Carcinoma neuroendocrino de células grandes en la unión esofagogástrica. Una entidad extremadamente infrecuente / Large cell neuroendocrine carcinoma at the esophagogastric junction. An extremely rare entity

Presentamos el tercer caso descrito hasta la fecha de carcinoma neuroendocrino de células grandes localizado en la unión esofagogástrica (CNECG). Los tumores neuroendocrinos esofágicos representan el 0,03-0,05% de todos los tumores malignos esofágicos. Dentro de los TNE esofágicos. El CNECG representa el 1% de los TNE esofágicos. Este tipo tumoral se caracteriza por elevar unos marcadores determinados: sinaptofisina, cromogranina A y CD56. De hecho, el 100% de los pacientes tendrán cromogranina o sinaptofisina, o al menos uno de estos tres marcadores. A su vez, el 78% tendrán invasión linfovascular y el 26% invasión perineural. Únicamente el 11% de los pacientes tendrán un estadio I-II, circunstancia que implica un curso agresivo y un peor pronóstico.(AU)

A rare case of an esophageal neuroendocrine carcinoma arising on an endoscopic resection scar / Un caso raro de esófago carcinoma neuroendocrino que surge en una endoscópica cicatriz de resección

Here we report a case of a 61-year-old woman who underwent en-bloc endoscopic submucosal dissection (ESD) of a 10mm depressed lesion (Paris 0-IIc, Figure A) in the mid-esophagus. Histopathology showed a lesion with high-grade squamous dysplasia (R0). On follow-up endoscopy at 6 and 12 months the scar was regular, without signs of recurrence. Seven months after the last endoscopy, the patient presented with chest pain and dysphagia. Endoscopy showed an ulcero-vegetating tumor with 3cm at the same location of previous ESD (Figure B), and biopsies showed a poorly differentiated small cell neuroendocrine carcinoma (NEC). Subsequent computed tomography identified peri-tumor and hilar lymph nodes, and an extensive periceliac nodal conglomerate adherent to the liver (stage IV). This is, to our knowledge, the first case described of esophageal NEC arising on the endoscopic resection scar.(AU)

Primary esophageal follicular lymphoma: a rare entity / Linfoma folicular esofágico primario: una entidad rara

A 63-year-old woman presented with dyspepsia de novo. An esophagogastroduodenoscopy revealed a 30 mm flat yellowish esophageal lesion, 28 cm from the incisors (Fig. 1a), with the stomach and duodenum without any lesion. Helicobacter pylori infection was excluded. Histological examination was suggestive of a lymphoproliferative process (Fig. 1b). Immunohistochemistry showed diffuse positivity for CD20 (Fig. 1c) and BCL-2 (Fig. 1d), dim CD10 and BCL-6 staining, a Ki-67 of 20-25%, no CD21 or cyclin D1 expression, all these features compatible with low-grade follicular lymphoma. Physical examination was unremarkable. Computed tomography of the neck, chest, and abdomen revealed no lymph node enlargement, hepatosplenomegaly or metastasis. Blood routine tests and tumor markers were at normal levels. Bone marrow biopsy showed no involvement by lymphoma. Therefore, a diagnosis of primary follicular lymphoma of the esophagus was made. The patient opted for a watch-and-wait strategy and there is no evidence of disease progression after four years of follow-up.(AU)

Impacto del manejo multidisciplinar digestivo-quirúrgico en los pacientes con atresia de esófago / Impact of digestive-surgical cross-disciplinary management in patients with esophageal atresia

Objetivos: El objetivo de este estudio es analizar si los pacientesintervenidos de atresia de esófago (AE) se benefician de un programade seguimiento multidisciplinar, basado en las guías clínicas actuales,implantado en nuestro centro. Material y métodos: Estudio retrospectivo, observacional y analí-tico incluyendo los pacientes intervenidos de AE entre 2012 y 2022. Seanalizaron los resultados de la implantación en 2018 de un programa deconsultas conjuntas de gastroenterología y cirugía pediátrica aplicandoun protocolo basado en las nuevas guías ESPGHAN-NASPGHAN. Sedividieron a los pacientes tratados antes y después de 2018 y se compararon las variables cuantitativas: pérdidas de seguimiento, inicio y duracióndel tratamiento antirreflujo e inicio de nutrición enteral, y cualitativas:prevalencia de reflujo gastroesfoágico, realización de cirugía antirreflujo,infecciones respiratorias, estenosis de la anastomosis, refistulizaciones,disfagia, episodios de impactación, necesidad de gastrostomía y resul-tados de las endoscopias. Resultados: Se incluyeron 38 pacientes. Un 63,2% presentaronreflujo gastroesofágico. El 97,4% tomaron tratamiento antirreflujo el primer año de vida que posteriormente se retiró en el 47,4%. El tiempo deretirada se redujo una media de 24 meses tras la aplicación del programa(p< 0,05). Se realizaron 4,6 veces más pHmetrías tras la implantacióndel programa. El protocolo estandarizó la realización de endoscopiasen pacientes asintomáticos al cumplir 5 y 10 años. Se realizaron 25endoscopias con tomas de biopsia después de 2018, detectando alteraciones histológicas en un 28%. El número de pérdidas de seguimiento seredujo de forma significativa tras la implantación del protocolo (p< 0,05). Conclusiones: El seguimiento multidisciplinar digestivo-quirúrgicode los pacientes con AE genera un impacto positivo en su evolución.(AU)

