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.

Gynecological anomalies in patients with anorectal malformations.

PURPOSE: The association of gynecological anomalies in all anorectal malformations (ARM) is firmly established. Our goal is to study this pathology in our patients to focus attention to this important issue. METHODS: Retrospective study of female patients operated for ARM and who underwent magnetic resonance imaging in our center. The type of malformation, the presence and type of vaginal, uterine, tubaric and urological anomalies were studied. RESULTS: 63 patients were included: 34.9% cloaca, 28.6% vestibular and 12.7% perineal. Half of patients had some type of müllerian anomaly; 19 vaginal, most frequent being the longitudinal vaginal septum (66.7%); 30 had uterine alterations, most frequent being the uterus didelphys (60%). Eighty percent of patients with complex ARM (cloaca, exstrophy) presented some type of gynecological malformation compared to 21.8% found in simple ARM (stenosis, perineal, vestibular) (p < 0.001). Vaginal anomalies are associated with a uterine anomaly in 100% of cases. Conversely, patients with uterine anomalies have concurrent vaginal anomaly in 63.3% of cases. CONCLUSION: Screening for gynecological anomalies is indicated in all patients with ARM. We recommend a vaginal examination in any girl with ARM during definitive repair and a subsequent MRI during follow-up. Collaboration with a gynecologist is essential.

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.

Corynebacterium pseudotuberculosis Infection in Patagonian Huemul ( Hippocamelus bisulcus ).

Corynebacterium pseudotuberculosis is an intracellular bacteria and the etiologic agent of caseous lymphadenitis in domestic and wildlife species. We report C. pseudotuberculosis infection in Patagonian huemul ( Hippocamelus bisulcus ) from the Cerro Castillo National Reserve, Region of Aysen, Chile. Subcutaneous abscesses in the abdominal and pectoral regions from two animals were sampled and bacteriologic isolation was performed. In both cases, we isolated a C. pseudotuberculosis strain belonging to the ovine genotype. In addition, one isolate was resistant to ciprofloxacin and streptomycin. We report that H. bisulcus is a susceptible species to this bacterium, which is transmitted by direct or indirect contact with domestic sheep ( Ovis aries ) and which represents a potential conservation threat to populations of H. bisulcus . Additional research and prevention efforts should be addressed.

Three-Dimensional Custom-Made Titanium Ribs for Reconstruction of a Large Chest Wall Defect.

Reconstruction of large chest wall defects always demand surgeons of having lots of means available (both materials and resourceful) to apply a cover to chest wall defects which can range from a few centimeters to the lack of a few entire ribs. In this study, we present the case of a teenager who suffered from a complete resection of three ribs because of Ewing sarcoma dependent on the sixth rib. Given the size of the defect, a multidisciplinary approach was chosen to provide rigid and soft tissue coverage and minimal functional and aesthetic impact. Custom-made titanium implants were designed based on three-dimensional computed tomography scan reconstruction. The surgical specimen via a left lateral thoracotomy (fifth, sixth, and seventh entire ribs) was resected, leaving a defect of 35 × 12 × 6 cm. A Gore-Tex patch (W. L. Gore & Associates, Arizona, United States) was placed and, after that, the implants were anchored to the posterior fragment of the healthy ribs and to the costal cartilage anteriorly. Finally, the surgical site was covered with a latissimus dorsi flap. The postoperative course was uneventful. After 9 months of follow-up, the patient has full mobility. This case shows that the implant of custom-made ribs, combined with other techniques, is a good surgical choice for reconstruction of large chest wall defects. The implant of custom-made ribs, combined with other techniques, is a good surgical choice for reconstruction of large chest wall defects.

Acute gastric volvulus and congenital diaphragmatic hernia, case report and review.

