Primary Esophageal Anastomosis Research Articles

Thoracoscopic Stage Internal Traction Repair Reduces Time to Achieve Esophageal Continuity in Long Gap Esophageal Atresia.

Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared. The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis-Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively-all patients had a contrast study-and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B. Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.

Use of Staged vs. Primary Repair in Thoracoscopic Esophageal Atresia Repair

IntroductionMorbidity after thoracoscopic primary repair of esophageal atresia (EA) is still high in many centers. We retrospectively assessed the outcomes of a center-specific standardized approach in a group of newborns with EA that had been classified into one of two surgical management groups. Methods38 consecutive newborns with EA (median birth weight: 2570 g, range: 1020–3880) were treated between 2013 and 2022. The patients were classified into one of two groups: one-stage or multi-stage approach. The decision was based on the patients' general condition, the results of preoperative tests and/or by local conditions during thoracoscopy. ResultsThirty patients (all with type C EA) underwent primary esophageal anastomosis and 8 patients (21%) underwent multi-stage surgery and delayed anastomosis. The decision to take a multi-stage approach was made in the following cases: hemodynamic instability (n = 3), severely hypoplastic (up to 2 cm) distal esophagus (n = 1), extremely high position of the proximal esophagus (n = 2) and in all patients with type A EA (n = 2). In the multi-stage group, the second-stage procedure was performed after a median of 13 days (range: 7–42). Overall survival for all patients was 89%, with a median follow-up of 4.5 years. We did not note either anastomotic leaks or conversion to the open technique in our cohort. ConclusionIn selected cases, the multi-stage approach can affect patient safety in terms of surgical morbidity. Considering multi-stage correction of EA in advance can positively affect outcomes, especially in terms of lower rates of anastomosis leakage and of conversion to open surgery. Level of evidenceIII.

A Minimally Interventional Approach to Oesophageal Atresia Repair With Early Enteral Feeding is Safe, Optimises Neonatal Outcomes, and Reduces Resource use

PurposeRecent series of newborn Oesophageal Atresia (OA) repair continue to report widespread use of chest drains, gastrostomy, routine contrast studies and parenteral nutrition (PN) despite evidence suggesting these are superfluous. We report outcomes using a minimally interventional approach to post-operative recovery. MethodsEthically approved (15/WA/0153), single-centre, retrospective case-note review of consecutive infants with OA 2000–2022. Infants with OA and distal trache-oesophageal fistula undergoing primary oesophageal anastomosis at initial surgery were included (including those with comorbidities such as duodenal atresia, anorectal malformation and cardiac lesions). Our practice includes routine use of a trans-anastomotic tube (TAT), no routine chest drain nor gastrostomy, early enteral and oral feeding, no routine PN and no routine contrast study. Data are median (IQR). ResultsOf total 186 cases of OA treated during the time period, 157 met the inclusion criteria of which 2 were excluded as casenotes unavailable. TAT was used in 150 infants. A chest drain was required in 13 (8%) and two infants had a neonatal gastrostomy. Enteral feeds were started on postoperative day 2 (2–3), full enteral feeds established by day 4 (4–6) and oral feeds started on day 5 (4–8). PN was required in 15%. Median postoperative length of stay was 10 days (8–17). Progress was quicker in term infants than preterm. One infant died of cardiac disease prior to neonatal discharge. Two planned post-operative contrast studies were performed (surgeon preference) and a further 7 due to clinical suspicion of anastomotic leak. Contrast study was therefore avoided in 94%. There were 2 anastomotic leaks; both presented clinically at day 4 and day 8 after oral feeds had been started. ConclusionOur minimally interventional approach is safe. It facilitates prompt recovery with lower resource use, reduced demand on nursing staff, reduced radiation burden, and early discharge home compared to published series without adversely affecting outcomes. Level of EvidenceLevel 4.

Clinical features of a tracheoesophageal fistula associated with complete tracheal rings.

