|Year : 2016 | Volume
| Issue : 3 | Page : 106-109
Clinical profile and management options of children with congenital esophageal stenosis: A single center experience
Jujju Jacob Kurian, Susan Jehangir, Isaac Tharu Varghese, Reju Joseph Thomas, John Mathai, Sampath Karl
Department of Paediatric Surgery, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Web Publication||18-May-2016|
Jujju Jacob Kurian
Department of Paediatric Surgery, Christian Medical College, Vellore, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Aim: The aim of the study is to review 7 patients with congenital esophageal stenosis treated in our institution from a diagnostic and therapeutic point of view. Materials and Methods: This is a retrospective cohort study of 7 patients treated in Christian Medical College, Vellore from 2008 to 2014. The data were analyzed with regards to age at onset of symptoms, investigative findings, age at definitive treatment, pathology, modalities of treatment, and outcomes. Results: Symptoms started within the 1 st year of life in all children with a median age of 4 months. The time of delay in diagnosis ranged from 8 months to 81 months with a mean period of 37 months. About 6 patients had a lower esophageal stenosis and 1 patient had a mid-esophageal stenosis. About 4 of the 7 children underwent endoscopic balloon dilatation from elsewhere, with 2 of the above 4 undergoing a myotomy for a wrongly diagnosed achalasia. The number of dilatations ranged from 2 to 7 with a mean of 4 dilatations. Resection of the stenotic segment with end to end anastomosis was employed in 6 of the 7 patients, and a transverse colon interpositioning was done in 1 patient. An antireflux procedure was performed in one patient. Histopathological examination of the resected specimen revealed tracheobronchial remnant in 3 patients, fibromuscular thickening in 3 patients, and membranous web in 1 patient. Postoperatively, 2 of the 7 patients had asymptomatic gastroesophageal reflux and 1 patient had postoperative stricture requiring one session of endoscopic balloon dilatation. The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or gastroesophageal reflux. There was a statistically significant increase in the weight for age after the operation. Conclusion: Congenital esophageal stenosis is rare and often confused with other causes of esophageal obstruction. Although endoscopic balloon dilatation offers an effective temporary relief, we feel that definitive surgery is curative. Long-term results following definitive surgery have been good, especially with respect to symptoms and weight gain.
Keywords: Congenital esophageal stenosis, fibromuscular stenosis, membranous diaphragm, tracheobronchial remnant
|How to cite this article:|
Kurian JJ, Jehangir S, Varghese IT, Thomas RJ, Mathai J, Karl S. Clinical profile and management options of children with congenital esophageal stenosis: A single center experience. J Indian Assoc Pediatr Surg 2016;21:106-9
|How to cite this URL:|
Kurian JJ, Jehangir S, Varghese IT, Thomas RJ, Mathai J, Karl S. Clinical profile and management options of children with congenital esophageal stenosis: A single center experience. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2019 Dec 9];21:106-9. Available from: http://www.jiaps.com/text.asp?2016/21/3/106/182581
| Introduction|| |
Congenital esophageal stenosis (CES) is a rare anomaly characterized by an intrinsic esophageal narrowing due to an abnormality of the esophageal wall. The incidence is 1 in 25,000-50,000 live births,  with the highest incidence in Japan. , Although there is no gender predilection, approximately one-third of cases are associated with esophageal and intestinal atresias, anorectal malformations, cardiac anomalies, and chromosomal anomalies. , Symptoms usually start with initiation of solid feeds and common presentations include vomiting, dysphagia, failure to thrive, and recurrent lower respiratory infections.
