Intestinal Atresia
Kara Baker
Intestinal stenosis and atresia are usually grouped together as a spectrum of congenital disorders that cause narrowing or complete obstruction anywhere along the intestine.
These disorders are generally classified by anatomic location: duodenal, jejunoileal, or colonic.
The first case report describing duodenal atresia was published by Calder in 1733.
Clinical findings were further described by Cordes in 1901, but it was not until 1905 and 1914 that the first surgical repairs were reported by surgeons in France and the Netherlands, respectively.1
The first enterostomy to correct jejunoileal atresia was performed by Voisin in 1804.
The first attempted resection and anastomosis was performed by Wanitschek in 1894, but Fockens is credited with the first successful anastomosis in 1911.1
Colonic atresia was the earliest to be described in 1673 by Binninger.
In 1922, Gaub treated a patient with sigmoid atresia with a proximal diverting colostomy, documenting the first record of a survivor of the condition.
In 1947, a surgeon by the name of Potts performed the first known primary anastomosis of the transverse colon in a newborn.1
RELEVANT ANATOMY
The small intestine is composed of 3 parts: the duodenum, jejunum, and ileum.
The duodenum is mostly retroperitoneal, outside of the proximal 2.5 cm.
It extends from the end of the pyloric canal to the duodenojejunal junction.
There is no clear boundary between the jejunum and ileum, but instead a gradual transition is noted by decreases in both wall thickness and presence of plicae circularis.2
The jejunum and ileum lie within the boundary of the colon and are attached to the posterior abdominal wall by mesentery, which also carries the blood and lymphatic supply.
EPIDEMIOLOGY AND ETIOLOGY
The duodenum is the most common site of neonatal intestinal obstruction, accounting for nearly half of all cases (Table 24.1).
The incidence of small bowel stenosis and atresia is believed to be 1:5000 to 1:10 000 live births, and they affect males more commonly than females.
Colonic stenosis and atresia are less common, occurring in 1:20 000 live births.
The etiology is believed to be due to failure of recanalization in the fetal gastrointestinal (GI) tract, which typically occurs during the 11th week of gestation.
They may also be due to intrauterine vascular disruption during fetal development, causing ischemia, necrosis, and reabsorption of affected segments of small or large bowel.
Intestinal stenosis and atresia are commonly associated with other congenital and chromosomal abnormalities, including trisomy 21 (30% of patients), Hirschsprung disease, cystic fibrosis, malrotation, neural tube defects, congenital heart diseases, and other GI atresias.
Fetal risk is increased by conditions that decrease fetal blood supply, such as vasoconstrictive medications and maternal smoking in the first trimester. Use of methylene blue has also been associated with intestinal atresia.3
Small bowel atresias are further classified into types I to IV (Figure 24.1).
CLINICAL PRESENTATION
Most cases will present with signs of obstruction: polyhydramnios in utero, abdominal distention, feeding intolerance, and vomiting after birth.
Emesis may be bilious or nonbilious based on the location of the obstruction relative to the ampulla of Vater.
Patients will also have failure to pass meconium with complete obstruction. Incomplete obstruction may allow passage of mucous plugs or blood per rectum.
TABLE 24.1 Classification of Duodenal and Jejunoileal Atresias | ||||||||||||||||||||||||
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