Transient hypogammaglobulinemia of infancy and unclassified syndromic immunodeficiencies are highly common in oesophageal atresia patients.

Due to the high rate of post-operative sepsis and other infectious complications, a routine immunological screening protocol has been initiated since 2015 in our paediatric surgery clinic for all patients admitted with oesophageal atresia (EA) and warrant a delayed definitive treatment. In our study, we aimed to evaluate the immunodeficiencies in EA patients, by comparing them to healthy age-matched controls. As a prospective cohort study, EA patients admitted between 2015 and 2022, who had their definitive operation after the newborn period (>28 days of age) were included. On admission, serum concentrations of IgG, IgA, IgM, lymphocyte subset levels, C3 and C4 levels, specific IgG antibody responses against hepatitis B, hepatitis A, measles, varicella zoster were evaluated. The patients were age-matched with healthy controls to compare the results and followed up until three years of age. If a humoral immunodeficiency was detected, intravenous immunoglobulin treatment was administered before major oesophageal surgery and during follow-up. 31 EA patients (18 M/13F) with a mean age of 13.3 ± 9.0 months were compared with 40 age-matched healthy controls. Mean serum IgG levels were found to be statistically lower than controls in all age groups (P < .05). Transient hypogammaglobulinemia of infancy (THI) and unclassified syndromic immunodeficiencies (USI) were found to be strikingly high, accounting for 29.0% and 22.5%, respectively, adding up to 51.5% of EA patients. This is the first study evaluating immunodeficiencies in EA patients found in the reviewed literature. More than half of EA patients that required delayed surgery had humoral immunodeficiency, so preoperative screening and immunology referral may improve patient outcomes.

A rare complication of pediatric liver transplantation: Post-transplant diaphragmatic hernia.

AIM: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed. METHODS: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated. RESULTS: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period. CONCLUSION: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases.

Long-term surgical outcomes in pediatric ovarian neoplasms: 20-year single-center experience.

PURPOSE: The management of pediatric ovarian neoplasms (ON) is based on finding a balance between adequate surgical treatment and future reproductive capacity. We aimed to evaluate long-term results of patients who underwent surgery for ON. METHODS: A retrospective cohort study design was used. Medical records of patients with ON were reviewed. They were invited to participate in a telephone-based survey assessing complaints, menstrual status, and post-surgical recurrence. RESULTS: Eighty-five patients were operated for ON between 1995 and 2015. Median age at surgery was 14.7 years. 62.4% of patients had ovary-sparing surgery (OSS). Median tumor size in oophorectomy group was significantly larger than OSS group (p = 0.029). Median length of follow-up was 5.1 years. Recurrent/metachronous disease was not significantly different between OSS and oophorectomy groups (p = 1.000). In OSS group, irregular menses (p = 0.004) and painful menses (p = 0.002) were significantly higher than oophorectomy group. CONCLUSION: The main goal of treatment in pediatric ON is to find the right balance between adequate and appropriate tumor resection and maximal effort for fertility preservation. Our results showed no difference between oophorectomy and OSS in the terms of recurrence. Although irregular and painful menses were found to be significantly higher in the OSS group, longer follow-up and prospective studies are needed to clarify this issue.

Fiberoptic endoscopic evaluation of swallowing (FEES) study: the first report in children to evaluate the oropharyngeal dysphagia after esophageal atresia repair.

BACKGROUND: The aim of this study was to evaluate the swallowing problems by fiberoptic endoscopic evaluation of swallowing (FEES) study in both short- and long-gap patients after esophageal atresia (EA) repair. METHODS: Hospital records of patients who had undergone surgery for EA were reviewed retrospectively. Patients were divided into two groups as short-gap (SG) group (n:16) and long-gap (LG) group (n:10) to compare the swallowing problems. FEES study was performed, and the results were discussed in detail. RESULTS: There were twenty-six (16 M/10 F) patients with a mean age at evaluation was 7.52 ∓ 3.68 years. Mean follow-up period was 75.35 ∓ 44.48 months. In FEES study, pharyngeal phase abnormalities were detected in 10 patients (38.4%). Pharyngeal phase abnormalities were detected significantly higher in LG group (p:0.015). Laryngeal penetration/aspiration was seen in four patients on FEES study (15.3%). All of them was in LG group (40%). Laryngeal penetration/aspiration was seen significantly higher in LG group (p:0.014). CONCLUSION: This is the first study to conduct FEES study in children after esophageal atresia repair to evaluate their swallowing conditions. Even though our sample is small, swallowing problems are more common than expected in the cases of LG when compared to SG.

