Current Environment:

Thompson K, Zendejas B, Svetanoff WJ, Labow B, Taghinia A, Ganor O, Manfredi M, Ngo P, Smithers CJ, Hamilton TE, Jennings RW. Evolution, lessons learned, and contemporary outcomes of esophageal replacement with jejunum for children. Surgery. 2021 Jul;170(1):114-125. doi: 10.1016/j.surg.2021.01.036. Epub 2021 Apr 1. PMID: 33812755.

Review of a contemporary cohort of 55 patients who have undergone a jejunal interposition. We reflect on the lessons learned along the way and highlight the importance of microvascular augmentation (supercharging) to the prevention of esophageal anastomotic leaks. 

Firriolo JM, Nuzzi LC, Ganske IM, Hamilton TE, Smithers CJ, Ganor O, Upton J 3rd, Taghinia AH, Jennings RW, Labow BI. Supercharged Jejunal Interposition: A Reliable Esophageal Replacement in Pediatric Patients. Plast Reconstr Surg. 2019 Jun;143(6):1266e-1276e. doi: 10.1097/PRS.0000000000005649. PMID: 31136495.

Review of 20 patients who underwent a jejunal interposition with a supercharged microvascular anastomosis and overview of their favorable outcomes.

Thompson K, Yu JW, Hamilton TE, Nandivada P, Kaufman T, Ganor O, Labow B, Taghinia A, Zendejas B, Jennings RW. Jejunal interposition for esophageal replacement after lye ingestion in a pediatric patient. Videoscopy. Vol. 31, No. 2 April 2021 https://doi.org/10.1089/vor.2020.0680

Case report of a staged approach to esophageal replacement in a child who suffered a long-segment esophageal stricture after accidental lye ingestion. We performed first an anterolateral thigh flap (skin tube) as a neo-esophagostomy to his pharynx, allowed this to heal and later removed his prior failed colonic conduit performed elsewhere and reconstructed him with a supercharged jejunal interposition with a favorable outcome.

Ganske IM, Firriolo JM, Nuzzi LC, Ganor O, Hamilton TE, Smithers CJ, Jennings RW, Upton J 3rd, Labow BI, Taghinia AH. Double Supercharged Jejunal Interposition for Late Salvage of Long-gap Esophageal Atresia. Ann Plast Surg. 2018 Nov;81(5):553-559. doi: 10.1097/SAP.0000000000001520. PMID: 29905609.

Case reports of three patients with prior failed esophageal replacements who underwent reconstruction with a double supercharged jejunal interposition with favorable outcomes. 

Bairdain S, Foker JE, Smithers CJ, Hamilton TE, Labow BI, Baird CW, Taghinia AH, Feins N, Manfredi M, Jennings RW. Jejunal Interposition after Failed Esophageal Atresia Repair. J Am Coll Surg. 2016 Jun;222(6):1001-8. doi: 10.1016/j.jamcollsurg.2015.12.001. Epub 2015 Dec 13. PMID: 26774491.

Initial report of the technique and outcomes of the first 10 patients who underwent jejunal interposition in our center. 

International EA-QOL Group. Establishment of a condition-specific quality-of-life questionnaire for children born with esophageal atresia aged 2-7 across 14 countries. Front Pediatr. 2023 Oct 23;11:1253892. doi: 10.3389/fped.2023.1253892. PMID: 37936889; PMCID: PMC10626467.

Based on parent input, the collaboration between experts, patient stakeholders, and instrument developers, a linguistic version of the EA-QOL (quality of life) questionnaire for children ages 2–7 for use in and across 14 countries has been established. These efforts have set the conditions for a cross-cultural field test of the EA-QOL questionnaire and will open the doors for a new chapter in outcome research, registries, and clinical practice concerning children with EA. In the long term, this will help increase knowledge of the disease’s burden, promote patient-centeredness, exchange of information between nations, and strengthen evidence-based treatments for children born with EA.

