: In this series of 29 pediatric patients who underwent instrumented spinal fusion due to deformity, we reported one case of reinfection (3.4%). Given that 96.6% of infections were resolved, we suggest treatment with I&D procedures with retention of implants to treat acute SSI.

Selective Fusion in Lenke 1 B/C: Before or After Menarche?

STUDY DESIGN: A retrospective, comparative study. OBJECTIVE: To determine the radiological behavior of the lumbar curve in selective fusions in premenarchal girls with adolescent idiopathic scoliosis (Lenke 1 B/C). METHODS: A retrospective, comparative study was conducted. Selective fusion was performed in 21 patients younger than 18 years. The patients were divided into 2 groups: group A, after menarche (n = 12) and group B, before menarche (n = 9). Angles (preoperative, and at 1 and 2 years postoperatively) of the fused thoracic curves and the corresponding lumbar curves were measured and compared. For statistical analysis, the t test was used with a significance level of P < .05. RESULTS: Mean preoperative angle value of the proximal/main thoracic curve was 61° in group A and 57° in group B (P = .44), and 21° and 20°, respectively, in the first year postoperatively (P = .61). Mean preoperative angle value of the lumbar curve was 43° in group A and 42° in group B (P = .87), while at 1 year after surgery, this curve was 19° in both groups (P = 0.91), and at 2 years postoperatively, the curve was 16° in group A and 17° in group B (P = .75). CONCLUSIONS: Over a 2-year follow-up, we did not find significant radiological differences in lumbar curves between patients who underwent surgery before and after menarche.

Surgical treatment of segmental spinal dysgenesis: a report of 19 cases.

STUDY DESIGN: Retrospective review. OBJECTIVE: To describe clinical presentation, surgical management, long-term results, and complications in patients with segmental spinal dysgenesis (SSD). In addition, we sought to emphasize early surgery for this complex congenital abnormality. SSD is a rare congenital malformation characterized by focal stenosis, spinal subluxation, kyphosis, and absence of the nerve roots. Neurologic function ranges from normal to complete paraplegia. Progression of the deformity and neurologic deterioration is the rule. METHODS: An independent spinal surgeon reviewed the complete records of 19 patients with SSD, between 1998 and 2015 at a single institution. Mean follow-up was 10 years and 6 months (2-14 years). RESULTS: We evaluated 11 males and 8 females, with a mean age of 2 years and 9 months (5 months-15 years). The dysgenetic segment involved an average of 2.9 vertebrae (1-5); the upper thoracic region was most commonly involved in ten cases. Fifteen patients had severe spinal stenosis. 14 patients presented neurological deficits and 15 patients had associated organ and musculoskeletal anomalies.Twenty-seven surgeries were performed, a mean of 1.76 procedures (1-5) to obtain solid fusion. Neurologic function improved in four, deteriorated in three, and remained unchanged in 12 patients Seven complications were recorded. CONCLUSION: We strongly recommend decompression and fusion as soon as possible to preserve or prevent neurologic deterioration. Although challenging, it was possible to achieve a solid instrumented fusion in all cases; however, a high rate of patients may deteriorate or not recover neurological status after surgery. LEVEL OF EVIDENCE: Level IV evidence.

Long-term results of hemivertebra excision: How does the spine behave after the peak of puberty?

INTRODUCTION: Early hemivertebra (HV) excision and posterior spinal fusion (PSF) is advocated as the treatment of choice in congenital kyphoscoliosis. We assessed global spinal balance and spinal deformity status once the pubertal peak has taken place, in children with a history of HV excision and PSF at age younger than 5 years. METHODS: Twenty-seven children with congenital kyphoscoliosis without co-existing proximal/distal congenital spinal abnormalities who underwent HV excision and PSF of ≤ 5 levels at age younger than 5 years and who had reached the peak of puberty at the last follow-up visit were evaluated. RESULTS: Twenty-seven HV excision were performed. Mean age at surgery was 3 years and 2 months. A mean of 3.2 segments were fused. Imbalance of the trunk was observed in 80% of thoracic and 75% of thoracolumbar HV excision. No arthrodesis technique (4 cases) presented 100% of spine decompensation. All children younger than 2 years at the time of surgery developed spinal imbalance. Eighteen patients (67%) had global spine imbalance; 81% male population and 63% of the female population. Mean age at surgery was 3 years + 2 months. Mean age of the patients at the time of the study was 15 years + 5 months. Mean follow-up was 12 years + 3 months. CONCLUSION: Many of these patients developed spinal imbalance and scoliosis worsened at the final follow-up. Early age at surgery, preoperative scoliosis severity, HV location, no arthrodesis technique, and the adding-on phenomenon may be involved.

