medvisМедицинская визуализацияMedical Visualization1607-07632408-9516RDS-Media Ltd.10.24835/1607-0763-2017-2-85-89medvis-396Research ArticleБРЮШНАЯ ПОЛОСТЬ И ЗАБРЮШИННОЕ ПРОСТРАНСТВОABDOMEN AND RETROPERITONEALМонолобарная форма болезни Кароли с внутрипеченочным конкрементом (клиническое наблюдение)Monolobar Form of Caroli’s Disease with Intrahepatic Stone (Clinical Case)ГалянТ. Н.GalyanT. N.

Галян Татьяна Николаевна – кандидат медицинских наук, старший научный сотрудник отделения рентгенодиагностики и компьютерной томографии 

Для корреспонденции: 119991 Москва, Абрикосовский пер., д. 2. 

Tatiana N. Galyan – cand. of med. sci., the senior research of XRay and CT diagnostic department 

Contact:  Abrikosovsky 2, Moscow, Russia, 119991. 

galmedi05@gmail.comТарбаН. С.TarbaN. S.ХовринВ. В.KhovrinV. V.ПолищукЛ. О.PolizhukL. O.БагметН. Н.BagmetN. N.ФисенкоЕ. П.FisenkoE. P.ФГБНУ “Российский научный центр хирургии им. акад. Б.В. Петровского”РоссияB.V. Petrovsky National Research Centre of SurgeryRussian Federation201728042017028589Copyright © Галян Т.Н., Тарба Н.С., Ховрин В.В., Полищук Л.О., Багмет Н.Н., Фисенко Е.П., 20172017Галян Т.Н., Тарба Н.С., Ховрин В.В., Полищук Л.О., Багмет Н.Н., Фисенко Е.П.Galyan T.N., Tarba N.S., Khovrin V.V., Polizhuk L.O., Bagmet N.N., Fisenko E.P.This work is licensed under a Creative Commons Attribution 4.0 License.https://medvis.vidar.ru/jour/article/view/396

Болезнь Кароли (коммуникативная кавернозная эктазия желчевыводящих путей) является редким наследственным заболеванием, которое характеризуется наличием необструктивных мешотчатых или веретенообразных расширений внутрипеченочных желчных протоков. Выделяют 2 типа болезни Кароли: истинный тип с расширением внутрипеченочных желчных протоков, с поражением, как правило, одного сегмента и со склонностью к формированию камней и развитием холангита и второй тип, который сочетается с врожденным печеночным фиброзом. Истинный тип болезни Кароли необходимо дифференцировать с первичным склерозирующим холангитом, поликистозом, холангиолитиазом и другими заболеваниями. В статье приведены обзор литературы и собственное клиническое наблюдение монолобарной формы болезни Кароли с наличием внутрипеченочного конкремента. Показаны трудности дооперационной дифференциальной диагностики.

Caroli’s disease is a rare inherited disorder characterized by nonobstructive saccular or fusiform dilation of the intrahepatic bile ducts. Two main types of Caroli’s disease are reported, the true type with isolated dilation of intrahepatic bile ducts of single liver segment with formation of stones and development of cholangitis. The second type is combined with a congenital hepatic fibrosis. The true type of Caroli’s disease is distinct from primary sclerosing cholangitis, polycystic disease, cholangiolithiasis and other diseases. This paper presents a literature review and a case of monolobar Caroli’s disease with intrahepatic stones. The difficulties of preoperative differential diagnosis are shown. 

