we came across a neonate who presented with repeated chest infections and respiratory distress . on evaluation by ct
a full term female baby was born after an uneventful pregnancy . at 1 month of age ,
a plain radiograph of the chest showed increased opacification of the right lung with reduced lung volume , air bronchograms , and mild compensatory hyperinflation of the left lung [ figure 1 ] . following this , a computed tomography ( ct ) scan
was performed , which showed hypoplasia of the right lung with multiple air bronchograms . in the mediastinum ,
two air - filled tubes were visualized , that is , trachea and esophagus [ figure 2a ] . only left mainstem bronchus was visualized at the tracheal bifurcation .
the right mainstem bronchus originated from distal esophagus and coursed superiorly to aerate the right lung [ figure 2b ] .
nasogastric tube was inserted that delineated the retracted air filled esophagus on the right side of the trachea .
dilute barium was slowly injected through the nasogastric tube , which established the diagnosis of bronchoesophageal communication by opacifying the bronchial tree on the right side [ figure 3 ] .
imaging advances such as volume rendered ct and virtual bronchoscopy showed anomalous origin of the right mainstem bronchus from the esophagus [ figure 4a ] and only one opening at the tracheal bifurcation [ figure 4b ] .
chest radiograph shows increased opacification of right lung with air bronchograms , mediastinal shift , and herniation of the contralateral lung towards right ( a ) axial image shows two closely placed air - filled tubes esophagus ( small arrow ) and trachea ( large arrow ) in the mediastinum .
( b ) coronal reformatted image depicts the origin of right the mainstem bronchus(small arrow ) from distal esophagus ( large arrow ) with associated collapse - consolidation of the right lung dilute barium has entered the right bronchial tree , thus , establishing the bronchoesophageal communication ( a ) volume rendered computed tomography scan shows the single left mainstem bronchus at carinal level .
the right mainstem bronchus originates from the distal esophagus and courses superiorly to aerate the right lung .
( b ) virtual bronchoscopy shows the single opening of the left mainstem bronchus ( arrow ) at the tracheal bifurcation .
right mainstem bronchus is not visualized at surgery , a hypoplastic consolidated lung was discovered on the right side .
there was a firm cartilaginous tube ( right mainstem bronchus ) coming from the distal esophagus and entering into the right lung [ figure 5 ] .
right pneumonectomy with enbloc resection of esophageal bronchus was performed , and abnormal opening in the esophagus was ligated and repaired .
operative findings : small consolidated right lung with a cartilaginous right mainstem bronchus(long arrow ) , communicating with the esophagus(short arrow )
sometimes only a segmental bronchus may arise from the lower esophagus with rest of the lung being normal .
diagnosis is usually established within the first 8 months of life , even though late presentations in adulthood are also described .
because of the proximity of the right mainstem bronchus with esophagus , right side is frequently affected .
furthermore , clinical manifestations may vary from recurrent chest infections to severe respiratory failure , depending on the type of malformation .
patients presenting with recurrent chest infections or cough on intake of fluids or food should be investigated with chest radiograph followed by contrast study of esophagus and chest ct .
vascular studies can be further conducted for preoperative mapping as well as to rule out sequestration .
barium esophagogram is the investigation of choice in such cases and should be the ideal choice in patients with recurrent refractory chest infections .
embryologically , the respiratory system develops as a ventral diverticulum from the foregut . as laryngotracheal tube elongates , the tracheoesophageal ridge develops and separates the laryngotracheal tube from rest of the foregut
later , tracheoesophageal ridge fuses to form the septum and divides the foregut into ventral and dorsal portions .
any abnormal development of tracheoesophageal groove along with the differential elongation of trachea and esophagus results in these anomalies .
known associations of esophageal lung include cardiac anomalies , esophageal atresia , and tracheooesophgeal fistula .
foregut duplication cysts , divericulae , intralobar or extralobar pulmonary sequestrations come under the noncommunicating variety .
communicating type is one where there is communication between the respiratory and gastrointenstinal systems . in 1966 ,
braimbridge and keith suggested a classification for congenital fistulas depending upon the communication between esophagus and bronchus / sequestrated lobe .
further , srikanth et al . classified the communicating bronchopulmonary foregut malformations as follows : 1a : total sequestered lung communicating with the foregut , associated with esophageal atresia and tracheoesophageal fistula to the distal pouch 1b : sequestered anatomic lobe or segment communicating with the foregut , associated with esophageal atresia and tracheoesophageal fistula to the distal pouch ii : total sequestered lung communicating with the lower esophagus ; absent ipsilateral mainstem bronchus iii : isolated anatomic lobe or segment communicating with the foregut iv : portion of the normal bronchial system communicating with the esophagus our case falls into category iv where the entire lung was aerated by the right mainstem bronchus , which originated from the lower esophagus .
the entire lung was hypoplastic and consolidated with the left mainstem bronchus arising from the distal esophagus .
the differential diagnosis includes pulmonary sequestration , congenital cystic adenomatoid malformation , and iatrogenic , inflammatory , or neoplastic fistulas .
the two main methods of treatment are division and suturing of the ends of the fistula and complete resection . till today
, less than 20 cases of esophageal lung have been reported , and most of the cases were associated with esophgeal atresia . in our case , there was no esophageal atresia as the nasogastric tube easily passed into the stomach .
bronchopulmonary foregut malformations are rare entities where there is abnormal communication between the respiratory and upper alimentary system . early recognition of this rare entity could guide an appropriate treatment with better clinical outcome .
 we report a rare case of esophageal lung in a neonate who presented with repeated chest infections and respiratory distress . 
 chest radiograph revealed increased opacification of the right lung with reduced lung volume and air bronchograms . 
 further evaluation with computed tomography ( ct ) showed the presence of only left mainstem bronchus at the tracheal bifurcation . 
 right mainstem bronchus originated from distal esophagus and aerated the right lung . 
 nasogastric tube was inserted into the stomach with injection of small amount of dilute barium through it , which established the communication of right mainstem bronchus with esophagus . 
 advanced ct scan imaging by virtual bronchoscopy and volume rendering further delineated the anatomical abnormality precisely prior to surgery . 
 surgical findings confirmed the diagnosis . 
Introduction
Case Report
Discussion
Conclusion
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