Finck C, Zendejas B, Knod JL, Tramontozzi PJ, Jungbauer D, Anderson M,  Middlesworth W, Scholz S, Kunisaki SM, Perioperative management and outcomes in long-gap esophageal atresia: A retrospective analysis from the Eastern Pediatric Surgery Network. Journal of Pediatric Surgery Open. Vol 3. 2003, https://doi.org/10.1016/j.yjpso.2023.100029

Multicenter collaborative study across children’s hospitals in the Eastern Pediatric Surgery Network evaluating management and outcomes of children with LGEA. 

Mohammed S, Hamilton TE. Advances in Complex Congenital Tracheoesophageal Anomalies. Clin Perinatol. 2022 Dec;49(4):927-941. doi: 10.1016/j.clp.2022.07.003. PMID: 36328608.

Overview of complex congenital tracheo-esophageal anomalies

Izadi S, Smithers J, Shieh HF, Demehri FR, Mohammed S, Hamilton TE, Zendejas B, The History and Legacy of the Foker Process for the Treatment of Long Gap Esophageal atresia, Journal of Pediatric Surgery, https://doi.org/10.1016/j.jpedsurg.2023.12.020, Epub ahead of print. 19 December 2023

Historical overview of the history of the Foker process

Evanovich DM, Wang JT, Zendejas B, Jennings RW, Bajic D. From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution. Front Surg. 2022 Mar 9;9:799052. doi: 10.3389/fsurg.2022.799052. PMID: 35356503; PMCID: PMC8959439.

Overview of the different EA types, disease severity and survival rates at our center from 2009-2020

Harrington AW, Riebold J, Hernandez K, Staffa SJ, Meisner JW, Zurakowski D, Jennings R, Hamilton T, Zendejas B. Feeding and Growth Outcomes in Infants with Type C Esophageal Atresia Who Undergo Early Primary Repair. J Pediatr. 2022 Feb;241:77-82.e1. doi: 10.1016/j.jpeds.2021.10.012. Epub 2021 Oct 21. PMID: 34687688.

Infants with esophageal atresia who undergo early primary repair can achieve standard growth curves by 3 years of age. However, poor discharge weight for age z-score (WAZ) was predictive of poor WAZ score at 1 year. Efforts to identify at-risk patients and institute targeted inpatient and outpatient nutrition interventions are needed to improve their growth trajectory.

Harrington AW, Riebold J, Hernandez K, Staffa SJ, Svetanoff WJ, Zurakowski D, Hamilton T, Jennings R, Mehta NM, Zendejas B. Nutrition delivery and growth outcomes in infants with long-gap esophageal atresia who undergo the Foker process. J Pediatr Surg. 2021 Dec;56(12):2133-2139. doi: 10.1016/j.jpedsurg.2021.07.014. Epub 2021 Jul 24. PMID: 34366132.

Throughout infancy, most primary Foker process LGEA patients have weight for age that is below average. Using targeted nutritional intervention, those who present with malnutrition can still achieve adequate growth despite prolonged and complicated hospital courses.

Shieh HF, Jennings RW. Long-gap esophageal atresia. Semin Pediatr Surg. 2017 Apr;26(2):72-77. doi: 10.1053/j.sempedsurg.2017.02.009. Epub 2017 Feb 3. PMID: 28550874.

Bairdain S, Zurakowski D, Vargas SO, Stenquist N, McDonald M, Towne MC, Miller DT, Jennings RW, Kantor DB, Agrawal PB. Long-Gap Esophageal Atresia Is a Unique Entity within the Esophageal Atresia Defect Spectrum. Neonatology. 2017;111(2):140-144. doi: 10.1159/000449241. Epub 2016 Oct 19. PMID: 27756070; PMCID: PMC5290190.

LGEA represents a unique type of EA. Compared to non-LGEA, it is more likely to be an isolated defect and associated with trisomy 21. Further, it is less commonly seen with VACTERL anomalies. Our findings suggest the involvement of unique pathways that may be distinct from those causing non-LGEA.

Kelly DP, Bairdain S, Zurakowski D, Dodson B, Harney KM, Jennings RW, Trenor CC. Quality improvement program reduces venous thromboembolism in infants and children with long-gap esophageal atresia (LGEA). Pediatr Surg Int. 2016 Jul;32(7):691-6. doi: 10.1007/s00383-016-3902-5. Epub 2016 Jun 4. PMID: 27262479.

