Duodenal atresia

Gastrointestinal Duodenal atresia Agenesis of appendix and cecum Mai rotation Imperforate anus... 

In addition to phocomelias, other teratogenic effects include eye and ear abnormalities, esophageal and duodenal atresias, and defects in internal organs such as the heart and kidneys. Congenital defects of the kidneys and nervous system may persist throughout life. Very large doses taken with ethanol have been associated with transient hypotension. Bradycardia has been rarely reported with therapeutic use. Acute toxicity appears infrequent. 

Hydroxylation. The 2)8-hydroxylated derivative of cholic acid, 2, 3a,7a,12a-tetrahydroxy-5i8-cholanate, was characterized as a minor component of gastric contents of neonates with duodenal atresia [202], Identification was facilitated by the availability of the authentic compound, arapaimic acid, which was isolated from bile of Arapaima gigas (Chapter 10) and was synthesized [204], This tetrol is also a minor urinary metabolite of cholate in patients with intrahepatic cholestasis [123]. A polar bile acid formulated as a 2,3,6,7-tetrol obtained from urine of healthy newborn infants [199] apparently differs from an unidentified urinary tetrol from patients with cholestasis [164],... 

Hydroxylation. Urinary acids hydroxylated at position 6, especially 6a, include hyodeoxycholate, hyocholate, 3a,6 /, 12a-trihydroxy- and 3a,6a,7a,12 -tetrahy-droxy-5 -cholanates. Hyocholate, the major bile acid in urine, serum and duodenal fluid of a child with intrahepatic cholestasis [109], is a major urinary metabolite of chenodeoxycholate in patients with intrahepatic cholestasis, while 3a,6a,12a-trihy-droxy-5j3-cholanate is only a minor metabolite of deoxycholate [123]. The 3a,6a,7a,12a-tetrahydroxy acid, obtained from urine of patients with liver diseases [194] and from gastric contents of neonates with duodenal atresia [203] is another metabolite of cholic acid [123] several unidentified tetrols have yet to be char-... 

The main causes for a double bubble are duodenal atresia, annular pancreas, and midgut volvulus. Less frequently it may be secondary to duodenal web, Ladd s band, or preduodenal portal vein (Crowe and Sumner 1978). 

Fig. 1.4a,b. Duodenal atresia, a Plain radiograph obtained 6 h after birth shows absence of air in the gastrointestinal tract of this neonate with severe lung disease, b Radiograph made after inflation ofthe stomach through a nasogastric tube demonstrates complete duodenal obstruction ( double bubble sign)... 

Fig. 1.5a-c. Annular pancreas. Supine (a) and lateral (b) views of the abdomen show complete obstruction at duodenal level identical to that seen in duodenal atresia. The stomach (st) and duodenal bulb (d) are distended and there is no distal air. c Surgical image shows the pancreatic tissue (arrow) encircling the duodenum (d)... 

The diagnosis is usually apparent on the plain films. The abdominal radiograph shows a few dilated bowel loops (three or four air bubbles), more than in the case of duodenal atresia and fewer than in ileal atresia or in other causes of low bowel obstruction (Fig. 1.14). The loop just proximal to the site of the atresia is frequently disproportionately dilated with a bulbous end. There is no air in the lower portion of the abdomen this is observed most clearly in the upright film (Rathaus and Grunebaum 1992) (Fig. 1.15). The colon cannot be identified and air... 

Connolly B, Alton DJ, Ein SH et al (1995) Partially reduced intussusception when are repeated delayed reduction attempts appropriate Pediatr Radiol 25 104-107 Cotran RS, Kumar V, Robbins SL (1989) Appendix. In Saunders WB (ed) Robbins pathologic basis of disease, 4th edn. Saunders, Philadelphia, pp 902-904 Crowe JE, Sumner TE (1978) Combined esophageal and duodenal atresia without tracheoesophageal fistula characteristic radiographic changes. AJR Am J Roentgenol 130 167-168... 

Nelson LH, Clark CE, Fishburne JI (1982) Value of serial sonography in the in utero detection of duodenal atresia. Obstet Gynecol 59 657-660... 

Fig. 2.6. Esophageal atresia without distal tracheoesophageal fistula. The abdomen is gasless. Duodenal atresia was diagnosed at the time of surgical repair...

If the stomach is too big in the third trimester on two consecutive studies, the main cause is duodenal atresia with the typical double-bubble appearance. A total of 30% of these patients will have trisomy 21 (Buonomo et al. 1998). These patients also have an increased risk of an esophageal atresia, in which case they will not always present with a double-bubble appearance due to the obstruction of the GI tract more proximally, unless they have an associated... 

Gastric rupture is mainly seen in neonates due to acute distention of the stomach, ischemic necrosis associated with perinatal asphyxia and distal obstruction such as annular pancreas (Ibach and Inouye 1965) or duodenal atresia/stenosis. On the other hand, duodenal rupture in neonates is rare. 

Fig. 5.3a,b. A neonate with a highly distended duodenum and stomach due to duodenal atresia. No passage of gas seen distally... 

In the case of duodenal obstruction with similar clinical signs as atresia, but with air distal to the duodenum, intestinal malrotation should be ruled out. 

A distended abdomen is virtually always present the more distal the level of intestinal obstruction. Failure to pass meconium maybe an additional symptom. Atresia of the jejunum and ileum is twice as common as duodenal atresia, about equal in jejunum and ileum, with an incidence of 1 in 4-500 live births. Multiple atresias occur in 10%-20% of cases (Carty et al. 2005 Parker 2003 Grier 1999 Devos and Meradji 2003 Berrocal et al. 1999). Intestinal vascular accidents, such a thromboem-... 

Preduodenal portal vein (PPV) is a rare congenital anomaly associated with additional various anomalies, including intestinal malrotation, situs anomalies, IVC anomalies, polysplenia, pancreatic anomalies, duodenal atresia, and cardiac anomalies (Tsuda et al. 1991 Gayer et al. 1999). On CT, the preduodenal and prepancreatic localization of the portal vein is demonstrated, as well as any associated anomalies (Fig. 9.5). The diagnosis of a PPV before surgical intervention in the upper abdomen is important to prevent its inadvertent ligation or transection (Tsuda et al. 1991). 

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