Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry.

 The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA).  The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500 g), LWB = 1,500-2,500 g), and normal BW (NBW; >2,500 g).  Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p < 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases (p < 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis (p < 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p < 0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis.  The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.

Oztan MO ，Soyer T ，Oztorun CI ，Firinci B ，Durakbaşa ÇU ，Dokumcu Z ，Gollu G ，Akkoyun I ，Demirel D ，Karaman A ，Ciftci I ，Ilhan H ，Parlak A ，Ozden O ，Cömert HSY ，Oral A ，Tekant G ，Kiyan G ，Erginel B ，Guvenc U ，Erdem AO ，Erturk N ，Yildiz A ... -  《-》

The effect of azygos vein preservation on postoperative complications after esophageal atresia repair: Results from the Turkish Esophageal Atresia Registry.

Preservation of the azygos vein (AV) maintains normal venous drainage of the mediastinum and decreases postoperative congestion. The modification of esophageal atresia (EA) repair by preserving AV may prevent postoperative complications and may lead to better outcomes. The data from the Turkish Esophageal Atresia Registry (TEAR) were evaluated to define the effect of AV preservation on postoperative complications of patients with EA. Data from TEAR for a period of five years were evaluated. Patients were enrolled into two groups according to the preservation of AV. Patients with divided (DAV) and preserved AV (PAV) were evaluated for demographic and operative features and postoperative complications for the first year of life. The DAV and PAV groups were compared according to the postoperative complications, such as fistula recanalization, symptomatic strictures, anastomotic leaks, total number of esophageal dilatations, and anti-reflux surgery. In addition, respiratory problems, which required treatment, were compared between groups. Among 502 registered patients; the data from 315 patients with the information of AV ligation were included. The male female ratio of DAV (n = 271) and PAV (n = 44) groups were 150:121 and 21:23, respectively (p > 0.05). The mean body weight, height, gestational age, and associated anomalies were similar in both groups (p > 0.05). The esophageal repair with thoracotomy was significantly higher in DAV group, when compared to the PAV group (p < 0.05). The rates of primary anastomosis and tensioned anastomosis were similar in both groups (p > 0.05). There was no difference between DAV and PAV groups for anastomotic leaks, symptomatic anastomotic strictures, fistula recanalization, and the requirement for anti-reflux surgery (p > 0.05). The rate of respiratory problems, which required treatment, was significantly higher in the DAV group (p < 0.05) CONCLUSION: The data in the TEAR demonstrated that preserving the AV during EA repair led to no significant advantage on postoperative complications, with exception of respiratory problems. AV should be preserved as much as possible to maintain a normal mediastinal anatomy and to avoid respiratory complications.

Soyer T ，Öztorun Cİ ，Fırıncı B ，Durakbaşa ÇU ，Bahadır GG ，Karaman A ，Dökümcü Z ，Akkoyun İ ，Demirel BD ，Öztan MO ，Çiftçi İ ，İlhan H ，Yalçın S ，Özden Ö ，Tekant GT ，Kıyan G ，Oral A ，Güvenç Ü ，Parlak A ，Erginel B ，Yıldız A ，Erdem AO ，Uzunlu O ，Ertürk N ，Aydın E ，Samsum H ，Arslan UE ... -  《-》

Outcome of primary repair in extremely and very low-birth-weight infants with esophageal atresia/distal tracheoesophageal fistula.

The optimal surgical management of extremely (ELBW) and very low-birth-weight (VLBW) neonates with esophageal atresia and distal tracheoesophageal fistula (EA/TEF) (Gross type C) is still debated. The aim of this study was to evaluate the surgical outcome of primary repair in these patients and compare it to ≥1500g neonates. Medical records of neonates with repaired EA from 2002 to 2016 were reviewed. 4 ELBW, 7 VLBW, and 24 ≥1500g infants had type C EA/TEF and underwent primary repair. Anastomotic leakage occurred in 0% ELBW, 0% VLBW and 8.3% ≥1500g patients and anastomotic stricture in 25% ELBW, 28.5% VLBW and 37.5% ≥1500g patients. 50% ELBW, 14.2% VLBW and 20.8% ≥1500g patients underwent secondary fundoplication. One patient of the VLBW group and one patient of the ≥1500g group died postoperatively of causes not related to EA/TEF. In extremely and very low-birth-weight neonates with type C EA/TEF surgical outcome after primary repair is comparable to the outcome in ≥1500g neonates. Primary repair can be performed in most of these patients and staged repair can be restricted to unstable patients. Treatment study level III.

Schmidt A ，Obermayr F ，Lieber J ，Gille C ，Fideler F ，Fuchs J ... -  《-》

Esophageal atresia/tracheoesophageal fistula in very low-birth-weight neonates: improved outcomes with staged repair.

Petrosyan M ，Estrada J ，Hunter C ，Woo R ，Stein J ，Ford HR ，Anselmo DM ... -  《-》

Staged repair improves outcome of high-risk premature infants with esophageal atresia and tracheoesophageal fistula.

Alexander F ，Johanningman J ，Martin LW 《JOURNAL OF PEDIATRIC SURGERY》