болезнь Кароливнутрипеченочный конкрементМР-холангиографиякомпьютерная томографиямагнитно-резонансная томографияCaroli’s Diseasecholangiolithiasismagnetic resonance cholangiopancreatographycomputed tomographymagnetic resonance tomographyReferencesCaroli J., Soupault R., Kossakowski J., Plocker L., Pardowska M. La dilatation polykystique congenitale des voies biliares intra-hepatiques. Essai de classification. Sem. Hop. Paris. 1958; 34: 488–495.Caroli J., Soupault R., Kossakowski J., Plocker L., Pardowska M. La dilatation polykystique congenitale des voies biliares intra-hepatiques. Essai de classification. Sem. Hop. Paris. 1958; 34: 488–495.Yoshizawa K., Kiyosawa K., Yabu K., Usada S., Shimizu S., Fujimori Y., Mukawa K., Tanaka E., Sodeymana T., Furuta S. Caroli's disease in three siblings (in Japanese with English abstract). Gastroenterol. Jpn. 1992; 27 (6): 780–784.Yoshizawa K., Kiyosawa K., Yabu K., Usada S., Shimizu S., Fujimori Y., Mukawa K., Tanaka E., Sodeymana T., Furuta S. Caroli's disease in three siblings (in Japanese with English abstract). Gastroenterol. Jpn. 1992; 27 (6): 780–784.Ciambotti G.F., Ravi J., Abrol R.P., Arya V. Right-sided monolobar Caroli's disease with intrahepatic stones: nonsurgical management with ERCP. Gastrointest. Endosc. 1994; 40 (6): 761–764.Ciambotti G.F., Ravi J., Abrol R.P., Arya V. Right-sided monolobar Caroli's disease with intrahepatic stones: nonsurgical management with ERCP. Gastrointest. Endosc. 1994; 40 (6): 761–764.Zangger P., Grossholz M., Mentha G., Lemoine R., Graf J.D., Terrier F. MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom. Imaging. 1995; 20: 361–364.Zangger P., Grossholz M., Mentha G., Lemoine R., Graf J.D., Terrier F. MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom. Imaging. 1995; 20: 361–364.Miller W.J., Sechtin A.G., Campbell W.L., Pieters P.C. Imaging findings in Caroli's disease. Am. J. Roentgenol. 1995; 165: 333–337.Miller W.J., Sechtin A.G., Campbell W.L., Pieters P.C. Imaging findings in Caroli's disease. Am. J. Roentgenol. 1995; 165: 333–337.Ribeiro A., Reddy R.K., Bernstein D., Jeffers L., Schiff E. Caroli's syndrome in twin sisters. Am. J. Roentgenol. 1996; 91 (5): 1024–1026.Ribeiro A., Reddy R.K., Bernstein D., Jeffers L., Schiff E. Caroli's syndrome in twin sisters. Am. J. Roentgenol. 1996; 91 (5): 1024–1026.Карельская Н.А., Кармазановский Г.Г. Магнитно-резонансная холангиопанкреатография. М.: ВИДАР-М, 2014. 272 с. Karelskaya N.A., Karmazanovsky G.G. MR cholangiopancreatography. M.: VIDAR-M, 2014. 272 р. (In Russian)Карельская Н.А., Кармазановский Г.Г. Магнитно-резонансная холангиопанкреатография. М.: ВИДАР-М, 2014. 272 с. Karelskaya N.A., Karmazanovsky G.G. MR cholangiopancreatography. M.: VIDAR-M, 2014. 272 р. (In Russian)Barros J.L., Polo J.R., Sanabia J., Garcia-Sabrido J.L., Gom é z-Lorenzo F.J. Congenital cystic dilatation of the intrahepatic bile ducts (Caroli's disease): Report of a case and review of the literature. Surgery. 1979; 85: 589–591.Barros J.L., Polo J.R., Sanabia J., Garcia-Sabrido J.L., Gom é z-Lorenzo F.J. Congenital cystic dilatation of the intrahepatic bile ducts (Caroli's disease): Report of a case and review of the literature. Surgery. 1979; 85: 589–591.Taylor A.C., Palmer K.R. Caroli's disease. Eur. J. Gastroenterol. 1998; 10 (2): 105–108.Taylor A.C., Palmer K.R. Caroli's disease. Eur. J. Gastroenterol. 1998; 10 (2): 105–108.Dagli Ü ., Atalay F., Sasmaz N., Bostanoglu S., Temucin G., Sahin B. Caroli's disease: 1977–1995 experiences. Eur. J. Gastroenterol. 1998; 10 (2): 109–112.Dagli Ü ., Atalay F., Sasmaz N., Bostanoglu S., Temucin G., Sahin B. Caroli's disease: 1977–1995 experiences. Eur. J. Gastroenterol. 1998; 10 (2): 109–112.Boyle M.J., Doyle G.D., McNulty J.G. Monolobar Caroli's disease. Am. J. Gastroenterol. 1989; 84: 1437– 1444.Boyle M.J., Doyle G.D., McNulty J.G. Monolobar Caroli's disease. Am. J. Gastroenterol. 1989; 84: 1437– 1444.Ros E., Navarro S., Bru C., Gilabert R., Bianchi L., Druguera M. Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli's syndrome. Lancet. 1993; 342: 404–406.Ros E., Navarro S., Bru C., Gilabert R., Bianchi L., Druguera M. Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli's syndrome. Lancet. 