Nihoul-Fékété et al. in 1987 classified CES into three forms, namely (a) tracheobronchial remnants (TBRs), (b) fibromuscular stenosis, and (c) membranous diaphragm (MD) in the wall of the esophagus based on the histopathological analysis.  TBRs are thought to result from the failure of the separation of the respiratory system from the foregut, whereas MDs occur as a result of incomplete recanalization.  Herein, we review our experience with various types of CES over a 6-year period.
| Materials and methods|| |
This is a retrospective cohort study of 7 patients with CES treated in Christian Medical College, Vellore between 2008 and 2014. Children were identified by an electronic search of Pediatric Surgery database and operation records. The hospital outpatient records, operation notes, radiological investigations, pathology reports, and discharge summaries were reviewed. The preoperative and postoperative weight along with findings of barium upper gastrointestinal series (Ba UGI) and UGI endoscopy were recorded. Based on hospital data, the family was contacted by phone or followed up in the outpatient department. The following follow-up questions were asked to the respondent: Presence or absence of (a) recurrent vomiting, (b) dysphagia to solids/liquids/both, (c) pain in the chest, and (d) recurrent lower respiratory tract infections. The weight at the last follow-up was noted and charted on a modified Agarwal's weight for age chart and compared with the preoperative weight. 
The preoperative and follow-up weight for age was analyzed using GraphPad Quickcalcs (CA, USA: http://www.graphpad.com/quickcalcs/). A two-tailed P value was calculated using the Student's t-test and Chi-square test with or without Yates correction. A P < 0.05 was considered as statistically significant.
| Results|| |
Of the 7 children, 3 were boys and 4 were girls. All children were symptomatic within the 1 st year of life (median age of 4 months). All children had vomiting at presentation. In addition, 3 (43%) children had dysphagia, 3 (43%) had a failure to thrive, and 1 (14%) had recurrent lower respiratory tract infections. None of the children had any known associated anomalies. One child had a poorly functioning kidney with systemic hypertension as a result of reflux nephropathy.
A Ba UGI showed a stenosis in the distal third of the esophagus in 6 (86%) children and in the middle third in 1 child (14%) [Figure 1]. UGI endoscopy confirmed the site with the mucosa at the site of stenosis being normal. The median time from onset of symptoms to diagnosis was 25 months (range 8-81 months).
|Figure 1: Preoperative and postoperative barium esophagogram of a mid-esophageal congenital esophageal stenosis and after successful correction|
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Initial endoscopic balloon dilatations were done from elsewhere in 4 of the 7 children before referral to our center for persistent symptoms. The number of dilatations done ranged from 2 to 7 (mean of 4 dilatations) at 3-6 months interval. Two of these children also underwent a myotomy as they were wrongly diagnosed as achalasia cardia before referral to our center.
Definitive surgery was undertaken in all. The approach was via a laparotomy in 2 children for whom the site of stenosis was close to the gastroesophageal (GE) junction (2-4 cm), and a left thoracotomy in 4. A combined left thoracotomy and laparotomy was done in one child who required a transverse colon interpositioning as the gap was too long to perform a primary anastomosis. The stenotic segment was resected, and primary anastomosis was possible in 6 children. In addition, an antireflux procedure was performed in one patient who had the stenotic segment very close to the GE junction. Histopathological examination of the resected specimen revealed the cause of stenosis to be a TBR in 3 patients, fibromuscular thickening in 3 patients, and MD in 1 patient. This data have been summarized in [Table 1].
The child who required transverse colon interpositioning developed an adhesive intestinal obstruction in the immediate postoperative period and required a relaparotomy. A 3-month postoperative barium esophagogram was done in all patients. About 2 of the 7 patients had asymptomatic gastroesophageal reflux (GER). One patient had a postoperative stricture. He underwent a single session of endoscopic balloon dilatation and is now asymptomatic at 32 months follow-up.