Congenital diaphragmatic hernia (CDH) is the result of the incomplete fusion and closure of the pleuroperitoneal canal during the fetal development. CDH is usually diagnosed prenatally but, if undiagnosed, the clinical presentation ranges from asymptomatic children to serious respiratory or gastrointestinal symptoms. Acute gastric volvulus associated with CDH is a rare surgical emergency in children. We report two cases of acute gastric volvulus associated with CDH and review the literature.

Efecto de la hemorragia aguda en un modelo experimental de trauma / Effects of acute hemorrhage in a experimental trauma model

Mediante la utilización de un modelo establecido de trauma se evaluó la asociación de la hemorragia aguda comprometió 30% del volumen sanguíneo circulante en animales de experimentación. A las 24 horas de ser introducidas las variables se constató una significación reducción de la inmunidad celular al cuantificarse la incorporación de Thymidina en los linfocitos T esplénicos, se evidenció además una disminución significativa en la capacidad de respuesta ante un proceso séptico al inducir una sepsis de origen intra-abdominal. A nivel intestinal, la presencia de edema y áreas focales de fragmentación en la mucosa intestinal permiten el paso de bacterias a través de ella, alcanzando los nódulos linfáticos mesentéricos. De este estudio se concluye que la asociación de estas dos variables ocasionan alteraciones importantes sobre el sistema inmune, incrementando la susceptibilidad a desarrollar infecciones e inducen el fenomeno de translocación bacteriana. Este último ha sido implicado en la etiología de la sépsis y la falla de múltiples sistemas debido a la reacción inflamatoria producida por los micro-organismos que translocan en los diferentes órganos de la economía

Efecto del ácido ascórbico en un modelo experimental de sepsis de origen abdominal / Effect of ascorbic acid in an experimental sepsis model of abdominal origin

Ciento treinta y nueve ratones de la cepa Balb-C con edades comprendidas entre las 8 y 12 semanas fueron sometidos a sepsis intra-abdominal utilizando el modelo experimental de ligadura y perforación del ciego. Los ratones fueron divididos en un grupo control el cual recibió solución salina normal y dos grupos experimentales que recibieron ácido ascórbico a las dosis de 30 y 60 mg/kg/día. El ácido ascórbico fue administrado en pequeños volúmenes por vía intra-peritoneal inmediatamente después de producirse la ligadura y perforación del ciego y durante los dos días subsiguientes. La administracción de ácido ascórbico incrementó de forma significativa la sobrevida ante la sépsis, siendo la mortalidad del 100% en el grupo control, comparado con 79.7 y 72.5% en los grupos experimentales que recibieron ácido ascórbico a los 30 y 60 mg/kg/día respectivamente alcanzando una significancia estadística con un valor de P < 0.05 en ambos casos

Papel de los murciélagos en el ciclo silvestre de la infección chagásica en la Guayana Venezolana / The role of bats in the life cycle of chagasic infection in the Venezuelan Guiana

A total of 44 wild bats from El Manteco (Bolívar State) were examined for trypanosomes, using three different sensitive parasitological methods. Trypanosoma cruzi like parasites were not found in Artibeus, Carollia. Mimon and Desmodus species which suggest a minimal role of bast in selvatic transmission cycles of Chagasic infection in Venezuelan Guiana

Estudio comparativo de reticulina en el granuloma de formas clínicas de lepra humana y lepra experimental del armadillo / Comparative study of reticulin in the granulome of clinicals forms of human and experimental leprae of armadillo

Se practican biopsias de piel y se tiñen con la coloración para retículo (método de Gomori) en 30 pacientes con Enfermedad de Hansen en sus diferentes formas clínicas, observándose la reticulina conservada en zonas donde el macrófago está inactivo como en la Lepra Lepromatosa y desaparición de la reticulina en áreas extensas del granuloma en la Lepra Tuberculoide practicándose estudio comparativo con biopsias de piel del granuloma lepromatoso del armadillo (Sabanicola Dasypus) y con piel sana del armadillo (Dasypus Novencinetus), apreciándose escasa formación de fibras de reticulina, en éstas