無脾症候群の一剖検例

An Autopsy Case of the Asplenic Syndrome

はじめに

　無脾症候群とは先天性無脾症に心血管系の異常，内臓の形成・位置異常等を伴う稀な疾患で，先天性心奇形の約3％に認められる^1）。本症候群はその多彩な奇形群と，胎生初期の胎児の状態との類似性が注目され，発生学的関心を集めている。最近我々は臨床的にWilson—Mikity症候群が疑われ，剖検により無脾症候群であることが確認された1例を経験したので報告する。

An autopsy case of asplenic syndrome was reported in 11/2 months old infant. Since birth the patient had been suffering from disturbance in respiration with a peculiar roentgenographic picture suggesting Wilson-Mikity syndrome.

Autopsy revealed complicated developmentalanomalies; asplenia, cardiovascular abnormalities, bilateral three-lobed lung, symmetry of the liver, situs inversus of the stomach and common mes-entery. A tumor was found arising from left adrenal medulla with the histology of neuroblas-toma.