The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or GER. There was a statistically significant increase in the weight for age after the operation. The data have been summarized in [Table 2].
|Table 2: The pre- and post-operative weight for age and follow-up barium upper gastrointestinal series findings|
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| Discussion|| |
The differential diagnoses of childhood esophageal obstruction are post-GER stricture, achalasia cardia, and CES, with a clinical differentiation being difficult due to the similarity in presentation. Preoperative Ba UGI series along with UGI endoscopy may be useful in differentiating CES from other causes of congenital esophageal obstruction. CES on Ba UGI series may show an abrupt or tapered stenosis anywhere in the esophagus, in contrast to achalasia and a UGI endoscopy will reveal the site of stenosis in the esophagus with a smooth mucosal lining, in contrast to Barrett's stricture. In achalasia cardia, though the Ba UGI series shows narrowing at the GE junction, the UGI endoscopy passes easily into the stomach without revealing any stenotic segment. In post-GER stricture, the site of stenosis is always in the lower third of the esophagus with the UGI endoscopy showing a stenotic area with mucosal abnormality.
However, the definitive diagnosis of CES is by histopathological examination of the resected esophageal segment. The three types as differentiated histopathologically have clinical relevance. Several studies have suggested that while TBRs require surgery, Fibro muscular thickening (FMT), and Membranous web (MWs) may be treated by endoscopic balloon dilatations. ,,,, It has been suggested that a Ba UGI series might help in preoperative differentiation where an abrupt stenosis occurs in TBR type, whereas a tapered stenosis correlates with the FM type. Recent studies have however disproved these findings.  In our study, we found no correlation of abrupt/tapered stenosis to the pathological type of CES. Recent reports have shown that endoscopic ultrasound can demonstrate hypoechoic TBRs at the site of stenosis. This along with magnetic resonance imaging holds promise in the preoperative differentiation of TBR and non-TBR types of CES. ,,
Surgical approach is determined by the site of stenosis. In case of stenosis involving the lower third of the esophagus, the approach depends on how close is the stenosis to the GE junction. A laparotomy will suffice if the stenosis is within 3-4 cm of the GE junction. In lesions of the thoracic esophagus, the approach is usually via a left or right thoracotomy. In lesions of the cervical esophagus, the approach is usually via the neck.
Although segmental resection of the stenotic segment is the gold standard, circular myotomy and endoscopic balloon dilatation have been used with varying degrees of success. In our series, 4 of the 7 children underwent endoscopic dilatations from elsewhere with 2 of the above-mentioned 4 undergoing a myotomy for a mistakenly diagnosed achalasia. Although these procedures resulted in an initial improvement of symptoms, all children eventually required definitive corrective surgery. Anecdotally, 3 of the 4 children who underwent dilatations were of the TBR type and therefore less likely to respond to dilatations. ,,,,
Determining the site of stenosis is another important step in surgery. As described by Amae et al., after isolating the esophagus, we insert a Foleys catheter through the mouth into the stomach. The bulb is then inflated and the pulled back into the esophagus to note the distal margin of stenosis. The site of stenosis will be revealed by the inability of the inflated catheter to pass through. After opening the distal end, the catheter is inserted from below up, and the bulb inflated. Pulling down on the catheter will reveal the proximal margin of stenosis. 
Studies have shown that the various procedures done for CES are not without complications. While esophageal dilatation can cause esophageal perforation, aspiration pneumonia, and cardiac arrest, ,, surgery may be complicated by leak and restenosis. Gastroesophageal reflux disease (GERD) due to an alteration in the esophago-gastric angle can occur following surgery. Asymptomatic GERD detected incidentally while doing a routine postoperative barium esophagogram, requires no treatment as seen in our series. However, symptomatic GERD will need further medical and possible surgical management. Postoperative strictures at the site of esophageal anastomosis are usually amenable to endoscopic balloon dilatation as seen in our study.
| Conclusion|| |
CES is a rare cause of esophageal obstruction in infants and needs to be distinguished from GER and achalasia cardia. A Ba UGI series and UGI endoscopy may be a useful adjunct in the preoperative differentiation of the above. Although endoscopic balloon dilatation can provide temporary relief, surgical excision of the stenotic segment offers a permanent cure and is the preferred method of managing CES in our institution. Long-term results following surgery are good, especially with respect to symptoms and weight gain.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2]