J Korean Surg Soc.  2010 Jul;79(1):75-78. 10.4174/jkss.2010.79.1.75.

Heterotaxia Syndrome with Intraluminal Duodenal Diverticulum

• Kim JH
• Seo HI
• Kim HS
• Kim DH
• Jeon TY
• Song GA
• Kim S

• KMID: 1455491
• DOI: http://doi.org/10.4174/jkss.2010.79.1.75

Abstract

Both heterotaxia and intraluminal duodenal diverticulum (IDD) are uncommon congenital anomalies. Heterotaxia is a group of situs anomalies and IDD is a type of duodenal atresia. Heterotaxia is commonly associated with intraabdominal abnormalities causing intestinal obstruction, but heterotaxia with IDD is extremely rare. Herein we report a case of 21-year-old female who presented with symptoms of duodenal obstruction due to IDD associated with heterotaxia. This is the second case found as a result of a search through PubMed. We first used 3-dimentional virtual computed tomograph endoscopy for making the diagnosis and the treatment plan of this patient.

Keyword

Heterotaxia; Situs ambiguous; Polysplenia; Intraluminal duodenal diverticulum; Virtual endoscopy

MeSH Terms

Diverticulum
Duodenal Obstruction
Endoscopy
Female
Humans
Intestinal Obstruction
Young Adult
Duodenal Obstruction

Figure

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  Fig. 1 Duodenography shows a contrast filled sac (arrows) that lies entirely in the reversed duodenal loop. The sac is surrounded by a narrow radiolucent line. It forms the wind sock configuration (the duodenal wind sock sign). This is a typical finding of intraluminal duodenal diverticulum.

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  Fig. 2 The CT image obtained at the level of the pancreas head clearly shows a blind-ending saccular structure within the dilated, reversed duodenum (arrows).

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  Fig. 3 The CT findings of heterotaxia syndrome. They show a midline liver, a right-sided stomach and polysplenia (arrows). There is no inferior vena cava (IVC) and note the dilatation of the azygous and hemiazygous veins. LV = liver; ST = stomach; AZ = azygous vein; HV = hemiazygous vein.

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  Fig. 4 Virtual CT endoscopy. It shows the intraluminal duodenal diverticulum with a small eccentric aperture (arrows) in the medial side of the 2nd portion of the duodenum.

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