See discussions, stats, and author profiles for this publication at: https://www.researchgate.

net/publication/12364603

Caroli syndrome

Article  in  Pediatric Surgery International · February 2000

DOI: 10.1007/s003839900323 · Source: PubMed

CITATIONS READS

19 33

2 authors, including:

M M Harjai
Armed Forces Medical College
141 PUBLICATIONS   453 CITATIONS   

SEE PROFILE

All content following this page was uploaded by M M Harjai on 13 November 2015.

The user has requested enhancement of the downloaded file.

Pediatr Surg Int (2000) 16: 431±432
CASE REPORT
Man Mohan Harjai á R. K. Bal
Caroli syndrome
Accepted: 17 May 1999
Abstract We describe a case of
Caroli syndrome (Caroli's disease
and congenital hepatic ®brosis) in a
10-year-old boy with bilobar in-
volvement and numerous renal cysts.
Liver transplantation o€ers the only
hope for such patients.
Key words Caroli syndrome á
Caroli's disease á Congenital hepatic
®brosis á Endoscopic retrograde
cholangiography á Intrahepatic
dilatation of biliary radicals
Introduction
Caroli syndrome (CS) consists of
Caroli's disease (CD) and congenital
hepatic ®brosis (CHF) [1]. It is an
uncommon congenital disorder of
the intrahepatic bile ducts that is
presumably of autosomal recessive
hereditary character. A classic case
of CS is presented. Non-surgical
management with an endoprosthesis
is discussed.
Case report
A 10-year-old boy was admitted with pain
in the upper abdomen, fever, vomiting, and
an abdominal mass of 2 12 months' duration.
M. M. Harjai (&) á R. K. Bal
Department of Surgery,
Armed Forces Medical College,
Pune-411 040,
India
There was no family history of hepatic
disease. His past medical history was un-
remarkable. On physical examination he
was poorly developed, with weight 20 kg
and height 128 cm. He was febrile (39 °C);
no icterus was present. There was upper-
abdominal fullness due to ®rm, nontender
hepatomegaly palpable 7 cm below the
right costal margin. Features of portal hy-
pertension in the form of ascites, spleno-
megaly, and distended abdominal-wall
veins were present.
Laboratory investigations revealed a
hemoglobin level of 6.2 g%, leucocyte
count 12,200/mm3, serum bilirubin 2.8 mg
%, alanine aminotransferase 21 IU/l,
aspartate aminotransferase 56 IU/l, alkaline
phosphatase 89 IU/l, total protein 8.5 g/dl,
albumin (A) 3.0 g/dl, globulin (G) 5.6 g/dl,
and A:G ratio 0.55:1. Hepatitis B serum
antigen was negative; serum amylase was 92
SU. Ultrasonography (US) revealed multi-
ple cystic dilatations in both lobes of the
liver and both kidneys (renal polycystosis).
Computed tomography (CT) (Fig. 1) and
Fig. 1 Computed tomography scan demon-
strating multiple cysts scattered throughout
liver, single large cyst in posterior-inferior
aspect of right lobe
Ó Springer-Verlag 2000
endoscopic retrograde cholangiography
(ERC) (Fig. 2) con®rmed the presence of
multiple communicating dilatations of the
bile ducts a€ecting the whole of the liver but
mainly the right lobe. Needle biopsy of the
liver revealed periductal ®brosis with sec-
ondary biliary cirrhosis.
Initial management consisted of naso-
gastric aspiration, broad-spectrum antibi-
otics, a blood transfusion, and other
supportive measures. Endoscopic papillo-
tomy and nasobiliary drainage (endopros-
thesis) was carried out to decompress the
biliary tree. The cholangitis resolved and
the patient is currently awaiting liver
transplantation (LT) due to deteriorating
hepatocellular function as a result of the
hepatic ®brosis.
Fig. 2 Endoscopic retrograde cholangiogra-
phy showing multiple communicating dilated
intrahepatic bile ducts, mainly in right lobe of
liver
432
Discussion
Suspicion of CD is often delayed
owing to its rarity. It is characterized
by ectasias of the intrahepatic bile
ducts without other abnormalities.
