Gallenwegzysten bei Kindern und Jugendlichen

ZUSAMMENFASSUNG

Gallenwegzysten sind seltene angeborene Fehlbildungen des Gallenwegsystems, welche zumeist bereits im Kindesalter diagnostiziert werden. Sie sind oft symptomlos, können sich aber auch schon im Kindesalter mit Zeichen einer unklaren zystischen Struktur im rechten Oberbauch, mit Zeichen einer akuten oder chronischen Obstruktion der Gallenwege oder einer Pankreatitis manifestieren. Die am weitesten verbreitete Einteilung der Zysten erfolgt nach Todani, welcher fünf Typen unterscheidet. Typ I und IV stellen dabei die häufigsten Zystenarten dar. Die genaue Beschreibung und Einteilung der Zysten ist von großer Bedeutung, da hiervon die Prognose, aber auch das weitere therapeutische Vorgehen abhängen. Dabei ist es inzwischen allgemein anerkannt, dass Gallenwegzysten eine Präkanzerose darstellen. Die Therapie der Wahl besteht daher in einer möglichst vollständigen Resektion des gesamten extrahepatischen Gallengangsystems mit Anlage einer biliodigestiven Anastomose. Es wird empfohlen, die Operation bereits im Kindesalter durchzuführen, da mit zunehmendem Alter das Risiko einer malignen Entartung signifikant ansteigt, aber auch die Rate an operativen Komplikationen zunimmt.

ABSTRACT

Biliary cysts are rare congenital malformations of the biliary tract. They are usually diagnosed in childhood and present with signs of a cystic mass in the right upper abdomen or with symptoms of acute or chronic biliary tract obstruction or pancreatitis. In some cases, they are diagnosed incidentally with no or only mild symptoms. The most common classification of biliary cysts was introduced by Todani, who discriminated five subtypes. The most common types are type I and IV. A presice description and classification of the biliary cysts is of utmost importance because prognosis and treatment depend on this. Today it is widely accepted that biliary cysts are a premalignant condition. In most types the treatment of choice is the surgical resection of the complete extrahepatic biliary tract with subsequent biliodigestive anastomosis. Because of the increasing risk of developing malignancy with age as well as an increased risk of operative complications in older patients, it is suggested to perform the operation already in childhood.

