Pediatric Plexiform Fibromyxoma: A Case Report.

The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed.

[Endocrine disruptors and hypospadias in Gran Canaria island (2012-2015)]. / Disruptores endocrinos e hipospadias en Gran Canaria (2012-2015).

OBJECTIVE: Hypospadias represents the most frequent male congenital malformation in children, with a prevalence of 1 in 200-300 new born or 4-6 in 1000 male new born. Genetic and environmental factors play a strong role on developing this virilization defect acting as endocrinal disruptors (ED). The objective of the present study was to evaluate the environmental factors that may have influenced the development oh hypospadias in children treated at the Maternal and Child University Hospital od Las Palmas of Gran Canaria Island during the years 2012-2015. METHODS: The present study represents a retrospective analysis of patients treated for hypospadias in the Maternal-Paediatric University Hospital of Las Palmas of Gran Canaria during 4 years (2012 -2015) by the use of medical records and telephone survey to families' patients. To perform the statistical analysis of the data, the SPSS program was used in version 15.0. The level of statistical significance was established for an error α = 0.05. The chi-square test and Fisher's correction were applied if necessary to test the associations between the qualitative variables. RESULTS: From 2012 to 2015, 128 patients were treated; we could only follow 116 patients. If we analyse patients' distribution throught those years we can resume that 33% of them were treated in 2012, 23% in 2013, 24% in 2014 and 20% in 2015. We analyse the study background of both parents: 45.5% of mothers had primary studies, 37.5% had secundary studies, 15.2% had university studies and 1.8% were students; 54.3% of fathers had primary studies, 32.4% had secundary studies and 13.3% had university studies. We did not found any statistically significant diference between the place of residence and the study background both in mothers (p=0,262) and fathers (p=0,052). Within all the patients followed, 78 had a clinical history of exposition to ED as a direct family background. CONCLUSIONS: The chronical exposure to ED, very frequent in our society, is a very important risk factor in the development of urologic pathologies as hypospadias; for this reason, awareness measures and prevention in social areas and workplace must be carried out to avoid unnecessary exposures.

OBJETIVO: El hipospadias es la malformación congénita genital masculina más frecuente en niños, con una prevalencia de 1 cada 250 recién nacidos de sexo masculino. Se especula que pueden existir factores genéticos y/o medioambientales que predisponen a este defecto de virilización actuando como disruptores endocrinos (DE). El objetivo del presente estudio fue evaluar los factores medio ambientales que puedan haber influido en el desarrollo del hipospadias en los niños tratados en el Hospital Universitario Materno Infantil de Las Palmas de Gran Canarias en los años 2012-2015. METODOS: El presente estudio representa un análisis retrospectivo de los casos de hipospadias tratados en el Hospital Universitario Materno Infantil de Las Palmas de Gran Canaria entre el 2012 y el 2015, tras la revisión de las historias clínicas y la realización de una encuesta telefónica a los padres de los pacientes tratados. Para realizar el análisis estadístico de los datos, se utilizó el programa SPSS en la versión 15.0. El nivel de significación estadística se estableció para un error α=0.05. Se aplicó el test de chi cuadrado y la corrección de Fisher en caso de que fuera necesario para testar las asociaciones entre las variables cualitativas. RESULTADOS: Durante el periodo de estudio se trataron a 128 pacientes de los cuales se siguieron a 116. Si analizamos su distribución por años, el 33% de los pacientes fueron tratados en el año 2012, el 23% en el 2013, el 24% en el 2014 y el 20% en el 2015. En relación al nivel de estudio de la familia, el 45.5% de las madres tenían estudios primarios, el 37.5% estudios secundarios, el 15.2% estudios universitarios y el 1.8% eran estudiantes. En relación a los padres, el 54.3% tenían estudios primarios, el 32.4% estudios secundarios y el 13.3% estudios universitarios. No se encontraron diferencias estadísticamente significativas entre el municipio de residencia y el nivel de estudio de las madres (p=0,262) y de los padres (p=0,052). De los 116 pacientes analizados, 78 tenían antecedentes familiares directos con exposición a disruptores endocrinos. CONCLUSIONES: La exposición a agentes químicos nocivos y pesticidas, cada vez más habitual en nuestra sociedad, constituye un factor de riesgo para el padecimiento de enfermedades del tracto urológico inferior como el hipospadias, por lo que se deberían tomar medidas de concienciación y prevención en el ámbito social y laboral frente a la exposición a dichos agentes.

Disruptores endocrinos e hipospadias en Gran Canaria (2012-2015) / Endocrine disruptors and hypospadias in Gran Canaria island (2012-2015)

