Global Advanced Research Journal of Medicine and Medical Sciences (GARJMMS) ISSN: 2315-5159
July 2017 Vol. 6(7), pp. 149-155
Copyright © 2017 Global Advanced Research Journals


Case Report 

Caroli’s disease: management of a rare condition during pregnancy

Nisa Félix* and Fátima Palma

Department of Maternal-Fetal Medicine, Dr. Alfredo da Costa Maternity, Lisbon, Portugal.

*Corresponding Author E-mail:

Accepted 17 July, 2017


Caroli’s disease is a rare congenital condition characterized by dilation of the intrahepatic biliary tree. There are about 200 cases described in the international literature and only three in pregnant women. In this article we describe a forth case dominated by the presence of severe cholestasis, with initial bile acids (BA) higher than 400μmol/L. We purposed a monitoring plan based on our knowledge about intrahepatic cholestasis of pregnancy (ICP), given its high prevalence in the obstetric population.

Keywords: Caroli’s disease, Caroli’s syndrome, cholestasis, bile acids, pregnancy 


List of Abbreviations

AF, alkaline phosphatase; BA, bile acids; CPAP, continuous positive airway pressure; CT, computed tomography; CTG, cardiotocography; ERCP, endoscopic cholangiopancreatography; GGT, gamma-glutamyl transpeptidase; H, hours; ICP, intrahepatic cholestasis of pregnancy; IM, intramuscular; MRCP, resonance cholangiopancreatography; NICU, neonatal intensive care unit; RDS, respiratory distress syndrome; UDCA, ursodeoxycholic acid; W, weeks



Adair C, Castillo R, Quinlan R, Ramos E, Gaudier F (1995). Caroli’s diseasecomplicatingpregnancy. Southern Med. J. 88:7.

Al Inizi S, Gupta R, Gale A (2006). Fetal tachyarrhythmia with atrial flutter in obstetric cholestasis. Int. J. Gynaecol. Obstet. 93: 53–54.

Ananthakrishnan A, Saeian K (2007). Caroli’s disease: identification and treatment strategy. Cur. Gastroenterol.  Reports. 9:151-155.

Bacq Y, Sentilhes L, Reyes HB, Glantz A, Kondrackiene J, Binder T, et al (2012). Efficacy of ursodeoxycholic acid in treating intrahepatic cholestasis of pregnancy: a meta-analysis. Gastroenterol. 143(6):1492-1501.

Beuers U (2010). Drug insight: mechanisms and sites of action of ursodeoxycholic acid in cholestasis in rat liver. Hepatol. 52:1758-1768.

Brites D (2002). Intrahepatic cholestasis of pregnancy: changes in maternal-fetal bile acid balance and improvement by ursodeox-ycholic acid. Ann. Hepatol. 1:20–8.

Brites D, El-Mir Y, Oliviera N, Marin JJG (1997). Amniotic fluid bile acid changes in the course of ursodeoxycholic acid therapy in intrahepatic cholestasis of pregnancy (abstract). J. Hepatol . 26:164A.

Brites D, El-Mir Y, Rodrigues CMP, van Zeller H, Marin JJG (1998). Bile acid composition of amniotic fluid and maternal serum in cholestasis of pregnancy and effect of ursodeoxycholic acid (abstract). J. Hepatol. 28:127A.

Brouwers L, Koster MPH, Page-Christiaens GCML, et al (2015). Intrahepatic cholestasis of pregnancy: maternal and fetal outcomes associated with elevated bile acid levels. Am. J. Obstet. Gynecol. 212:100.e1-7.

Campos GA, Guerra FA, Israel EJ (1986). Effects of cholic acid infusion in fetal lambs. Acta. Obstet. Gynecol. Scand. 65:23–26.

Geenes V, Chappell LC, Seed PT, Steer PJ, Knight M, Williamson C (2014). Association of severe intrahepatic cholestasis of pregnancy with adverse pregnancy outcomes: a prospective population-based case-control study. Hepatol. 59:1482-1491.

Germain A, Kato S, Carvajal J, Valenzuela G, Valdes G, Glasinovic J (2003). Bile acids increase response and expression of human myometrial oxytocin receptor. Am. J. Obstet. Gynecol . 189:577-582. 15.

Giovanardi O (2003). Monolobar Caroli’s disease in an adult. Case report. Hepatogastroenterol. 50: 2185-2187.

Glantz A, Marschall H, Mattsson L (2004). Intrahepatic cholestasis of pregnancy: relationship between bile acids level and fetal complication rates. Hepatol. 40:467-474.

Gorelik J, Harding SE, Shevchuk AI, et al (2002). Taurocholate induces changes in rat cardiomyocyte contraction and calcium dynamics. Clin. Sci. (Lond). 103:191-200.

Gorelik J, Shevchuk A, de Swiet M, Lab M, Korchev Y, Williamson C (2004). Comparison of the arrhythmogenic effects of tauro- and glycoconjugates of cholic acid in an in vitro study of rat cardiomyocytes. BJOG. 111:867-870.

Green–top Guideline No. 43. Obstetrics cholestasis. Royal college of obstetricians and gynecologists 2011.

Hussman K, Friedwald J, Gollub M, Melamed J (1991). Caroli’sdisease associated with infantile polycystic kidney disease.Prenatal sonographic appearance. J. Ultrasound Med. 10:235–237.

