ABSTRACT
Introduction: Esophageal strictures in children are in most cases associated with a benign etiology. There are multiple conditions that are associated with esophageal strictures including congenital stenosis, secondary to surgical repair of esophageal atresia, caustic burns following ingestion of acids or alcalis, radiation therapy and secondary to some pathologies as gastroesophageal reflux disease, eosinophilic esophagitis, scleroderma, epidermolysis bulllosa and idiopathic. Esophageal dilation can be performed with different techniques that include endoscope dilators, dilations performed over the wire and using the endoscope itself. Some cases require other adjunctive techniques that improve the results after failed progressive dilation. This therapies includes medical treatment and surgical derivations, with some cases known as recalcitrant. Also, esophageal strictures and its management could produce motility disorders. There is no consensus in the literature regarding the management process, especially in frequency of interventions, use of adjunctive therapies and alternatives for conservative management. This problem is more evident in developing countries. Objective(s): The objective of this study was to describe the cases of esophageal strictures and its management in children between 2016 and 2022 in the Instituto Nacional de Pediatria in Mexico City. Method(s): This was a six year retrospective study involving pediatric patients with esophageal stricture. We retrospectively reviewed the medical records of 23 pediatric patients who underwent endoscopic treatments for esophageal strictures, between January 2016 and May 2022 in the Comprehensive Pediatric Gastroenterology Diagnostic Unit in the Instituto Nacional de Pediatria in Mexico City. Result(s): The mean age at diagnosis was 24 months (Q1 15, Q3 35), 12 patients were male (52%) and 11 patients were female (48%). The most prevalent etiology was caustic strictures in 10 patients (43%). Six patients (26%) had esophageal atresia (4 type III, 1 type I and 1 type V), all whose received surgical management in the first days of life. All required repeated pneumatic dilation (between 1 and 11) for the management of postsurgical stenosis. Other etiologies that were found include Schatzki Ring, congenital stenosis, esophageal fibrosis associated with congenital dyskeratosis, epidermolysis bullosa, graft-versus-host disease and gastroesophageal reflux disease (one patient for each cause). In one patient the etiology remains unknown. Seventeen patients had one stricture, 5 patients had two strictures and 1 had 3 strictures. Ten patients had esophageal pseudodiverticula and two had mucosal fold. Six patients underwent dilation with Savary-Guilliard dilators combined with pneumatic balloon dilation. Four patients received mitomycin- C as an adjuvant therapy during dilations. The average diameter of stenosis was increased from 7 mm (range 4-15 mm) to 13,5 mm (range 8-18mm). Two patients had severe complications, one had a esophageal perforation associated with dilation. The other one had a pneumothorax related with anesthetic management. In the outcome 6 patients are asymptomatic, 1 patient persist with dysphagia after completed treatment, 9 patients are under treatment, 1 patient died secondary to its underlying disease and 6 patients lost follow up. Conclusion(s): Post-corrosive esophagitis and post-esophageal atresia anastomotic strictures were the most frequent types of cicatricial esophageal strictures. The conservative treatment was the first management strategy in the majority of patients, being the endoscopic balloon dilation the first choice. The SARS-COV-2 sanitary emergency limited the progressive intervention rate and appropriate clinical follow up of patients, reason why there is an important loss of follow up in the described group. A number of patients are currently on management, reason why their outcomes will be assessed in the future.
ABSTRACT
Dyskeratosis Congenita (DC) is a rare and heterogeneous disease. This disorder is resulted from a defect in the telomere maintenance in stem cells. Telomerase RNA component, shelterin complex, and telomerase reverse transcriptase are mutated in this disease. Many studies have previously confirmed shorter leukocyte telomere length in DC. On the other hand, the association between telomere length and Coronavirus disease 2019 (COVID-19) indicated that people with a short telomere background mostly show more severe symptoms related to COVID-19, and the mortality rate among them increases as well. Because patients with DC have an abnormally short telomere length, in the current study, we hypothesized that they are at higher risk of developing symptomatic COVID-19 that requires further clinical care.