A tracheoesophageal fistula (TEF) associated with complete tracheal rings (CTR) is extremely rare. This study aimed to elucidate the clinical features of this combination. Records of 39 patients diagnosed with TEF with or without CTR between January 2013 and February 2023 were retrospectively reviewed. Data collected included location of the TEF and CTR, esophageal gap, surgery of TEF/esophageal atresia (EA), and symptoms of CTR. Seven patients had CTR, while 32 patients did not have CTR. TEF was frequently located higher than the T4 vertebra, and the median esophageal gap was shorter in patients with CTR than in those without (5/7 [71.4%] vs. 6/32 [18.8%], P = 0.012; 0 (range: 0-15) mm vs. 13.3 (range: 0-40) mm, P = 0.017, respectively). TEF was located just above the beginning of the CTR in all patients. All patients with CTR underwent primary esophageal anastomosis. Additionally, two patients were diagnosed with CTR before TEF/EA repair. A tracheal tube was prevented from contacting the CTR during TEF/EA repair, and respiratory insufficiency did not occur. Our results may help pediatric surgeons in the management of TEF with CTR.

Perioperative management and outcomes in long-gap esophageal atresia: A retrospective analysis from the Eastern Pediatric Surgery Network

BackgroundLess than 10% of infants born with esophageal atresia (EA) have a long gap that precludes primary anastomosis at birth. The purpose of this study was to evaluate the diagnosis, management, and outcomes of infants with long-gap esophageal atresia (LGEA) within a regional consortium of children's hospitals. MethodsAfter IRB approval, a multicenter, retrospective cohort study was conducted of LGEA patients managed by 13 member institutions between 2009 and 2018. LGEA was defined as Type A or B esophageal atresia with the inability to perform a primary esophageal anastomosis at birth based on preoperative imaging or operating findings. Study outcomes, including operative repair, postoperative outcomes, and complications, were collected to detect significant associations between variables (p < 0.05). ResultsThere were 62 LGEA patients identified, including 50 (81%) with Type A and twelve (19%) with Type B esophageal atresia. Most (77%) were diagnosed prenatally with 98% undergoing a gastrostomy before attempted EA repair. The mean gap length at repair was 3.24 ± 1.59 cm (n = 21). Most (95%) were managed with delayed repair (median age at repair of 96 days (IQR: 67.5-131), and 22 (35%) underwent an esophageal traction-induced lengthening process. Two (3.2%) required esophageal replacement. There was no significant difference in complications between different treatment strategies. ConclusionsIn this collaborative descriptive study of 62 infants with LGEA, delayed primary repair with or without traction was the preferred approach, with outcomes that were comparable between strategies, and with high rates of esophageal preservation. We encountered variability in gap length measurement and reporting. This study highlights a critical need for a prospective, multi-institutional registry with uniform care pathways to help aid in the development of evidence-based guidelines for these challenging patients.

Outcome of long gap esophageal atresia at 6 years: A prospective case control cohort study

Summary Background DataEA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years. MethodsA multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed. ResultsThirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group. ConclusionsLong gap strongly influenced digestive morbidity at age 6 years.

Suture approximation of esophageal atresia leads to esophageal recanalization

Esophageal atresia is a congenital pathology in 1:4000 live newborns, which can occur isolated, associated with tracheoesophageal fistula or long gap (>2 vertebral bodies); the surgical objective is the ligation of the fistula and the esophageal anastomosis in the same surgery. However, this may not be possible in all cases [1]. The staged approach initially begins with the ligation of the tracheoesophageal fistula and gastrostomy, and definitive surgery focuses on the esophageal anastomosis. It is performed when the patient presents adequate clinical and anatomical conditions. Spontaneous recanalization of esophageal atresia is rare. The exact mechanism by which it occurs is unknown. Gastric reflux, being in contact with the esophageal distal end, generates inflammation and erosion of the wall, producing communication between the esophageal segments. We propose approximating the proximal and distal esophageal segments utilizing sutures produces an inflammatory process due to a foreign body generating a fistulous tract between the two esophageal components and esophageal recanalization [2]. We present two cases of esophageal recanalization to describe the clinical presentation, diagnostic and therapeutic approaches through imaging tests and endoscopy, contributing to the management of this unusual condition. • Esophageal atresia is a challenge; it presents variations in the location of the tracheoesophageal fistula and the length of the esophageal ends. It occurs isolated or associated with malformations. The pediatric surgeon must individualize treatment for each patient. • In all cases, extreme premature patients have hemodynamic alterations and fragile esophageal tissue, which do not allow performing a primary esophageal anastomosis. • Upper gastrointestinal endoscopy is a diagnostic and therapeutic resource in patients with spontaneous esophageal recanalization or the presence of a recurrent tracheoesophageal fistula.

