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Key Points

  • Accounts for up to 15% of intestinal atresias. Incidence is about 1 in 20,000 livebirths.

  • On prenatal sonogram appears as multiple dilated loops.

  • Fetal MRI may demonstrate dilated proximal colon and absence of meconium in distal colon.

  • Colonic atresia can be associated with gastrointestinal anomalies or Hirschsprung's disease.

  • Diagnosis has no implications for timing or route of delivery.

  • Overall prognosis is excellent.


Colonic atresia is a rare cause of intestinal obstruction and accounts for less than 10% to 15% of all cases of intestinal atresia (Sturim and Ternberg, 1966; Coran and Eraklis, 1969; Bowles et al., 1976; Powell and Raffensperger, 1982; Touloukian, 1993a). Colonic atresia was first recognized in 1673 by Bininger (Powell and Raffensperger, 1982). The first survivor with this condition, treated by colostomy, was not reported until 1922 (Gaub, 1922). Potts (1947) was the first to report a survivor after primary anastomosis. Few series of appreciable size have been reported subsequently because of the rarity of isolated colonic atresia or stenosis (Philippart, 1986). The pathogenesis is thought to be similar to the mechanism responsible for jejunoileal atresia and stenosis (see Chapter 73). The majority of colonic atresias, which occur proximal to the splenic flexure, include a significant segment of absent colon with distal microcolon (Rescorla and Grosfeld, 1985). Anomalies associated with colonic atresia are unusual, but can include gastroschisis and jejunal atresia, Hirschsprung's disease, as well as ocular and skeletal anomalies (Bowles et al., 1976; Powell and Raffensperger, 1982; Rescorla and Grosfeld, 1985; Jackman and Brereton, 1988; Etensel et al., 2005; Draus et al., 2007). The skeletal anomalies most often seen with isolated colonic atresia include syndactyly, polydactyly, absent radius, and clubfoot (Philippart, 1986). Major cardiac anomalies and genetic defects are rare, although they have been reported (Robertson et al., 1994). Colonic atresias may be seen as a complicating factor in abdominal wall defects such as gastroschisis, omphalocele, or vesicointestinal fistulas (Bowles et al., 1976; Powell and Raffensperger, 1982). Hirschsprung's disease has also been reported to occur in association with colonic atresia (Johnson and Dean, 1981). However, it is important to remember that in two-thirds of the cases, colonic atresia occurs as an isolated defect without associated abnormalities. Multiple colonic atresias have been reported but are extremely rare (Touloukian, 1993b).

The classic demonstration by Louw and Barnard (1955) that jejunal atresia results from an in utero mesenteric vascular accident is widely accepted and is thought to also apply to colonic atresia. Vascular compromise may occur as a result of a primary vascular accident in utero, or it may be secondary to a mechanical event, such as intestinal volvulus. Because colonic atresia, proximal and distal to the splenic flexure, differs, the cause ...

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