Israel E, Guzman M, Campos G (1986). Maximal response to oxytocin of the isolated myometrium from pregnant patients with intrahepatic cholestasis. Acta. Obstet. Gynecol. Scand. 65:581-582.

Jin J, Pan SL, Huang LP, Yu YH, Zhong M, Zhang GW (2015). Risk factors for adverse fetal outcomes among women with early- versus late-onset intrahepatic cholestasis of pregnancy. Int. J. Gynaecol. Obstet. 128: 236-240.

Kawakita T, Parikh LI, Ramsey PS, et al (2015). Predictors of adverse neonatal outcomes in intrahepatic cholestasis of pregnancy. Am. J. Obstet. Gynecol. 213:570.e1-8.

Laatikainen TJ (1975). Fetal bile acid levels in pregnancies compli-cated by maternal intrahepatic cholestasis. Am. J. Obstet. Gyne-col. 122:852–856.

Marschall HU, Wagner M, Zollner G, Fickert P, Diczfalusy U, Gumhold J, et al (2005). Complementary stimulation of hepatobili-ary transport and detoxification systems by rifampicin and ursodeoxycholic acid in humans. Gastroenterol. 129:476–485.

Puljic A, Kim E, Page J, et al (2015). The risk of infant and fetal death by each additional week of expectant management in intrahepatic cholestasis of pregnancy by gestational age. Am. J. Obstet. Gynecol. 212:667.e1–5.

Rioseco AJ, Ivankovic MB, Manzur A, et al (1994). Intrahepatic cholestasis of pregnancy: a retrospective case-control study of perinatal outcome. Am. J. Obstet. Gynecol. 170: 890-895.

Sato Y, Ren X, Nakanuma Y (2012). Caroli’s disease: current knowledge of its biliary pathogenesis obtained from an orthologous rat model. Int.  J. Hepatol. 107945.

Sepulveda WH, Gonzalez C, Cruz MA, Rudolph MI (1991). Vasoconstrictive effect of bile acids on isolated human placental chorionic veins. Eur. J. Obstet. Gynecol. Reprod. Biol. 42:211-215.

Serrano MA, Brites D, Larena MG, Monte MJ, Bravo MP , Oliveira N, et al (1998). Beneficial effect of ursodeoxycholic acid on alterations induced by cholestasis of pregnancy in bile acid transporta cross the human placenta. J. Hepatol. 28(5):829-839.

Sgro M, Rossetti S, Barozzino T, Toi A, Langer J, Harris P, Harvey E, Chitayat D (2004). Caroli’s disease: prenataldiagnosis, postnataloutcome na geneticanalysis. Obstet. Gynecol. 23: 73-76.

Shaw D, Frohlich J, Wittmann BA, Willms M (1982). A prospective study of 18 patients with cholestasis of pregnancy. Am. J. Obstet. Gynecol. 142:621–625.

Strhlow S, Pathak B, Goodwin T, Perez B, Ebrahimi M, Lee R (2010). The mechanical PR interval in fetuses of women with intrahepatic cholestasis of pregnancy. Am. J. Obstet. Gynecol. 203:455.e1-5.

Suchy F (2013). Caroli’s disease. Uptodate.

Tsunoda M, Ohba T, Uchino K, Katabuchi H, Okamura H, Kaneki S (2008). Pregnancy complicated by Caroli’s disease with polycystic kidney disease: A case report and following observations. J. Obstet. Gynaecol. 34:599-602.

Williamson C, Geenes V (2014). Intrahepatic cholestasis of pregancy. Obstet. Gynecol. 124:120-133.

Williamson C, Gorelik J, Eaton BM, Lab M, de Swiet M, Korchev Y (2001). The bile acid taurocholate impairs rat cardiomyocyte function: a proposed mechanism for intra-uterine foetal death in obstetric cholestasis. Clin. Sci. Lond. 100:363-369.

Williamson C, Hems LM, Goulis DG, et al (2004). Clinical outcome in a series of cases of obstetric cholestasis identified via a patient support group. BJOG. 111:676-681.

Yu L, Ding Y, Huang T, Huang X (2014). Effect of bile acid on fetal lung in rat model of intrahepatic cholestasis of pregnancy. Int.  J.  Endocrinol. 308274.

Yuksel A, Has R, Isikoglu M, Suoglu O (2002). Prenataldiagnosisof Caroli’s syndrome. Ultrasound Obstet. Gynecol. 19:525– 530.

Zecca E, Costa S, Lauriola V, Vento G, Papacci P, Romagnoli C (2004). Bile acid pneumonia: a “new” form of neonatal respiratory distress syndrome? Pediatrics. 114:269–272.

Zecca E, Luca D, Marras M, Caruso A, Bernardini T (2006). Intrahepatic cholestasis of pregnancy and neonatal respiratory distress syndrome. Pediatrics. 117:1669-1672.

Zhang D, Ji Z, Sheng X, Liu H, Pan B, Dong L (2012). Caroli’s disease: a report of 14 patients and review of the literature. J. Digest. Dis. 13:491-495.

Zhou L, Qui H, Luo X (2013). Analysis of clinical characteristics and perinatal outcomes of early onset intrahepatic cholestasis of pregnancy. Zhanghua Fu Chen KeZaZhi.  48:20-24.

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