同胞に発症した先天性幽門閉鎖症

概要

論文の詳細を見る
Congenital pyloric atresia is extremely rare. We have experienced the disease occurred in siblings (older and younger brothers). This paper reports a younger case of congenital pyloric atresia complicated with epidermolysis bullosa and the review of the literature concerning the familiar occurrence. A baby boy, antenately diagnosed as pyloric atresia by ultrasonography was delivered normally with 33 weeks gestation, weighning 2,450 gr. Since 26th week of pregnancy, hydramnios was noted. He was admitted to our department just after birth. Abdominal plain X-p showed single bubble sign. At on day of age, a laparotomy was performed. Pyloric atresia due to the cord structure was found and the resection of the pylorus with end-to-end gastroduodenostomy was performed. His postoperative course was uneventful, but mild epidermolysis bullosa was found in this case postoperatively. The investigation of HLA typing, and the pedigree will be needed to clarify the hereditary transmission, as the high incidence of familiar occurrence was noted.
日本小児外科学会の論文
1983-10-20