Cystic diseases of the biliary tract and liver Invited Editor

Abstract

Cystic diseases of liver are recognized in infancy and childhood initially Cystic diseases of liver and biliary tract are choledocal cysts autosomal recessive and autosomal dominant polycystic kidney disease congenital hepatic fibrosis and Caroli disease cystic dilatation of intrahepatic bile ducts Choledochal cysts and Caroli disease do not allow biliary flow cause chronic or obstructive cholestasis and progressive liver disease In congenital hepatic fibrosis and polycystic kidney disease there is cystic formations at terminal interlobular bile ducts but cholestasis is not seen They don’t cause liver and biliary tract functional disturbances Turk Arch Ped 2008; 43: 40 5 Key words: Biliary tract cystic diseases liver renal

Keywords

• -

Karaciğer ve safra yollarının kistik hastalıkları Çağrılı Editör

Öz

Karaciğerin kistik hastalıkları süt çocukluğu ve çocukluk çağında tanı alırlar Karaciğer ve safra yollarının kistik hastalıklarını koledok kistleri otozomal çekinik resesif ve otozomal baskın dominant polikistik böbrek hastalığı doğumsal hepatik fibroz ve Caroli hastalığı karaciğer içi safra yollarının kistik genişlemesi oluşturmaktadır Koledok kisti ve Caroli hastalığı safra akımını engeller kronik veya tıkayıcı kolestaza ve ilerleyici karaciğer hastalığına neden olur Doğumsal hepatik fibroz ve polikistik böbrek hastalığında terminal interlobuler safra kanallarında kistik oluşumlar yer alır kolestaz yoktur Karaciğer ve safra yolları işlev bozukluğuna yol açmazlar Türk Ped Arş 2008; 43: 40 5 Anahtar kelimeler: Böbrek karaciğer kistik hastalık safra yolları

Anahtar Kelimeler

• Böbrek, karaciğer, kistik hastalık, safra yolları

Kaynakça

1. Lipsett PA, Pitt HA, Colombani PM, et al. Choledochal cyst di- sease. A changing pattern of presentation. Ann Surg 1994;220:644-52.
2. Stain SC, Guthrie CR, Yellin AE, et al. Choledochal cyst in the adult. Ann Surg 1995;222:128-33.
3. Stringer MD, Dhawan A, Davenport M, et al. Choledochal cysts: lessons from a 20 year experience. Arch Dis Child 1995; 73:528-31.
4. Evans SJ. Abnormalities of the bile ducts.In:Wyllie R, Hyams SJ (eds). Pediatric gastrointestinal disease. Philadelphia: W.B.Saunder company, 1993:901-33.
5. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: Classification, operative procedures, and review of thir- ty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977 ;134:263-9.
6. Todani T, Watanabe Y, Toki A, et al. Classification of conge- nital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg 2003;10:340-4.
7. 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;37:1568-73.
8. Okada A, Nakamura T, Higaki J, et al. Congenital dilatation of the bile duct in 100 instances and its relationship with anoma- lous junction. Surg Gynecol Obstet 1990;171:291-8.