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

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.

Detail correction for Gross classification of esophageal atresia based on 434 cases in China.

Detail correction for Gross classification of esophageal atresia based on 434 cases in China.

Influence of birth weight on primary surgical management of newborns with esophageal atresia

BackgroundTo determine if birth-weight (BW) influences primary surgical management of newborns undergoing operation for esophageal atresia and tracheo-esophageal fistula (EA-TEF). MethodsNewborns undergoing repair of esophageal atresia at a single specialist centre between 1999 and 2017 were categorised into three groups based on BW; Group A < 1.5 kg, Group B <2.5 kg and Group C >2.5 kg. Outcome data analysed were (i) technical ability of the surgeon to perform primary esophageal anastomosis, (ii) anastomotic leak, (iii) anastomotic stricture, (iv) esophageal replacement, (v) need for other procedures notably fundoplication, aortopexy, tracheostomy and (vi) mortality. Statistical analysis was performed using a two-tailed Fisher's exact test and logistic regression. Results198 patients underwent surgery for EA-TEF during the study period, Group A (n = 13), Group B (n = 73) and Group C (n = 112). Inability to perform a primary anastomosis was significantly higher in Group A vs Group B (p = 0.003) and Group C (p = 0.004). Birthweight was a significant variable in the ability to perform a primary esophageal anastomosis (OR 1.009, p = 0.004). Mortality rate was significantly higher in Group A vs Group C (P = 0.0158). ConclusionsVery low birth weight infants are less likely to achieve a definitive primary anastomosis during emergent repair of esophageal atresia, and have a higher mortality.

Association Between Administration of Antacid Medication and Anastomotic Stricture Formation After Repair of Esophageal Atresia

BackgroundAnastomotic stricture is a significant cause of morbidity after repair of esophageal atresia (EA). Exposure to gastric acid has been postulated to contribute to stricture development and severity leading to prophylactic antacid use by some surgeons. We investigated the association between administration of antacid medication and the development of anastomotic strictures. MethodsRetrospective case-note review of consecutive infants undergoing repair of EA with distal tracheoesophageal fistula (type C) between January 1994 and December 2014. Only infants who underwent primary esophageal anastomosis at initial surgical procedure were included. Stricture-related outcomes were compared initially for infants who received prophylactic antacid medication (PAAM) versus no prophylaxis, and the role of PAAM in stricture prevention was explored in a multivariate model. Outcomes were also compared for infants grouped by antacid use at any stage. ResultsOne hundred fourteen infants were included. Sixteen infants received PAAM at surgeon preference. Of the remaining 98 infants, 44 subsequently received antacid as treatment for gastroesophageal reflux (GER) and 54 never received antacid medication. There was no statistically significant association between incidence of stricture in the first year (10 of 16 versus 41 of 98; P = 0.18) nor time to first stricture (median, 57 d [41-268] versus 102 d [43-320]; P = 0.89) and administration of PAAM. Similarly, there were no statistically significant associations between incidence of stricture, age at first stricture and number of dilatations, and administration of antacid medication either as prophylaxis nor when given as treatment for symptoms or signs of GER. ConclusionsThese data do not support the hypothesis that PAAM reduces the incidence or severity of anastomotic stricture after repair of EA. Treatment with antacids may be best reserved for those with symptoms or signs of GER. Further prospective investigation of the role of antacid prophylaxis on stricture formation after EA repair is warranted.

