Bilier Kistler Tanı ve Tedavi Yaklaşımları
Özet
Biliyer kistler (koledok malformasyonları), safra ağacı boyunca tek başına veya çoklu olarak meydana gelebilen ve taş oluşumu, kolanjit, pankreatit, rüptür ve sekonder biliyer siroz gibi ciddi komplikasyonlara yol açabilen kistik dilatasyonlardır. Kadınlarda ve çocuklarda daha sık görülen bu kistler, Todani klasifikasyonuna göre altı ana tipe ayrılmaktadır. En yaygın türleri Tip I ve Tip IV kistler olup, bu iki tür özellikle kolanjiokarsinom gibi yüksek malignite riski taşımaktadır. Kistlerin patogenezinde anormal pankreatobiliyer bileşke (APBJ) önemli bir rol oynamakta, enzimatik reflü safra epitelinde hasara yol açmaktadır. Klinik olarak hastalar genellikle karın ağrısı, sarılık ve ele gelen kitle üçlüsüyle başvururlar. Tanıda transabdominal ultrason, BT ve özellikle iyonlaştırıcı radyasyon içermeyen MRCP öncelikli olarak tercih edilirken; altın standart direkt kolanjiyografidir (ERCP). Tedavi yaklaşımı kistin tipine göre değişmektedir. Yüksek kanser riski barındıran Tip I ve Tip IV kistlerde kistin Roux-en-Y hepatikojejunostomi ile tamamen cerrahi eksizyonu önerilirken, Tip II kistler basit eksizyonla, Tip III kistler ise endoskopik sfinkterotomi ile tedavi edilebilmektedir; intrahepatik tutulumu olan Caroli hastalığında (Tip V) ise karaciğer nakli gerekebilmektedir.
Biliary cysts, also known as choledochal malformations, are cystic dilatations that can occur singly or multiply along the biliary tree, leading to significant complications such as stone formation, cholangitis, pancreatitis, rupture, and secondary biliary cirrhosis. More prevalent in females and children, these cysts are categorized into six primary types based on the Todani classification. Type I and Type IV cysts are the most common variants and carry a particularly high risk of malignancy, such as cholangiocarcinoma. An anomalous pancreaticobiliary junction (APBJ) plays a critical role in the pathogenesis, allowing pancreatic juice reflux to damage the biliary epithelium. Clinically, the classic presentation includes the triad of abdominal pain, jaundice, and a palpable mass. Diagnosis relies on transabdominal ultrasound, CT, and especially MRCP due to its non-ionizing nature, while direct cholangiography (ERCP) remains the gold standard. Treatment strategies depend heavily on the cyst type. Complete surgical excision with Roux-en-Y hepaticojejunostomy is strongly recommended for high-risk Type I and Type IV cysts. Conversely, Type II cysts can be managed with simple excision, Type III with endoscopic sphincterotomy, and diffuse intrahepatic Type V cysts (Caroli disease) may ultimately require liver transplantation.
Referanslar
Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977; 134:263.
Todani T, Watanabe Y, Toki A, Morotomi Y. Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg 2003; 10:340.
Cha SW, Park MS, Kim KW, et al. Choledochal cyst and anomalous pancreaticobiliary ductal union in adults: radiological spectrum and complications. J Comput Assist Tomogr 2008; 32:17.
Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst disease. A changing pattern of presentation. Ann Surg 1994; 220:644.
Zimmer V, Lammert F. Periampullary choledochal diverticulum. Clin Res Hepatol Gastroenterol 2020; 44:628.
Kagiyama S, Okazaki K, Yamamoto Y, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J Gastroenterol 1987; 82:641.
Law R, Topazian M. Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol 2014; 12:196.
Han SJ, Hwang EH, Chung KS, et al. Acquired choledochal cyst from anomalous pancreatobiliary duct union. J Pediatr Surg 1997; 32:1735.
Iwata F, Uchida A, Miyaki T, et al. Familial occurrence of congenital bile duct cysts. J Gastroenterol Hepatol 1998; 13:316.
Lu, SC. Biliary cysts. In: Textbook of gastroenterology, Yamada, T (Eds), Lippincott Williams and Williams, Philadelphia 1999. p.2292.
Tyler KL, Sokol RJ, Oberhaus SM, et al. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts. Hepatology 1998; 27:1475.
Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg 1977; 12:39.
Yamao K, Mizutani S, Nakazawa S, et al. Prospective study of the detection of anomalous connections of pancreatobiliary ducts during routine medical examinations. Hepatogastroenterology 1996; 43:1238.
Ragot E, Mabrut JY, Ouaïssi M, et al. Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC). World J Surg 2017; 41:538.
Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy. Langenbecks Arch Surg 2009; 394:159.
Li L, Yamataka A, Yian-Xia W, et al. Ectopic distal location of the papilla of vater in congenital biliary dilatation: Implications for pathogenesis. J Pediatr Surg 2001; 36:1617.
