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.

Use of non-invasive scales for detecting esophageal varices in paediatric patients with portal vein thrombosis. / Utilización de escalas no invasivas en la detección de varices esofágicas en pacientes con trombosis venosa portal.

INTRODUCTION: Portal vein thrombosis (PVT) is the most frequent cause of portal hypertension in paediatric population. Baveno VI Consensus considers endoscopic variceal ligation as the second therapeutic option after meso-Rex bypass (surgical shunt). AIM: Analyse the diagnostic profitability of non-invasive scales in order to predict the risk of oesophageal varices (OV) in children with PVT. MATERIAL AND METHODS: Descriptive retrospective study where every upper gastrointestinal endoscopy (UGE) carried on patients <15 years old with non-cirrhotic PVT were included. There were divided according to the presence of OV and sex, cause, age, previous gastrointestinal bleeding or treatments, results of UGE and scales (Clinical Prediction Rule - CPR), Varices Prediction Rule - VPR), King's Variceal Prediction Score - K-VaPS) and Platelet count/Spleen diameter Ratio - PSR). Qualitative variables were expressed as absolute frequency and percentage, and quantitative variables as median and interquartile range. U Mann-Whitney and Hanley-McNeil tests were used for comparisons. RESULTS: Forty-five UGE were analysed. 80% (n=36) presented OV: median of 3 (2-3) and 33.3% (n=12) required endoscopic variceal ligation. Statistical differences were demonstrated between both groups: CPR (142.39 [132.22-166.53] vs. 122.75 [115.24-133.15]; p=0.003), VPR (9.91 [9.36-11.75] vs. 5.6 [3.34-8.39]; p=0.001), K-VaPS (117.86 [99.66-126.58] vs. 99.64 [94.88-10.18]; p=0.019), PSR (2384.62 [1902.22-3201.63] vs. 1252.5 [579.6-2144.42]; p=0.05), with and area under the curve AUROC>75%, without statistical differences between scales. CONCLUSIONS: In paediatric patients with non-cirrotic PVT non-invasive scales can be used as a tool to predict the presence of OV and raise the indication of UGE.

Enuresis: clinical evolution of patients attended at specialized unit of paediatric urology.

OBJECTIVE: To analyse the clinical evolution, the therapeutic strategies and the characteristics of the patients presenting enuresis attended at our outpatient clinic. MATERIAL AND METHODS: Retrospective study of patients <14 years old(yo) diagnosed of enuresis attended at our outpatient clinic (2011-2019) and completed their follow-up (remission or aged 15). Urotherapy was offered to all patients as initial management. The therapeutic strategies were classified as: first line (desmopressin or clock alarm), second line (desmo-pressin+alarm) and third line(anticholinergics). The remission rate during follow-up, the number of consultations needed until remission and the treatments used were calculated. Statistical tests used:Kaplan-Meier, actuarial survival. Multivariate analysis:Cox regression.Statistical significance:p<0.05. RESULTS: Data were collected from 125 patients (mean age: 8.6±2.45yo). Family history of enuresis was present in 38.9%. The mean follow-up was 2.37±1.55yo and the average number of consultations was 7.54±5.06. The remission rate (RE) was 84%(n=105), with a median remission interval:2.66 years (2.34-2.991[95%CI]). The average number of treatments required for remission was 2.74±1.27. RE with urotherapy alone was 20%(n=25); RE with first line:19.3%(n=17) and second line:16.7(n=11). In the remaining patients, a RE of 78.18%(n=43) was achieved by adding an anticholinergic. Patients aged > 8.7 years at the beginning of the follow-up required less time to achieve remission (p=.025). These patients had a higher RE (hazard ratio 1.15 (1.05-1.25))(p=.004). No other variables were significant. CONCLUSION: Staged therapeutic strategies are necessary to achieve remission. Only 25% remitted with urotherapy as single treatment. RE are higher when patients are >8.7 yo once they initiate their follow up.

Analysis of the Predictive Capacity of the Clinical Variables That Indicate the Performance of Voiding Urosonography in the Study of Vesicoureteral Reflux in Children.

