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Year : 2016  |  Volume : 5  |  Issue : 2  |  Page : 155-157

A child with hypertension and ascitis

1 Department of Pediatric, Pediatric Hepatobiliary Clinic, Mumbai, Maharashtra, India
2 Department of Pediatric Surgery, Pediatric Hepatobiliary Clinic, B J Wadia Hospital for Children, Mumbai, Maharashtra, India

Date of Web Publication5-Jul-2016

Correspondence Address:
Ira Shah
1/B Saguna, 271/B St. Francis Road, Vile Parle (W), Mumbai - 400 056, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-8632.185456

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Caroli disease is a rare disorder characterized by congenital dilatation of the intrahepatic biliary tree and may be associated with polycystic kidney disease. Patients usually present with intermittent abdominal pain, hepatomegaly, and recurrent cholangitis. Renal failure as a presenting complaint of Caroli disease is rare. We present a child with Caroli disease who presented to us with renal failure.

Keywords: Caroli disease, cholangitis, hypertension, polycystic kidney disease, renal failure

How to cite this article:
Shah I, Bhatnagar S. A child with hypertension and ascitis. J NTR Univ Health Sci 2016;5:155-7

How to cite this URL:
Shah I, Bhatnagar S. A child with hypertension and ascitis. J NTR Univ Health Sci [serial online] 2016 [cited 2020 May 25];5:155-7. Available from: http://www.jdrntruhs.org/text.asp?2016/5/2/155/185456

  Introduction Top

Caroli disease is a nonobstructive dilatation of the intrahepatic bile ducts. This is a rare congenital disorder that classically causes saccular ductal dilatation that usually is segmental. There are two forms of Caroli disease, one form associated with congenital hepatic fibrosis and a simpler form occurring alone. The former, called Caroli syndrome, is associated with portal hypertension. The latter, known as Caroli disease, may be associated with autosomal recessive polycystic kidney disease or rarely with autosomal dominant polycystic kidney disease. [1],[2] We present a child with Caroli disease and renal failure.

  Case report Top

A 5-year-old boy born of nonconsanguineous marriage presented with fever, epistaxis, and breathlessness, 10 days ago, for which he was admitted in another hospital and treated with intravenous antibiotics and blood transfusion. He was referred for further management. His birth history was normal. He was unimmunised and milestones were normal. Family history was noncontributory. On examination, he was in respiratory distress (heart rate = 130/min and respiratory rate = 60/min) with hypertension [Blood pressure (BP) = 170/110 mmHg]. He was malnourished (Height = 96 cm, Weight = 10 kg, <5 th centile), had pallor, and had generalized edema. There were no dysmorphic features or signs of rickets. Systemic examination revealed hepatosplenomegaly with ascites. Other systems were normal. Investigations showed hemoglobin of 6.9 g/dL with white blood cell count of 11,400/cumm and platelet count of 1,35,000/cumm. He had hyperkalemia (serum potassium = 5.9 mmoL/L) with normal serum sodium. His blood urea nitrogen was 101 mg% and serum creatinine was 6.6 mg/dL. He had hypocalcemia (serum calcium = 6.8 mg/dL) and hyperphosphatemia (serum phosphorus = 10 mg%) with normal alkaline phosphatase. Venous blood gas showed metabolic acidosis (pH = 6.91 and bicarbonate = 1.5 mmoL/L). His liver function tests were normal [bilirubin = 0.6 mg/dL, serum glutamic oxaloacetic transaminase (SGOT) = 44 IU/L, serum glutamic pyruvic transaminase (SGPT) = 31 IU/L, total proteins = 6.3 g/dL, and serum albumin = 3.6 g/dL]. Ultrasound of the abdomen showed multiple cysts in the liver with bilateral hydronephrosis and hydroureter with splenomegaly and multiple collaterals. Computed tomography (CT) of the abdomen showed central intrahepatic cysts with dilated biliary radicles with bilateral polycystic kidneys [Figure 1] and [Figure 2]. Esophagogastroscopy was normal. Ultrasound of kidneys of the parents were normal. He was thus diagnosed as a Caroli disease. He was treated with bicarbonate infusion, calcium supplements blood transfusion, and antihypertensives with potassium binders but he continued to have hyperkalemia and his creatinine increased to 8.3 mg/dL. He was advised dialysis and renal transplant, which the parents refused. The child took discharge against medical advice.
Figure 1: T1 weighted image of MRI of the abdomen shows central intrahepatic cysts with dilated biliary radicles

