저자들은 다발성 선천기형 및 태아 곤란증의 의진하에 반복 제왕술을 통하여 분만되어 5분만에 사망한 신생아의 부검소견상 뇌의 심한 분화 및 형성부전을 보이는 Holoprosencephaly, 폐형성부전, 생식기 및 항문의 무형성, 비장, 췌장의 무형성을 동반한 요로기형과 복벽의 결손을 가진 prune-belly syndrome 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

The Prune-belly syndrome is the most common term for congenital absence, deficiency or hypoplasia of the abdominal musculature accompained by a large hypotonic bladder, dilated and tortuous ureters and bilateral cryptochidism. This full-blown syndrome, which was associated with alobar holosencephaly with hydrocephalus, hypoplasia of lung and libs, markedly dilated cyst with hypertrophied muscular layer, mild hydroureter, polycystic kidney, colon atresia, agenesis of pancreas, spleen and genital organ and equinovalgus and genuvalgus, has been carefully observed in accordance with review of references.