INTRODUCTION: Voiding urosonography (VUS) is a dynamic imaging technique which evaluates the lower urinary tract by introducing sonographic contrast into the bladder, preferably used in the diagnosis of vesicoureteral reflux (VUR). Our goals were to describe the clinical indications for performing a VUS for VUR's diagnosis and analyse its diagnostic reliability. MATERIAL AND METHODS: Cross-sectional study carried on patients under 15 years old with a VUS taken between November 2013-2020. Sex, age, indications (lower urinary tract infection-LUTI; dilatated lower urinary tract-DLUT; duplex collecting system-DCS), results (presence/absence of VUR, score and side) and complications were analysed. U Mann-Whitney and Chi-squared tests were used and 2 predictive capacity models for the indications used to detect VUR were employed (logistic binomial regression and multilayer perceptron neural network). Statistical significance p < 0.05. RESULTS: 415 VUS were completed correctly (male 51.8%, median age 7.3 (3.1-15.3) months). Indications were: LUTI (67.5%), DLUT (33.5%) and DCS (10.2%); presenting 1, 2 and 3 indications in 86.5%, 12.8% and 0.7% respectively. VUR was diagnosed in 34.7% cases. A tendency towards statistically significance was showed related to male sex (p = 0.052) and the only significative clinical indication for VUR was DCS (p = 0.007). Patients with 2 simultaneous indications had higher probability of VUR (p = 0.012). DCS presence or male sex were the only predictive variables of VUR: DCS = OR 1.89 (1.54-6.52) (p = 0.006) and male sex = OR 1.56 (1.03-2.35) (p = 0.035). CONCLUSIONS: VUS is a thriving technique, radiation free and with a low complications rate. Presence of DCS, male sex or 2 simultaneous indications for VUS increase the probability of presenting VUR.

Hendidura laringotraqueoesofágica: presentación clínica y corrección quirúrgica / Laryngotracheoesophageal cleft: Clinical presentation and surgical treatment

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Enfisema subcutáneo neonatal / Neonatal subcutaneous emphysema

No disponible

Utilización de escalas no invasivas en la detección de varices esofágicas en pacientes con trombosis venosa portal / Use of non-invasive scales for detecting esophageal varices in paediatric patients with portal vein thrombosis

Introducción La trombosis portal (TVP) es la causa más frecuente de hipertensión portal en población pediátrica. El Consenso de Baveno VI considera la ligadura endoscópica de varices como segunda opción terapéutica tras el meso-Rex-bypass (shunt quirúrgico). Objetivo Analizar la rentabilidad diagnóstica de escalas no invasivas para predecir el riesgo de varices esofágicas en niños con TVP. Material y métodos Estudio descriptivo retrospectivo donde se incluyeron endoscopias digestivas altas (EDA) en pacientes<15 años con TVP no cirróticos. Se dividieron según la presencia de varices esofágicas y se estudiaron sexo, etiología, edad, hemorragia digestiva o tratamientos previos, resultados de EDA y las escalas (Regla Predicción Clínica-CPR, Regla Predicción Varices-VPR, King's Variceal Prediction Score-K-VaPS y ratio plaquetas/bazo-RPB). Las variables cualitativas se expresaron mediante frecuencia absoluta y porcentaje, y las cuantitativas mediante mediana y rango intercuartílico. Para las comparaciones se emplearon los test U de Mann-Whitney y Hanley-McNeil. Resultados Se realizaron 45 EDA. Un 80%(n=36) presentaron varices esofágicas: mediana de 3(2 – 3) y un 33,3%(n=12) precisó ligadura endoscópica de varices. Se demostraron diferencias estadísticamente significativas entre ambos grupos: CPR (142,39 [132,22 - 166,53] vs. 122,75 [115,24 – 133,15] p=0,003), VPR (9,91 [9,36 – 11,75] vs. 5,6 [3,34 – 8,39] p=0,001), K-VaPS (117,86 [99,66 - 126,58] vs. 99,64 [94,88 - 110,18] p=0,019), RPB (2384,62 [1902,22 - 3201,63] vs. 1252,5 [579,6 - 2144,42] p=0,05), con un área bajo la curva>75%, sin demostrarse diferencias entre escalas. Conclusiones En pacientes pediátricos con TVP no cirróticos se pueden emplear escalas no invasivas como herramienta para predecir la presencia de VE y plantear con ello la indicación de EDA. (AU)