Long-term outcome and efficiency of symptom-selective approach to assess gastroesophageal reflux prior to gastrostomy in neurologically impaired children.

PURPOSE: To evaluate the necessity of preoperative screening for gastroesophageal reflux (GER) prior to gastrostomy in neurologically impaired children. METHODS: Medical records of neurologically impaired children, who have undergone laparoscopic gastrostomy between January, 2004 and June, 2018, were retrospectively reviewed. Before the year of 2014, all patients who required gastrostomy had been routinely screened for GER pre-operatively, but after the year of 2014, only the ones with GER-related symptoms were tested. The characteristics and outcomes of Routine Screening (RS) and Selective Screening (SS) periods were compared. RESULTS: There were 55 and 54 patients in the RS and SS periods, respectively. Demographics, primary pathologies, and mean follow-up durations (> 2 years) were similar. The rate of GER screening was significantly lower in the SS period (29.6% vs. 63.6%). The rate of Laparoscopic Nissen Fundoplication (LNF) combined with gastrostomy was significantly lower in the SS period (14.8% vs. 38.2%). During follow-up, the rates of new-onset GER symptoms (13% vs. 11.7%) and LNF requirement later on (6.5% vs. 8.8%) were statistically similar between the two periods. CONCLUSION: Routine screening for GER is not necessary prior to gastrostomy in neurologically impaired children. Symptom-selective screening algorithm is safe and efficient in the long term.

Intracellular localization of beta-catenin expression plays a possible prognostic role on the outcome of hepatoblastoma patients.

PURPOSE: Wnt/Beta-catenin pathway plays an essential role in liver development and regeneration. Abnormal activation in this pathway leads to development of hepatoblastoma (HB). Although its importance has invoked attention, its prognostic role is debatable. We aimed to evaluate the significance of intracellular localization of beta-catenin (BC) expression in the outcome of hepatoblastoma patients. METHODS: Medical records of HB patients between 2004 and 2018 were reviewed. Patients were grouped according to intracellular localization of BC expression by immunohistochemistry as being cytoplasmic or nuclear. Demographics, radiological images, PRETEXT classifications, vascular involvement, risk groups, chemotherapy responses, and survival rates were analyzed and compared between groups. RESULTS: There were 41 patients. Thirteen patients were excluded for unavailability of records in four, negative/unclear BC expressions in seven. Cytoplasmic expression of BC was observed in 17 patients whereas 13 patients displayed nuclear expression. Demographics were similar in both groups. Cytoplasmic BC expression was associated with poor chemotherapy response (p = 0.001) and increased vascular involvement (p = 0.0162) requiring more extensive surgeries (p = 0.039). CONCLUSION: Although the numbers are limited in our series, the intracellular localization of BC expression has been found to be a promising determining factor for hepatoblastoma prognosis. With larger patient series, more reliable results can be achieved.

Pulmonary Metastasectomy of Sarcoma: Is the Ratio of Surgical Margin to Nodule Size a Prognostic Factor?

