先天性表皮水疱症を合併した先天性幽門閉鎖症の 1 例

概要

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A newborn baby was admitted to the Center presenting with vomiting, abdominal distension and cyanosis. The initial diagnosis was sepsis because of high fever, blood count and analysis, CRP test and other clinical signs. In the course of treatment congenital pyloric atresia was diagnosed by X-ray examination. He also presented with blisters at the forarm, groin and soles. After adequate infusion and antibiotics treatment, a laparotomy was performed at the age of 10 days. There was an induration at the pylorus. A gastrotomy revealed an interruption of the gastric and duodenal mucosal membrane at the site of the induration. A Finney type pyloromyotomy was successfully performed. Postoperative course was complicated again by blisters appearing in both extremities. The boy was discharged at 36 days of life with a few remaining but recovering blisters. The combination of congenital pyloric atresia and epidermolysis bullosa was reported in ten cases in the world literatures. This is the first report in Japan. The prognosis is determined by the type of the skin lesion. Epidermolysis bullosa of simplex type has better prognosis than that of dystrophyic type. The inevitability of the combination of the two congenital diseases and the clinical significance of epidermolysis as a pile mark to detect the congenital pyloric atresia were discussed.
日本小児外科学会の論文