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1
題名:Monolobar Caroli's Disease: Report of Two Cases within a Family     (12點)
單葉性卡羅里氏病:同一家族兩病例報告
著者:Min-Chieh Shieh(謝閔傑) ;Ching-Chung Chiang(江慶鐘) ;Chi-Wen Tu(涂?文) ;Chao-Ming Shih(史兆明) ;Chi-Szu Liao(廖志思) ;Chen-Hsien Su(蘇振賢)
出版地區:台灣
出版城市:台北市
學科:醫學綜合
關鍵字:Caroli's disease ; autosomal dominant polycystic kidney disease ; hepatic resection
刊名:Formosan journal of surgery
卷期:40卷3期(2007.6)
頁碼:136-141
語言:英語
摘要: 中文摘要PDF ; 英文摘要PDF

卡羅里氏病是肝臟的先天性疾病,肝內膽管會出現多發性囊狀異常擴張。此種疾病常與體細胞顯性遺傳或隱性遺傳的多發性腎臟囊腫疾病合併出現。我們提出同一家族內出現兩個肝臟單葉性卡羅里氏病的案例,在接受部分肝臟切除手術後得到滿意的成果。 案例1是一位74歲女性病患,已診斷罹患體細胞顯性遺傳多發性腎臟囊腫疾病十年並已進展至慢性腎衰竭。她以反覆發生急性膽管炎及菌血症作?臨床表現。腹部電腦斷層及核磁共振檢查都證實左葉肝內膽管出現囊狀異常擴張、肝內結石及總膽管結石。案例2是一位43歲男性病患,同時是案例1的兒子,在兩年前也診斷罹患體細胞顯性遺傳多發性腎臟囊腫疾病。他以阻塞性黃膽合併急性膽管炎作?臨床表現。腹部電腦斷層發現左側肝內膽管異常擴張、肝內結石及總膽管結石。兩位案例在接受膽囊切除、總膽管截石及左外側肝臟接除手術後,術後恢復都相當順利。病患術後分別追蹤4個月及14個月,臨床上都無膽管炎復發的症狀出現。卡羅里氏病的確切診斷需要依賴影像學檢查。經內試鏡逆行性胰膽管攝影是最好的診斷方法,它能提供非侵入性、安全及準確的診斷。對侷限於單葉或單節肝臟的卡羅里氏病患者,進行部份肝臟切除可以提供治癒的機會。
Caroli's disease is a congenital disorder characterized by segmental communicating saccular dilatation of larger intrahepatic bile ducts. This malformation may occur in association with autosomal dominant or recessive polycystic kidney disease of varying severity. This study reports the successful management of two cases with monolobar Caroli's disease within a family by partial hepatic resection. Case one was a 74 year-old female, who had a known diagnosis of autosomal dominant polycystic kidney disease, and clinically manifested repeated episodes of bacterial cholangitis and septicemia. Abdominal computed tomography and magnetic resonance cholangiopancreatography confirmed the presence of saccular dilatation of intrahepatic bile ducts confined to the left lobe, hepatolithiasis and choledocholithiasis. Case two was a 43 year-old male, who was the son of case one, had autosomal dominant polycystic kidney disease, and clinically manifested acute bacterial cholangitis. Abdominal computed tomography demonstrated the presence of dilated left intrahepatic bile ducts, hepatolithiasis and choledocholithiasis. Both patients underwent cholecystectomy, choledocholithotomy and left lateral segmentectomy and the post-operative recovery was uneventful in both cases. There was no recurrence of cholangitis during a 14-month and a four-month follow-up, respectively. The diagnosis of Caroli's disease was established by imaging studies. Magnetic resonance cholangiopancreatography is preferred and it could provide a noninvasive, safe and accurate diagnosis of Caroli's disease. Partial hepatic resection may be curative in patients with Caroli's disease confined to a single lobe or segment of the liver.


    

本卷期目次
Formosan journal of surgery 40卷3期 (2007.6)
Concomitant Myocardial Revascularization and Modified Radical Mastectomy: Report of a Case/ Shao-jung LiWen-Je KoShu-Hsun ChuShoei-Shan Wang
Extradural Tophaceous Gout of Lumbosacral Spine Report of a Case/ Tai-Tzung KuoChen-Chia TsaiCheng-Kuei ChangMing-Fu Chiang
Magnetic Resonance Image for Preoperative Localization of Insulinoma/ Keng-Hao LiuMing-Chin YuWEI-CHEN LEEKuang-Tse PanJenn-Hui JenYi-Yin JanMiin-Fu Chen
Laparoscopic Treatment of Bezoar-induced Small Bowel Obstruction: Report of a Case/ Heng-Fu LinJiann-Ming WuKuo-Hsin ChenLi-Ming TsengShih-Horng Huang
Volvulus of Gallbladder: Report of a Case/ Ching-Chuan HsiehChia-Siu WangWen-Ke WangWen-Shih Huang
Monolobar Caroli's Disease: Report of Two Cases within a Family/ Min-Chieh ShiehChing-Chung ChiangChi-Wen TuChao-Ming ShihChi-Szu LiaoChen-Hsien Su
Endoscope-Assisted Minimally Invasive Surgery for Coronary Artery Bypass/ Kuan-Ming ChiuTzu-Yu LinShao-jung LiJer-Shen ChenChih-Yang ChanShu-Hsun Chu
Magnetic Resonance Image for Preoperative Localization of Insulinoma/ Keng-Hao LiuMing-Chin YuWei-Chen LeeKuang-Tse PanJenn-Hui JenYi-Yin JanMiin-Fu Chen
Endoscope-Assisted Minimally Invasive Surgery for Coronary Artery Bypass/ Kuan-Ming ChiuTzu-Yu LinShao-Jung LiJer-Shen ChenChih-Yang ChanShu-Hsun Chu
Concomitant Myocardial Revascularization and Modified Radical Mastectomy: Report of a Case/ Shao-Jung LiWen-Je KoShu-Hsun ChuShoei-Shan Wang
Extradural Tophaceous Gout of Lumbosacral Spine Report of a Case/ Tai-Tzung KuoChen-Chia TsaiCheng-Kuei ChangMing-Fu Chiang
Laparoscopic Treatment of Bezoar-induced Small Bowel Obstruction: Report of a Case/ Heng-Fu LinJiann-Ming WuKuo-Hsin ChenLi-Ming TsengShih-Horng Huang
Volvulus of Gallbladder: Report of a Case/ Ching-Chuan HsiehChia-Siu WangWen-Ke WangWen-Shih Huang
Monolobar Caroli's Disease: Report of Two Cases within a Family/ Min-Chieh ShiehChing-Chung ChiangChi-Wen TuChao-Ming ShihChi-Szu LiaoChen-Hsien Su
 
   
 
   

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