Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia.

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.

Impact of Esophageal Atresia on the Success of Fundoplication for Gastroesophageal Reflux.

OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P < .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.