BACKGROUND: Main prognostic factors of improved survival after pulmonary metastasectomy (PM) for osteogenic and soft tissue sarcomas are suggested as histological type, number and size of pulmonary nodules, and disease-free interval (DFI). METHODS: Sixty-nine patients who underwent PM between January 1999 and December 2017 were evaluated retrospectively. Relations between parameters and prognostic risk factors for overall survival (OS) and disease-free survival (DFS) were evaluated. RESULTS: Osteosarcoma was the most common histologic type (36.2%) and 21 of 25 cases were seen under the age 20 years (p < 0.001). Comparison of patient groups including osteosarcoma and nonosteosarcoma patients showed significant difference according to age (p < 0.001), nodule size (p = 0.033), ratio of surgical margin to nodule size (p = 0.007), and DFI (p = 0.039). Univariate analysis showed that the number of nodules (p = 0.008), ratio of surgical margin to nodule size (p = 0.001), and localization of nodule (p = 0.039) were significant factors associated with DFS. Also, nodule size (p = 0.042), number of nodules (p = 0.003), ratio of surgical margin to nodule size (p < 0.001), and laterality (p = 0.027) were significant prognostic factors associated with OS. Cut-off values of ratio of surgical margin to nodule size for DFS and OS were calculated as 0.94. Logistic regression analysis determined the ratio of surgical margin to nodule size as the common significant risk factor for DFS and OS. CONCLUSIONS: Our study showed that the ratio of surgical margin to nodule size ≥ 1 should be taken as a common risk factor for DFS and OS. Therefore, resection of nodules with the possible widest surgical margin is an important point of PM.

Integration of radiology and clinical score in pediatric appendicitis.

BACKGROUND: The efficacy of ultrasonography (US) and abdominal X-ray in combination with Pediatric Appendicitis Score (PAS) is complicated in the diagnosis of acute appendicitis. Abdominal X-ray is as useful as US with clinical assessment when evaluated by experienced pediatric radiologist in acute appendicitis. The aim of this study was to determine the value of US and abdominal X-ray for appendicitis in children when combined with clinical assessment based on PAS, and to establish a practical pathway for acute appendicitis in childhood. METHODS: A prospective, observational cohort study was conducted at an urban, academic pediatric emergency department. Patients were classified at low (PAS 1-4), intermediate (PAS 5-7), or high (PAS 8-10) risk for appendicitis. Low-risk patients were discharged with telephone follow-up in ≤10 days; those at intermediate risk underwent X-ray and US. High-risk patients received immediate surgical consultation. Patients were grouped on histopathology as having either proven acute appendicitis or no appendicitis. RESULTS: A total of 288 children were analyzed. Surgery was performed in 134 patients (46.5%), and 128 (95.5%) had positive histopathology. Mean PAS in the patients with and without appendicitis was 7.09 ± 1.42 and 4.97 ± 2.29, respectively (P = 0.00). The rate of missed cases was 6/288 (2%), and the negative appendectomy rate was 6/134 (4.4%). When the score cut-off was set at 6, the sensitivity and specificity of PAS was 86.7% and 63.1%, respectively. The diagnostic performance of daytime US had a sensitivity of 91.1% and specificity of 71.1%. Also, positive US or PAS >6 or both, had sensitivity and specificity 96.7% and 59.9%, respectively. CONCLUSION: US or abdominal X-ray in children with possible appendicitis should be integrated with PAS to determine the next steps in management. In the case of discordance between the clinical findings and radiology, prolonged observation or further imaging are recommended.

Thoracoscopic treatment of left-to-right shunt in a child with scimitar syndrome.

Scimitar syndrome represents a rare variant of partial anomalous pulmonary venous connection with right lung hypoplasia, dextrocardia, and concomitant airway-vessel abnormalities. Surgical correction is preferred in symptomatic patients or in patients with increased left-to-right shunt. In this report, the first case of scimitar syndrome with dual arterial supply and venous drainage to be treated with thoracoscopic approach is presented.

Changing bulking agent may require change in injection volume for endoscopic treatment of vesicoureteral reflux.