Objective: The objective of this study was to analyze whether patients undergoing esophageal atresia (EA) surgery benefit from a cross-disciplinary follow-up program, based on current clinical guidelines,implemented in our institution. Materials and methods: An observational, analytical, retrospectivestudy of patients undergoing EA surgery from 2012 to 2022 was carriedout. The results of a joint pediatric surgery and gastroenterology consultation program –which was implemented in 2018 and applies a protocolbased on the new ESPGHAN-NASPGHAN guidelines– were analyzed.Patients were divided according to whether they had been treated before or after 2018. Quantitative variables –follow-up losses, anti-refluxtreatment initiation and duration, and enteral nutrition initiation– andqualitative variables –prevalence of gastroesophageal reflux, anti-refluxsurgery, respiratory infections, anastomotic stenosis, re-fistulizations,dysphagia, impaction episodes, need for gastrostomy, and endoscopicresults– were compared. Results: 38 patients were included. 63.2% had gastroesophagealreflux. 97.4% received anti-reflux treatment in the first year of life, withtreatment being subsequently discontinued in 47.4%. Discontinuationtime decreased by a mean of 24 months following program implementation (p< 0.05). A 4.6-fold increase in the frequency of pH-metries wasnoted following program implementation. The protocol standardizedendoscopies in asymptomatic patients when they turn 5 and 10 years old. 25 endoscopies with biopsy were carried out after 2018, with histologicaldisorders being detected in 28% of them. The number of follow-up lossessignificantly decreased following protocol implementation (p< 0.05). Conclusions: Digestive-surgical cross-disciplinary follow-up of EApatients has a positive impact on patient progression. Applying the guidelines helps optimize treatment and early diagnosis of complications.(AU)

Niveles séricos de IGF-1 e IGFBP-3 en pacientes con esófago de Barrett y adenocarcinoma de esófago. Estudio longitudinal / Serological levels of IGF-1 and IGFBP-3 in patients with Barrett's esophagus and esophageal adenocarcinoma: Longitudinal study

Antecedentes: El esófago de Barrett (EB) es una entidad con una progresión histológica a malignidad conocida. Los factores de crecimiento insulínico (IGF, de insulin-like growth factor) están involucrados en la carcinogénesis asociada a la obesidad y se han asociado con el riesgo de padecer algunos tipos de cáncer. Objetivos: Evaluar los niveles serológicos de IGF-1 e IGFBP-3 en pacientes con EB y adenocarcinoma de esófago. Pacientes y métodos: Estudio prospectivo de pacientes con EB y adenocarcinoma de esófago explorados con gastroscopia entre septiembre 2012 y diciembre 2015 a los que se realizó una extracción de sangre para la determinación de IGF-1 e IGFBP-3. Se incluyó un grupo control. Resultados: Se incluyeron 116 pacientes: 36 controles, 62 con EB (42 sin displasia y 20 con displasia) y 18 con adenocarcinoma. El IGF-1 y la ratio molar IGF-1/IGFBP-3 presentaron un aumento progresivo en los grupos con EB y adenocarcinoma comparado con los controles (IGF-1: 135,55±66,07ng/ml; 148,33±81,5ng/ml; 108,19±46,69ng/ml, respectivamente; p=0,049) (ratio molar: 0,23±0,91; 0,29±0,11; 0,19±0,06, respectivamente; p=0,001), sin diferencias entre los diferentes grados histológicos. Cincuenta y cuatro de los 65 pacientes con EB fueron seguidos durante una mediana de 58,50 meses (12-113) y 11 de ellos (20,4%) presentaron progresión a displasia de bajo grado (n=8) o displasia de alto grado/adenocarcinoma (n=3), sin encontrar diferencias en el sistema IGF comparado con los que no progresaron. Conclusiones: Los pacientes con EB y adenocarcinoma esofágico presentan cambios en el sistema IGF aunque los niveles de IGF-1 e IGFBP-3 no se correlacionan con la progresión histológica del EB.(AU)

