Development of a Multi-institutional Registry of Long Gap Esophageal Atresia: Results from a New Regional Pediatric Surgery Consortium

Background: Long gap esophageal atresia (LGEA) occurs in 4-8% of esophageal atresia (EA) cases and there is significant heterogeneity in the diagnosis, management, and outcomes. A regional pediatric surgery research collaborative sought to develop a retrospective multi-institutional registry to characterize the demographics, diagnosis, management and outcomes of LGEA patients.

Methods: Characteristics of patients diagnosed with LGEA between December 2009 and December 2018 were entered into a HIPAA compliant REDCap multicenter research database comprised of pediatric tertiary referral centers. LGEA was defined as the inability to bring the esophageal ends together during an initial surgical procedure or by the treating physician on pre-operative imaging. Re-operative LGEA patients were excluded. Charts were reviewed for demographics, perioperative management, and outcomes. Descriptive statistics were calculated using SAS.

Results: Sixty-nine patients with LGEA were entered by eight institutions (Table 1). Twenty-five (36%) patients were prenatally diagnosed (72% had an absent stomach and 68% had polyhydramnios). Average birth weight was 2.2 kg (SD = 0.6) with an average gestational age of 35.4 weeks (SD = 3.2). Common comorbidities included congenital heart disease (50%) and VACTERL (23%). Eighty three percent of patients had definitive repair at the institution where LGEA was diagnosed and 19% were transferred in from another facility. Most patients (60%) had a delay in an attempt at a primary anastomosis to enable growth of the neonate (defined as >1 month from birth), of which the majority (56%) were delayed between 2-3 months and 23% delayed 4-6 months. The average gap length at time of delayed repair was 3.5cm (SD = 1.3, n = 12) and in vertebral bodies was 2.8 (SD = 1.3, n = 16). Nearly one third of patients (28%) had traction sutures placed as a part of their LGEA management. There were one reported case of esophageal replacement (gastric tube). Complications at 30 days post-op included anastomotic leak (23%), airway complications (10%), and anastomotic stricture (21%). Airway complications consisted of prolong intubation (3%); tracheomalacia (3%) and other (3%).Gastroesophageal reflux disease was diagnosed in 39% of patients. There was no mortality reported at 30 days post-op (n = 63). Table 2 provides an overview of oral intake over the course of 90 days post-op. 47 patients (68%) completed 90-day follow-up.

Conclusion: In rare congenital diseases such as LGEA, there is a benefit in collaboration between regional institutions to attain the patient numbers needed to evaluate perioperative strategies. This early review of 69 LGEA patients highlights that use of esophageal replacement is uncommon and preservation of the native esophagus is possible in most cases. Continued enrollment will help provide further insight into the nuances of LGEA management across a regional tertiary pediatric surgery consortium.