Histiocytosis in the pediatric spine: a clinical and radiographic analysis of 50 patients.

STUDY DESIGN: Descriptive, retrospective. Scientific level of evidence IV. OBJECTIVES: The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine. MATERIAL AND METHODS: A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed. RESULTS: Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2-12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment. CONCLUSIONS: Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

A few challenges in mucopolysaccharidosis type I. / Algunos desafíos en mucopolisacaridosis tipo I.

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care.

Se describen como desafíos actuales en mucopolisacaridosis I la necesidad de una clasificación adecuada, vinculándola a las indicaciones terapéuticas; el diagnóstico temprano desde la pesquisa neonatal, sus ventajas y dificultades hasta la sospecha clínica de las formas grave y atenuada; el cuidado de la patología espinal y oftalmológica, desde el diagnóstico, el seguimiento y el tratamiento; las reacciones alérgicas por terapia de reemplazo enzimático, su diagnóstico y tratamiento. Por último, la transición hacia el cuidado adulto.

New recommendations for the care of patients with mucopolysaccharidosis type I. / Nuevas recomendaciones para el cuidado de los pacientes con mucopolisacaridosis tipo I.

Considering the advances made on mucopolysaccharidosis type I after the consensus study published by a group of experts in Argentina in 2008, recommendations about genetic testing, cardiological follow-up, airway care, hearing impairment detection, spinal and neurological conditions, as well as current treatments, were reviewed. Emphasis was placed on the need for early diagnosis and treatment, as well as an interdisciplinary follow-up.

Dados los avances sobre mucopolisacaridosis I con posterioridad al consenso publicado en la Argentina por un grupo de expertos en 2008, se revisan recomendaciones respecto a estudios genéticos, seguimiento cardiológico, cuidado de la vía aérea, alertas sobre aspectos auditivos, de la patología espinal y neurológica. Se hace revisión de la terapéutica actual y se enfatiza en la necesidad de un diagnóstico y tratamiento precoces, así como de un seguimiento interdisciplinario.

Antibiotic prophylaxis in high-risk pediatric spine surgery: Is cefazolin enough?

STUDY DESIGN: Retrospective study. OBJECTIVE: To describe pathogens found in SSI during pediatric-instrumented spine surgery, and to assess the relationship between pathogens and the etiology of the spinal deformity. Surgical site infection (SSI) after pediatric spine fusion is a well-known complication with incidence rates between 0.5 and 42%, associated with the patient underlying disorder. Pathogens involved in SSI seem to be related to patient characteristics, such as the etiology of the spinal deformity. GNB (gram-negative bacilli) are more frequent in neuropathic, muscular, and syndromic conditions. High-risk pediatric patients with a spine deformity undergoing instrumented surgery might benefit from receiving perioperative intravenous prophylaxis for GNB. METHODS: We conducted a retrospective study at our tertiary-care pediatric hospital from January 2010 to January 2017. We reviewed records of all episodes of SSI that occurred in the first 12 months postoperatively. All patients who underwent instrumented spine surgery were included in this study. RESULTS: We assessed 1410 pediatric-instrumented spine surgeries; we identified 68 patients with deep SSIs, overall rate of 4.8%. Mean age at instrumented spine surgery was 12 years and 9 months. Time elapsed between instrumented surgery and debridement surgery was 28.8 days. Cultures were positive in 48 and negative in 20. Of the 48 positive culture results, 41 (72%) were GNB, 12 (21%) gram-positive cocci (GPC), three (5%) gram-positive anaerobic cocci (GPAC), and one (2%) coagulase-negative staphylococci (CoNS). Of the 68 patients with primary SSIs, 46 were considered to have a high risk of infection, which reported GNB in 81%, GPC in 15%, GPAC in 2%, and CoNS in 2%. CONCLUSION: Cefazolin prophylaxis covers GPC and CoNS, but GNB with unreliable effectiveness. Gram-negative pathogens are increasingly reported in SSIs in high-risk patients. Adding prophylaxis for GNB in high-risk patients should be taken into account when considering spine surgery. LEVEL OF EVIDENCE: IV.

Aneurysmal Bone Cysts of the Spine in Children: A Review of 18 Cases.