Background: Barrett's esophagus (BE) is an entity with a known histological progression to malignancy. The insulin-like growth factor (IGF) system is involved in the carcinogenesis through obesity-related mechanisms that include IGF and it has been associated with several types of cancer. Objectives: To evaluate the serological levels of IGF-1 and IGFBP-3 in patients with BE and esophageal adenocarcinoma. Patients and methods: Prospective study of patients with BE and esophageal adenocarcinoma who underwent upper endoscopy between September 2012 and December 2015. A baseline determination of IGF-1 and IGFBP-3 was performed. We included a control group of patients without BE. Results: One hundred sixteen patients were included: 36 controls, 62 with BE (42 without dysplasia and 20 with dysplasia) and 18 with adenocarcinoma. IGF-1 and IGF-1/IGFBP-3 molar ratio showed a progression to high levels in BE and adenocarcinoma than in controls (IGF-1: 135.55±66.07ng/ml, 148.33±81.5ng/ml, 108.19±46.69ng/ml, respectively; P=.049) (molar ratio: 0.23±0.91, 0.29±0.11, 0.19±0.06, respectively; P=.001), without differences between the histological types of BE. Fifty-four out of the 65 patients with BE were followed up (median of 58.50 months, range 12–113) and 11 of them (20.4%) presented progression to low-grade dysplasia (n=8) or high-grade dysplasia/adenocarcinoma (n=3), without differences in the IGF system compared with patients without progression. Conclusions: Patients with BE and esophageal adenocarcinoma have changes in the IGF system although the serological levels of IGF-1 and IGFBP-3 do not correlate with histological progression of BE.(AU)

Incidence of opioid-induced esophageal dysfunction / Incidencia de disfunción esofágica inducida por opiáceos

Background: Retrospective studies have suggested that long-term use of opioids can cause esophageal motility dysfunction. A recent clinical entity known as opioid-induced esophageal dysfunction (OIED) has been postulated. There is no data from prospective studies assessing the incidence of opioid-induced effects on the esophagus. Aim: Evaluate the incidence of OIED during chronic opioid therapy. Methods: From February 2017 to August 2018, all patients seen in the Pain Unit of the hospital, who started opioid treatment for chronic non-neoplastic pain and who did not present esophageal symptoms previously, were included. The presence of esophageal symptoms was assessed using the Eckardt score after 3 months and 1 year since the start of the study. In February 2021, the clinical records of all included patients were reviewed to assess whether esophageal symptoms were present and whether opioid therapy was continued. In patients presenting with esophageal symptoms, an endoscopy was performed and, if normal, a high-resolution esophageal manometry was performed. For a confidence level of 95%, a 4% margin of error and an estimated prevalence of 4%, a sample size of 92 patients was calculated. Results: 100 patients were included and followed while taking opioids, for a median of 31 months with a range between 4 and 48 months. Three women presented with dysphagia during the first 3 months of treatment, being diagnosed with esophagogastric junction outflow obstruction; type II and type III achalasia. The cumulative incidence of OIED was 3%; 95%-CI: 0–6%. Conclusions: Chronic opioid therapy in patients with chronic non-neoplastic pain is associated with symptomatic esophageal dysfunction.(AU)

Antecedentes: Estudios retrospectivos han sugerido que el uso crónico de opiáceos puede causar disfunción esofágica. Se ha postulado una entidad clínica reciente denominada disfunción esofágica inducida por opioides (DEIO). No existen estudios prospectivos que evalúen la incidencia de esta entidad. Objetivo: Evaluar la incidencia de DEIO durante el tratamiento crónico con opiáceos. Métodos: Desde febrero de 2017 hasta agosto de 2018, se incluyeron todos los pacientes atendidos en la Unidad del Dolor de nuestro hospital, que iniciaron opiáceos por dolor crónico no neoplásico sin síntomas esofágicos previos. La clínica esofágica se valoró mediante la escala de Eckardt a los tres meses y al año. En febrero de 2021, se revisaron las historias clínicas de todos los pacientes para evaluar la presencia de clínica esofágica y si continuaban con opiáceos. En los pacientes con síntomas esofágicos, se realizó una gastroscopia y, si era normal, una manometría esofágica de alta resolución. Para un nivel de confianza del 95%, una precisión del 4% y una prevalencia estimada del 4%, se calculó un tamaño muestral de 92 pacientes. Resultados: Se incluyeron 100 pacientes que fueron seguidos mientras tomaban opiáceos, con una mediana de 31 meses y un rango entre 4 y 48 meses. Tres mujeres presentaron un trastorno motor esofágico durante el seguimiento (obstrucción funcional de la unión esofagogástrica; acalasia tipo II y tipo III). La incidencia acumulada fue del 3%; IC 95%: 0-6%. Conclusiones: El tratamiento crónico con opiáceos en pacientes con dolor crónico no neoplásico se asocia a disfunción esofágica sintomática.(AU)

