congenital esophageal stenosis ( ces ) is a rare condition in which a malformation of esophageal wall architecture results in stenosis of the esophagus . most cases reported in the literature are small segmental strictures which are managed with segmental resection and anastomosis of the esophagus . the index case had a long segment stricture in which segmental resection was precluded and was managed by an innovative gastroesophagoplasty . a 5-year - old boy presented with the complaints of difficulty in swallowing since infancy which was gradually increasing in severity . at the time of presentation barium swallow was performed which revealed long segment narrowing in the lower part of the esophagus [ figure 1 ] . esophagoscopy showed a narrowing in the lower esophagus approximately 20 cm from the incisors which could not be negotiated by the scope . however , the mucosa just proximal to the narrowing looked normal . a radionuclide gastroesophageal reflux scan revealed mild to moderate gastroesophageal reflux . a provisional diagnosis of ces was made . the full thickness of the strictured esophagus was laid open , and a fundus of stomach patch akin to that used in fundoplication post - hellers myotomy was sutured over the esophageal defect . the postoperative dye study showed no esophageal leak , but there was a persistent narrowing in the midesophagus [ figure 2a ] . symptomatically , the child initially improved with acceptance of semisolids but after 2 - 3 weeks was able to swallow only liquids . exploratory laparotomy was performed to perform further transhiatal mobilization of the strictured esophagus followed stricturoplasty . the procedure had to be abandoned due to vascular adhesions of the bowel near the hiatus with a future plan to explore again after 3 months through a thoracotomy . after 3 months , a thoracic approach was planned with an option to conversion to thoracoabdominal access . the patient was placed in supine position and incision was given across the left 7 intercostal space till the midline . the part of esophagus proximal to the previous site of myotomy was narrowed and had gritty feeling on palpation suggestive of the presence of cartilage . the uppermost part of the stomach which was used as a patch over the previous esophagostomy was opened transversely and sutured to the newly created defect in the esophagus . the postoperative contrast study revealed a patent normal looking esophagus till the midthoracic region [ figure 2b ] . distal to the midthoracic region the contrast was going down both through the native esophagus and through the gastroesophagoplasty . the child was asymptomatic in the postoperative period , and once he had an adequate oral intake of solids , feeding jejunostomy was removed . the child presented again after 3 months with difficulty in accepting solids for which he underwent esophageal dilatation . barium swallow ap view ( a ) and lateral view ( b ) showing a stenosis in the lower esophagus at the level of 8 thoracic vertebrae ( white arrowhead ) . the part distal to the stenosed part is also not significantly distended ( a ) barium swallow following transhiatal mobilization , stricturotomy and fundal patch shows a persistent narrowing in the esophagus at t8 level ( white arrowhead ) . the distal esophagus is normally distended ( white arrow ) and the fundal patch is seen in the left hemithorax ( black arrowhead ) . ( b ) barium swallow following complete stricturoplasty and esophagogastric anastomosis with no evident stricture . the contrast is passive through the native esophagus ( white arrow ) as well as esophagogastric anastomosis ( white arrow ) ces is most commonly classified based upon classification suggested by nihoul - fkt et al . they classified ces into 3 types : ( 1 ) tracheobronchial remnants ( tbr ) ( 2 ) fibromuscular stenosis ( fms ) : segmental hypertrophy of the muscularis and diffuse fibrosis of the submucosa ( 3 ) membranous diaphragm ( md ) . of these , the tbr is the most common whereas md is the least common . overall ces is more common in the lower third of the esophagus and the treatment for the most common type , i.e. tbr is essentially surgical which may involve segment resection and anastomosis of the esophagus , circular myectomy or enulceation of the cartilage . preoperative diagnosis is often confusing , especially in olden children , where acquired causes such as strictures secondary to reflux may also be present . on esophagogram , tbr will often show an abrupt stenosis whereas fms may show a gradual tapering . esophagoscopy reveals a normal mucosa overlying the stenosed segment which rules out strictures secondary to corrosive or reflux . lately , endoscopic ultrasonography is being used to reliably differentially between tbs and other types which helps in dictating management . the presence of symptoms since infancy , normal mucosa on esophagoscopy , findings in esophagogram and intraoperative findings supported the diagnosis of a ces in the index case . another aspect that is unique in this case is the long segment of the esophagus was involved rather than 1 - 2 cm that is usually described . the gold standard for diagnosis the tbr type is a histopathological examination of the resected esophageal wall which shows cartilage rests . since the latter was not performed in our case , the diagnosis can be debated on . however what is more important and clinically relevant is the management of these rare and difficult cases where the standard procedures such as resection and anastomosis of esophagus , enucleation , and myectomy are not possible . in management of severe varieties of ces , procedures like cardioplasty irrespective of the etiology of the esophageal stricture , which may be acquired or congenital , the aim of the treatment is to allow the patients to eat normally and be asymptomatic . anderson et al . suggested that stricturotomy can be considered in difficult cases before condemning the native esophagus in favor of replacement . the first procedure which we performed wherein the stricture was laid open and a fundal patch was applied is a described procedure for management of peptic esophageal stricture . the third surgery that we performed was unpremeditated and was performed as the fundal patch could be easily brought to the laid open esophagus . ces is a rare entity but should be suspected in children with dysphagia who are symptomatic since infancy . tbr type is the most common and needs surgical intervention . in severe or long strictures , in such cases , the surgeon should have an open mind and use whatever is available to him while maintaining basic surgical principles as was done in this case . the surgical procedures have to be tailored based on the individual cases for an optimum result .
congenital esophageal stenosis ( ces ) is a rare cause of dysphagia in children . 
 diagnosis is often apparent on esophagogram . 
 surgical treatment for the subtype with tracheobronchial remnants ( tbr ) includes resection and anastomosis of the stenosed segment , myectomy , enucleation of cartilage , etc . , these procedures are not suitable if the stenosed segment is long . 
 we present a case of a 5-year - old boy who was diagnosed as ces and was successfully managed with stricturoplasty with some innovative modifications .