Ando K, Miyano T, Kohno S, Takamizawa S, Lane G
Department of Pediatric Surgery, Juntendo University, School of Medicine, Shizuoka Children's Hospital Tokyo, Japan.
Eur J Pediatr Surg. 1998 Feb;8(1):23-5. doi: 10.1055/s-2008-1071113.
Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.
在我院接受治疗的187例小儿先天性胆管囊肿病例中，我们遇到13例自发性穿孔。所有病例均在4岁以下。8例发现有胆汁性腹膜炎，5例为闭合性穿孔。肝外胆管形态呈囊性者8例，梭形者5例。穿孔周围的囊肿壁呈薄膜状，可见胆汁从变薄的壁中渗出。9处穿孔为单发，4例有多处穿孔。17处穿孔中有4处在囊肿壁后部。仅1例穿孔与共同通道内的蛋白栓有关，而10例因胆管内压力升高迹象而需要经皮胆道引流的先天性胆管囊肿病例中，有7例发现有蛋白栓。我们认为，先天性胆管囊肿的自发性穿孔在婴儿期并不罕见。穿孔的病因必定是由于胰胆管合流异常导致胰液反流引起的胆道上皮刺激，加之婴儿期的壁层不成熟，而非导管压力异常升高或某一点的先天性壁层薄弱。