Underlying etiology associated with the diagnosis of absent contractility on high resolution esophageal manometry / Etiologías subyacentes asociadas al diagnóstico de contractilidad ausente en la manometría esofágica de alta resolución

Background/Aims: Absent contractility is considered a disorder of peristalsis. The literature about the etiology and clinical characteristics is scarce and the evidence on systemic diseases associated with this esophageal disorder is limited. Therefore, we aimed to determine the etiology of absent contractility in our population using the clinical algorithm recently described in the literature. Methods: We conducted a retrospective, descriptive study at a single tertiary hospital of all patients diagnosed of absent contractility between May 2018 and February 2020. Data on demographic characteristics, medication, comorbidities, and laboratory and paraclinical tests were recorded from clinical records. Results: A total of 72 patients with absent contractility were included for analysis. There was a predominance of female sex (n=43, 59.7%), with a mean age of 55.4 (±15.0) years. We identified a systemic disorder associated with absent contractility in 64 (88.9%) patients. From these, 31 (43.1%) patients were diagnosed with a systemic autoimmune disease, 26 (36.1%) patients were considered to have absent contractility secondary to pathological exposure to acid-reflux and 15 (20.8%) patients were diagnosed with other non-autoimmune systemic disorders. In the remaining eight (11.1%) patients, there were no underlying systemic disorders that could justify the diagnosis of absent contractility. Conclusions: A systematic approach to search for an underlying cause in patients diagnosed with absent contractility is warranted. Up to 90% of patients with absent contractility have a systemic disorder associated with this condition.(AU)

Antecedentes: La contractilidad ausente se considera un trastorno de la peristalsis esofágica. La literatura que existe sobre la etiología y las características clínicas es escasa y la evidencia sobre enfermedades sistémicas asociadas a este trastorno esofágico es limitada. Nuestro objetivo fue determinar la etiología de la contractilidad ausente en nuestra población utilizando el algoritmo clínico recientemente descrito en la literatura. Métodos: Se realizó un estudio descriptivo retrospectivo en un hospital terciario de todos los pacientes diagnosticados de ausencia de contractilidad entre mayo de 2018 y febrero de 2020. Se recogieron datos de características demográficas, medicación, comorbilidades y pruebas de laboratorio y estudios paraclínicos. Resultados: Se incluyeron para el análisis un total de 72 pacientes con ausencia de contractilidad. Predominó el sexo femenino (n=43, 59,7%), con una edad media de 55,4 (±15,0) años. Identificamos un trastorno sistémico asociado con la ausencia de contractilidad en 64 (88,9%) pacientes. De estos 31 (43,1%) pacientes fueron diagnosticados de una enfermedad autoinmune sistémica, 26 (36,1%) pacientes se consideraron con ausencia de contractilidad secundaria a exposición patológica al reflujo ácido y 15 (20,8%) fueron diagnosticados con otras enfermedades no autoinmunes sistémicas. En los 8 pacientes restantes (11,1%) no hubo trastornos sistémicos subyacentes que pudieran justificar el diagnóstico de contractilidad ausente. Conclusiones: Un enfoque sistemático está justificado para investigar una causa subyacente en pacientes diagnosticados de contractilidad ausente. Hasta el 90% de los pacientes con contractilidad ausente tienen un trastorno sistémico asociado con esta afectación de la motilidad esofágica.(AU)

Papel de la yeyunoplastia en la reconstrucción esofágica compleja / Role of jejunoplasty in complex esophageal reconstruction

