Use of pedicled flaps after oncologic resections in pediatric patients.

The aim of this study is to review the indications of pedicled flaps and analyze the results. A observational retrospective study of under 18-year-old oncology patients who required reconstructive surgery with pedicled flaps between 2011 and 2022 was performed. Demographic and clinical variables, indications, complications, and outcomes were collected. 236 patients were reviewed and 13 met inclusion criteria, eight girls and five boys (mean age: 10.6 years). Indications were Ewing's sarcoma (5), osteosarcoma (5), neuroblastoma, desmoid tumor, and neurofibroma. Preoperative PET-CT, MRI and bone scintigraphy were performed. The flaps were used on costal and extremity reconstruction: latissimus dorsi (5), pectoralis (2), medial gastrocnemius (2), combined latissimus dorsi, trapezius and serratus muscle, biceps femoris, fascio-neuro-cutaneous saphenous and cutaneous advancement-rotation. Two were performed on allograft and eight on prosthesis. All allowed immediate and complete closure. Six patients received intraoperative radiotherapy. One flap infection and two vascular complications were reported, a total necrosis, which required a new flap, and a partial necrosis, treated with a local plasty. Chemotherapy was resumed after 21 days (15-31). Mean follow-up time was 5.34 years. Flaps are an effective therapeutic option allowing reconstruction of large defects after pediatric oncologic surgeries. The most frequent complication was vascular.

Juvenile Xanthogranuloma as Differential Diagnosis of a Vulvar Mass: A Case Report.

Vulvar masses in children are an unusual finding but their differential diagnosis is extensive. In case of solid masses, rhabdomyosarcoma (RMS) must always be considered due to the fact that it is the most common tumor in external genitals during childhood. However, RMS has a radiological appearance very similar to juvenile xanthogranuloma (JXG). We present a 16-month-old girl with a 2 cm solid mass on her left labia majora, with four overlying cutaneous papules. After imaging tests, an excisional biopsy was programmed due to high malignancy suspicion. Histopathology of the mass and one of the papules was diagnostic for JXG. After a 12-month follow-up, the patient shows no signs of relapse or complication. Deep JXG is an uncommon entity in childhood and exceptional in the genital area. Therefore, it must be included in the differential diagnosis of a solid vulvar mass, especially if accompanying yellowish xanthomatous cutaneous lesions are present.

Management and results of thyroidectomies in pediatric patients with MEN 2 syndrome.

AIM OF THE STUDY: To evaluate the outcome of prophylactic thyroidectomies (PT) in patients with MEN 2 syndrome in a tertiary center. METHODS: A retrospective study was designed, including all patients with MEN 2 syndrome who underwent PT between 2000 and 2019. Demographics, gene mutation, postoperative complications and histopathological findings were registered. MAIN RESULTS: 30 patients were included (29 MEN 2A and 1 MEN 2B) with a median age at surgery time of 7.0 ± 3.2 years. Familiar history was present in all but 3 patients. A therapeutic thyroidectomy was performed in 2 patients due to evidence of medullary thyroid carcinoma (MTC, both were late diagnosis), and in the other 28 cases, a PT was performed. 8 patients had a RET mutation ranked as Moderate Risk (American Thyroid Association): median age at surgery was 7.2 ± 4.2 years, and histological findings were C-cell hyperplasia (n = 6) and no alterations (n = 2). 16 patients had a high risk mutation; median age at surgery time was 6.9 ± 2.8 years and histological findings were normal thyroid gland (n = 1), C Cell Hyperplasia (n = 8), microcarcinoma (n = 6), and MTC (n = 1). The mean hospital stay was 1.4 ± 0.68 days. No intraoperative complications or recurrent laryngeal nerve injuries were registered. 7 patients presented a transient hypoparathyroidism and 1 patient had permanent hypoparathyroidism. CONCLUSIONS: Early PT in patients with MEN 2 syndrome is a safe procedure when performed by an experienced team of Pediatric Surgeons and with a multidisciplinary approach. Early genetic analysis and familial counselling is essential to prevent the development of a MTC.

Complications of Central Venous Access Devices in Patients With Sickle Cell Disease and Thalassemia Major.

Pediatric patients with sickle cell disease and thalassemia major present clinical characteristics that could lead to a higher incidence of central venous access devices-associated complications (CVAD-C). With the objective of analyzing the safety of the use of CVAD in these patients, a retrospective review including all pediatric patients with these pathologies who required the implantation of a CVAD between 2004 and 2019 was performed. In all, 54 patients with 100 CVAD (65 totally implantable venous access port with subcutaneous reservoir, 35 single-lumen or double-lumen partially tunneled catheter) were included. During 60,410 days at risk of suffering a CVAD-C, 55 complications (complication rate [CR]/1000 catheter-days at risk=0.91) were reported in 46 CVAD: 19 mechanicals (CR=0.32), 32 infectious (CR=0.53), and 4 thrombotic complications (CR=0.066). Incidence of mechanical and infectious complications was significantly higher in double-lumen partially tunneled catheter than in totally implantable venous access port with subcutaneous reservoir (P<0.001). Lower age at insertion was related with a higher incidence of any complication (odds ratio=0.88/y, P=0.02). Patients who required a stem cell transplantation (31 patients and 65 CVAD) had no significant higher incidences of CVAD-C. In conclusion, our study supports the safety of using CVAD in these patients, with a low incidence of infectious, thrombotic, and mechanical complications.

