Two Sibling Cases of Congenital duodenal Membranous Stenosis in Adolescence

Koji Fujimoto, Satoshi Matsusue, Hiroshi Takeda, Sadao Kashihara, Norihiko Terada*

Department of Abdominal Surgery, Tenri Hospital
*Terada Hospital

Case 1 is a 29-year-old woman who came to our hospital complaining of high grade fever. Blood chemistry showed markedly elevated biliary enzyme levels. Hypotonic duodenography and duodenoscopy revealed a circular stenosis in the second portion of the duodenum. A congenital duodenal membranous stenosis (web) was suspectd and a laparotomy was performed. Intraoperative cholangiography showed that the caliber of the common bile duct was dilated and the wall was irregular. The papilla of Vater was located proximal to the web. She was diagnosed to have congenital duodenal membranous stenosis with cholangitis. The web was excised and Roux-en-Y choledocho-jejunostomy was performed. Case 2 is a 25-year-old woman (a younger sister of case 1) who came to a local hospital complaining of vomiting. Upper GI series and duodenoscopy revealed a circular stenosis in the second portion of the duodenum. She was suspected of having annular pancreas and a laparotomy was performed. However, stenosis in the second portion of the duodenum instead of annular pancreas was detected. Gastrojejunostomy was performed. These two sibling cases of congenital duodenal membranous stenosis in adolescence are the first reported in Japan. Moreover, case 2 is also important with respect to the fact that cholangitis is the presenting symptom.

Key words
congenital duodenal membranous stenosis in adolescence, sibling cases, retrograde cholangitis

Reprint requests
Koji Fujimoto Department of Abdominal Surgery, Tenri Hospital
200 Mishima-cho, Tenri, 632 JAPAN

Accepted
November 11, 1992

To read the PDF file you will need Abobe Reader installed on your computer.