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Figure 2: T1 weighted image of MRI of the abdomen shows cysts in the kidney

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  Discussion Top

Caroli disease is a rare bile duct abnormality characterized by congenital dilatations of the intrahepatic bile ducts concerning a segment, a lobe, or both lobes of the liver. About 155 cases have been reported in literature. [3] Stenosis and dilatation cause initial biliary stasis leading to intrahepatic biliary lithiasis and secondary infection. Thus, most patients present with recurrent biliary infections such as cholangitis, septicemia, and intrahepatic and subphrenic abscesses. [3] It may be complicated by portal hypertension. [4] Usually, renal complication may be seen in infants with enlarged cystic kidneys and progressive renal failure or patients may have normal-appearing kidneys with minimal renal dysfunction. In our patient, imaging showed bilateral polycystic kidneys and he had renal failure at 5 years of age. [5] Additionally, because ultrasound of kidneys of the parents was normal, it was suggestive of autosomal recessive inheritance. Complications of Caroli disease are cholelithiasis, polycystic kidney disease, cirrhosis, and cholangiocarcinoma. Stone passage may cause pancreatitis. In fact, cholangiocarcinoma can occur in approximately 7% of cases and is due to prolonged exposure of the ductal epithelium to high concentration of unconjugated secondary bile acids. [6],[7]

Diagnosis of Caroli disease is based on imaging and by demonstrating the cystic lesions in continuation with the biliary tree. Irregular cystic dilatation of the large proximal intrahepatic bile ducts with normal ducts in between is seen. [8] In our patient, both ultrasound as well as CT abdomen showed dilated intrahepatic ducts.

Treatment of Caroli disease consists of antibiotics for cholangitis and ursodeoxycholic acid for intrahepatic cholelithiasis. [9] Surgical intervention for the removal of stones may be required and bile flow can be maintained by hepaticojejunostomy. In cases of localized stasis, lobectomy may decrease the risk of cholangiocarcinoma and for refractory cholangitis, liver transplantation may be considered. [10] For renal failure due to polycystic kidney disease, renal transplantation is the treatment of choice.

Practice points

  • Caroli disease should be suspected in a child with liver and renal disease.
  • Though cholangitis and portal hypertension are common clinical features, renal failure may also be seen.
  • Diagnosis is based on imaging and demonstrating the cystic lesions in liver.
  • Treatment consists of antibiotics for cholangitis and ursodeoxycholic acid for intrahepatic cholelithiasis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Bersnstein J, Slovio TI. Polycystic diseases of the kidney. In: Edelmann CM Jr, editor. Pediatric Kidney Disease. Vol. 2. Boston: Little Brown; 1992. p. 1139-53.  Back to cited text no. 1
Jordan D, Harpaz N, Thung SN. Caroli′s disease and adult polycystic kidney disease: A rarely recognized association. Liver 1989;9:30-5.  Back to cited text no. 2
Madjov R, Chervekov P, Madjova V, Balev B. Caroli′s disease. Report of 5 cases and review of literature. Hepatogastroenterology 2005;52:606-9.  Back to cited text no. 3
Senyüz OF, Yesildag E, Kuruoglu S, Yeker Y, Emir H. Caroli′s disease in children: Is it commonly misdiagnosed? Acta Paediatr 2005;94:117-20.  Back to cited text no. 4
Friedman J. Caroli disease. Available from: . [Last accessed on 2005 Dec 12].  Back to cited text no. 5
Bismuth H, Krissat J. Choledochal cystic malignancies. Ann Oncol 1999;10(Suppl 4):94-8.  Back to cited text no. 6
Lowenfels AB. Does bile promote extra-colonic cancer? Lancet 1978;2:239-41.  Back to cited text no. 7
Karim AS. Caroli′s disease. Indian Pediatr 2004;41:848-50.  Back to cited text no. 8
Ros E, Navarro S, Bru C, Gilabert R, Bianchi L, Bruguera M. Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli′s syndrome. Lancet 1993;342:404-6.  Back to cited text no. 9
Jonas MM, Perez-Atayde AR. Fibrocystic liver disease. In: Suchy FJ, Sokol RJ, Balistreri WF, editors. Liver Disease in Children. 2 nd ed. Philadelphia: Lippincott Williams & Wilkins; 2001. p. 904-5.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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