Matsumoto S, Tanaka M, Ikeda S, Yoshimoto H. Sphincter of Oddi motor activity in patients with anomalous pancreaticobiliary junction. Am J Gastroenterol 1991; 86:831.
Craig AG, Chen LD, Saccone GT, et al. Sphincter of Oddi dysfunction associated with choledochal cyst. J Gastroenterol Hepatol 2001; 16:230.
Xia HT, Wang J, Yang T, et al. Sphincter of Oddi Dysfunction and the Formation of Adult Choledochal Cyst Following Cholecystectomy: A Retrospective Cohort Study. Medicine (Baltimore) 2015; 94:e2088.
Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg 2009; 52:506.
Shah OJ, Shera AH, Zargar SA, et al. Choledochal cysts in children and adults with contrasting profiles: 11-year experience at a tertiary care center in Kashmir. World J Surg 2009; 33:2403.
Badebarin D, Aslanabadi S, Teimouri-Dereshki A, et al. Different clinical presentations of choledochal cyst among infants and older children: A 10-year retrospective study. Medicine (Baltimore) 2017; 96:e6679.
Lee SE, Jang JY, Lee YJ, et al. Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 2011; 146:1178.
de Kleine RH, Schreuder AM, Ten Hove A, et al. Choledochal malformations in adults in the Netherlands: Results from a nationwide retrospective cohort study. Liver Int 2020; 40:2469.
Sastry AV, Abbadessa B, Wayne MG, et al. What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management? World J Surg 2015; 39:487.
Elnemr A, Ohta T, Kayahara M, et al. Anomalous pancreaticobiliary ductal junction without bile duct dilatation in gallbladder cancer. Hepatogastroenterology 2001; 48:382.
Sugiyama M, Abe N, Tokuhara M, et al. Pancreatic carcinoma associated with anomalous pancreaticobiliary junction. Hepatogastroenterology 2001; 48:1767.
Hidaka E, Yanagisawa A, Seki M, et al. High frequency of K-ras mutations in biliary duct carcinomas of cases with a long common channel in the papilla of Vater. Cancer Res 2000; 60:522.
Kim Y, Hyun JJ, Lee JM, et al. Anomalous union of the pancreaticobiliary duct without choledochal cyst: is cholecystectomy alone sufficient? Langenbecks Arch Surg 2014; 399:1071.
Drabek J, Keil R, Stovicek J, et al. The role of endoscopic retrograde cholangiopancreatography in choledochal cysts and/or abnormal pancreatobiliary junction in children. Prz Gastroenterol 2017; 12:303.
Jabłońska B. Biliary cysts: etiology, diagnosis and management. World J Gastroenterol 2012; 18:4801.
Oduyebo I, Law JK, Zaheer A, et al. Choledochal or pancreatic cyst? Role of endoscopic ultrasound as an adjunct for diagnosis: a case series. Surg Endosc 2015; 29:2832.
Akhan O, Demirkazik FB, Ozmen MN, Ariyürek M. Choledochal cysts: ultrasonographic findings and correlation with other imaging modalities. Abdom Imaging 1994; 19:243.
Lam WW, Lam TP, Saing H, et al. MR cholangiography and CT cholangiography of pediatric patients with choledochal cysts. AJR Am J Roentgenol 1999; 173:401.
Park DH, Kim MH, Lee SK, et al. Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts? Gastrointest Endosc 2005; 62:360.
Kim SH, Lim JH, Yoon HK, et al. Choledochal cyst: comparison of MR and conventional cholangiography. Clin Radiol 2000; 55:378.
Lambie H, Cook AM, Scarsbrook AF, et al. Tc99m-hepatobiliary iminodiacetic acid (HIDA) scintigraphy in clinical practice. Clin Radiol 2011; 66:1094.
Ouaissi M, Kianmanesh R, Ragot E, et al. Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts. World J Gastrointest Surg 2016; 8:427.
Nakano K, Mizuta A, Oohashi S, et al. Protein stone formation in an intrapancreatic remnant cyst after resection of a choledochal cyst. Pancreas 2003; 26:405.
Yamada T, Furukawa K, Yokoi K, et al. Liver cyst with biliary communication successfully treated with laparoscopic deroofing: a case report. J Nippon Med Sch 2009; 76:103.
Ouaïssi M, Kianmanesh R, Belghiti J, et al. Todani Type II Congenital Bile Duct Cyst: European Multicenter Study of the French Surgical Association and Literature Review. Ann Surg 2015; 262:130.
Antaki F, Tringali A, Deprez P, et al. A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video). Gastrointest Endosc 2008; 67:163.
Tao KS, Lu YG, Wang T, Dou KF. Procedures for congenital choledochal cysts and curative effect analysis in adults. Hepatobiliary Pancreat Dis Int 2002; 1:442.
