Four cancer cases after esophageal atresia repair: Time to start screening the upper gastrointestinal tract

Floor Wt Vergouwe, Madeleine Gottrand, Bas Pl Wijnhoven, Hanneke IJsselstijn, Guillaume Piessen, Marco J Bruno, René Mh Wijnen, Manon Cw Spaander, Floor Wt Vergouwe, Madeleine Gottrand, Bas Pl Wijnhoven, Hanneke IJsselstijn, Guillaume Piessen, Marco J Bruno, René Mh Wijnen, Manon Cw Spaander

Abstract

Esophageal atresia (EA) is one of the most common congenital digestive malformations and requires surgical correction early in life. Dedicated centers have reported survival rates up to 95%. The most frequent comorbidities after EA repair are dysphagia (72%) and gastroesophageal reflux (GER) (67%). Chronic GER after EA repair might lead to mucosal damage, esophageal stricturing, Barrett's esophagus and eventually esophageal adenocarcinoma. Several long-term follow-up studies found an increased risk of Barrett's esophagus and esophageal carcinoma in EA patients, both at a relatively young age. Given these findings, the recent ESPGHAN-NASPGHAN guideline recommends routine endoscopy in adults born with EA. We report a series of four EA patients who developed a carcinoma of the gastrointestinal tract: three esophageal carcinoma and one colorectal carcinoma in a colonic interposition. These cases emphasize the importance of lifelong screening of the upper gastrointestinal tract in EA patients.

Keywords: Adenocarcinoma; Barrett’s esophagus; Esophageal atresia; Esophageal cancer; Screening; Squamous cell carcinoma.

Conflict of interest statement

Conflict-of-interest statement: All authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Chest computed tomography scan (CT scan) (case 1, tumor 2) demonstrating a tumor mass in the cervical native esophagus with suspected tumor invasion in the left thyroid gland.
Figure 2
Figure 2
Findings at upper endoscopy and chest computed tomography scan (CT scan) (case 2). A: Upper endoscopy revealing a stenotic ulcerative tumor in the proximal esophagus, 22-29 cm from incisors. Histological examination of esophageal biopsies confirmed the diagnosis esophageal squamous cell carcinoma. B: Chest CT scan showing a tumor mass in the proximal esophagus with suspected tumor invasion in the trachea.
Figure 3
Figure 3
Initial findings at positron emission tomography-computed tomography scan (PET-CT scan) (case 3), showing PET-positive lesion in the distal esophagus without metastasis.
Figure 4
Figure 4
Initial findings at positron emission tomography-computed tomography scan (PET-CT scan) (case 4). A: Chest CT scan image with a circumferential wall thickening of the thoracic colonic interposition over a length of 10 cm, not clearly separated from the thyroid and left brachiocephalic vein. Locoregional suspected lymph nodes (