In CS, the congenital intrahepatic
ductal dilatation is also associated
with features of CHF and the renal
lesion of autosomal recessive poly-
cystic kidney [2]. CD and CS are
inherited in an autosomal-recessive
fashion. The etiology is unknown.
Ductal-plate malformation and in-
fantile obstructive cholangiopathy
theories have been postulated. Males
and females are equally a€ected.
The clinical features re¯ect re-
current bouts of cholangitis due to
bile stasis. The classic features of CS
are well-exempli®ed by our case. The
child has scattered intrahepatic di-
lated, communicating cysts with
features of CHF, poor liver reserve,
and portal hypertension in the form
of ascites, splenomegaly, and dis-
tended abdominal-wall veins. US,
CT, percutaneous transhepatic
cholangiography, or ERC patterns
are usually diagnostic of CS by
showing dilatation of the ®rst- and
second-order branches of the intra-
hepatic biliary ducts.
De®nitive therapy for this disease
is limited. Emergency surgery in the
presence of acute cholangitis in
adults, for whom ®gures are readily
available, is associated with high
View publication stats
mortality (20%±40%) and morbidi-
ty (44%±84%) [3]. Percutaneous
transhepatic biliary drainage for de-
compression has a complication rate
of 7%±40% [4]. Endoscopic sphinc-
terotomy and drainage procedures
are established modes of treatment
for acute cholangitis in adults with
high success rates and low morbidity
and mortality, but have seldom been
used in children [5]. An extensive
search of the available literature re-
vealed only a few case reports of
endoscopic stenting in pediatric pa-
tients.
In this case we performed endo-
scopic retrograde biliary drainage,
leaving the nasobiliary stent in the
right hepatic duct. The endopros-
thesis e€ectively reduced the stasis
and cholangitis and the patient's
condition improved. Hence, biliary
stenting is a safe and e€ective mode
of treatment for patients with CD
with severe cholangitis and deterio-
rating liver function who are await-
ing LT. Apart from biliary drainage,
a nasobiliary catheter o€ers other
advantages like allowing follow-up
cholangiograms and ¯ushing the
biliary tree to remove bile sludge.
The other modalities of treatment
include hepatic lobectomy for iso-
lated lobar disease. For panhepatic
disease, orthotopic LT is the only
therapeutic option.
CS should be included in the dif-
ferential diagnosis of chronic chole-
stasis of unknown cause. If recurrent
cholangitis occurs in spite of medical
therapy, the outlook is poor: a sig-
ni®cant number of these patients die
5 to 10 years after the appearance of
cholangitis [6]. A frequency of chol-
angiocarcinoma of 7% has been
reported as complicating this condi-
tion [7].
References
1. Caroli J (1973) Diseases of the intrahe-
patic biliary tree. Clin Gastroenterol 2:
147±161
2. Mall J, Ghahremani G, Boyer J (1974)
Caroli's disease associated with congen-
ital hepatic ®brosis and renal tubular
ectasia. Gastroenterology 46: 1029±1035
3. Magun A (1990) Acute cholangitis ±
endoscopic drainage or emergency sur-
gery. Gastroenterology 99: 1530±1531
4. Huang SM, Yu Sc, Tsang Ym, Wei Tc,
Hsu Sc, Chen KM (1989) Complication
of percutaneous transhepatic cholangi-
ography and biliary drainage. J Clin
Gastroenterol 96: 446±452
5. Chawla YK, Sharma BC, Dilawari JB
(1993) Endoscopic nasobiliary drainage
in acute suppurative cholangitis. Indian
J Gastroenterol 12: 97±98
6. Baker S, Beker B (1995) Cysts of the
liver. In: Haubrich W, Scha€ner F, Berk
JE (eds) Bockus gastroenterology, 5th
edn. Saunders, Philadelphia, pp 2400±
2401
7. Ludwig J, Wiesner RH, LaRusso NF
(1982) Focal dilatation of intrahepatic
bile ducts (Caroli's disease), cholangio-
carcinoma, and sclerosis of extrahepatic
bile ducts: a case report. J Clin Gastro-
enterol 4: 53±57