Low gestational age is associated with less anastomotic complications after open primary repair of esophageal atresia with tracheoesophageal fistula

BackgroundThe aim of this study was to evaluate anastomotic complications after primary one-staged esophageal atresia (EA) repair relating to the patients` gestational age (GA).MethodsRetrospective data analyses of patients who underwent closure of tracheoesophageal fistula (TEF) and primary esophageal anastomosis from 01/2007 to 12/2018 in two pediatric surgical centers. Exclusion of EA other than Gross type C, long-gap EA, minimal invasive or staged approach. Postoperative complications during the first year of life were assessed. Associated malformations, the incidence of infant respiratory distress syndrome (IRDS) and intraventricular bleeding were analyzed.ResultsInclusion of 75 patients who underwent primary EA repair. Low GA was associated with significantly lower incidence of anastomotic complications (p = 0.019, r = 0.596, 95% CI 0.10–0.85). Incidence of anastomotic leakage (0% vs. 5.5%; p = 0.0416), recurrent TEF (0% vs. 5.5%; p = 0.0416) und anastomotic stricture (0% vs. 14.5%; p = 0.0019) was significantly lower in patients < 34 gestational weeks. Incidence of IRDS (55% vs. 0%; p < 0.0001) and intraventricular bleeding (25% vs. 3.6%; p = 0.0299) was significantly higher in patients < 34 gestational weeks.ConclusionsDespite prematurity-related morbidity, low GA did not adversely affect surgical outcome after primary EA repair. Low GA was even associated with a better anastomotic outcome indicating feasibility and safety of primary esophageal reconstruction.

Repair of oesophageal atresia by consultants and supervised trainees results in similar outcomes.

Consultants and trainees require exposure to complex cases for maintaining and gaining operative experience. Oesophageal atresia (OA) repair is a neonatal surgical procedure with indicative numbers for completion of training. A conflict of interest may exist between adequate training, maintaining consultant experience and achieving good outcomes. We aimed to review outcomes of procedures performed primarily by trainees and those performed by consultants. We carried out a retrospective case note review of all consecutive infants who underwent surgical repair of OA with distal tracheooesophageal fistula (TOF) between January 1994 and December 2014 at our institution. Only cases that underwent primary oesophageal anastomosis were included. Surgical outcomes were compared between cases that had a trainee and those that had a consultant listed as the primary operator. One hundred and twenty-two cases were included. A total of 52 procedures were performed by trainees, and 68 by consultants. Two cases were undeterminable and excluded. Infant demographics, clinical characteristics and duration of follow-up were similar between groups. All infants survived to discharge. Procedures performed by trainees and those performed by consultants as primary operators had a similar incidence of postoperative pneumothorax (trainees 4, consultants 3; p=0.46), anastomotic leak (trainees 5, consultants 3; p=0.29) and recurrent TOF (trainees 0, consultants 2; p=0.5). Overall 52% of cases had an anastomotic dilatation during follow-up, with no difference between the trainee and consultant groups (50% vs 53%; p=0.85). Surgical outcomes for repair of OA/TOF are not adversely affected by trainee operating. Trainees with appropriate skills should perform supervised OA/TOF repair. These data are important for understanding the interrelationship between provision of training and surgical outcomes.

Delayed Diagnosis in Esophageal Atresia and Tracheoesophageal Fistula

Timely identification of esophageal atresia is challenging. Diagnosis may be suspected antenatally with a combination of polyhydramnios, associated with a small or absent stomach bubble or other anomalies. Esophageal atresia can be suspected postnatally in the presence of tachypnea, increased oral secretions, and an inability to advance an orogastric tube. Failure to recognize an esophageal atresia can have life-threatening implications. A 5-day-old infant with a history of failure to thrive and respiratory distress presented in a community emergency department following a prolonged apnea associated with a breastfeed. Delayed postnatal diagnosis of esophageal atresia and tracheoesophageal fistula. During stabilization in the emergency department, a nasogastric tube was placed to decompress the stomach. A subsequent chest and abdominal radiograph identified the nasogastric tube curled in the upper esophagus, confirming an esophageal atresia. The abdominal radiograph demonstrated gaseous distension, suggesting the presence of a distal tracheoesophageal fistula. The neonate had a primary esophageal anastomosis and fistula ligation in a surgical neonatal unit. He was discharged home at 29 days of life. Understanding the challenges of an antenatal diagnosis and awareness of postnatal presentation with a view to improving postnatal recognition and better-quality outcomes for infants with an esophageal atresia and tracheoesophageal fistula.