1993; 342: 404–406.Borges Pinto R., Pereira J., Reverbel T., Grigoletti J., De Mello E., Silva G. Caroli's disease: Report of 10 cases in children and adolescents in Southern Brazil. J. Pediatr. Surg. 1998; 33 (10): 1531–1535.Borges Pinto R., Pereira J., Reverbel T., Grigoletti J., De Mello E., Silva G. Caroli's disease: Report of 10 cases in children and adolescents in Southern Brazil. J. Pediatr. Surg. 1998; 33 (10): 1531–1535.Лузин Е.В., Митин Н.А., Погребняк В.Ю., Федорова Л.В. Болезнь Кароли: трудности диагностики и возможности лечения. Клиническая медицина. 2013; 10 (91): 57–60. Luzin E.V., Mitin N.A., Pogrebnjak V.Ju., Fedorova L.B. Caroli’s disease: difficulties in diagnosing and treatment options. Clinicheskaya meditsina. 2013; 10 (91): 57–60. (In Russian)Лузин Е.В., Митин Н.А., Погребняк В.Ю., Федорова Л.В. Болезнь Кароли: трудности диагностики и возможности лечения. Клиническая медицина. 2013; 10 (91): 57–60. Luzin E.V., Mitin N.A., Pogrebnjak V.Ju., Fedorova L.B. Caroli’s disease: difficulties in diagnosing and treatment options. Clinicheskaya meditsina. 2013; 10 (91): 57–60. (In Russian)Mero ñ o-Carbajosa E.A., Celdran-Uriarte A., MorenoCaparros A., Solera-Arroyo J.C., Marijuan-Martin J.L. Caroli's disease: Study of six cases, including one with epithelial dysplasia. Int. Surg. 1993; 78: 46–49.Mero ñ o-Carbajosa E.A., Celdran-Uriarte A., MorenoCaparros A., Solera-Arroyo J.C., Marijuan-Martin J.L. Caroli's disease: Study of six cases, including one with epithelial dysplasia. Int. Surg. 1993; 78: 46–49.Pavone P., Laghi A., Catalano C., Materia A., Basso N., Passariello R. Caroli's disease. Evaluation with MR cholangiopancreatography (MRCP). Abdom. Imaging. 1996; 21: 117–119.Pavone P., Laghi A., Catalano C., Materia A., Basso N., Passariello R. Caroli's disease. Evaluation with MR cholangiopancreatography (MRCP). Abdom. Imaging. 1996; 21: 117–119.Asselah T., Ernst O., Sergent G., L'hermine C., Paris J-C. Caroli's disease: A magnetic resonance cholangiopancreatography diagnosis. Am. J. Gastroenterol. 1998; 93 (1): 109–110.Asselah T., Ernst O., Sergent G., L'hermine C., Paris J-C. Caroli's disease: A magnetic resonance cholangiopancreatography diagnosis. Am. J. Gastroenterol. 1998; 93 (1): 109–110.Maurea S., Mollica C., Imbriaco M., Fusari M., Camera L., Salvatore M. Magnetic resonance cholangiography with mangafodipir trisodium in Caroli's disease with pancreas involvement. J. Pancreas. 2010; 11 (5):460–463.Maurea S., Mollica C., Imbriaco M., Fusari M., Camera L., Salvatore M. Magnetic resonance cholangiography with mangafodipir trisodium in Caroli's disease with pancreas involvement. J. Pancreas. 2010; 11 (5):460–463.Lefere M., Thijs M., De Hertogh G., Verslype C., Laleman W., Vanbeckevoort D., Van Steendergen W., Claus F. Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur. J. Gastroenterol. Hepatol. 2011; 23 (7): 578–585.Lefere M., Thijs M., De Hertogh G., Verslype C., Laleman W., Vanbeckevoort D., Van Steendergen W., Claus F. Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur. J. Gastroenterol. Hepatol. 2011; 23 (7): 578–585.Fozard J.B.J., Wyatt J.L., Hall R.I. Epithelial dysplasia in Caroli's disease. Gut. 1990; 30: 1150–1153.Fozard J.B.J., Wyatt J.L., Hall R.I. Epithelial dysplasia in Caroli's disease. Gut. 1990; 30: 1150–1153.Moslim M.A., Gunasekaran G., Vogt D., Cruise M., MorrisStiff G. Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature. J. Gastrointest. Surg. 2015; 19 (11): 2019–2027.Moslim M.A., Gunasekaran G., Vogt D., Cruise M., MorrisStiff G. Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature. J. Gastrointest. Surg. 2015; 19 (11): 2019–2027.Salvadori P.S., Torres U.S., D'Ippolito G. Contrastenhanced magnetic resonance cholangiography with gadoxetic-acid-disodium for the detection of biliary-cyst communication in Caroli disease. Gastroenterol. Hepatol. 2016; 39 (10): 669–670.Salvadori P.S., Torres U.S., D'Ippolito G. Contrastenhanced magnetic resonance cholangiography with gadoxetic-acid-disodium for the detection of biliary-cyst communication in Caroli disease. Gastroenterol. Hepatol. 2016; 39 (10): 669–670.

The authors declare that there are no conflicts of interest present.