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).