INTRODUCTION: Various bulking agents were utilized for endoscopic correction of VUR. A study reviewing multi-institutional data showed that the amount of injection material has increased over time with the purpose of improving success rates, which also resulted in costs. We noticed an opposite trend in our center since we started using a new bulking agent. The aim of this study was to evaluate evolution of our practice with different bulking agents. PATIENTS AND METHODS: Records of VUR patients who underwent subureteric injection with polyacrylate polyalcohol copolymer (PPC) and dextronomere hyaluronic acide (DxHA) between 2005 and 2014 were reviewed. Variation of different parameters throughout the study period was evaluated along with the success rate. Success was defined as complete resolution of reflux. RESULTS: A total of 260 patients with 384 refluxing units were included. The success rate was higher in PPC group compared to DxHA group. There was no statistically significant difference between years regarding distribution of VUR grade, body weight, patient height, and age in PPC group. Despite significant reduction in injection volume, success rate did not decrease through the years with PPC. CONCLUSION: Different bulking agents may require different injection volumes to achieve the same success rate in endoscopic treatment of vesicoureteral reflux. Habits gained with previous experience using other materials should be revised while using a new agent.

Laparoscopic Treatment of Median Arcuate Ligament Syndrome: A Rare Cause of Chronic Severe Abdominal Pain.

Median arcuate ligament syndrome is a rare disorder characterized by chronic postprandial abdominal pain and weight loss caused by compression on celiac artery. A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. The compression on celiac artery was detected on Doppler ultrasound and diagnosis was confirmed by computed tomography angiography. The patient underwent laparoscopic release of median arcuate ligament. There were no intraoperative complications; however, partial pain response was observed postoperatively that necessitated para-spinal ganglion blockage. The patient is symptom-free in 1-year follow-up period.

Acquired right diaphragmatic hernia following pediatric living donor orthotopic liver transplantation.

ADH following OLT is a rare entity. Herein, we report a case of Alagille syndrome who developed ADH secondary to OLT, and possible etiological causes are discussed in light of the literature.

Two-stage approach in the management of thoracic neuroenteric cyst with spinal extension: thoracoscopic excision following dorsal laminectomy.

BACKGROUND: Neuroenteric cysts (NC) are rare pathologies and localized generally in posterior mediastinum or abdomen where they may extend to spinal canal through a vertebral defect. Isolated spinal lesions require dorsal/ventral laminectomy and thoracic ones thoracotomy or thoracoscopy. Posterolateral approach via thoracotomy is generally performed for lesions with both thoracic and spinal components. Minimal invasive excision of a thoracic NC with spinal extension in an infant is presented herein. CASE REPORT: A term female newborn with an antenatal (26th week) diagnosis of congenital diaphragmatic hernia (CDH) was admitted. On physical examination, she was normal except mild dyspnea and CDH were excluded on radiogram. Left parenchymal opacity necessitated thorax tomography that revealed lobulated cystic lesion (6 × 3.5 × 4.5 cm) in posterior mediastinum. MRI showed intraspinal extension of the lesion through a hemivertebrae (6th). Two-stage procedure was planned for suspected neuroenteric cyst. First, intraspinal component was excised with dorsal laminectomy and the connection was closed. Then, the thoracic component was excised thoracoscopically. Histopathological evaluation confirmed the diagnosis. Total parenteral nutrition and high dose somatostatin analog was needed due to transient left chylothorax on postoperative course. She was well and symptom-free in postoperative period. CONCLUSION: Neuroenteric cysts may lead to misdiagnoses in antenatal period. MRI is critical to show spinal and vertebral pathologies in suspected cases. Thoracoscopy may safely be performed for thoracic lesions with spinal extension in two-stage approach following closure of the connection and excision of the spinal component.

Pulmonary arteriovenous malformation in children.

Although pulmonary arteriovenous malformations (PAVM) are relatively rare in children, they are important in the differential diagnosis of common pulmonary problems, such as hypoxemia, hemoptysis and dyspnea on exertion. We report the cases of two PAVM patients with different presentations and describe the treatment strategies.

The effect of postoperative ventilation strategies on postoperative complications and outcomes in patients with esophageal atresia: Results from the Turkish Esophageal Atresia Registry.

OBJECTIVES: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. STUDY DESIGN: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. RESULTS: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. CONCLUSION: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

Living With My Baby With Congenital Anomaly: A Qualitative Case Report.