Introducción: La reconstrucción esofágica es un proceso quirúrgico técnicamente muy complejo, gravado por una importante morbilidad. Clásicamente se han utilizado la gastroplastia y la coloplastia, aunque la yeyunoplastia ya fue descrita por Roux en 1907. Parece demostrado que la plastia de yeyuno libre es una muy buena opción en el tratamiento de la enfermedad del esófago cervical, pero no está tan claro el papel de la yeyunoplastia supercharged en la reconstrucción del esófago torácico. El objetivo de este estudio es el análisis de las reconstrucciones esofágicas realizadas en nuestra unidad y que precisaron de un injerto de yeyuno. Métodos: Estudio retrospectivo de las reconstrucciones esofágicas realizadas con yeyunoplastias en nuestra unidad entre enero de 2011 y diciembre de 2019. Se analizan datos epidemiológicos, indicaciones, técnica quirúrgica y morbimortalidad. Resultados: Se realizaron 67 procedimientos quirúrgicos de reconstrucción esofágica compleja de los que 10 fueron yeyunoplastias: 5 yeyunos libres en esófago cervical y 5 supercharged en esófago torácico con abordaje transesternal. La morbilidad, mortalidad, estancia media y tiempo de retirada de la alimentación enteral fueron menores en los yeyunos libres que en los supercharged. Conclusiones: En nuestro grupo la yeyunoplastia supercharged es la última opción para la reconstrucción del esófago torácico; el acceso por esternotomía media nos permite un excelente abordaje del mediastino anterior y los vasos mamarios internos. El yeyuno libre sería la primera elección —con indemnidad del resto de esófago— en la reconstrucción del esófago cervical. (AU)

Introduction: Esophageal reconstruction is a very complex surgical procedure, burdened by significant morbidity. Gastroplasty and coloplasty have classically been used. Free jejunal plasty has shown to be a very good option in the treatment of cervical esophagus pathology, but the role of supercharged jejunoplasty in thoracic esophagus reconstruction is still controversial. Methods: A retrospective study of esophageal reconstructions with jejunoplasties performed in our unit between January 2011 and December 2019. Epidemiological data, indications, surgical technique, and morbidity and mortality were analyzed. Results: 67 procedures of esophageal reconstruction were performed, 10 of which were jejunoplasties: 5 free jejunums and 5 supercharged. Morbidity, mortality, mean stay and withdrawal time from enteral feeding were lower in free than in supercharged jejunums. Conclusions: Supercharged jejunoplasty was the last option for reconstruction of the thoracic esophagus. Median sternotomy access provides an excellent approach to the anterior mediastinum and the internal mammary vessels. The free jejunum would be the first choice, with the indemnity of the rest of the esophagus, in the reconstruction of the cervical esophagus. (AU)

Gastric barotrauma induced by the Sellick maneuver performed to enable endoscopic submucosal dissection

A 55-year-old male underwent endoscopic submucosal dissection (ESD), under general anesthesia and orotracheal intubation, of a 22 mm Paris 0-IIa+0-IIb lesion located in the greater curvature of the medium/distal antrum. It was not possible to start the procedure after several attempts due to permanent belching and inability to maintain adequate gastric distension. ESD was only feasible after performing the Sellick maneuver (manual pressure application at the cricoid cartilage to occlude the upper esophagus), which allowed sustained gastric distension throughout the procedure (60 minutes). (AU)

Peroral endoscopic myotomy in a 9-year-old girl with achalasia and annular stenosis in the lower esophagus

We present a case report of peroral endoscopic myotomy (POEM) in the treatment of pediatric patient with achalasia and annular stenosis in lower esophagus. A 9-year-old girl was newly diagnosed with achalasia. After assessment, the patient underwent POEM procedure and the retrievable stent was implanted in the esophagus. Postoperatively, lung CT suggested esophagopleural fistula, while gastroscopy showed that the metal stent was displaced to the esophageal lumen. A fistula was found in the lower esophageal segment after the stent was removed. Emergency operation of endoscopic perforation repair and esophageal stent replacement were performed. The patient recovered well in the next 6 months of follow-up. (AU)

An unexpected complication of prophylactic esophageal stenting: esophageal stent impaction after thread dislocation

A 47-year-old woman was referred to our department with opportunistic endoscopic findings of two submucosal esophageal bulges, approximately half the circumference of the esophagus, both nearly 2.0 cm in size, and 24-27 cm from the incisors. Ultrasound endoscopy diagnosed smooth muscle tumors originating from the muscularis propria layer and she next underwent submucosal tunneling endoscopic resection. Intraoperatively, part of the tumor could not be separated from the muscularis propria layer and a U-shaped tumor was finally resected. A fully covered self-expanding esophageal nitinol stent was then inserted, covering the full circumference esophageal mucosa. The stent was fixed by ears with knotted thread and proton pump inhibitors were given for 1 week. (AU)