He Yuxin, Yu Zhuwen, Chen Weichang
Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Soochow University, 188 Shizi Road, Suzhou, Jiangsu Province, China.
BMC Gastroenterol. 2020 Sep 21;20(1):306. doi: 10.1186/s12876-020-01445-2.
Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease.
We report a 65-year-old male patient with jaundice, decreased appetite, and itchy skin. His biochemistry test results indicated obstructive jaundice disease. Cross-sectional imaging showed a type IVA choledochal cyst accompanied by autosomal dominant polycystic kidney disease (ADPKD). Due to economic difficulties, the patient achieved percutaneous transhepatic cholangial drainage (PTCD) instead of surgery.
To our knowledge, this is the second case report of the coexistence of type IVA choledochal cysts and ADPKD. We conclude that it is vital to be aware that the above condition is a possibility. This case report will aid earlier diagnosis and management and possibly prevent further damage to liver and kidney function.
胆管囊肿分为5种类型。医生认为卡罗里病（指V型胆管囊肿）是一种由PKHD1基因突变引起的特殊类型胆管囊肿，与常染色体隐性遗传性多囊肾病（ARPKD）相关。目前其他类型的胆管囊肿与多囊肾病之间尚无明确关联。
我们报告一名65岁男性患者，有黄疸、食欲减退和皮肤瘙痒症状。其生化检查结果显示为梗阻性黄疸疾病。横断面成像显示为IVA型胆管囊肿并伴有常染色体显性遗传性多囊肾病（ADPKD）。由于经济困难，该患者接受了经皮肝穿刺胆管引流术（PTCD）而非手术治疗。
据我们所知，这是IVA型胆管囊肿与ADPKD并存的第二例病例报告。我们得出结论，意识到上述情况有可能发生至关重要。本病例报告将有助于早期诊断和治疗，并可能防止对肝脏和肾脏功能造成进一步损害。
