色素沉着-息肉综合征伴多发性肠息肉、肠套叠、肠坏死1例

A report of the pigmentation- polyposis syndrome with polypi intussusception and intestinal necroses

患者男， 10岁，以肠梗阻收院。检查见唇部有针头、米粒大小不等，点状墨黑色色素沉着斑，境界清晰，有的相互融合成片，口腔粘膜有片状黑色斑可见。两手背、手指，两足背、趾端，有散在针头大小黑色色素斑。经剖腹探查，证实为空肠上段息肉，息肉系多发性，小如蚕豆，大如乒乓球。即行肠息肉、部分坏死肠段切除及肠吻合术，术后恢复顺利。其色素斑未作特殊处理。

A 10- year- old male patient was hospitalized for an intestinal obstruction. Different sizes of ink- melanin of pigmented macules from pinhead to rice were found on the lips. The patches were well- defined, and some of it were confluent. The piece- pigmented macules were found on the oral mucosa.Some pinhead- sized melanin- pigmented macules on the backs of the hands, the fingers, the backs of the feet and the end of the toes were also found. It was proved to be the polyposis of superior segment jejumum, after abdominal exploration. There are many polypi. The smaller one has the same size as a pea, and the larger as a table tennis. The intestinal polypi and part of the intestinal necroses are resected. Then, the intestinal anastomosis is done. He is recovered successfully after the operation.