Schlüsselwörter

Gallenwegzysten - Choledochuszyste - Todani-Klassifikation - Malignomentstehung

Key words

Biliary cysts - choledochal cyst - Todani classification - malignancy

• Literatur

• 1 Alonso-Lej F, Rever Jr WB, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Int Abstr Surg 1959; 108 (01) 1-30
• 2 Arda IS, Tuzun M, Aliefendioglu D. et al Spontaneous rupture of extrahepatic choledochal cyst: two pediatric cases and literature review. Eur J Pediatr Surg 2005; 15: 361-363
• 3 Babitt D. Congenital choledochal cysts: New etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann Radiol 1968; 12: 231-240
• 4 de Vries JS, de Vries S, Aronson DC. et al Choledochal cysts: age of presentation, symptoms, and late complications related to Todani’s classification. J Pediatr Surg 2002; 37 (11) 1568-1573
• 5 Feng A, O’hara SM, Gupta R, Fei L, Lin TK. Normograms for the extrahepatic bile duct diameter in children. JPGN 2017; 64 (03) e61-e64
• 6 Foo D, Wong K, Lan L, Tam P. Impact of prenatal diagnosis on choledochal cysts and the benefit of early excision. J Pediatr Child Health 2009; 45: 28-30
• 7 Fumino S, Iwai N, Deguchi E. Spontaneous rupture of choledochal cyst with pseudocyst formation – report on 2 cases and literature review. J Pediatr Surg 2006; 41: E19-21
• 8 Hamada Y, Ando H, Kamisawa T. et al Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci 2016; 23: 342-346
• 9 Huang CT, Lee HC, Chen WT. et al Usefulness of magnetic resonance cholangiopancreatography in pancreatobiliary abnormalities in pediatric patients. Pediatr Neonatol 2011; 52 (06) 332-336
• 10 Jablońska B. Biliary cysts: etiology, diagnosis and management. World J Gastroenterol 2012; 18 (35) 4801-4810
• 11 Kato T. Etiological relationships between choledochal cyst and anomalous junction of the pancreatobiliary ductal system. Keio J Med 1989; 38 (02) 136-151
• 12 Kobayashi S, Asano T, Yamasaki M. et al Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999; 126: 939-944
• 13 Kobayashi S, Ohnuma N, Yoshida H. et al Preferable operative age of choledochal dilation types to prevent patients with pancreaticobiliary maljunction from developing biliary tract carcinogenesis. Surgery 2006; 139 (01) 33-38
• 14 Komi N, Tamura T, Miyoshi Y. et al Nationwide survey of cases of choledochal cyst. Analysis of coexistent anomalies, complications and surgical treatment in 645 cases. Surg Gastroenterol 1984; 3: 69-73
• 15 Kozumi K, Kadama T. A case of cystic dilatation of the common bile duct and its pathology. Tokyo J Med Sci 1916; 30: 1413-1426
• 16 Lipsett PA, Pitt HA. Surgical treatment of choledochal cysts. J Hepatobiliary Pancreat Surg 2003; 10 (05) 352-359
• 17 Miyano T, Suruga K, Suda K. “The choledocho-pancreatic long common channel disorders” in relation to the etiology of congenital biliary dilatation and other biliary tract disease. Ann Acad Med Singap 1981; 10 (04) 419-426
• 18 Moss RL, Musemeche CA. Successfull management of ruptured choledochal cyst by primary cyst excision and biliary reconstruction. J Pediatr Surg 1997; 32 (10) 1490-1491
• 19 Nicholl M, Pitt HA, Wolf P. et al Choledochal cysts in western adults: complexities compared to children. J Gastrointest Surg 2004; 8: 245-252
• 20 Ohashi T, Wakai T, Kubota M. et al Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. J Gastroenterol Hepatol 2013; 28: 243-247
• 21 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: 360-366
• 22 Rao KLN, Chowdhary SK, Kumar D. Choledochal cyst associated with portal hypertension. Pediatr Surg Int 2003; 19: 729-732
• 23 Remmele W. Hrsg Pathologie Band 3. Zweite neu bearbeitete Auflage. Berlin, Heidelberg: Springer-Verlag; 1997: 27-31
• 24 Sacher VY, Davis JS, Sleeman D, Casillas J. Role of magnetic resonance cholangiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol 2013; 5 (08) 304-312
• 25 Singham J, Schaeffer D, Yoshida E, Scudamore C. Choledochal cysts: analysis of disease pattern and optimal treatment in adult and paediatric patients. HPB (Oxford) 2007; 9: 383-387
• 26 Singham J, Yoshida EM, Scudamore CH. Choledochal cysts. Part 1 of 3: Classification and pathogenesis. Can J Surg 2009; 52 (05) 434-440
• 27 Singham J, Yoshida EM, Scudamore CH. Choledochal cysts. Part 2 of 3: Diagnosis. Can J Surg 2009; 52: 506-511
• 28 Singham J, Yoshida EM, Scudamore CH. Choledochal cysts. Part 3 of 3: Management. Can J Surg 2010; 53 (01) 51-56
• 29 Soares KC, Arnaoutakis DJ, Kamel I. et al Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg 2014; 219 (06) 1167-1180
• 30 Soares KC, Goldstein SD, Ghaseb MA. et al Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int 2017; 33: 637-650
• 31 Ten Hove A, de Meijer VE, Hulscher JBF, Kleine RHJ. Meta-analysis of risk of developing malignancy in congenital choledochal malformation. Brit J Surg 2018; 105: 482-490
• 32 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 (02) 263-269
• 33 Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979; 44: 1134-1141
• 34 Tsai M, Lin W, Hsu W. et al Clinicopathologic feature and surgical outcome of choledochal cyst in different age groups: the implication of surgical timing. J Gastrointest Surg 2008; 12: 2191-2195
• 35 van den Eijnden MHA, de Kleine RHJ, de Blaauw I. et al Nederlandse Studiegroep voor Choledochus Cysten/malformaties. Choledochal malformation in children: lessons learned from a Dutch National Study. World J Surg 2017; 41: 2631-2637
• 36 Vater A, Elzer CS. Dissetatio de scirrhis viscerum occasione setionis viri tympanite defunte. Wittenburgae 1723; 881: 1-22
• 37 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6: 207-212
• 38 Wiseman K, Buczkowski AK, Chung SW. et al Epidemiology, presentation, diagnosis, and outcome of choledochal cysts in adults in an urban environment. Am J Surg 2005; 189 (05) 527-531
• 39 Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg 1980; 140 (05) 653-657
• 40 Yamashiro Y, Miyano T, Suruga K. et al Experimental study of the pathogenesis of choledochal cyst and pancreatitis, with special reference to the role of bile acids and pancreatic enzymes in the anomalous choledocho-pancreatico ductal junction. J Pediatr Gastroenterol Nutr 1984; 3 (05) 721-727
• 41 Yotsuyanagi S. Contribution to the aetiology and pathogeny of idiopathic cystic dilatation of the common bile-duct with report of three cases: A new aetiological theory based on supposed unequal epithelial proliferation at the stage of the physiological epithelial occlusion of the primitive choledochus. Gann 1936; 30: 601-650