DOZ047.36: Thoracoscopic esophageal internal axial traction for long- gap esophageal atresia

Abstract Aim of the Study Long-gap esophageal atresia (LGEA) remains a surgical challenge. This study aimed to report the results of thoracoscopic esophageal axial internal traction in LGEA. Methods This multicenter observational study included retrospectively neonates who underwent primary thoracoscopic esophageal axial internal traction for LGEA between June 2017 and July 2018. LGEA was defined as the technical impossibility to perform a primary esophageal anastomosis. The Ethical Review Board of our institution approved the study. Main Results Eight neonates were included with a median gestational age at birth of 35 weeks [25; 37] and a median birth weight of 2266 g [890; 3800], 6 types I and 2 types II according to Ladd's classification. Initial median gap between 2 esophageal ends was 5 vertebral bodies [4.5; 7]. Internal traction was performed at a median age of 5 weeks of life [1; 17] with a median operative time of 87 minutes. Four patients required at least 2 internal traction procedures. After a mean traction time of 1.5 weeks [1; 13.5], esophageal anastomosis was successfully performed in 7 patients (5 thoracoscopies, 2 thoracotomies) with a median operative time of 165 minutes. One patient needed a colonic interposition. Five of these 7 patients required an esophageal endoscopic dilatation (median number: 4 [2; 6]). Median follow-up was 9.75 months [3; 16]. Conclusions Thoracoscopic esophageal axial internal traction for LGEA was a safe and feasible procedure that allowed an esophageal anastomosis in 7 of the 8 patients. Improvement of the procedure requires setting a common protocol concerning the timing of the first internal axial traction and the duration of traction before considering esophageal anastomosis.

Primary repair of long gap esophageal atresia in a neonate employing circular myotomy on upper pouch and a novel hemicircular myotomy on the distal pouch

Abstract Introduction Pure esophageal atresia (EA) with long gap between the pouches remains a challenge for primary repair. Several techniques to facilitate primary repair in long gap EA have been described: complete mobilization of proximal and/or distal esophageal pouch, single or double circular myotomies or spiral myotomy of upper pouch, circular myotomy of both upper and lower pouch, flaps raised from the upper pouch to bridge the gap and external traction sutures. Some of these techniques are not routinely performed for anatomical reasons or unfamiliarity with the technique. Complete mobilization of upper pouch with circular myotomy is standard and often favored approach. Complete mobilization of lower pouch with or without circular myotomy is described. It has a risk of impairing the blood supply to the lower pouch due to segmental nature of its blood supply. We present a novel case of primary repair of this defect using a circular myotomy on upper pouch along with a hemicircular myotomy of the lower pouch without complete circumferential mobilization of the lower esophageal pouch. This allowed adequate lengthening of the lower pouch without compromising its blood supply facilitating a minimal tension primary repair. Case We report the case of a 38 4/7 term infant with intra-uterine growth retardation (IUGR) and Tetralogy of Fallot (TOF) with a long 4.5 cm gap pure esophageal atresia who was repaired aged 16 weeks. Per-operative gap assessment revealed the gap to more than 4 vertebral bodies. Even after complete mobilization of the upper esophageal pouch, a 2.5 cm (cm) gap persisted. The upper pouch was narrow and hence unsuitable to facilitate flap reconstruction. A single circular myotomy was performed on the upper esophageal pouch approximately 1 cm proximal to its blind end. The lower pouch was then mobilized, taking care that its blood supply was not compromised. This still left a one cm gap that was overcome by performing a hemicircular (180°) myotomy on the lower esophageal pouch approximately 1 cm away from the blind end. A tension free primary esophageal anastomosis was then performed. Post operatively patient developed a stricture which was dilated successfully to 12 Fr (4 mm). No other complications are reported. Conclusion Hemicircular myotomy of the distal esophageal pouch can be employed successfully to achieve primary repair in cases of long gap esophageal atresia. This technique does not require full circumferential mobilization of lower pouch as required with full circular myotomy and has less risk of compromising blood supply to the distal esophageal pouch decreasing risks of anastomotic leak, failure and esophageal motility of lower pouch.

Management of esophageal atresia and early predictive factors of mortality and morbidity in a developing country.

