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

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. 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. 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. 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. Treatment study. III.

Lal DR ，Gadepalli SK ，Downard CD ，Ostlie DJ ，Minneci PC ，Swedler RM ，Chelius TH ，Cassidy L ，Rapp CT ，Billmire D ，Bruch S ，Burns RC ，Deans KJ ，Fallat ME ，Fraser JD ，Grabowski J ，Hebel F ，Helmrath MA ，Hirschl RB ，Kabre R ，Kohler J ，Landman MP ，Leys CM ，Mak GZ ，Raque J ，Rymeski B ，Saito JM ，St Peter SD ，von Allmen D ，Warner BW ，Sato TT ，Midwest Pediatric Surgery Consortium ... -  《-》

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula.

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. 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. 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%). 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. Type of study: Treatment study. Level IV.

Lal DR ，Gadepalli SK ，Downard CD ，Ostlie DJ ，Minneci PC ，Swedler RM ，Chelius T ，Cassidy L ，Rapp CT ，Deans KJ ，Fallat ME ，Finnell SME ，Helmrath MA ，Hirschl RB ，Kabre RS ，Leys CM ，Mak G ，Raque J ，Rescorla FJ ，Saito JM ，St Peter SD ，von Allmen D ，Warner BW ，Sato TT ，Midwest Pediatric Surgery Consortium ... -  《-》

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

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. 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. 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). 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 III.

Lal DR ，Gadepalli SK ，Downard CD ，Minneci PC ，Knezevich M ，Chelius TH ，Rapp CT ，Billmire D ，Bruch S ，Carland Burns R ，Deans KJ ，Fallat ME ，Fraser JD ，Grabowski J ，Hebel F ，Helmrath MA ，Hirschl RB ，Kabre R ，Kohler J ，Landman MP ，Leys CM ，Mak GZ ，Ostlie DJ ，Raque J ，Rymeski B ，Saito JM ，St Peter SD ，von Allmen D ，Warner BW ，Sato TT ，Midwest Pediatric Surgery Consortium ... -  《-》

Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair.

Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems. Retrospective review of 66 consecutive patients 2009-2016 (55 referrals and 11 local) who underwent repair via reoperative thoracotomy or cervicotomy for recTEF and acqTEF (IRB P00004344). Our surgical approach used complete separation of the airway and esophagus, which reliably revealed the TEF (without need for cannulation) and freed the tissues for primary closure of the trachea and frequently resection of the tracheal diverticulum. For associated esophageal strictures, stricturoplasty or resection was performed. Separation of the suture lines by rotational pexy of the both esophagus and the trachea, and/or tissue interposition were used to further inhibit re-recurrence. For associated severe tracheomalacia, posterior tracheopexy to the anterior spinal ligament was utilized. The TEFs were recurrent (77%), acquired from esophageal leaks (26%), in addition to persistent or missed H-type (6%). Seven patients in this series had multiple TEFs of more than one category. Of the acqTEF cases, 6 were esophagobronchial, 10 esophagopulmonic, 2 esophagotracheal (initial pure EA cases), and 2 from a gastric conduit to the trachea. Upon referral, 18 patients had failed endoluminal treatments; and open operations for recTEF had failed in 18 patients. Significant pulmonary symptoms were present in all. During repairs, 58% were found to have a large tracheal diverticulum, and 51% had posterior tracheopexy for significant tracheomalacia. For larger esophageal defects, 32% were treated by stricturoplasty and 37% by segmental resection. Rotational pexy of the trachea and/or esophagus was utilized in 62% of cases to achieve optimal suture line separation. Review with a mean follow-up of 35months identified no recurrences, and resolution of pulmonary symptoms in all. Stricture treatment required postoperative dilations in 30, and esophageal replacement in 6 for long strictures. There was one death. This retrospective review of 66 patients with postoperative recurrent and acquired TEF following esophageal atresia repair is the largest such series to date and provides a new categorization for postoperative TEF that helps clarify the diagnostic and therapeutic challenges for management.

Smithers CJ ，Hamilton TE ，Manfredi MA ，Rhein L ，Ngo P ，Gallagher D ，Foker JE ，Jennings RW ... -  《-》

Clinical outcomes following implementation of a management bundle for esophageal atresia with distal tracheoesophageal fistula.

This study evaluated compliance with a multi-institutional quality improvement management protocol for Type-C esophageal atresia with distal tracheoesophageal fistula (EA/TEF). Compliance and outcomes before and after implementation of a perioperative protocol bundle for infants undergoing Type-C EA/TEF repair were compared across 11 children's hospitals from 1/2016-1/2019. Bundle components included elimination of prosthetic material between tracheal and esophageal suture lines during repair, not leaving a transanastomotic tube at the conclusion of repair (NO-TUBE), obtaining an esophagram by postoperative-day-5, and discontinuing prophylactic antibiotics 24 h postoperatively. One-hundred seventy patients were included, 40% pre-protocol and 60% post-protocol. Bundle compliance increased 2.5-fold pre- to post-protocol from 17.6% to 44.1% (p < 0.001). After stratifying by institutional compliance with all bundle components, 43.5% of patients were treated at low-compliance centers (<20%), 43% at medium-compliance centers (20-80%), and 13.5% at high-compliance centers (>80%). Rates of esophageal leak, anastomotic stricture, and time to full feeds did not differ between pre- and post-protocol cohorts, though there was an inverse correlation between NO-TUBE compliance and stricture rate over time (ρ = -0.75, p = 0.029). Compliance with our multi-institutional management protocol increased 2.5-fold over the study period without compromising safety or time to feeds and does not support the use of transanastomotic tubes. Level II. Treatment Study.

Bence CM ，Rymeski B ，Gadepalli S ，Sato TT ，Minneci PC ，Downard C ，Hirschl RB ，Amin RA ，Burns RC ，Cherney-Stafford L ，Courtney CM ，Deans KJ ，Fallat ME ，Fraser JD ，Grabowski JE ，Helmrath MA ，Kabre RD ，Kohler JE ，Landman MP ，Lawrence AE ，Leys CM ，Mak GZ ，Port E ，Saito JM ，Silverberg J ，Slidell MB ，St Peter SD ，Troutt M ，Walker S ，Wright T ，Lal DR ，Midwest Pediatric Surgery Consortium ... -  《-》