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.

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.

Manejo del dolor posoperatorio por cirugía de columna en la población pediátrica: Resultados preliminares / Pain management following spinal surgeries in pediatric patients. Preliminary results

Introducción: Aproximadamente el 80% de los pacientes pediátricos sometidos a cirugía de columna refiere dolor moderado o severo en el posoperatorio inmediato. Objetivos: Describir la presencia de dolor y su intensidad durante el posoperatorio de una cirugía de columna en pacientes pediátricos y evaluar la eficacia del tratamiento analgésico actual. Materiales y Métodos: Se evaluaron pacientes sometidos a cirugía de columna, de entre 10 y 18 años, con capacidad mental para entender el propósito del estudio. Se utilizó el esquema de medicación estándar actual posoperatorio del hospital. Se constataron la presencia del dolor, la hora de comienzo, la localización y la intensidad (escala de valoración numérica), entre otras variables. Resultados: Se evaluó a 84 pacientes pediátricos, con una edad promedio de 12 años y 9 meses, al momento de la cirugía. Las etiología fueron: idiopática (41 casos), neuropática (14 casos), sindrómica (10 casos), muscular (7 casos) y misceláneas (12 casos). El puntaje promedio en la escala de valoración numérica fue de 2,1 antes de la cirugía; de 7,3 el día 1 posoperatorio y de 3,3 el día 6 posoperatorio/alta. El tiempo promedio de internación fue de 6.3 días, el costo económico global de la internación fue de USD1090 por paciente. Conclusiones: Un importante porcentaje de pacientes pediátricos refiere dolor moderado o severo en el posoperatorio de una cirugía de columna, tan solo la mitad recibe un esquema de manejo de dolor estandarizado y adecuado. El objetivo ulterior de este estudio será protocolizar el manejo del dolor con un enfoque multimodal. Nivel de Evidencia: III

Introduction: Around 80% of pediatric patients who undergo spine surgery report moderate to severe pain in the immediate postoperative (POP) period, and only half of them are treated according to an adequate and standardized pain management scheme. Objectives: To describe the type and intensity of POP pain in children who underwent spinal surgery and evaluate the effectiveness of the current pain management protocol. Materials and Methods: We assessed children between 10 and 18 years of age who were able to understand the purpose of the study. We recorded presence of pain, time of POP pain onset, location, and referred intensity of the pain using a numeric rating scale (NRS), among other variables. Results: Overall, 84 pediatric patients were evaluated. Mean age at surgery: 12 years and 9 months. Etiology: idiopathic (41 patients), neuropathic (14 patients), syndromic (10 patients), muscular (7 patients), and miscellaneous (12 patients). Mean preoperative NRS was 2.1 and 7.3 on POP day 1, 6.6 on POP day 2, 6.2 on POP day 3, 5.1 on POP day 4, 3.7 on POP day 5, and 3.3 on POP day 6/at discharge. Mean hospital stay was 6.3 days. The mean cost of hospital stay was USD 1090 per patient. Conclusions: A high percentage of pediatric patients who underwent spinal surgery reported moderate to severe pain in the POP period, and just half of them received a standardized pain management protocol. A pain management protocol with a multimodal focus should be considered in a near future. Level of Evidence: III