Congenital esophageal stenosis (CES) is a rare entity with an estimated incidence of 1:25,000–50,000 live births. Three causes for CES have been described: tracheobronchial remnant, segmental fibromuscular hypertrophy, and membranous web. CES can be associated with other anomalies such as esophageal atresia, chromosomal anomalies, cardiac anomalies, intestinal atresia, anorectal malformation, and trisomy 21.
Main symptoms are dysphagia, vomiting during meals, regurgitation of undigested food, insufficient growth, and relapsing pneumonia due to aspiration and typically occur when solid foods are introduced in diet.
Diagnosis of esophageal stenosis is achieved with barium esophagogram and endoscopic study, but differential diagnosis among the three forms of CES can be difficult. Endoscopic ultrasonography may help in identifying correct diagnosis of cartilaginous remnant.
First-line treatment consists of esophageal dilations which lead to resolution of stenosis in most of the cases. Tracheobronchial remnants typically are unresponsive to dilations and require resection of the stenotic tract followed by end-to-end anastomosis. The most serious complication is the esophageal perforation after dilation.
Despite various therapeutic options, a consistent part of patients continues to complain of dysphagia and requires a follow-up for a long time.
Appignani A (1993) Stenosi esofagee congenite. In: Dòmini R, Lima M (eds) Chirurgia delle malformazioni digestive. Piccin, BolognaGoogle Scholar
van Poll D, van der Zee DC (2012) Thoracoscopic treatment of congenital esophageal stenosis in combination with H-type tracheoesophageal fistula. J Pediatr Surg 47(8):1611–1613CrossRefPubMedGoogle Scholar
Nihoul-Fèkètè C, De Backer A, Lortat-Jacob S et al (1987) Congenital esophageal stenosis: a review of 20 cases. Pediatr Surg Int 2:86–92CrossRefGoogle Scholar
Vasudevan SA, Kerendi F, Lee H, Ricketts RR (2002) Management of congenital esophageal stenosis. J Pediatr Surg 37(7):1024–1026CrossRefPubMedGoogle Scholar
Michaud L, Coutenier F, Podevin G et al (2013) Characteristics and management of congenital esophageal stenosis: findings from a multicenter study. Orphanet J Rare Dis 8:186CrossRefPubMedPubMedCentralGoogle Scholar
Bocus P, Realdon S, Eloubeidi MA, Diamantis G, Betalli P, Gamba P, Zanon GF, Battaglia G (2011) High-frequency miniprobes and 3-dimensional EUS for preoperative evaluation of the etiology of congenital esophageal stenosis in children (with video). Gastrointest Endosc 74(1):204–207CrossRefPubMedGoogle Scholar
Romeo E, Foschia F, de Angelis P et al (2011) Endoscopic management of congenital esophageal stenosis. J Pediatr Surg 46(5):838–841CrossRefPubMedGoogle Scholar
Jones DW, Kunisaki SM, Teitelbaum DH, Spigland NA, Coran AG (2010) Congenital esophageal stenosis: the differential diagnosis and management. Pediatr Surg Int 26(5):547–551CrossRefPubMedGoogle Scholar
Chao HC, Chen SY, Kong MS (2008) Successful treatment of congenital esophageal web by endoscopic electrocauterization and balloon dilatation. J Pediatr Surg 43(1):e13–e15CrossRefPubMedGoogle Scholar
Takamizawa S, Tsugawa C, Mouri N, Satoh S, Kanegawa K, Nishijima E, Muraji T (2002) Congenital esophageal stenosis: therapeutic strategy based on etiology. J Pediatr Surg 37(2):197–201CrossRefPubMedGoogle Scholar
Amae S, Nio M, Kamiyama T, Ishii T, Yoshida S, Hayashi Y, Ohi R (2003) Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases. J Pediatr Surg 38(4):565–570CrossRefPubMedGoogle Scholar
Maeda K, Hisamatsu C, Hasegawa T, Tanaka H, Okita Y (2004) Circular myectomy for the treatment of congenital esophageal stenosis owing to tracheobronchial remnant. J Pediatr Surg 39(12):1765–1768CrossRefPubMedGoogle Scholar
Saito T, Ise K, Kawahara Y, Yamashita M, Shimizu H, Suzuki H, Gotoh M (2008) Congenital esophageal stenosis because of tracheobronchial remnant and treated by circular myectomy: a case report. J Pediatr Surg 43(3):583–585CrossRefPubMedGoogle Scholar