Congenital abnormalities

Atresia and tracheo-oesophageal fistula

Congenital atresia of the oesophagus is usually associated with a tracheo-oesophageal fistula. In 85 per cent of cases it is the Lower segment that communicates with the trachea (Fig. 50.13).

It is important to be aware of this abnormality because its recognition within 48 hours of birth, and subsequent surgical correction, is the only hope of survival.

Suspect atresia in early feeding problems

Clinical features

The newborn baby regurgitates all of its first and subsequent feeds. Saliva pours almost continuously from its mouth. This is the sign of oesophageal atresia — it does not occur in any other condition. Attacks of coughing and cyanosis occur on feeding. It should be suspected in all cases of hydramnios, a condition that is present in 50 per cent of cases of atresia. Oesophageal atresia may occur as part of the VACTER group of anomalies (V — vertebral body segmentation defects; A — anal atresia; C — cardiovascular: patent ductus arteriosus, ventricular septal defect; TE — tracheo-oesophageal fistula; R

      radial ray hypoplasia, unilateral renal agenesis).

 

Clinical confirmation of the diagnosis

A nasogastric tube typically comes against an obstruction within 10 cm. If this occurs the diagnosis is virtually certain.

Radiological confirmation

A lateral chest X-ray shows a lucent proximal pouch that may displace the trachea anteriorly. If bowel gas is present there must be a fistula to the distal oesophagus (Fig. 50.14). In cases of doubt air (preferred) or a small amount of contrast can be injected through the nasogastric tube. If contrast is injected it should be remembered that there may be a fistula between the upper segment and the trachea.

Remember to locate the aortic arch

The side that the aortic arch is on needs to be determined because a thoracotomy will be done on the side opposite to the aortic arch. If the position of the aorta is not clear from the chest X-ray an echocardiogram should be done.

Treatment

Corrective surgery is normally performed shortly after the diagnosis is made. If there is a long distance between the two ends there is no need for hurry because there is no fistula to the trachea. It is usual to wait for several weeks so that the two ends of the oesophagus can grow towards each other to reduce the distance between them. In the meantime the child is fed through a gastrostomy tube.

Operation

The best approach is through a thoracotomy on the side opposite to the aortic arch, usually the right, at the level of the fifth intercostal space. The lower segment is divided at its entrance into the trachea and the fistula closed. It is usually possible to perform an anastomosis between the blind upper segment and the lower segment. If this is not possible part of the colon may be interposed.

Prognosis

The two most feared complications are pneumonia and leak­age from the anastomosis. Following recovery from the immediate postoperative period the long-term prognosis is excellent. Most enter adult life with an essentially normal upper GI tract. Some have problems with gastro-oesophageal reflux and there can sometimes be a problem with a-peristalsis of the distal segment giving rise to dysphagia.

Oesophageal stenosis

This is a rare congenital organic narrowing of the lumen of the oesophagus and is a cause of dysphagia. It occurs any­where in the oesophagus.

Dysphagia lusoria

Several vascular anomalies may produce dysphagia by compression of the oesophagus. Classically this is due to an aberrant right subclavian artery [arteria lusoria (Fig. 50.15)]. However, the oesophagus is more commonly compressed by vascular rings, such as a double aortic arch. Dysphagia occurs in only a minority of cases and usually presents early in childhood, although it can occur in the late teens. Treatment is usually by division of the nondominant component of the ring.

‘Short’ oesophagus with hiatus hernia is mentioned only to say that it is not a congenital abnormality. True shortening of the oesophagus is secondary to severe reflux oesophagitis (see below).