Intestinal atresia (from Greek meaning “non-perforated”)
denotes a complete obliteration of the intestinal lumen and is commonly
used to describe congenital conditions. Intestinal webs, which are thin intraluminal diaphragms
that are lined with mucosa, are a form of atresia. Webs may be stretched
out to give the appearance of a windsock. Stenosis is
a narrowing of the intestinal lumen. Intestinal atresias and webs
may be subclassified based on anatomical location. They may occur
in the esophagus, stomach, duodenum, jejunum, ileum, colon, or anus.1
The underlying pathophysiology in all children with atresias
and webs is obstruction to the flow of intestinal contents. The
mode of presentation can be acute, with obvious features of regurgitation,
vomiting, abdominal pain, and abdominal distension. Neglected cases
may present with systemic decompensation and shock. Presentation
can also be subtle especially when the obstruction is incomplete
as may be seen with intestinal stenosis. Patients may have chronic
intermittent abdominal pain, intermittent vomiting, and distension.
They may have long-standing nutritional compromise or weight loss;
however, some children will present with normal weight as they adjust
their diet to minimize symptoms. Early diagnosis can be prompted
by careful evaluation of clinical findings as well as antenatal
history, and subsequent accurate interpretation of radiographic
findings. Initial care should include appropriate resuscitation
and identification of associated anomalies in preparation of the
child for definitive therapy. It is critical that early in the course
of the work-up, conditions that threaten the child’s life
or lead to loss of intestinal length, such as heart disease, malrotation,
or intestinal perforation, are identified and treated. Secondary investigation
could then be performed safely. Treatment of intestinal atresia
is surgical. The goal of the surgical treatment is relief of intestinal
obstruction while preserving intestinal length and function. Prior
to the advent of safe pediatric anesthesia and intensive care, as
many as 50% of the children did not survive treatment.1–3 Mortality
declined sharply starting in the 1950s, and currently acute mortality
is less than 4–8%. Most patients can expect a
good quality of life after surgical correction but a significant
burden of disease is borne by those with inadequate intestinal length.
Esophageal atresia (EA) is a congenital malformation in which
the esophagus is interrupted. Congenital EA occurs approximately
in 1 in 3000 live births.4 The embryological basis for
esophageal anomalies is still poorly understood. The esophagus and trachea
separate 28–37 days following fertilization. Failure of
the tracheal bud to separate from the primordial foregut is thought
to result in EA. EA is often associated with other congenital anomalies,
most commonly cardiac, and can be a component of vertebral, anorectal,
cardiac, tracheoesophageal, renal, and limb anomalies (VACTERL)
complex of anomalies (Table 15–1).
Table 15–1. Associated
Anomalies in Babies with Esophageal Atresia
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