Congenital tracheo-oesophageal fistula in an adult

Abstract

The incidence of tracheo-oesophageal fistula is approximately 1 in 4500 live births. Double fistulae, between the trachea and upper and lower segments of the oesophagus, occur in less than 0.3 percent'. This report describes the management of an adult patient with an undiagnosed proximal fistula who suffered repeated pulmonary complications following aspiration of gastric contents during anaesthesia. Case reportA 26-year-old Caucasian presented for elective caesarean section and requested general anaesthesia. She had undergone tracheo-oesophageal fistula and atresia repair as a neonate, but was in good health. Physical examination revealed the scars of a right thoracotomy and previous caesarean sections. Routine preoperative medication included ranitidine 150mg and magnesium trisilicate mixture 20ml orally. During rapid-sequence induction of anaesthesia there were no signs of regurgitation. After delivery, an orogastric tube was passed, 15ml of fluid were aspirated and magnesium trisilicate mixture 20 ml was given via the tube, which was then removed. Soon afterwards, respiration became noisy and a quantity of fluid, contaminated with medication, was obtained on tracheal suction. On completion of surgery, neuromuscular blockade was reversed and the patient was extubated awake in the left lateral position. Cyanosis and wheezing developed progressively, despite prompt treatment with bronchodilators, physiotherapy and oxygen. Chest radiography revealed patchy shadowing at the left base, and blood gas analysis confirmed the presence of severe hypoxia despite an inspired oxygen concentration of 100per cent (Pa,o2, 6.5 kPa; 3.9 kPa). After 24 h intensive care and continuation of bronchodilator and antibiotic therapy, the hypoxaemia resolved.Subsequently, the patient admitted to mild dysphagia with attacks of coughing and wheezing at night since childhood, which were worse if she slept with fewer than three pillows and during pregnancy. Review of the medical records showed that oesophageal atresia, confirmed by contrast radiography, had been diagnosed shortly after her birth. Right thoracotomy confirmed oesophageal atresia with distal tracheooesophageal fistula and primary reconstruction was performed. Postoperatively cyanotic attacks delayed her discharge home, but contrast studies failed to demonstrate a fistula or anastomotic stricture. In her 19th year, a barium swallow investigation showed only a 'web' at the repair site and retrograde oesophageal peristalsis. More recently, there was clinical, microbiological and radiographic evidence of pneumonia following all three previous caesarean sections (one under epidural and two under general anaesthesia). On one of these occasions the lower lobes of both lungs were involved and on the other two occasions only the left lung was involved. Heavy growths of Streptococcus pneumoniae and Haemophilus influenzae had been isolated from the sputum following her first two caesarean sections. After this latest caesarean section, tracheo-oesophageal communication was suspected and f...