Introduction Portal vein thrombosis (PVT) is the most frequent cause of portal hypertension in paediatric population. Baveno VI Consensus considers endoscopic variceal ligation as the second therapeutic option after meso-Rex bypass (surgical shunt). Aim Analyse the diagnostic profitability of non-invasive scales in order to predict the risk of oesophageal varices (OV) in children with PVT. Material and methods Descriptive retrospective study where every upper gastrointestinal endoscopy (UGE) carried on patients <15 years old with non-cirrhotic PVT were included. There were divided according to the presence of OV and sex, cause, age, previous gastrointestinal bleeding or treatments, results of UGE and scales (Clinical Prediction Rule – CPR), Varices Prediction Rule – VPR), King's Variceal Prediction Score – K-VaPS) and Platelet count/Spleen diameter Ratio – PSR). Qualitative variables were expressed as absolute frequency and percentage, and quantitative variables as median and interquartile range. U Mann–Whitney and Hanley–McNeil tests were used for comparisons. Results Forty-five UGE were analysed. 80% (n=36) presented OV: median of 3 (2–3) and 33.3% (n=12) required endoscopic variceal ligation. Statistical differences were demonstrated between both groups: CPR (142.39 [132.22-166.53] vs. 122.75 [115.24-133.15]; p=0.003), VPR (9.91 [9.36-11.75] vs. 5.6 [3.34-8.39]; p=0.001), K-VaPS (117.86 [99.66-126.58] vs. 99.64 [94.88-10.18]; p=0.019), PSR (2384.62 [1902.22-3201.63] vs. 1252.5 [579.6-2144.42]; p=0.05), with and area under the curve AUROC>75%, without statistical differences between scales. ConclusionsIn paediatric patients with non-cirrotic PVT non-invasive scales can be used as a tool to predict the presence of OV and raise the indication of UGE. (AU)

Analysis of the Predictive Capacity of the Clinical Variables That Indicate the Performance of Voiding Urosonography in the Study of Vesicoureteral Reflux in Children

Introduction: Voiding urosonography (VUS) is a dynamic imaging technique which evaluates the lower urinary tract by introducing sonographic contrast into the bladder, preferably used in the diagnosis of vesicoureteral reflux (VUR). Our goals were to describe the clinical indications for performing a VUS for VUR’s diagnosis and analyse its diagnostic reliability. Material and Methods: Cross-sectional study carried on patients under 15 years old with a VUS taken between November 2013-2020. Sex, age, indications (lower urinary tract infection—LUTI; dilatated lower urinary tract—DLUT; duplex collecting system—DCS), results (presence/absence of VUR, score and side) and complications were analysed. U Mann-Whitney and Chi-squared tests were used and 2 predictive capacity models for the indications used to detect VUR were employed (logistic binomial regression and multilayer perceptron neural network). Statistical significance p < 0.05. Results: 415 VUS were completed correctly (male 51.8%, median age 7.3 (3.1-15.3) months). Indications were: LUTI (67.5%), DLUT (33.5%) and DCS (10.2%); presenting 1, 2 and 3 indications in 86.5%, 12.8% and 0.7% respectively. VUR was diagnosed in 34.7% cases. A tendency towards statistically significance was showed related to male sex (p = 0.052) and the only significative clinical indication for VUR was DCS (p = 0.007). Patients with 2 simultaneous indications had higher probability of VUR (p = 0.012). DCS presence or male sex were the only predictive variables of VUR: DCS = OR 1.89 (1.54-6.52) (p = 0.006) and male sex = OR 1.56 (1.03-2.35) (p = 0.035). Conclusions: VUS is a thriving technique, radiation free and with a low complications rate. Presence of DCS, male sex or 2 simultaneous indications for VUS increase the probability of presenting VUR (AU)