The aim of this study is to investigate management and outcome in esophageal atresia (EA) and to identify early predictive factors of morbidity and mortality in a developing country. Charts of neonates with repaired EA from 2007 to 2016 were reviewed. Patients' characteristics, operative details, and postoperative outcomes were collected. Statistical analyses were performed to identify predictors of complicated evolution. Forty-two cases were collected. There were 14 girls and 28 boys. Only one patient had antenatal diagnosis (2.3%). The mean gestational age was 38 weeks. Nine patients (21.4%) weighed less than 2.5 kg. Seventeen (40.4%) patients had associated malformations most commonly cardiac (9/17). Thirteen patients had delayed diagnosis (30.9%). Thirty-nine (92.8%) patients underwent primary esophageal anastomosis. Overall survival was 76.2%. Nineteen patients (57% of survivals) had complicated evolution before the age of one year and 15 patients (46.8% of survivals) developed complications after the age of one year. Perinatal variables associated with mortality were prematurity (p=0.004, OR=5.4, IC95%=[1.13-25.80]), low birth weight (p=0.023, OR=7, IC95%=[1.38-35.47]), cardiac malformations (p=0.006, OR=10.5, IC95%=[2.03-54.27]) and delayed diagnosis (p=0.005, OR=10.11, IC95%=[2.005-50.980]). Variables associated with short-term and middle-term complications were duration of intubation (p=0.019, OR=0.118, IC95%=[0.019-0.713]) and the presence of short-term complications (p=0.016, OR=7.33, IC95%=[1.467-36.664]) respectively. These factors may be used to identify patients who will benefit from more intensive follow-up program.

Recurrent tracheoesophageal fistula in an adolescent without persistent symptoms

Rationale:Congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) ininfants have been treated well with surgery. Approximately 10% of children displayed recurrent fistula. In the present case, we reported recurrent TEF in an adolescent as a complication of EA/TEF in infancy.Patient concerns:An infant was diagnosed with gross type C congenital EA and TEF and subsequentlyunderwent repair in early infancy, with division of the TEF and primary esophageal anastomosis. Postoperative esophageal strictures developed and were relieved by bougienage of the esophagus partially. Then, the child had normal growth with mild symptoms, mainly choking when drinking water. At 11 years of age, the child developed fever and cough, and massive bronchiectasis in lobus inferior pulmonis sinister was found.Diagnosis:Recurrent tracheoesophageal fistula.Interventions:Division of the TEF and esophageal replacement with gastric tube was performed as treatmentOutcomes:The child recovered well.Lessons:Recurrent tracheoesophageal fistula aftercongenital EA and TEF could be diagnosed in adolescence. Massive bronchiectasis might develop without apparent symptoms.

Esophageal atresia and Tracheoesophageal fistula with associated anomalies in a tertiary care hospital of north India

Background: Esophageal atresia (EA) with or without Tracheoesophageal fistula (TEF) has been described as the epitome of pediatric surgery and the management of these neonates require high degree of skill, manpower and tertiary care. This clinical study was undertaken to identify the EA and TEF cases along with the pattern of associated anomalies causing mortality in Varanasi .Methods: This prospective study included 53 patients of EA with TEF who underwent primary esophageal anastomosis. Their clinical profile, time of presentation, various associated anomalies, sex distribution, and their effect on mortality and morbidity were observed.Results: Mortality was high (41.64%) among patients who reached the tertiary centre late i.e. &gt;24 hours. The incidence of low birth weight babies is approximately 75% and mortality rate increased with low birth weight. Almost 2/3 of patients had clinical or radiological evidence of pneumonia. The mortality was very high in severe pneumonia 66.7% as compared to 15% patients without pneumonia. Septicaemia and leak were most common complications. The mortality in septicemic patients is high (77.8%). The overall incidence of associated anomalies was 47%. Early surgical intervention improved the survival of EA with TEF with major GIT associated anomalies. The overall survival rate was 60.37% and mortality was 39.67%.Conclusions: Although we have improved a lot in managing these cases of EA with TEF, but we have to do much more to achieve our goal of near 100% survival.

13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count

BackgroundThe presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. MethodsBabies with EA from January 2005 – December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. ResultsSeventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs.Paradoxically, 10 patients with esophageal atresia +/− trachea-esophageal atresia (EA +/− TEF) and supernumerary ribs underwent primary repair.Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap.Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587.VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. ConclusionWe found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/− TEF. Level of evidencePrognosis study – level IV.