多発奇形を合併した Prune Belly Syndrome の1例 : 本邦報告例110例の統計的観察

概要

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A newborn male baby was brought to our hospital because of a thin, flaccid abdominal wall. Clinical examination revealed multiple anomalies such as high imperforate anus, malrotation of the intestine, patent urachus, omphalocele, megacystis, urethral stenosis and bilateral undescended testis, so that colostomy, repair of malrotation, excision of the urachus, one stage repair of the abdominal wall and vesicostomy was performed at the day of birth. Further examination of urinary tract after the first operation revealed agenesis of the right kidney and bilateral vesicoureteric reflux (VUR). He is unable to expel urine per urethra at the age of two years and bouginage of the urethra is done for the purpose of intermittent catheterization and antireflux operation for VUR is scheduled to be done in the near future. A review of the literature to date in Japan has brought to light an additional 110 cases of the prune belly syndrome, and discussion was made on these cases. High mortality rate (40.5%) was observed and the causes of death were chiefly pulmonary failure, sepsis and renal failure. Therefore more proper treatment should be given to serve good renal function and to prevent urinary and respiratory tract infection.
日本小児外科学会の論文
1991-04-20