Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium.

PURPOSE: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. METHODS: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. RESULTS: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). CONCLUSION: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. LEVEL OF EVIDENCE: Level III.

Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium.

PURPOSE: Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF. METHODS: The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers. RESULTS: 292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p>0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p<0.001), but no difference in the incidence of recurrent fistula (p=0.3). Empiric postoperative antibiotics for >24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p>0.3). Hospital volume was not associated with postoperative complication rates (p>0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7. CONCLUSION: Morbidity after primary repair of proximal EA and distal TEF patients is substantial, and many common practices do not appear to reduce complications. Specifically, this large retrospective series does not support the use of prophylactic antibiotics beyond 24h and empiric acid suppression may not prevent complications. Use of a transanastomotic tube was associated with higher rates of stricture, and interposition of prosthetic material was associated with higher leak rates. Routine postoperative esophagram can be safely obtained on day 5 resulting in earlier initiation of oral feeds. STUDY TYPE: Treatment study. LEVEL OF EVIDENCE: III.

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula.

BACKGROUND/PURPOSE: Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS: A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS: Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION: Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE: Type of study: Treatment study. Level IV.

The Relationship of Endoscopic Proficiency to Educational Expense for Virtual Reality Simulator Training Amongst Surgical Trainees.

With the introduction of Fundamentals of Endoscopic Surgery, training methods in flexible endoscopy are being augmented with simulation-based curricula. The investment for virtual reality simulators warrants further research into its training advantage. Trainees were randomized into bedside or simulator training groups (BED vs SIM). SIM participated in a proficiency-based virtual reality curriculum. Trainees' endoscopic skills were rated using the Global Assessment of Gastrointestinal Endoscopic Skills (GAGES) in the patient care setting. The number of cases to reach 90 per cent of the maximum GAGES score and calculated costs of training were compared. Nineteen residents participated in the study. There was no difference in the average number of cases required to achieve 90 per cent of the maximum GAGES score for esophagogastroduodenoscopy, 13 (SIM) versus11 (BED) (P = 0.63), or colonoscopy 21 (SIM) versus 4 (BED) (P = 0.34). The average per case cost of training for esophagogastroduodenoscopy was $35.98 (SIM) versus $39.71 (BED) (P = 0.50), not including the depreciation costs associated with the simulator ($715.00 per resident over six years). Use of a simulator appeared to increase the cost of training without accelerating the learning curve or decreasing faculty time spent in instruction. The importance of simulation in endoscopy training will be predicated on more cost-effective simulators.

Training techniques in laparoscopic donor nephrectomy: a systematic review.

The learning curve to achieve competency in laparoscopic donor nephrectomy (LDN) is poorly outlined. Online databases were searched for training in LDN. Abstracts and manuscripts were excluded if they did not address introduction of a laparoscopic technique for donor nephrectomy. Relevant manuscripts were reviewed for surgical technique, use of animal models, co-surgeons, surgeon specialty and training, institution type/volume, and assessment of training method. Forty-four met inclusion criteria, with 75% describing the evolution from open to LDN. Eighty-two percent were from academic centers, and 36% were from centers performing <25 donor nephrectomies each year. The learner was an attending surgeon 80% of the time, mostly urologists with prior laparoscopy or open nephrectomy experience. The learning curve, defined by decreased operating time, averaged 35 cases. Improved intra-operative, patient, and recipient outcomes were observed for centers performing ≥50 LDNs annually. The United Network of Organ Sharing requires 15 cases as surgeon or assistant to be certified as the primary LDN surgeon. This falls below the described learning curve for LDN. The assessment of training and competency for LDN is heterogeneous, and objective learner-based metrics could help surgeons and institutions reach a quality standard for performing this operation.