STUDY DESIGN: Retrospective study. OBJECTIVE: The aim of this study was to evaluate the clinical presentation, treatments, outcome, complications, and recurrence rate in the surgical and nonsurgical management of spinal aneurysmal bone cyst (ABC) in a series of 18 pediatric patients. METHODS: Between 1988 and 2014, we evaluated 18 pediatric patients diagnosed with ABC confirmed by pathology studies. We analyzed clinical and radiological features, non-surgical and surgical treatment, outcome, and complications. RESULTS: The series included 12 male and 6 female patients with a mean age of 10 years and 4 months, with a mean follow-up of 5 years. Location of the ABC was lumbar in 8, cervical in 7, thoracic in 2, and sacral in 1 case. Axial pain was the most common symptom followed by radicular involvement. Surgery was performed in the presence of spine instability or neurological involvement (tumor resection) and in the remaining, nonsurgical treatment (percutaneous intralesional injection of methylprednisolone and calcitonin). Recurrence was observed in 4 patients requiring reintervention. There were no procedure-related complications. CONCLUSION: In patients without neurological involvement or spinal instability, nonsurgical treatment is the treatment of choice. Total or subtotal removal combined with posterior instrumented spinal fusion is recommended in cases with a neurological deficit. Both procedures have shown good long-term results.

Proximal junction kyphosis after posterior spinal fusion for early-onset scoliosis.

STUDY DESIGN: Retrospective study. OBJECTIVE: The aim of this study was to assess the presence of proximal junctional kyphosis (PJK) in our population of children with early-onset scoliosis (EOS) and to identify the predisposing factors for the development of PJK in the postoperative period after posterior spinal fusion (PSF). Few studies have been conducted to evaluate the incidence of proximal junction kyphosis (PJK) in children after early-onset scoliosis (EOS) after posterior spinal fusion (PSF). MATERIALS AND METHODS: Overall, 114 pediatric patients aged < 10 years who underwent surgery for scoliosis or kyphoscoliosis at a single center between 2013 and 2015 were evaluated. Forty-five patients submitted to PSF of five or more levels met the inclusion criteria. The sample included 12 female and 10 male patients. Mean age at surgery was 7 years and 8 months. RESULTS: PJK was observed in 22 patients (48.9%). Overall, the mean proximal junctional angle at 12 and 36 months was 17.1° and 22°, respectively. The uppermost instrumented vertebra (UIV) with the highest PJK rate was T6-T7. The lowest instrumented vertebra (LIV) with the highest PJK rate was L2. Etiology was idiopathic in 4, neuromuscular in 11, congenital in 14, and syndromic in 16. According to underlying disorder, prevalence of PJK was 78% in those with a congenital, 50% in those with a syndromic, 12% in those with idiopathic, and 9% in those with a neuromuscular EOS. Surgical revision rate was 4% (one patient). Mean postoperative follow-up was of 3 years and 4 months (range 3-4 years and 1 month). CONCLUSION: Congenital and syndromic etiology, but not age at PJK onset or sex of the patient, significantly affected the incidence rate of PJK. The UIV with the highest PJK rate was T6-T7 and the LIV with the highest PJK rate was L2. The patients had a low surgical revision rate. LEVEL OF EVIDENCE: Level IV.

Scoliosis in spinal muscular atrophy: is the preoperative magnetic resonance imaging necessary?

PURPOSE: To determine the prevalence of intraspinal alterations in scoliosis due to Spinal Muscular Atrophy (SMA). METHODS: Cross-sectional, observational, descriptive study. Fifty-six patients with SMA diagnosis required surgical treatment due to scoliosis. INCLUSION CRITERIA: scoliosis/kyphoscoliosis > 50 degrees in the coronal plane, clinical characteristics of Spinal Muscular Atrophy, accurate diagnosis by means of molecular or genetic study. Prior to the spinal surgery, and to find related intraspinal alterations, MRI of the spine and posterior cranial fossa was performed. RESULTS: Forty females, 16 males, mean age 11 years (range 6-14 years). 94% of the patients had Spinal Muscular Atrophy type 2. The mean angle value was 81 degrees (range 53-122 degrees) in the coronal plane and 62 degrees (range 35-80 degrees) in the sagittal plane. The prevalence of intraspinal alterations was 1.78%. One patient with cervical hydromyelia and no neurological surgical procedure prior to the spinal deformity surgery was reported. CONCLUSIONS: In the context of preoperative planning and strategy of patients with scoliosis due to Spinal Muscular Atrophy, MRI may have not to be requested.

Inpatient versus outpatient halo-gravity traction in children with severe spinal deformity.

