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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

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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.

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Congenital Esophageal Atresia

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Definitions and Epidemiology

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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).

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Table 15–1. Associated Anomalies in Babies with Esophageal Atresia

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