Digital thoracic drainage: a new system to monitor air leaks in pediatric population.

INTRODUCTION: New digital thoracic drainage systems allow an objective measurement of air leakage. They have proven their usefulness in the postoperative thoracic surgery in adults, but there is little experience with its use in the pediatric population. The objective of our study is to analyze their safety and effectiveness in the postoperative period of the pediatric patients. METHOD: A prospective consecutive observational study was done. All patients submitted to pulmonary resection between 2011 and 2017 and in whom digital thoracic drainage system was used (Thopaz Chest Drain System, Medela, Switzerland) were prospectively enrolled in this study. We analyzed variables: duration of chest tube (CT), days of hospitalization and radiographs in the immediate postoperative period related to the presence of CT. This group was compared with a historical cohort of patients (from 2011 to 2015) with a pulmonary resection in whom the traditional thoracic drainage was used. For the statistical analysis, the Mann-Whitney U-Test was used for independent samples. RESULT: Twenty-six patients were included, Digital drainage system was used in13 patients and traditional drainage was used in 13 patients. The median age was 18 months (12 days-14 years). The mean number of days with the chest tube was 1.69 ±â€¯0.6 in digital drainage group versus 5.38 ±â€¯4 days in traditional drainage group (p < 0.05) The mean number of postoperative radiographs was 2.8 ±â€¯1.1 in digital drainage group versus 6.23 ±â€¯5.2 radiographs in traditional drainage group (p < 0.05). The average hospital stay in digital drainage group was 5.69 ±â€¯2.7 days versus 7 ±â€¯4.7 days in the traditional drainage group (p > 0.05). No complications related to the use of digital drainage group were registered. CONCLUSION: The digital thoracic drainage systems provide an objective measurement of air leakage, allowing early chest tube removal and decreasing the number of radiographs performed postoperatively. Its use in the pediatric population appears to be safe and potentially beneficial. LEVEL OF EVIDENCE: II.

Rinolitiasis: una entidad en el olvido / No disponible

Los rinolitos son masas nasales mineralizadas poco frecuentesque se producen como resultado de la calcificaciónde un cuerpo endógeno o exógeno en el interior de lafosa nasal. Presentamos un casos de rinolitiasis exponiendosu etiología, manifestaciones clínicas, hallazgosde la tomografía computerizada, así como su diagnosticodiferencial. Su tratamiento de elección es la extracciónquirúrgica. Hacemos hincapié en la rareza de esta enfermedad,así como en la importancia de los estudiosradiológicos en el diagnóstico de esta entidad tan olvidada

Rhinoliths are uncommon nasal mineralized massesresulting from calcification of an endogenous orexogenous nidus within the nasal cavity. The etiology,clinical presentation, CT findings and differential diagnosisin one cases of rhinolith are presented in thisreport. The treatment of choice is surgical removal. Weunderline the rarity of this patology an the importantrole of radiological studies in the diagnosis of such aforgotten entity

Purification of neutral protease by dye affinity chromatography.

Twenty triazinic dyes were assayed as ligands for the chromatographic affinity purification of a neutral protease from Flavourzyme, a commercial preparation. Screening at pH 4.0 allowed the selection of eight dyes on the basis of their high protease adsorption. When the pH was set to 5.0 in order to increase selectivity, only Yellow HE-4R, Red HE-3B, and Cibacron Blue F3G-A maintained protease adsorption at high values. Neither maximum capacities nor dissociation constants calculated from isotherms measured at 8 and 25 degrees C showed great differences. By contrast, a strong temperature effect was evidenced in the elution step: elution at 8 degrees C allowed 70, 81, and 98% recovery of adsorbed protease with Yellow HE-4R, Red HE-3B, and Cibacron Blue F3G-A, respectively, whereas only 20% recovery was attained at 25 degrees C. Based on the results obtained, a purification process for the neutral protease contained in Flavourzyme with Cibacron Blue F3G-A as the affinity ligand was developed, yielding 96% of electrophoretically pure enzyme in a single step, the specific activity rising from 850 to 3650 U/mg.

Cisplatino y 5-Fluorouracilo en perfusión continua de 96 horas para canceres recidivantes o metastasicos de cabeza y cuello / Cisplatine and 5-Fluorouracil in continuing perfusion during 96 hours to recurrents or metastasis in head and neck

A 36 pacientes con carcinoma epidermoide recurrente o metastásico de cabeza y cuello fueron tratados con cisplatino y 5-fluorouracilo, ambos en perfusión continua de 96 horas. Veintiocho presentaban recidiva local, seis metástasis y dos recidiva local y a distancia. Trece habían recibido previamente quimioterapia. Se consiguieron ocho respuestas completas (22 por ciento) y siete parciales (19 por ciento), con una respuesta objetiva del 41 por ciento. La supervivencia actuarial durante un periodo de seguimiento de 59 meses fue del 17 por ciento, con una supervivencia media de ocho meses. La supervivencia actuarial en los pacientes con respuesta completa fue del 50 por ciento a los 59 meses. Las manifestaciones de toxicidad más frecuentemente encontradas fueron estomatitis, leucopenia y vómitos. El esquema terapéutico resulta efectivo y beneficia principalmente a los pacientes con recidiva local, en los cuales es posible conseguir respuesta completa.