9. Han SJ, Hwang EH, Chung KS, et al . Acquired choledochal cyst from anomalous pancreatobiliary duct union. J Pediatr Surg 1997;32:1735-8.
10. Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg 1977;12:39-42.
11. Redkar R, Davenport M, Howard ER. Antenatal diagnosis of congenital anomalies of the biliary tract. J Pediatr Surg 1998;33:700-4
12. Okada T, Sasaki F, Ueki S, et al. Postnatal management for prenatally diagnosed choledochal cysts. J Pediatr Surg 2004;39:1055-8.
13. Kim WS, Kim IO, Yeon KM, et al. Choledochal cyst with or without biliary atresia in neonates and young infants: US dif- ferentiation. Radiology 1998;209:465-9.
14. Lugo-Vicente HL. Prenatally diagnosed choledochal cysts: observation or early surgery? J Pediatr Surg 1995;30:1288- 90.
15. Suita S, Shono K, Kinugasa Y, et al. Influence of age on the presentation and outcome of choledochal cyst. J Pediatr Surg 1999;34:1765-8.
16. Poddar U, Thapa BR, Chhabra M, et al. Choledochal cysts in infants and children. Indian Pediatr 1998;35:613-8.
17. Ando K, Miyano T, Kohno S, et al. Spontaneous perforation of choledochal cyst: a study of 13 cases. Eur J Pediatr Surg 1998;8:23-5.
18. Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg 1980;140:653- 7.
19. Iwai N, Deguchi E, Yanagihara J, et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg 1990;25:1261-3.
20. Franko J, Nussbaum ML, Morris JB. Choledochal cyst cho- langiocarcinoma arising from adenoma: case report and a re- view of the literature. Curr Surg 2006;63:281-4.
21. Bancroft JD, Bucuvalas JC, Ryckman FC, et al. Antenatal di- agnosis of choledochal cyst. J Pediatr Gastroenterol Nutr 1994;18:142-5.
22. Yamataka A, Ohshiro K, Okada Y, et al. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg 1997;32:1097-102.
23. Miyano T, Yamataka A, Kato Y, et al. Hepaticoenterostomy after excision of choledochal cyst in children: a 30-year expe- rience with 180 cases. J Pediatr Surg 1996 ;31:1417-21.
24. Metcalfe MS, Wemyss-Holden SA, Maddern GJ. Manage- ment dilemmas with choledochal cysts. Arch Surg 2003;138:333-9.
25. Li DY, Schwarz BK. Congenital and structural abnormalities of the liver. In: Kelly AD (ed). Liver disease in children. Second edition. Oxford; Blackwell, 2004: 162-82.
26. Keane F, Hadziç N, Wilkinson ML, et al. Neonatal presentati- on of Caroli's disease. Arch Dis Child Fetal Neonatal Ed 1997;77:F145-6.
27. Levy AD, Rohrmann CA Jr, Murakata LA, et al. Caroli's disea- se: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179:1053-7.
28. Desmet VJ. Pathogenesis of ductal plate malformation. J Gastroenterol Hepatol 2004;19: S356-60.
29. Desmet VJ. Ludwig symposium on biliary disorders-Part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 1998;73:80-9.
30. Giovanardi RO. Monolobar Caroli's disease in an adult. Case report. Hepatogastroenterology 2003; 50: 2185-7
31. Gupta AK, Gupta A, Bhardwaj VK, et al. Caroli's disease. In- dian J Pediatr 2006; 73: 233-5.
32. Yonem O, Bayraktar Y. Clinical characteristics of Caroli's di- sease. World J Gastroenterol 2007;13:1930-3.
33. Sharma R, Mondal A, Taneja V, et al. Radionuclide scintig- raphy in Caroli's disease. Indian J Pediatr 1997; 64: 105-7.
34. Asselah T, Ernst O, Sergent G, et al. Caroli's disease: a mag- netic resonance cholangiopancreatography diagnosis. Am J Gastroenterol 1998; 93: 109-10.
35. Marchal GJ, Desmet VJ, Proesmans WC. Caroli disease: high- frequency US and pathologic findings. Radiology 1986 ; 158: 507-11.
36. De Kerckhove L, De Meyer M, Verbaandert C, et al. The pla- ce of liver transplantation in Caroli's disease and syndrome. Transpl Int 2006; 19: 381-8.
37. Patil S, Das HS, Desai N, et al. Caroli's syndrome-a rare cau- se of portal hypertension. J Assoc Physicians India 2004; 52: 261.
38. Ros E, Navarro S, Bru C, et al. Ursodeoxycholic acid treat- ment of primary hepatolithiasis in Caroli's syndrome. Lancet 1993;342:404-6.
39. Bockhorn M, Malago M, Lang H, et al. The role of surgery in Caroli's disease. J Am Coll Surg 2006; 202: 928-32.
