Introduction

Hydatidosis is a zoonosis that is generally caused by infection with Echinococcus granulosus. The disease is endemic in many countries, such as those around the Mediterranean Sea, as well as in Central and Eastern Asia and Latin America (1). The liver is the most common site of hydatid cysts (1, 2). Rupture of a hydatid cyst into the biliary tract is common and is manifested as obstructive jaundice or cholangitis (1, 2). In case of intrabiliary rupture, endoscopic retrograde cholangiography (ERC) with endoscopic sphincterotomy or bile duct stenting achieves decompression of the biliary tract from intracystic debris and prevents the recurrence of obstructive jaundice. We herein describe a case of hepatic hydatid obstructive jaundice after hepatectomy.

Case Report

A 79-year-old woman with jaundice and right hypochondralgia was referred to our

hospital in January 2008. Right hepatectomy was performed in April 2003 because of

hepatic hydatid disease, and the margin was found to be positive for hydatid cysts. Her

height and weight were 151 cm and 42 kg, respectively; she had been treated with

albendazole.

Physical examination revealed right hypochondralgia and jaundice; her body

temperature was 37.6 ℃. Her white blood cell count was 12,900 /μL, and the levels of

C-reactive protein, alkaline phosphatase, and total bilirubin were 12.4 mg/dL, 1,135

U/L, and 18.5 mg/dL, respectively. Abdominal ultrasonography showed a dilated

intrahepatic bile duct, and abdominal computed tomography (CT) showed lesions with

calcifications and a dilated left intrahepatic bile duct (Fig. 1). Magnetic resonance

cholangiopancreatography revealed the obstruction of a 2 cm long bile duct (Fig. 2). A

diagnosis of acute cholangitis with obstructive jaundice was made, and intrabiliary

rupture of a relapsing hydatid cyst was suspected to be the cause of this condition.

ERC (Fig. 3A, B) and bile duct stenting (Cotton-HuibregtseⓇ Biliary Stent Sets;

Wilson-Cook Medical, Inc., NC, USA) were performed on January 22, 2008 (Fig.

4A-D), and the intracystic fluid and debris drained to the bile duct stent. Two months

after bile duct stenting, the level of total bilirubin was 1.5 mg/dL, and no dilated

intrahepatic ducts were observed on abdominal CT scans (Fig. 5). The patient was

discharged in March 2008; however, she was referred to our hospital again in July 2008

with jaundice and right hypochondralgia probably because of bile duct stent occlusion.

The patient was admitted to our hospital 5 times during a period of 3 years, and bile

duct stenting was performed 7 times, while endoscopic naso-biliary drainage was

performed once. The last bile duct stenting (plastic stent; 8.5Fr, 12 cm) was performed

in September 2010, and she survived for a period of 7 years and 8 months after right

hepatectomy.

Figure 1

Figure 1: Abdominal computed tomography (CT) showed a dilated intrahepatic duct and lesions with calcifications ().

Figure 2

Figure 2: Magnetic resonance cholangiopancreatography showed dilated intrahepatic ducts and obstruction of a 2 cm long bile duct ().

Figure 3

Figure 3: Endoscopic retrograde cholangiography (ERC) showed bile duct obstruction (A) and bile duct stenting (plastic stent; 8.5Fr, 12 cm; B) ().

Figure 4

Figure 4: Bile duct stenting. ERC was performed for cannulation into an orifice of Vater’s papilla (A), exchange of a guidewire (B), and insertion of a bile duct stent (C, D).

Figure 5

Figure 5: Abdominal CT showed the bile duct stent () and disappearance of the dilated intrahepatic ducts.

Figure 6

Table 1. Characteristics of the patients with hepatic hydatid obstructive jaundice who underwent endoscopic bile duct stentings from 1983 to 2010 based on the Japanese medical database

Discussion

Intrabiliary rupture of a hepatic hydatid cyst is a common complication and may

occur in 2 forms: an occult rupture, in which only the cystic fluid drains to the biliary

tree and is observed in 10-37% of the patients; and frank rupture, which has an overt

passage of intracystic material to the biliary tract and is observed in 3-17% of the

patients (3, 4). Intrabiliary rupture mainly occurs in centrally localized cysts, and an

intracystic water pressure up to 80 cm is also a predisposing factor for the rupture (5).

Intrabiliary rupture occurs in the right hepatic duct (55-60% cases), left hepatic duct

(25-30% cases), hepatic duct junction, common bile duct (CBD), or cystic duct

(8-11%); perforation into the gallbladder may be observed in 5-6% of cases (6-8). It was

thought that the present case was an occult rupture, because the intracystic fluid and

debris drained to the bile duct stent.

Intrabiliary ruptures of hydatid cysts have been diagnosed by imaging and

laboratory tests. Although these tests were proven to be ineffective in detecting

occult ruptures, certain findings from studies conducted in the USA provide essential

clues for the diagnosis of frank ruptures with obstruction. Echogenic material, without

posterior acoustic shadowing in extrabiliary ducts, implied the presence of intracystic

material (9). An abdominal CT scan may reveal a dilated CBD with low attenuation

intraluminal material, suggesting the presence of hydatid sand and cysts (10, 11).

Some cases of definitive endoscopic treatment of obstructive jaundice after

intrabiliary rupture have been reported; ERC has become the “gold standard” for the

assessment of intrabiliary rupture by achieving a detection rate of 86.6% to 100% (3,

12-15). ERC, which is a minimally invasive procedure, may obviate reoperation.

Endoscopy is a modality serving both diagnostic and therapeutic aims. Postoperative

ERC has several advantages: I-clarification of the causes of ongoing or recurrent

symptoms and laboratory abnormalities; II-resolution of obstructions or cholangitis due

to residual material in biliary ducts; III-management of postoperative external biliary

fistulae; and IV-provision of a realistic solution for secondary biliary strictures (12, 13,

16, 17).

ERC with bile duct stenting is performed for the treatment of intrabiliary ruptures

associated with obstructive jaundice, but this is rare in Japan. Ichushi Web, a Japanese

medical database, showed that only 4 Japanese cases, including the present case, were

reported from 1983 to 2010 (Table 1). In all cases, bile duct stenting was repeatedly

performed because of stent occlusion, but major complications were not encountered

after endoscopic procedures.

In conclusion, therapeutic endoscopy is a safe and valuable procedure for the

postoperative management of patients with hepatic hydatid disease.