This case report was made to understand the emotions, thoughts, and experiences of the mother, who was lying in the long-term neonatal intensive care unit. An individual in-depth interview was conducted once with the mother of the infant with the diaphragm hernia. The interview recorded and lasted approximately 30 min. The data were analyzed by inductive method and themes and codes were created. The mother of the infant with a diaphragmatic hernia was 31 years old, married with 2 children, and employed full-time. The infant was diagnosed antenatally at 37 weeks old, weighed 3.000 g, and was male. As a result of the interview, 3 main themes were identified: "Facing the disease," "Experiences in intensive care," and "Change in family life." The results show that having an infant with congenital anomaly affects the life of all family members and shows the problems experienced strikingly.

Is Temperature Monitoring Necessary in Pediatric Circumcision?

OBJECTIVE: Perioperative hypothermia occurs in the pediatric age group in the perioperative period at a high rate. In this study, it is aimed to reveal the incidence of perioperative hypothermia and the risk factors that play a role in its development in pediatric circumcision cases that have a brief operation duration. MATERIALS AND METHODS: This prospective observational cohort study included 100 children who underwent circumcision under general anesthesia. All patients were heated with a passive heater and hypothermia was interpreted as a drop in body temperature below <36˚C. The patients were divided into 2 groups: group 1 (patients with body temperature <36˚C) and group 2 (≥36˚C). Demographic data, the American Society of Anesthesiologists' Classification of Physical Health Score, premedication method, operation time, fluid amount, preoperative and postoperative temperature of patients as tympanic were recorded. RESULTS: The average age of the patients was 70 ± 40 months (median: 84) and 93% were ASA I. In 71% of patients, a decrease in body temperature and hypothermia developed in 39% compared to baseline. The duration of operation was longer in the group with hypothermia (30 min [15-70] vs. 25 min [15-60], P < .001). Only the duration of operation was determined as the independent risk factor associated with hypothermia (odds ratio: 1.103 [1.017-1.197], P=.018). CONCLUSION: In this study, it was found that high rates of hypothermia developed even in minor operations such as pediatric circumcision. The risk of hypothermia increases with the prolongation of surgery.

Comparison of the multi-epitope recombinant antigen DIPOL and hydatid fluid for the diagnosis of patients with cystic echinococcosis.

The use of serological tests containing multiple immunodominant antigens rather than single antigens have the potential to improve the diagnostic performance in Cystic Echinococcoses (CE) as a complement tool to clear the inconclusive imaging data. Here, we comparatively evaluated the diagnostic value of Hydatid Fluid (HF) and the recently described recombinant multi-epitope antigen DIPOL in IgG-ELISA in a clinically defined cohort of CE patients. The serum samples from 149 CE patients were collected just before surgical or Percutaneous- Aspiration- Injection- Reaspiration (PAIR) procedures. Additionally, serum samples of patients with other parasitic infections (n=49) and healthy individuals (n=21) were also included in the study as controls. To investigate the association between the genotype of the parasite and DIPOL, cyst materials from 20 CE patients were sequenced. In terms of overall sensitivity, HF was higher than DIPOL (82.55%,78.52%, respectively). However, while the sensitivity of HF was higher than DIPOL in patients with active and transitional cysts (83.3%, 75.4%, respectively), sensitivity of DIPOL in inactive cysts was higher compared to HF (95.6%, 78.3%, respectively). The sensitivity of DIPOL depending on cyst stage was statistically significant (P= 0.041). In terms of specificity, DIPOL was found to be better than HF (97.71%, 91.43%, respectively). By genotyping, the majority of 20 patients showed G1 genotype (80%). All patients harboring G3 and G1/G3 cyst genotypes were positive with both antigens, while 87.5% of patients with G1 genotype were seropositive with HF and 75% with DIPOL. The overall sensitivity and high specificity of DIPOL suggest that this recombinant protein containing immunodominant epitopes is a potential substitute for the HF by serological tests for the diagnosis of CE.

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

INTRODUCTION: 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). MATERIALS AND METHODS: 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). RESULTS: 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. CONCLUSION: 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.

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

AIM: 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. METHODS: 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. RESULTS: 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.