STUDY DESIGN: A retrospective, comparative study. OBJECTIVE: To compare the results, complications, and costs of preoperative halo-gravity traction in in- and outpatient settings. BACKGROUND DATA: Surgical management of severe spinal deformities remains complex and controversial. Preoperative halo-gravity traction results in a decreased need for aggressive surgical techniques, lower incidence of intraoperative neurologic complications, and improvement of nutritional parameters and preoperative cardiopulmonary function. METHODS: Twenty-nine patients younger than 18 years with kyphoscoliosis undergoing preoperative halo-gravity traction were divided into two groups: inpatients (n: 15) and outpatients (n: 14, home care or care at the Foundation). Traction time (weeks), traction weight (kg), radiographic curve correction, complications, and costs were compared. For statistical analysis, t test and odds ratio were calculated with a significance of p < 0.05. RESULTS: Mean traction time was 6 weeks for in- and 4 weeks for outpatients (p = 0.038). Initial traction weight was 6 kg in both groups, while final traction weight was 13 kg for in- and 15 kg for outpatients (p = 0.50). At the end of the traction period, coronal correction was 24° in in- and 28° in outpatients (p = 0.5), while sagittal correction was 27° and 29°, respectively (p = 0.80). Pin loosening was observed in 2 patients in each group, of whom 1 outpatient developed pin-site infection. In each group, one patient developed transient neurologic complications (odds ratio 1.091). Mean treatment cost per patient was 2.8-fold higher in inpatients. CONCLUSIONS: Considering complications and costs, our results show that preoperative halo-gravity traction in an outpatient setting is an option to be taken into account. LEVEL OF EVIDENCE: Grade III.

Clinical Manifestations and Surgical Management of Spinal Lesions in Patients With Mucopolysaccharidosis: A Report of 52 Cases.

STUDY DESIGN: Descriptive retrospective cohort of 52 pediatric patients with mucopolysaccharidosis (MPS) and spinal cord disease and surgical outcomes in a reference hospital. OBJECTIVES: To describe clinical manifestations and surgical management and outcomes of spinal lesions. METHODS: All medical records of 52 patients with mucopolysaccharidosis (I, II, III, IV, and VI) diagnosed between 1992 and 2011 were identified and followed at a single spine center of a pediatric hospital. Demographic, clinical manifestations, spinal cord lesions, and surgical management were the focus of the descriptive report. RESULTS: A total of 52 patients (32 males and 20 females), mean age at diagnosis of 8 ± 4 years (range 1-19), and with a mean follow-up of 11 ± 8 years were identified. Forty-three had cervical disease (the most frequent affection found was odontoid hypoplasia followed by atlantoaxial instability) and 14 patients had thoracolumbar kyphosis. Twenty-one patients presented neurologic compromise before surgery (quadriparesis as the most frequent manifestation), with the progression of neurologic impairment being the most common surgical indication. Surgery was performed in 38 patients (25 cervical and 13 thoracolumbar). Of the 21 patients with preoperative neurologic deficit, 6 patients showed neurologic improvement. The most common surgical complication found was proximal junctional kyphosis. CONCLUSION: This is the largest series published of mucopolysaccharidosis pediatric patients with a surgically treated spinal condition. We recommend early spinal cord decompression in mucopolysaccharidosis spine pathology to prevent or potentially reverse neurologic impairment. LEVEL OF EVIDENCE: Level IV.

When and how to discontinue bracing treatment in adolescent idiopathic scoliosis: results of a survey.

BACKGROUND: Currently, there is little consensus on how or when to discontinue bracing in adolescent idiopathic scoliosis (AIS). An expert spine surgeon national survey could aid in elucidate discontinuation of the brace.Few data have been published on when and how to discontinue bracing treatment in patients with AIS resulting in differences in the management of the condition. The aim of this study was to characterize decision-making of surgeons in the management of bracing discontinuation in AIS. METHODS: An original electronic survey consisting of 12 multiple choice questions was sent to all the members of the National Spine Surgery Society (497 surveyed). Participants were asked about their type of medical practice, years of experience in the field, society memberships, type of brace they usually prescribed, average hours of daily brace wearing they recommended, and how and when they indicated bracing discontinuation as well as the clinical and/or imaging findings this decision was based on. Exclusion criteria include brace discontinued because of having developed a curve that warranted surgical treatment. RESULTS: Of a total of 497 surgeons, 114 responded the survey (22.9%). 71.9% had more than 5 years of experience in the specialty, and 51% mainly treated pediatric patients. Overall, 95.5% of the surgeons prescribed the thoracolumbosacral orthosis (TLSO), indicated brace wearing for a mean of 20.6 h daily. Regarding bracing discontinuation, indicated gradual brace weaning, a decision 93.9% based on anterior-posterior (AP) and lateral radiographs of the spine and physical examination, considered a Risser ≥ IV and ≥ 24 months post menarche. CONCLUSIONS: The results of this study provide insight in the daily practice of spine surgeons regarding how and when they discontinue bracing in AIS. The decision of bracing discontinuation is based on AP/lateral spinal radiographs and physical examination, Risser ≥ IV, regardless of Tanner stage, and ≥ 24 months post menarche. Gradual weaning is recommended.