Fundamentos:El hipospadias es la malformación congénita genital masculina más frecuente en niños, con una prevalencia de 1 cada 250 recién nacidos de sexo masculino. Se especula que pueden existir factores genéticos y/o medioambientales que predisponen a este defecto de virilización actuando como disruptores endocrinos (DE). El objetivo del presente estudio fue evaluar los factores medio ambientales que puedan haber influido en el desarrollo del hipospadias en los niños tratados en el Hospital Universitario Materno Infantil de Las Palmas de Gran Canarias en los años 2012-2015. Métodos:El presente estudio representa un análisis retrospectivo de los casos de hipospadias tratados en el Hospital Universitario Materno Infantil de Las Palmas de Gran Canaria entre el 2012 y el 2015, tras la revisión de las historias clínicas y la realización de una encuesta telefónica a los padres de los pacientes tratados. Para realizar el análisis estadístico de los datos, se utilizó el programa SPSS en la versión 15.0. El nivel de significación estadística se estableció para un error alfa=0.05. Se aplicó el test de chi cuadrado y la corrección de Fisher en caso de que fuera necesario para testar las asociaciones entre las variables cualitativas. Resultados: Durante el periodo de estudio se trataron a 128 pacientes de los cuales se siguieron a 116. Si analizamos su distribución por años, el 33% de los pacientes fueron tratados en el año 2012, el 23% en el 2013, el 24% en el 2014 y el 20% en el 2015. En relación al nivel de estudio de la familia, el 45.5% de las madres tenían estudios primarios, el 37.5% estudios secundarios, el 15.2% estudios universitarios y el 1.8% eran estudiantes. En relación a los padres, el 54.3% tenían estudios primarios, el 32.4% estudios secundarios y el 13.3% estudios universitarios. No se encontraron diferencias estadísticamente significativas entre el municipio de residencia y el nivel de estudio de las madres (p=0,262) y de los padres (p=0,052). De los 116 pacientes analizados, 78 tenían antecedentes familiares directos con exposición a disruptores endocrinos. Conclusiones: La exposición a agentes químicos nocivos y pesticidas, cada vez más habitual en nuestra sociedad, constituye un factor de riesgo para el padecimiento de enfermedades del tracto urológico inferior como el hipospadias, por lo que se deberían tomar medidas de concienciación y prevención en el ámbito social y laboral frente a la exposición a dichos agentes

Background: Hypospadias represents the most frequent male congenital malformation in children, with a prevalence of 1 in 200-300 new born or 4-6 in 1000 male new born. Genetic and environmental factors play a strong role on developing this virilization defect acting as endocrinal disruptors (ED). The objective of the present study was to evaluate the environmental factors that may have influenced the development oh hypospadias in children treated at the Maternal and Child University Hospital od Las Palmas of Gran Canaria Island during the years 2012-2015. Methods: The present study represents a retrospective analysis of patients treated for hypospadias in the Maternal-Paediatric University Hospital of Las Palmas of Gran Canaria during 4 years (2012 -2015) by the use of medical records and telephone survey to families' patients. To perform the statistical analysis of the data, the SPSS program was used in version 15.0. The level of statistical significance was established for an error alfa = 0.05. The chi-square test and Fisher's correction were applied if necessary to test the associations between the qualitative variables. Results: From 2012 to 2015, 128 patients were treated; we could only follow 116 patients. If we analyse patients' distribution throught those years we can resume that 33% of them were treated in 2012, 23% in 2013, 24% in 2014 and 20% in 2015. We analyse the study background of both parents: 45.5% of mothers had primary studies, 37.5% had secundary studies, 15.2% had university studies and 1.8% were students; 54.3% of fathers had primary studies, 32.4% had secundary studies and 13.3% had university studies. We did not found any statistically significant diference between the place of residence and the study background both in mothers (p=0,262) and fathers (p=0,052). Within all the patients followed, 78 had a clinical history of exposition to ED as a direct family background. Conclusions: The chronical exposure to ED, very frequent in our society, is a very important risk factor in the development of urologic pathologies as hypospadias; for this reason, awareness measures and prevention in social areas and workplace must be carried out to avoid unnecessary exposures

Choledochal cyst in pediatric patients: a 10-year single institution experience.

BACKGROUND: Choledochal cysts (CCs) are rare congenital dilatations of the biliar tree. The incidence is 1:150,000. A correct diagnosis and surgical treatment are important because of the long term risks of infection and neoplasia. We report our experience with CCs in children. PATIENTS AND METHODS: Patients with CC disease, who were seen at the Hospital Italiano de Buenos Aires between 1999 and 2009, were identified retrospectively from our prospectively acquired hepato-pancreatico-biliary database. Prenatal, clinical, surgical and pathological records were analysed. RESULTS: We included 12 children (10 girls and 2 boys), all of them affected by a cystic dilatation type I of Todani classification. Between the 4 patients with antenatal diagnosis only one presented symptoms before surgery, expressed in acolic feces. Between the 8 patients with postnatal diagnosis, the most common clinical presentation was abdominal pain, occurring in all patients. Ultrasound was used for the diagnosis of all patients. Mean age at surgery was 74 months. All patients had intraoperative cholangiograms to definitively confirm the diagnosis. Five patients underwent open surgery and 7 laparoscopic approach. From patients with a laparoscopic approach, 1 had to be converted to open surgery due to intraoperative complications and 2 underwent an initial surgery for a cholecystectomy and a CC cyst excision. The complications observed were: anastomotic biliary fistula 1, residual lithiasis in the pancreatic portion of the duct 1 and anastomotic stenosis 1. The last 2 cases required reoperation. CONCLUSIONS: Once CCs are diagnosed, careful treatment decisions need to be taken because both intra hepatic and extra hepatic CCs, if untreated, have an increased risk of cancer. After a careful study of the cyst's characteristics, we recommend a laparoscopic approach when possible, as the best choice, after 3 months of life to increase the success of the treatment according to the size of the anatomic structure. We recommend early treatment, before 3 months of life, only in case of severe symptoms.