VTE risk-reduction program implementation (prophylactic anticoagulation) decreased symptomatic VTE incidence with associated decreases in ICU and hospital length of stay. Prophylactic anticoagulation can be utilized safely in a complicated pediatric surgical population.

Bairdain S, Dodson B, Zurakowski D, Waisel DB, Jennings RW, Boretsky KR. Paravertebral nerve block catheters using chloroprocaine in infants with prolonged mechanical ventilation for treatment of long-gap esophageal atresia. Paediatr Anaesth. 2015 Nov;25(11):1151-7. doi: 10.1111/pan.12736. Epub 2015 Aug 14. PMID: 26272104.

Short-term paravertebral nerve catheter placement decreases opioid and benzodiazepine exposure, weaning days and ICU stay in infants undergoing prolonged mechanical ventilation for LGEA repair.

Bairdain S, Dodson B, Zurakowski D, Rhein L, Snyder BD, Putman M, Jennings RW. High incidence of fracture events in patients with Long-Gap Esophageal Atresia (LGEA): A retrospective review prompting implementation of standardized protocol. Bone Rep. 2015 Jun 14;3:1-4. doi: 10.1016/j.bonr.2015.06.002. PMID: 28377960; PMCID: PMC5365208.

Episodes of muscle paralysis appeared to be the most significant risk factor for fractures in patients with LGEA who underwent the Foker procedure. The incidence of symptomatic fractures decreased significantly following implementation of a standardized protocol in this series of LGEA patients. The protocol includes limiting medications detrimental to bone health; repleting vitamin D both before and after paralysis; optimizing nutrients and minerals such as calcium and phosphorus in PN and in feeds; weekly labs during and after paralysis; multiple daily sessions with physical therapy (PT) for passive range of motion; educating family and care team about careful handling.

Dionigi B, Bairdain S, Smithers CJ, Jennings RW, Hamilton TE. Restoring esophageal continuity following a failed colonic interposition for long-gap esophageal atresia. J Surg Case Rep. 2015 Apr 23;2015(4):rjv048. doi: 10.1093/jscr/rjv048. PMID: 25907539; PMCID: PMC4407410.

Case report of salvage Foker process in a patient with a prior failed colonic interposition. 

Bairdain S, Hamilton TE, Smithers CJ, Manfredi M, Ngo P, Gallagher D, Zurakowski D, Foker JE, Jennings RW. Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery. J Pediatr Surg. 2015 Jun;50(6):933-7. doi: 10.1016/j.jpedsurg.2015.03.010. Epub 2015 Mar 25. PMID: 25841281.

Overview of the outcomes of 52 patients who underwent the Foker process. Study results show improved outcomes for those who underwent a primary Foker process compared to a rescue or secondary Foker process after prior attempts elsewhere. Early referral to a multidisciplinary esophageal center and a flexible approach to establish continuity in LGEA patients is recommended.

Bairdain S, Kelly DP, Tan C, Dodson B, Zurakowski D, Jennings RW, Trenor CC 3rd. High incidence of catheter-associated venous thromboembolic events in patients with long gap esophageal atresia treated with the Foker process. J Pediatr Surg. 2014 Feb;49(2):370-3. doi: 10.1016/j.jpedsurg.2013.09.003. Erratum in: J Pediatr Surg. 2015 Mar;50(3):504. PMID: 24528989.

First study that shed the light on the issue of increased risk of deep venous thrombosis (DVT) in children undergoing the Foker process. This led to the development of DVT prevention initiatives that have drastically reduced the risk of DVT. 

Liszewski MC, Bairdain S, Buonomo C, Jennings RW, Taylor GA. Imaging of long gap esophageal atresia and the Foker process: expected findings and complications. Pediatr Radiol. 2014 Apr;44(4):467-75. doi: 10.1007/s00247-013-2847-2. Epub 2013 Dec 24. PMID: 24366604.

Overview of the radiographic findings in patients undergoing treatment for long-gap esophageal atresia