Case report zyxwvu

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Br. J. Surg. 1985, Vol. 72, August, 6 5 1 4 5 2 Discussion
Involvement of the intrahepatic bile ducts in Caroli’s disease

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Surgical management of Caroli’s may be partial or total, with limited forms of the disease often
confined to the left side of the liver. Congenital cystic dilatation
disease involving both lobes of of the bile ducts may be associated with fibrocholangiomatosis
and with nephrospongiosis (Cacchi-Ricci disease). Symptoms
the liver

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and signs are due to biliary stasis, intraductal lithiasis and
infection. Symptoms can usually be traced back to childhood.
The most notable complications of Caroli’s disease are
P. Aeberhard cholangitis, Gram-negative sepsis, liver abscess and subphrenic
abscess. Amyloidosis and cholangiocarcinoma may occur late in
Department of Surgery, Kantonsspital CH-5000Aarau,
Switzerland

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Correspondence to: Professor P. Aeberhard

Congenital cystic dilatation of intrahepatic bile ducts‘ is a rare

disease and until recently only about 100 cases had been
reported in the literature’ -3. Its prevalence has probably been
underestimated in the past, as many cases must have remained
undiagnosed due to the lack of appropriate diagnostic methods.
The introduction of new imaging techniques such as ultrasound,
CT scan and endoscopic retrograde cholangiography has
increased the probability of diagnosing Caroli’s disease,
resulting in more case reports and a growing index of suspicion
when patients present with a history of recurrent episodes of
right upper quadrant pain, fever, and j a ~ n d i c e ~The
- ~ . author
presents a case of Caroli’s disease involving the whole of the left
lobe, the caudate lobe, and the posterior segment of the right
lobe (Couinaud‘s segments 6 and 7) and discusses the problems
of management.

Case report
The patient was a 28-year-old Portugese woman who had suffered bouts

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of cholangitis from the age of eight years. She was referred for
diagnosis and further treatment when cholangitis recurred following
cholecystectomy for presumed acalculous cholecystitis. CT scan and
endoscopic retrograde cholangiography demonstrated cystic dilatation
of intrahepatic bile ducts in the whole of the left lobe of the liver, the
caudate lobe. and the Dosterior segment of the right lobe (segments 6 and
7). The dilated ducts’were obstructed by hun&eds of deiselY Packed Figure 1 Retrograde cholangiogram showing normal bile ducts in the
stones (Figures 1-3). anterior segment of the right lobe (Couinaud’s segments 5 and 8). The
At operation we first performed a left hepatic IobectomY including
posterior segmental ducts are partially visualized, showing cystic
the caudate lobe. The opening of the obstructed duct of the Posterior djlatatjon and multiple stones. Ducts in the left lobe have not been
segment of the right lobe was too narrow to permit disimpaction of
rjsualized
stones. Therefore, the stone-filled msterior segmental ducts were
approached through a transparenchymal incision into a superficially
located part of the ductal system of segment 6, but the stones in segment
7 could not be mobilized. To deal with the remaining stones later,
biliary-enteric anastomosis to a Roux-en-Y jejunal loop at the hilum of
the liver was complemented by anastomosis of the peripheral part of
segmental duct 6 to the same loop. A catheter was introduced into the
Roux-en-Y limb through a Witzel tunnel and advanced through the
peripheral hepaticojejunostomy into the dilated duct of segment 6. The
peripheral end of the catheter was brought out through a stab incision
and secured to the skin (Figure 4). Two weeks later the catheter was
replaced by a guide wire, and a fibreoptic choledochoscope was
introduced over the wire. Most of the stones remaining in segments 6 and
7 could then be removed endoscopically using irrigation, a Dormia
basket and a Fogarty catheter. One week later some residual stones were
extracted using the same method. The patient made an uneventful
recovery. Follow-up is now 13 months. She has had no further episodes
of fever and jaundice.
99mTcHIDA sequential biliary scintigraphy performed one year
after the operation showed normal elimination of the
radiopharmaceutical from all areas of the remaining right liver lobe.
Elimination was through the anastomosis at the hilum of the liver,
whereas the peripheral anastomosis, which was shown widely open and
functional at four months after the operation, no longer contributed Figure 2 CTscun showing cystic dilatation ofintrahepatic bile ducts,

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significantly to the drainage of the abnormal bile ducts. with masses of partially calcified stones in the left lobe

0007-1323/85/080651-02%3.00 8 1985 Butterworth & Co (Publishers) Ltd 651

Case report zyxwvutsrqpon
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Figure 3 Dragram of involved and unaffected parts of the intrahepatic
biliary ductal system: only the right anterior segmental duets are normal
the course of the disease. Caroli’s disease associated with
fibrocholangiomatosis presents in the child as congenital
cirrhosis of the liver and carries a most unfavourable prognosis.
In pure Caroli’s disease the prognosis depends upon the
extent of the lesions. Localized forms may be cured by surgezy.
Caroli’s disease confined to one lobe of the liver can be cured by
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hepatic resection’ -‘I. Cases with total ductal involvement pose
a much more difficult therapeutic challenge which may be met
by using Praderi’s method of disimpaction and permanent
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percutaneous transhepatic intubation of the ducts of both halves
of the liver and hapaticojejunal MouielI4 has
recommended broad exposure of the intrahepatic lobar and
segmental ducts by resection of the anterior part of segment 4.
Complete disimpaction of stones requires multiple peripheral
hepaticotomies, and when all stones are removed, the
transparenchymal hepaticotomies are suture-closed with the
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opening in the main hepatic duct bifurcation anastomosed to a
Roux-en-Y loop. Mercadier has combined left-sided resection
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with disimpaction of stones in the right half of the liver and
Roux-en-Y hepaticojejunal anastomosis”.
The case reported in the present paper is unique in that only
the anterior segment of the right liver lobe (segments 5 and 8)
was normal. Left hepatic lobectomy including the caudate lobe
was straightforward. Clearing of the ducts of segments 6 and 7
had to be completed by repeated percutaneous instrumentation
through a peripheral hepaticojejunostomy. Biliary-enteric
anastomosis is considered essential for the prevention of
recurrent obstruction when resection has not been feasible.
Anastomosis is to the hepatic duct birfurcation, the main hepatic
or common duct or to the remaining hepatic duct following
lobectomy. Additional peripheral anastomosis affords access for
removal of residual stones by percutaneous instrumentation and
may offer enhanced protection from recurrent stone formation
and obstruction. A method of Roux-en-Y hepaticojejunostomy
in which the closed end of the loop is buried subcutaneously for
ready access in case of recurrent stone formation has been
published by Fag Kan and Chou Tsoung16 but was unknown to
this author when the patient reported in the present paper was
operated upon. Sequential biliary-scintigraphy is an elegant and
non-invasive method of follow-up after biliary-enteric
anastomotic procedures.
References
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4. Clemens M, Jost JO, Kautz G, Schwering H, Kessler B, Galansky
652
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Figure 4 a Concept of surgical management: resection of left lobe
including caudate lobe (segments 1-4); segments 5 and 8 left untouched;
disimpaction of stones in segments 6 and 7; b Completed operation:
central and peripheral (segment 6 )hepaticojejunostomy to the same Roux-
en- Y loop, percutaneous catheter .for later endoscopic access to patho-
logical posterior segments
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Paper accepted 19 March 1985
Br. J. Surg., Vol. 72, No. 8, August 1985