Choledochal Cyst in Infancy: A Follow-up Study

嬰兒期膽道囊腫: 追蹤研究

鄭明宗(Ming-Tzong Cheng) ； 張美惠(Mei-Hwei Chang) ； 許宏遠(Hong-Yuan Hsu) ； 倪衍玄(Yen-Hsuan Ni) ； 陳秋江(Chiu-Chiang Chen) ； 賴鴻緒(Hong-Shiee Lai) ； 陳維昭(Wei-Jao Chen) ； 謝豐舟(Fon-Jou Hsieh) ； 朱娟秀(Jan-Show Chu)

《Acta Paediatrica Taiwanica》 41卷1期 (2000/02) Pp. 13-17

https://doi.org/10.7097/APT.200002.0013

摘要

從1980年元月至1997年2月，19個膽道囊腫 （男8，女11）病患在一歲前被診斷，大部分病患在6全月大前被診斷 （79%），其中兩名為產前診斷。根據Todani分類，Ia型最多，其餘為Ic型 （26%）。有14名病患接受Roux-en-Y膽道空腸吻合術或肝門空腸吻合術及囊腫切除，3名病患接受葛西開刀法，有一名病患史接受引流術，另一名病患因染色體3套18症拒絕開刀。10名病患同時有肝臟組織學檢查，其中4名合併肝硬化，這19名病患追蹤期間由6個月至9年，平均4.1年，有4名病患不幸死亡7名合併膽道閉鎖者皆合併黃疸及灰白便，其中兩名病患有合併肝硬化，因延遲求診及開刀而死亡，另一名雖然常發現膽囊炎，但最後失去追蹤，剩餘3名則很健康。而12名無膽道閉鎖者，9名健康，1名失去追蹤，2名死亡，其中一名為染色體3套18症狀無法開刀，另一名則延遲開刀後因肝硬化而死亡。結論是：嬰兒期的膽道囊腫應考慮合併膽道閉鎖之可能性；超音波是一種良好的診斷工具，死亡之病患一般都與稽延性黃疸、膽道閉鎖、肝硬化、延遲開刀、敗血症或多重異常有關。另外若合併持績性黃疸與灰白便者應儘快開刀。

關鍵字

膽道囊腫 ； 產前診斷 ； 膽道閉鎖 ； 膽道空腸吻合術

並列摘要

From January 1980 to February 1997, 19 cases, 8 males and 11 females, of choledochal cyst were diagnosed before one year old. The majority of patients were diagnosed by ultrasonography before 6 months old (15/19; 79%), including two diagnosed prenatally. According to Todani’s classification, type I(subscript u) was the most common (74%), followed by type I(subscript c), (26%). Fourteen patients underwent Roux-en-Y choledocho-or hepatico-jejunostomy and cyst excision, 3 patients underwent Kasai operation, and I patient underwent external biliary drainage only. The remaining one patient with Trisomy 18 anomaly refused operation. Four of the 10 patients in whom liver histologic examinations were performed, had liver cirrhosis. The follow-up period of these patients ranged from 6 months to 9 years, with a mean of 4.1 years. We divided these 19 cases into 2 groups, according to the presence or absence of biliary atresia. In the 7 infants with biliary atresia (37%), all presented with jaundice and acholic stool. Two patients died due to delayed presentation and surgery, both had liver cirrhosis. One patient is living with liver cirrhosis. Another patient was lost to follow-up, but frequent cholangitis was noted till 8 months old. The remaining 3 patients are living and well. In the 12 without biliary atresia, 9 patients are living and well. Two patients died, one due to Trisomy 18 anomaly and the other with delayed surgery and liver cirrhosis. One case was lost to follow-up. In summary: 1) a possibility of the association of biliary atresia in infants with choledochal cyst should be carefully searched and considered as a unique group; 2) ultrasonography is a good diagnostic tool in choledochal cyst during prenatal or infancy period; 3) the mortality cases were characterized by prolonged bile stasis, biliary cirrhosis, delayed surgery, or multiple anomalies; 4) surgery should be performed as early as possible for those with persistent jaundice and light colored stools.

並列關鍵字

choledochal cyst ； prenatal diagnosis ； biliary atresia ； Roux-en-Y choledochojejunostomy