40. Habib S, Shakil O, Couto OF, et al. Caroli's disease and ort- hotopic liver transplantation. Liver Transpl 2006; 12: 416-21.
41. Berrebi G, Erickson RP, Marks BW. Autosomal dominant polycystic liver disease: a second family. Clin Genet. 1982;21:342-7.
42. Pirson Y, Lannoy N, Peters D, et al. Isolated polycystic liver disease as a distinct genetic disease, unlinked to polycystic kidney disease 1 and polycystic kidney disease 2. Hepatology 1996;23:249-52.
43. Karhunen PJ, Tenhu M. Adult polycystic liver and kidney di- seases are separate entities. Clin Genet 1986;30:29-37.
44. Waanders E, te Morsche RH, de Man RA, et al. Extensive mu- tational analysis of PRKCSH and SEC63 broadens the spec- trum of polycystic liver disease. Genetik Hum Mutat 2006; 27:830.
45. Drenth JP, Tahvanainen E, te Morsche RH, et al. Abnormal hepatocystin caused by truncating PRKCSH mutations leads to autosomal dominant polycystic liver disease. Hepatology 2004;39:924-31.
46. Qian Q, Li A, King BF, et al. Clinical profile of autosomal do- minant polycystic liver disease. Hepatology 2003;37:164-71.
47. Kerr DN, Okonkwo S, Choa RG. Congenital hepatic fibrosis: the long-term prognosis. Gut 1978;19:514-20.
48. Alvarez F, Bernard O, Brunelle F, et al. Congenital hepatic fib- rosis in children. J Pediatr 1989;99:370-5.
49. Desmet VJ. What is congenital hepatic fibrosis? Histopatho- logy 1992;20:465-77.
50. Perisic VN. Long-term studies on congenital hepatic fibrosis in children. Acta Paediatr 1995 ;84:695-6.
51. De Vos M, Barbier F, Cuvelier C. Congenital hepatic fibrosis. J Hepatol 1988;6:222-8.
52. Witzleben CL, Sharp AR. Nephronophthisis-congenital hepa- tic fibrosis": an additional hepatorenal disorder. Hum Pathol 1982;13:728-33.
53. Blankenberg TA, Ruebner BH, Ellis WG, et al. Pathology of re- nal and hepatic anomalies in Meckel syndrome. Am J Med Genet 1987;3: S 395-410.
54. Fiorillo A, Migliorati R, Vajro P, et al. Congenital hepatic fibro- sis with gastrointestinal bleeding in early infancy. Clin Pediatr 1982;21:183-5.
55. Abdullah AM, Nazer H, Atiyeh M, et al. Congenital hepatic fib- rosis in Saudi Arabia. J Trop Pediatr 1991;37:240-3.
56. Sessa A, Righetti M, Battini G. Autosomal recessive and do- minant polycystic kidney diseases. Minerva Urol Nefrol 2004; 56:329-38.
57. Sessa A, Ghiggeri GM, Turco AE. Autosomal dominant polyc- ystic kidney disease: clinical and genetic aspects. J Nephrol 1997;10:295-310.
58. Boucher C, Sandford R. Autosomal dominant polycystic kid- ney disease (ADPKD, MIM 173900, PKD1 and PKD2 genes, protein products known as polycystin-1 and polycystin-2). Eur J Hum Genet 2004;12:347-54.
59. Johnson CA, Gissen P, Sergi C. Molecular pathology and ge- netics of congenital hepatorenal fibrocystic syndromes. J Med Genet 2003 ;40:311-9.
60. Lina F, Satlinb LM. Polycystic kidney disease: the cilium as a common pathway in cystogenesis. Curr Opin Pediatr 2004;16:171-6.
61. Bennett WM, Elzinga LW. Clinical management of autosomal dominant polycystic kidney disease. Kidney Int Suppl 1993;42:S74-9.
62. Parfrey PS, Bear JC, Morgan J, et al. The diagnosis and prog- nosis of autosomal dominant polycystic kidney disease. N Engl J Med 1990 ;323:1085-90.
63. Wilcox DM, Weinreb JC, Lesh P. MR imaging of a hemorrha- gic hepatic cyst in a patient with polycystic liver disease. J Comput Assist Tomogr 1985;9:183-5.
64. Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: the clinical experience in North America. Pediatrics 2003;111:1072-80.
65. Zerres K, Rudnik-Schöneborn S, Senderek J, et al. Autosomal recessive polycystic kidney disease (ARPKD). J Nephrol 2003;16:453-8.
66. Murcia NS, Woychik RP, Avner ED. The molecular biology of polycystic kidney disease. Pediatr Nephrol 1998;12:721-6.
67. Capisonda R, Phan V, Traubuci J, et al. Autosomal recessive polycystic kidney disease: outcomes from a single-center ex- perience. Pediatr Nephrol 2003;18:119-26.
68. Roy S, Dillon MJ, Trompeter RS, Barratt TM. Autosomal re- cessive polycystic kidney disease: long-term outcome of ne- onatal survivors. Pediatr Nephrol 1997;11:302-6.