Gastrointestinal manifestations in OA (oesophageal atresia) /TOF (tracheo - oesophageal fistula)
Dysphagia (90%) and GORD (80%) are near-universal in children and adults born with OA/TOF, though many don't realise they have these as they have been present since birth. (1,2).
Presentation
Infants and small children:
- frequent vomiting
- poor weight gain and failure to thrive
- reluctance to feed, feeding with back arching
- excess saliva and dribbling
- multiple dental caries, tooth enamel damage and dental wear.
- poor sleep and irritability
- wheezing and coughing at night (3)
Older children and adults
- heartburn
- worsening of existing reflux symptoms
- worsening swallowing difficulties/food sticking
- regurgitation
- choking when eating
- chest pain
- faintness, diarrhoea and nausea post eating
- respiratory and ENT symptoms
- weight loss (1)
Causes
- GORD- found in 75% (1)
- hiatus hernia- 29% have this following their surgical repair. (4)
- dysmotility (2)
- oesophageal stricture (5)
- achalasia-like symptoms or high pressure lower oesophageal sphincter
- eosinophilic oesophagitis- 12% TOF/OA adolescents (6)
- Barrett’' oesophagus – prevalence rates range from 6-15% (7-9)
- Dumping syndrome- up to 12% long gap OA (1)
- oesophageal cancer (squamous or adenocarcinoma)(10-13)
- gastric neuroendocrine tumour due to long term PPI use
Contributor:
- Dr Caroline Love (August 2022)
- Associate Specialist in Dermatology at York Hospitals Trust; Adult born with OA/TOF and representative of TOFS charity
Reference:
- Hannon E, Eaton S, Curry JI, Kiely EM, Spitz L, De Coppi P. Outcomes in adulthood of gastric transposition for complex and long gap esophageal atresia. J Pediatr Surg. 2020 Apr;55(4):63945
- Deurloo JA, Klinkenberg EC, Ekkelkamp S, Heij HA, Aronson DC. Adults with corrected oesophageal atresia: is oesophageal function associated with complaints and/or quality of life? Pediatr Surg Int. 2008 May 1;24(5):537-41.
- Sidebotham, Emma. Gastro-oesophageal reflux. In: The TOF Book by David Crabbe and Vicki Martin
- Rintala RJ, Sistonen S, Pakarinen MP. Outcome of Oesophageal Atresia Beyond Childhood. Journal of Pediatric Gastroenterology and Nutrition. 2011 May;52:S35.
- Koivusalo AI, Pakarinen MP, Lindahl HG, Rintala RJ. Endoscopic Surveillance After Repair of Oesophageal Atresia: Longitudinal Study in 209 Patients. J Pediatr Gastroenterol Nutr. 2016 Apr;62(4):562-6.
- Lardenois E, Michaud L, Schneider A, Onea M, Rebeuh J, Gottrand-Aumar M, et al. Prevalence of Eosinophilic Esophagitis in Adolescents With Esophageal Atresia. Journal of Pediatric Gastroenterology and Nutrition. 2019 Jul 1;Publish Ahead of Print:1.
- Cartabuke RH, Lopez R, Thota PN. Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula. Gastroenterol Rep (Oxf). 2016 Nov;4(4):310-4.
- Connor MJ, Springford LR, Kapetanakis VV, Giuliani S. Esophageal atresia and transitional care - step 1: a systematic review and meta-analysis of the literature to define the prevalence of chronic long-term problems. The American Journal of Surgery. 2015 Apr 1;209(4):747-59.
- Schneider A, Michaud L, Gottrand F. Esophageal atresia: metaplasia, Barrett. Diseases of the Esophagus. 2013 May 1;26(4):425-7.
- Deurloo JA, van Lanschot JJB, Drillenburg P, Aronson DC. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. Journal of Pediatric Surgery. 2001 Apr 1;36(4):629-30.
- Jayasekera CS, Desmond PV, Holmes JA, Kitson M, Taylor ACF. Cluster of 4 cases of esophageal squamous cell cancer developing in adults with surgically corrected esophageal atresia - time for screening to start. Journal of Pediatric Surgery. 2012 Apr 1;47(4):646-51.
- Vergouwe FWT, IJsselstijn H, Biermann K, Erler NS, Wijnen RMH, Bruno MJ, et al. High Prevalence of Barrett's Esophagus and Esophageal Squamous Cell Carcinoma After Repair of Esophageal Atresia. Clin Gastroenterol Hepatol. 2018 Apr;16(4):513-521.e6.
- Adzick NS, Fisher JH, Winter HS, Sandler RH, Hendren WH. Esophageal adenocarcinoma 20 years after esophageal atresia repair. J Pediatr Surg. 1989 Aug 1;24(8):741–4.