Congenital Esophageal Atresia Long-Term Follow-Up—The Pediatric Surgeon’s Duty to Focus on Quality of Life

Abstract

Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon’s management and needs to be performed in each phase of a child’s development.

Disease Specific Quality of Life as Part of the Long-term Follow-up for Children Born With Esophageal Atresia in an Academic Unit in South Africa - a Pilot Study

“…Similar to our results, they reported worse scores in the "eating' category in younger children in their cohort [22]. We would concur with them in concluding that older children develop skills in coping with eating e.g., reminding themselves to drink uids when eating (more than half of children always reminded themselves to drink uid with meals) and to eat slower [22]. In addition, 38% and 54% respectively of the older children in our cohort were never bothered by choking or vomiting while eating, which might indicate their adaptation to these particular feeding di culties as they get older.…”

“…In comparison, more children than parents in the 8-17 years group reported eating being negatively. In 2022, Ardenghi et al conducted a QoL study of children born with EA in their unit [22]. Similar to our results, they reported worse scores in the "eating' category in younger children in their cohort [22].…”

“…In 2022, Ardenghi et al conducted a QoL study of children born with EA in their unit [22]. Similar to our results, they reported worse scores in the "eating' category in younger children in their cohort [22]. We would concur with them in concluding that older children develop skills in coping with eating e.g., reminding themselves to drink uids when eating (more than half of children always reminded themselves to drink uid with meals) and to eat slower [22].…”

Reliability and Validity of the Polish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia

“…As for reports on QOL in children after EA repair, it is clearly visible that research methodology is of key importance [21,30,31]. A literature review has shown various results, but overall suggests that total generic QOL scores are lowered in patients with EA [21].…”

Missed Proximal Tracheoesophageal Fistula (TEF) in a Neonate with Type D Esophageal Atresia

“…Esophageal atresia (EA) is a rare congenital malformation with a reported incidence up to 1 in 3,000 live births worldwide, and can be classified according to the Gross classification. 1 This classification anatomically defines EA as types A to E, based on the relation between the esophageal pouches and the trachea. Type D is the rarest EA with double tracheoesophageal fistula (TEF) from the proximal and distal esophageal pouch, which occurs in 1 to 3% of EA cases.…”