Per oral endoscopic myotomy in a pediatric patient with achalasia. / Miotomía endoscópica por vía oral (POEM) en un paciente pediátrico para tratamiento de la acalasia esofágica.

INTRODUCTION: Achalasia is the most common primary motor disorder of the esophagus. Its reported incidence is low, even more in pediatric patients. Laparoscopic Heller myotomy is the current stan dard of treatment. During the last years, per-oral endoscopic myotomy (POEM) has been positioned as a safe and effective therapeutic alternative as the Heller procedure for esophageal achalasia. Ob jective: To describe the POEM technique and report the first pediatric case in our country. CLINICAL CASE: 11-year-old patient, previously healthy, who presented with progressive dysphagia for solids and liquids and weight loss. The study concluded a type II achalasia. The patient underwent a POEM and had a postoperative course without incidents. One year after the intervention, symptomatic, endoscopic and manometric resolution have been documented. CONCLUSIONS: The described case is the first POEM in a pediatric patient in our country. Esophageal achalasia is uncommon in pediatrics and POEM has demonstrated clinical success and safety comparable to laparoscopic Heller myotomy in short and medium term. Long-term follow-up will determine its definitive role in the treatment of pediatric patients with esophageal achalasia.

Miotomía endoscópica por vía oral (POEM) en un paciente pediátrico para tratamiento de la acalasia esofágica / Per oral endoscopic myotomy in a pediatric patient with achalasia

INTRODUCCIÓN: La acalasia es el trastorno motor primario más frecuente del esófago. Su incidencia reportada es baja, aún más en pacientes pediátricos. La miotomía de Heller laparoscópica corresponde al estándar actual de tratamiento. Durante los últimos años la miotomía endoscópica por vía oral (POEM) se ha posicionado como una alternativa terapéutica segura y tan efectiva como el Heller para la acalasia esofágica. OBJETIVO: Describir la técnica de POEM y reportar el primer caso pediátrico en nuestro país. CASO CLÍNICO: Paciente de 11 años, previamente sano, que se presentó con disfagia ilógica progresiva y baja de peso. El estudio concluyó una acalasia tipo II. Fue sometido a POEM y cursó un postoperatorio sin incidentes. A un año de la intervención se ha documentado resolución de la sintomatología, seguimiento endoscópico y manométrico sin complicaciones. CONCLUSIONES: El caso descrito corresponde al primer POEM en un paciente pediátrico en nuestro país. La acalasia esofágica es infrecuente en pediatría y el POEM ha demostrado éxito clínico y seguridad comparables a la miotomía de Heller laparoscópica en el corto y mediano plazo. El seguimiento a largo plazo permitirá determinar su rol definitivo en el tratamiento de pacientes pediátricos con acalasia esofágica.

INTRODUCTION: Achalasia is the most common primary motor disorder of the esophagus. Its reported incidence is low, even more in pediatric patients. Laparoscopic Heller myotomy is the current stan dard of treatment. During the last years, per-oral endoscopic myotomy (POEM) has been positioned as a safe and effective therapeutic alternative as the Heller procedure for esophageal achalasia. OBJECTIVE: To describe the POEM technique and report the first pediatric case in our country. CLINICAL CASE: 11-year-old patient, previously healthy, who presented with progressive dysphagia for solids and liquids and weight loss. The study concluded a type II achalasia. The patient underwent a POEM and had a postoperative course without incidents. One year after the intervention, symptomatic, endoscopic and manometric resolution have been documented. CONCLUSIONS: The described case is the first POEM in a pediatric patient in our country. Esophageal achalasia is uncommon in pediatrics and POEM has demonstrated clinical success and safety comparable to laparoscopic Heller myotomy in short and medium term. Long-term follow-up will determine its definitive role in the treatment of pediatric patients with esophageal achalasia.

[Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts]. / Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares / Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts

Las dilataciones quísticas de las vías biliares, conocidas como lagunas biliares, se han descrito en el seguimiento de los pacientes sometidos a portoenterostomía o cirugía de Kasai por atresia de las vías biliares (AVB). Su aparición se ha asociado con el desarrollo de colangitis y consecuentemente a un peor pronóstico. OBJETIVO: Describir las características clínicas y pronóstico de pacientes con AVB, sometidos a cirugía de Kasai, con énfasis en aquellos que desarrollaron lagunas biliares. PACIENTES Y MÉTODO: Estudio retrospectivo de pacientes sometidos a portoenterostomía de Kasai por AVB durante los años 2008 a 2016. Se tabuló la información demográfica y variables asociadas a la portoenterostomía de Kasai, trasplante hepático, lagunas o quistes biliares y episodios de colangitis. Se crearon curvas de Kaplan Meier y comparación mediante el test de Log Rank para evaluar sobrevida global, libre de colangitis y con hígado nativo, considerando un valor p como significativo. RESULTADOS: Se analizó el seguimiento de 13 pacientes. El tiempo promedio de realización del Kasai fue a los 85 meses (rango 42-193, DS 40,3), seis pacientes (46%) fueron sometidos a cirugía de Kasai después de los 90 días de vida por derivación tardía. Cuatro (31%) desarrollaron lagunas biliares múltiples, todos presentaron episodios de colangitis. La sobrevida libre de colangitis fue significativamente menor para los portadores de lagunas biliares. Nueve pacientes (69%) recibieron un trasplante hepático, en promedio a los 16 meses de edad (rango 6-40, DS 12,1), en 3 de ellos la causa fue colangitis recurrente. No se encontraron diferencias significativas en la sobrevida con hígado nativo ni en sobrevida global entre portadores y no portadores de lagunas biliares. CONCLUSIONES: En esta cohorte, la incidencia de lagunas biliares luego de la portoenterostomía es similar a la descrita en la literatura. Los resultados concuerdan con la relación propuesta entre ellas y el desarrollo de colangitis. En los pacientes de nuestra serie, el diagnóstico y derivación de AVB fue realizado tardíamente, determinando un peor pronóstico.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.