Trachea-oesophageal Fistula and its management at PGIMER

Jun 18, 2017 17:25 | Chandigarh
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Chandigarh, 18 June, 2017 (kulbir singh kalsi): Trachea-oesophageal Fistula (TEF) is a condition characterized by abnormal communication between the esophagus (food pipe) and the trachea (wind pipe). It may be either present since birth (congenital) or developed due to some disease process (acquired). Congenital TEF is a common abnormality of the upper respiratory and digestive tract affecting nearly 1 in 2500 – 3000 live births with increased risk in case of family history of TEF. This disease is multi-factorial in causation, including factors like chromosomal abnormalities  and environmental, however all factors are not known.

In TEF, the food pipe is incompletely developed – the two formed pouches (upper and lower) of food pipe separated by a gap. Commonly seen types of TEF include a blind upper pouch and a lower pouch connected to the wind pipe, the second being - both upper and lower pouched without any connections (Pure esophageal atresia).

Most babies present on 1st day of life with a lot of saliva from mouth, inability to take in feeds and breathing difficulty. They need to be seen by a vigilant paediatrician (child doctor) or a GP who would refer them to a pediatric (child) surgeon. TEFs are frequently associated with other organ abnormalities – like the heart (13-34%), backbone (6-21%), limbs (5-9%), anus and rectum (10-16%) and the kidney (5-14%).

During pregnancy, the diagnosis of TEF includes maternal polyhydramnios (increased fluid in amniotic sac) and absent stomach bubble (normally seen) on multiple antenatal ultrasound help in antenatal diagnosis of TEF. After delivery, clinical complaints as mentioned above along with coiling/stopping of an orogastric tube (passed through the mouth) in the upper food pipe on chest xray help in diagnosis of TEF.

Management – TEFs require early surgical repair by highly trained and specialized pediatric surgeons. In more recent times key hole repair is also being done at PGIMER, Chandigarh. Surgery involves disconnecting the communication between the wind pipe and food pipe and stiching the upper and lower pouches together, so that the baby can attain normal feeding. Pure esophageal atresias  are managed with bypass surgery of the food pipe (saliva will drain out from the neck) and stomach (a pipe will be kept for feeding the baby) followed by reconstruction of food pipe late in life using different organs like stomach and colon.

At PGIMER Chandigarh, TEF is one of the commonest surgical emergencies and more than 250 cases are operated every year. The reason for this high number at our center according to Prof Ram Samujh, Professor and Head Department of Pediatric Surgery, is the large catchment area from Jammu &Kashmir, Punjab, Himachal Pradesh, Haryana, Uttrakhand and parts of western UP. Most of these areas are devoid of pediatric surgical expertise which is required for managing these babies. 

At present there were 19 patients operated in our neonatal surgical ICU for TEF and associated anomalies, the highest number at any given time at any place in India.

As per Prof J K Mahajan, Professor of Pediatric Surgery, patients with TEF need long term follow up with periodic contrast (dye xrays) studies because of risk of development of gastro-esophageal reflux (stomach contents go back to the esophagus), recurrent cough and chest infections and food pipe stitch site tightening. These long term complications may require anti reflux surgeries and upper GI endoscopy (to look inside the food pipe) with multiple esophageal dilatations. Survival and prognosis of operated TEFs depend upon prematurity, birth weight and associated organ abnormalities as mentioned before.