References

    1. Pedersen RN, Calzolari E, Husby S, Garne E; EUROCAT Working group. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child. 2012;97:227–232.
    1. Wang B, Tashiro J, Allan BJ, Sola JE, Parikh PP, Hogan AR, Neville HL, Perez EA. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res. 2014;190:604–612.
    1. Sulkowski JP, Cooper JN, Lopez JJ, Jadcherla Y, Cuenot A, Mattei P, Deans KJ, Minneci PC. Morbidity and mortality in patients with esophageal atresia. Surgery. 2014;156:483–491.
    1. Rintala RJ, Pakarinen MP. Long-term outcome of esophageal anastomosis. Eur J Pediatr Surg. 2013;23:219–225.
    1. Vergouwe FW, IJsselstijn H, Wijnen RM, Bruno MJ, Spaander MC. Screening and Surveillance in Esophageal Atresia Patients: Current Knowledge and Future Perspectives. Eur J Pediatr Surg. 2015;25:345–352.
    1. Huynh Trudeau V, Maynard S, Terzic T, Soucy G, Bouin M. Dysphagia among adult patients who underwent surgery for esophageal atresia at birth. Can J Gastroenterol Hepatol. 2015;29:91–94.
    1. Taylor AC, Breen KJ, Auldist A, Catto-Smith A, Clarnette T, Crameri J, Taylor R, Nagarajah S, Brady J, Stokes K. Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long-term follow-up study. Clin Gastroenterol Hepatol. 2007;5:702–706.
    1. Sistonen SJ, Koivusalo A, Nieminen U, Lindahl H, Lohi J, Kero M, Kärkkäinen PA, Färkkilä MA, Sarna S, Rintala RJ, et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann Surg. 2010;251:1167–1173.
    1. Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, Pakarinen MP. Cancer after repair of esophageal atresia: population-based long-term follow-up. J Pediatr Surg. 2008;43:602–605.
    1. Jayasekera CS, Desmond PV, Holmes JA, Kitson M, Taylor AC. Cluster of 4 cases of esophageal squamous cell cancer developing in adults with surgically corrected esophageal atresia--time for screening to start. J Pediatr Surg. 2012;47:646–651.
    1. Krishnan U, Mousa H, Dall’Oglio L, Homaira N, Rosen R, Faure C, Gottrand F. ESPGHAN-NASPGHAN Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Esophageal Atresia-Tracheoesophageal Fistula. J Pediatr Gastroenterol Nutr. 2016;63:550–570.
    1. Deurloo JA, van Lanschot JJ, Drillenburg P, Aronson DC. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg. 2001;36:629–630.
    1. Adzick NS, Fisher JH, Winter HS, Sandler RH, Hendren WH. Esophageal adenocarcinoma 20 years after esophageal atresia repair. J Pediatr Surg. 1989;24:741–744.
    1. Alfaro L, Bermas H, Fenoglio M, Parker R, Janik JS. Are patients who have had a tracheoesophageal fistula repair during infancy at risk for esophageal adenocarcinoma during adulthood? J Pediatr Surg. 2005;40:719–720.
    1. Pultrum BB, Bijleveld CM, de Langen ZJ, Plukker JT. Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia. J Pediatr Surg. 2005;40:e1–e4.
    1. Solomon BD, Baker LA, Bear KA, Cunningham BK, Giampietro PF, Hadigan C, Hadley DW, Harrison S, Levitt MA, Niforatos N, et al. An approach to the identification of anomalies and etiologies in neonates with identified or suspected VACTERL (vertebral defects, anal atresia, tracheo-esophageal fistula with esophageal atresia, cardiac anomalies, renal anomalies, and limb anomalies) association. J Pediatr. 2014;164:451–457.e1.
    1. Torre LA, Bray F, Siegel RL, Ferlay J, Lortet-Tieulent J, Jemal A. Global cancer statistics, 2012. CA Cancer J Clin. 2015;65:87–108.
    1. Kamangar F, Chow WH, Abnet CC, Dawsey SM. Environmental causes of esophageal cancer. Gastroenterol Clin North Am. 2009;38:27–57, vii.
    1. Zhang SK, Guo LW, Chen Q, Zhang M, Liu SZ, Quan PL, Lu JB, Sun XB. The association between human papillomavirus 16 and esophageal cancer in Chinese population: a meta-analysis. BMC Cancer. 2015;15:1096.
    1. Sandler RS, Nyrén O, Ekbom A, Eisen GM, Yuen J, Josefsson S. The risk of esophageal cancer in patients with achalasia. A population-based study. JAMA. 1995;274:1359–1362.
    1. Pajecki D, Zilberstein B, Cecconello I, Dos Santos MA, Yagi OK, Gama-Rodrigues JJ. Larger amounts of nitrite and nitrate-reducing bacteria in megaesophagus of Chagas’ disease than in controls. J Gastrointest Surg. 2007;11:199–203.
    1. Chapuy L, Pomerleau M, Faure C. Topical mitomycin-C application in recurrent esophageal strictures after surgical repair of esophageal atresia. J Pediatr Gastroenterol Nutr. 2014;59:608–611.

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