2 The procedure
2.1.1 Oesophageal atresia is a congenital condition in which there is a break in the continuity of the oesophagus between the mouth and stomach. In some patients, both the proximal and the distal ends of the oesophagus end in blind pouches; more commonly, one or both ends of the oesophagus are attached to the trachea, forming tracheo-oesophageal fistulae. Saliva and milk enter the lungs after pooling in the upper oesophagus or passing though a tracheo-oesophageal fistula, resulting in episodes of choking, coughing and cyanosis. If untreated, oesophageal atresia leads to death from aspiration pneumonia or malnutrition.
2.1.2 The vast majority of patients are treated by surgical division of the fistula and primary anastomosis of the oesophagus, to allow normal swallowing.
2.1.3 The definition of long-gap atresia varies, but generally it means the gap is greater than 3–3.5 cm. With long-gap atresia, any direct anastomosis is placed under significant tension. Therefore, any fistula is divided, and a gastrostomy is sited to enable enteral feeding. Repair is delayed for up to 3 months to allow the upper and lower pouches to elongate and hypertrophy, with the intention that anastomosis will then be possible. If it is not possible, alternative surgical approaches include pulling the stomach partially up into the thorax, or using a length of colon to join the oesophageal ends.
2.2.1 Using a transthoracic extrapleural approach, the fistula or fistulae are divided and oversewn. The oesophageal pouches are exposed and traction sutures are placed in the ends, brought out through the skin and fixed with silastic buttons. Traction is applied to the sutures, which stimulates elongation of the oesophagus by 1–2 mm per day. Once the ends of the oesophagus have come together, or are in close proximity, a primary anastomosis is performed. After the repair, the patient may be kept sedated and ventilated for a number of days to allow the anastomosis to heal. Oesophageal balloon dilatation may be performed if required.
2.3.1 Reported clinical outcomes varied considerably between studies, and were often qualitative only. One case series reported 70 infants with oesophageal atresia treated by primary repair: 10 of the patients had long-gap atresia and four of these were treated with the Foker technique. All four patients were eating excellently or satisfactorily at a mean follow-up of 8.8 years. After treatment, all 10 of the infants with long-gap atresia had gastro-oesophageal reflux requiring fundoplication. A second case series described 23 cases in which the babies were treated with external traction (atresia length ranged from 3.7 to 10 cm). A primary anastomosis was achieved in all cases.
2.3.2 Another case series reported that 67% (2/3) of patients achieved full oral feeding at up to 4 months after treatment with the Foker technique. Another found that 50% (1/2) of patients were eating solids normally at 1 year, and 50% (1/2) still required a gastric tube for feeding. The rate of successful anastomosis varied between studies from 100% (4/4 and 2/2) to 33% (1/3). For more details, refer to the Sources of evidence.
2.4.1 Disruption of sutures during the traction stage of the procedure occurred in 25% (3/12) of patients with long-gap atresia across all the studies identified, usually requiring the anastomosis to be performed under greater tension than intended. No deaths were reported that were directly related to repair of the oesophageal atresia.
2.4.2 The Specialist Advisors noted adverse events including stricture formation and gastro-oesophageal reflux. They also noted other possible adverse events including anastomotic leak, suture disruption during the period of traction, fistulae, gastric emptying problems and difficulties in swallowing.