Paediatrics: Congenital abnormalities: tracheo-oesophageal

Congenital abnormalities: tracheo-oesophageal

Tracheo-oesophageal fistula

TOF is usually associated with OA. However, an isolated TOF will pre-sent with:

•   choking or coughing during feeding;

•   abdominal distension;

•   recurrent LRTI.

Although symptoms are present from birth the diagnosis is frequently not made until later in childhood. The investigations of choice are a tube injec-tion of X-ray contrast into the oesophagus and bronchoscopy. Treatment is surgical division of the TOF through a neck incision.

Oesophageal atresia and tracheo-oesophageal fistula

The incidence of OA and TOF (Fig. 23.2) is 1/3500 live births.

•   75% babies with OA will have a TOF.

•   10% will have isolated OA, which is usually associated with a long gap or defect.

•   Rare isolated TOF.

•   Rare OA with both upper and lower pouch TOFs.

Maternal polyhydramnios is common, although antenatal diagnosis is rare.

Babies present at birth with:

•   excess mucus or ‘mucousy’;

•   choking and cyanosis on feeding;

•   associated malformations in 50%, usually the VACTERL association.

Diagnosis

Confirmed or excluded by:

•   Passage of a 10F NGT.

•   CXR: the tube stops in the upper thorax. Air in the stomach indicates a fistula between the trachea and the distal oesophagus (TOF).

Acute management

•   The baby should be kept warm and disturbed as little as possible.

•   The upper oesophageal pouch should be aspirated regularly by oropharyngeal suction or a Replogle tube.

•   Standard IV fluids started.

• Pre-operative antibiotics are not required unless there is evidence of aspiration pneumonia.

• Babies who require mechanical ventilation must be referred urgently for surgery because gas will escape down the TOF and produce progressive gastric distension, which impairs ventilation further, ultimately leading to gastric perforation.

Surgery

• Disconnection of the TOF and anastomosis of upper and lower oesophagus through a right thoracotomy.

• Long gap OA may require a feeding gastrostomy and a cervical oesophagostomy in the neonatal period followed by oesophageal replacement during infancy. Some specialist centres now perform the Foker operation, where prolonged internal ‘stretch’ makes the remnant of the upper and lower oesophagus ‘grow’!

• High-risk babies may have a staged procedure—the TOF is ligated and then the OA repaired a few days later.

• Complications include anastomotic leak, anastomotic stricture, gastro-oesophageal, and recurrent fistula.

Follow-up

• Respiratory morbidity in the early years after OA/TOF repair is relatively high, particularly in the winter months. Consider admitting these children during respiratory infections.

• Obstruction of the oesophagus by food boluses is common in toddlers and young children after OA repair. Usually, it is caused by meat that has not been chewed. Refer for urgent oesophagoscopy.