Fig. 8.1
Congenital stenosis of the distal third of the esophagus
The radiologic appearance is morphologically similar in all cases of esophageal stenosis: an important esophageal dilation followed by a threadlike narrowing. The site of the stricture can be useful to reach a first differential diagnosis. It has been seen how the three different forms of congenital cause have different favorite localizations.
The ectopic cartilaginous tissue is found in more than 90 % of cases in the lower third of the esophagus and especially near the cardia; fibromuscular stenosis is more frequently found between the middle and the lower third with a predilection for the proximal part of the latter; in achalasia instead, by definition, the stenotic portion is always located in the cardia.
A first rough differentiation between the three types of CES can be made by examining the patient. Symptoms caused by tracheobronchial remnants and fibromuscular stenosis are typical in early infancy, while those caused by achalasia usually occur later in school age; also peptic stricture typically affects older children. When radiographic examination is not able to identify the diagnosis, an endoscopic study can highlight the main characteristics of the lesion. The direct vision of the section affected by a peptic stricture will show an inflamed area also above the stenosis; the endoscopic images, associated with medical history and evaluation of symptoms, lead to the correct diagnosis. Regarding other types of stenosis, correct diagnosis can be made in the course of endoscopic examination, based on the ease or otherwise to overcome the restricted portion by the endoscope.
In case of achalasia, the cardia can be passed by the endoscope, forcing it kindly, and a mild dilation can be obtained, albeit only temporarily. In the presence of fibromuscular stenosis, the endoscope cannot exceed the stenotic tract that can be dilated with special probes. In ectopic cartilaginous ring, the direct vision does not allow the differential diagnosis, but the attempt to dilate the narrow tract is usually unsuccessful, and this causes the operator to desist. In case of cardia localization of fibromuscular stenosis, differential diagnosis with achalasia becomes more difficult and in some cases impossible even after endoscopy. Even if most of the patients’ endoscopic examination can lead to diagnosis, further informations can be obtained with a manometric examination. Also the outcome of dilations can be used as a diagnostic criterion: in case of achalasia, improvement will only be transitional, while it will be present and often progressive in case of congenital stenosis sensitive to expansion.
Endoscopic ultrasonography (EUS) is a useful tool in differential diagnosis between the three types of congenital stenosis. In particular, EUS is able to evidence the presence of cartilage which is difficult to be seen with CT scan or MRI. EUS is available for pediatric patients from 2001, and recently, a 3D miniprobe has been used to obtain multiplanar and oblique views of the stenosis [7–9].
8.5.1 Therapy
The first-line treatment of CES is dilation. It can be performed with hydrostatic balloon probe or with Savary-Gilliard bougie. There is no strong evidence on which is the better technique, and the choice depends on the expertise of each center. Jones et al. reported advantages of balloon dilation because of radial and localized force applied by the baloon, rather than axial shearing force applied on stenosis by the bougie [10]. On the other hand, Romeo et al. reported a higher risk of perforation with balloon dilation affirming that bouginage seems safer [9]. An endoscopic check after dilation is mandatory, and in case of deep mucosal laceration, an esophagogram is recommended.
Whatever the technique chosen, the dilations are usually performed every 15 days until reaching a stable esophageal caliber. The subsequent dilations are established on the basis of clinics, age, and results obtained on each patient.
Some authors reported success in endoscopic electrocauterization or partial resection of mucosal web added to dilations [11, 12].
Fibromuscular stenosis and mucosal web usually respond successfully to dilations, while in case of cartilaginous remnant or dilation failure, it is necessary to undergo surgery (Fig. 8.2).
Fig. 8.2
Barium esophagogram after six dilations. The patient continued to complain of severe dysphagia
In these cases, the treatment of choice is the resection of the stenotic tract followed by end-to-end anastomosis (Fig. 8.3) and eventual fundoplication if the stenosis is closed to gastroesophageal junction [2, 13]. The stricture can be identified externally by palpation or with the use of a catheter placed preoperatively. Maeda and Saito reported two patients affected by tracheobronchial remnant treated with circular extramucosal myectomy at the stenotic level followed by suture of the esophageal wall. This technique allows extirpation of the cartilage and muscular disarrangement avoiding lumen opening [14, 15].
