Swallowing-related quality of life in children with oesophageal atresia: a national cohort study.

Swallowing and feeding disorders are a major concern for children with oesophageal atresia (OA) after primary or staged OA repair. Primary OA repair is associated with higher rates of short-term complications in preterm infants with very low birth weight (VLBW) or extreme low birth weight (ELBW). On the other hand, primary repair may have the benefit of early commencement of oral feedings. We hypothesize that also in the medium-term, swallowing-related quality of life is better after primary oesophageal repair. We conducted a prospective cross-sectional study on swallowing quality in a national cohort of former VLBW and ELBW children with OA, using the structured paediatric swallowing quality of life (pedSWAL-QOL) questionnaire. Results were correlated with surgical approach and baseline clinical data. Principal component analysis of pedSWAL-QOL domains was performed. In total, 44 complete data sets of 78 children were available. The mean age of children was 8.5 years (SD = 7.4), and 23 children (52%) had primary OA repair. The overall median pedSWAL-QOL score was 2 (IQR = 0-3), representing a high swallowing-related quality of life, independent of surgical technique (p = 0.086). Children with a history of intracranial haemorrhage (ICH) (p = 0.002) and those with VACTERL association (p = 0.008) had significantly decreased enjoyment with eating. In addition, children with VACTERL association had problems to find suitable foods (p = 0.04). CONCLUSION: In this national cohort of VLBW and ELBW preterm-born children with OA, swallowing-related quality of life is good, mostly independent of initial surgery. Children with OA and ICH or VACTERL association may require more intense support with feeding. WHAT IS KNOWN: • Dysphagia, resembling feeding and swallowing disorders, is common in children and adults with repaired oesophageal atresia. Nevertheless, dysphagia in children with oesophageal atresia decreases with age. • Parents of younger children suffer from increased anxiety and fear regarding eating and swallowing abilities of their children. WHAT IS NEW: • Swallowing-related quality of life in former preterm children with oesophageal atresia is good, independent of initial surgical approach (primary vs. staged repair), even in very low birth weight or extreme low birth weight infants. • Children suffering from VACTERL association or intracranial haemorrhage show decreased enjoyment with eating.

Esophageal Perforation and EVAC in Pediatric Patients: A Case Series of Four Children.

Introduction: In pediatric patients, esophageal perforation (EP) is rare but associated with significant morbidity and mortality rates of up to 20-30%. In addition to standard treatment options, endoscopic esophageal vacuum-assisted closure (EVAC) therapy has shown promising results, especially in adult patients. Thus far, the only data on technical success and effectiveness of EVAC in pediatric patients were published in 2018 by Manfredi et al. at Boston Children's Hospital. The sparse data on EVAC in children indicates that this promising technique has been barely utilized in pediatric patients. More data are needed to evaluate efficacy and outcomes of this technique in pediatric patients. Method: We reviewed five cases of therapy using EVAC, ArgyleTM Replogle Suction Catheter (RSC), or both on pediatric patients with EP in our institution between October 2018 and April 2020. Results: Five patients with EP (median 3.4 years; 2 males) were treated with EVAC, RSC, or a combination. Complete closure of EP was not achieved after EVAC alone, though patients' health stabilized and inflammation and size of EP decreased after EVAC. Four patients then were treated with RSC until the EP healed. One patient needed surgery as the recurrent fistula did not heal sufficiently after 3 weeks of EVAC therapy. Two patients developed stenosis and were successfully treated with dilatations. One patient treated with RSC alone showed persistent EP after 5 weeks. Conclusion: EVAC in pediatric patients is technically feasible and a promising method to treat EP, regardless of the underlying cause. EVAC therapy can be terminated as soon as local inflammation and C-reactive protein levels decrease, even if the mucosa is not healed completely at that time. A promising subsequent treatment is RSC. An earlier switch to RSC can substantially reduce the need of anesthesia during subsequent treatments. Our findings indicate that EVAC is more effective than RSC alone. In some cases, EVAC can be used to improve the tissues condition in preparation for a re-do surgery. At 1 year after therapy, all but one patient demonstrated sufficient weight gain. Further prospective studies with a larger cohort are required to confirm our observations from this small case series.

Outcome of Patients With Esophageal Atresia and Very Low Birth Weight (≤ 1,500 g).

Introduction: Primary repair of esophageal atresia (EA) in infants with very low birth weight (VLBW) and extremely low birth weight (ELBW) has been widely performed in pediatric surgery. However, several studies have shown that complication rates in infants with VLBW are high. We hypothesize preterm children benefit from a shorter, less-traumatizing operation in the first days of life, as staged repair implies. Methods: Patients with EA and VLBW were retrieved from the database of a large national patient organization KEKS e.V. Structured questionnaires were sent to all the patients' families; the responses were pseudonymized and sent to our institution. Results: Forty-eight questionnaires from patients were analyzed. The mean birth weight was 1,223 g (720-1,500 g). Primary repair was performed in 25 patients (52%). Anastomotic insufficiency (AI) was reported in 9 patients (19%), recurrent fistula (RF) in 8 (17%), and anastomotic stenosis in 24 patients (50%). Although AI was almost twice as common after primary repair than after staged repair (24 vs. 13%; p = 0.5), the difference was not statistically significant. RF was more frequent after primary repair (28 vs. 4%; p = 0.04), gastroesophageal reflux was more frequent in the group after staged repair (78 vs. 52%; p = 0.04), and both correlations were statistically significant. Intracranial hemorrhage (ICH) was reported in 11 patients (23%) and was observed in 7 of them (64%, p = 0.4) after primary repair. ICH was reported in 60% of patients with ELBW and 75% of patients when ELBW was paired with primary repair. Conclusion: This study demonstrates the complication rate in patients with VLBW is higher than the average of that in patients with EA. The study indicates that a staged approach may be an option in this specific patient group, as less RF and AI are seen after staged repair. ICH rate in patients with ELBW seemed to be especially lower after staged repair. Interestingly, gastroesophageal reflux was statistically significantly higher in the group after staged repair, and postoperative ventilation time was longer. It is therefore necessary to individually consider which surgical approach is appropriate for this special patient group.