AIM: Intrabiliary rupture (IBR) is a common and serious compli-cation of hepatic hydatid cyst. The incidence varies from 1% to 25%. The treatment of IBR is still controversial. We aimed to design an algorithm for the treatment of hepatic hydatidosis with IBR by reviewing our cases.

METHODS: Eight cases of IBR were analyzed retrospectively. Patients were evaluated according to age, sex, clinical findings, cyst number and stage, abdominal ultrasonography and CT-scan, surgical methods, complica-tions, results and coincidental diseases.

RESULTS: Female/male ratio was 1/7. Mean age was 52.12±18.26 years (range 24-69 years). Right upper quadrant pain, flatulence, palpable hepatic mass were symptoms common in all patients. Cholestatic jaundice was found in four cases. In all patients, cyst evacuation and omento-plasty were performed, followed by either choledochod-uodenostomy, T-tube drainage, intracavitary suturing of the orifice, two cases in each. Whereas in two patients diagnosed post-operatively percutaneous drainage of biliary collection or ERCP and sphincteroplasty were added. Morbidity and hospital stay were higher in these cases.

CONCLUSION: When the diagnosis of IBR can be done pre-or intra-operatively, morbidity decreases. If a biliary fistula is seen post-operatively, endoscopic procedures such as ERCP, sphincteroplasty or nasobiliary drainage can be applied.

Intrabiliary rupture (IBR) is the most common and serious complication of hepatic hydatid cyst (HHC). It has also been reported as ‘cystobiliary fistula’ or ‘cystobiliary communication’ in the literature. The incidence varies from 1% to 25% although an incidence of 64.75% has been reported from a multicentric study in Tunisia[1-12].

Obstructive jaundice, fever, right-upper quadrant pain, nausea and vomiting, flatulence, palpable hepatic mass are the most commonly encountered symptoms in intrabiliary rupture[2,3,9].

Although there is some consensus on the medical and surgical treatment of hydatid cyst diseases, the treatment of intrabiliary rupture still remains controversial. Intracystic suturing of the orifice, T-tube drainage, double side drainage, cystobiliary disconnection, choledochoduodenostomy, choledochojejunostomy, endoscopic sphincterotomy and nasobiliary stent application have been used.

In this report, eight cases of intrabiliary rupture were presented and discussed in the light of literature review.

In this study, eight cases of hepatic hydatid cyst with cystobiliary fistulae treated by our team between 1994 and 2003 were presented. They began to undergo intra-operative ultrasonography (US) in 1997.

All patients’ records were reviewed and evaluated as to the age, sex, clinical findings, cyst number and stages according to Gharbi’s classification, abdominal ultrasono-graphy and CT-scan results, surgical methods, complications, results and coincidental diseases.

Intrabiliary rupture is a common and serious complication of hepatic hydatidosis. It occurs especially in centrally localized and high stage cyst. High intracystic pressure up to 80 cm H2O is a predisposing factor. The cyst rupture can occur in three clinical forms. Contained rupture occurs when the cyst contents are confined within the pericyst. Communicating rupture defines tearing of the pericyst and evacuation of cyst contents into the biliary tract or bronchioles. Direct rupture describes complete tear of the cyst wall and spillage of the cyst contents into the peritoneal or pleural cavity. Small cysto-biliary communications develop in 80-90% of all HHCs[16].

Furthermore, there are two different clinical settings associated with intrabiliary rupture: frank intrabiliary rupture and simple communication. In the former, the cyst content drains to biliary tract and causes cholestatic jaundice. In the latter simple communications are frequently overlooked and could cause biliary fistulae post-operatively[17]. If the cystobiliary opening was less than 5 mm, spontaneous drainage of the cystic content was uncommon and could be treated by suturing under the direct vision[18]. If the CBD diameter was larger than 5 mm, cystic content migration into the biliary tract would occur in 65% of the cases[19]. Vesicles, debris and purulent materials may be found in the biliary collection.

In all patients the most frequent symptoms were right upper quadrant pain and flatulence. Obstructive jaundice and fever have been recorded in 90% and 20% of the cases respectively. Nausea and vomiting were rare[2,3,9,20].

Diagnosis of IBR is difficult and can be established pre-, intra- and post-operatively. When obstructive jaundice is present, US, CT-scan, magnetic resonance imaging (MRI) and scintigraphic investigation can show the cyst and cystobiliary communications, but in patients with no jaundice, a correct diagnosis can be made in only 25%. Radiodiagnostic evaluation can also demonstrate cystic content in the gall bladder[7] and the CBD[1,4,19,21]. On the other hand, cholelit-hiasis and choledocholithiasis are common (81-61.53%) coincidental diseases[6,7]. Laboratory and serological tests can also be helpful for diagnosis.

If obstructive jaundice was not present and cystobiliary connection could not be seen pre-operatively, three findings should raise suspicion of cystobiliary fistulas, namely bile-stained cystic fluid, visualized and sutured bile leak orifice intra-operatively; intra-operative observation of CBD enlarg-ement or the presence of cyst content in the common bile duct; unexpected post-operative bile drainage from the cavity drains[8,22,23].

The orifice of bile leakage could be seen in 11.7-17.07% of the cases during the operation[3,23] while this was difficult in posteriorly localized cysts. In these cases, cholangiography could be done by a catheter pushed into the ductus cysticus or the cystobiliary fistula[3,4,8,22,23]. As an extreme procedure, puncture of choledochus and injection of radioopac solution or methylene blue are helpful to diagnose intrabiliary rupture or to see the orifice. Eleftheriadis[23] emphasized that choledochoscopy could also be helpful in the diagnosis of IBR.

When an intrabiliary rupture is diagnosed pre-or intra-operatively, there are alternative treatment modalities in addition to conventional surgery of hepatic hydatid cyst. Cystic evacuation, removal of germinative layer, disinfection of cystic cavity are necessary. If cystobiliary orifice is seen and no cystic content is observed in a normal caliber choledo-chus, suturing the orifice is sufficient to prevent the compl-ications. Videolaparoscopical suturing of the orifice has been reported in literature[8].

When cystic content is observed in a normal caliber choledochus, choledochotomy+evacuation of cystic content and debris from biliary tree±irrigation with 0.9% NaCl solution and T-tube drainage are enough. If CBD enlarge-ment is diagnosed with cystic content in it or in the gallbladder, choledochoduodenostomy is preferable. Some authors have reported the wide application of T-tube drainage in cases with high morbidity rates[3,4,6,7,9,22,24,25]. On the other hand, Roux and Y hepaticojejunostomy have been reported for the treatment of bile duct stricture associated intrabiliary rupture[20,26]. Open sphincteroplasty was also used in the last decades[25].

The presence of cholangitis has been reported as a risk factor. Even T-tube drainage itself could cause cholestatic icterus[3,17,27] and is also a source of infection[28]. Some auth-ors have used cystojejunostomy albeit the risk that cystic content drainage into the biliary tract could result in obstr-uctive jaundice and cholangitis[21,29].

When intrabiliary rupture was overlooked during the surgical treatment, biliary fistulae (up to 1 000 mL/d) were unavoidable and if this occurred ERCP would be nece-ssary. Post-operative biliary fistula rate was about 20% in all cases[22,23,30,31].

In the cases of overlooked cystobiliary fistulae, transsphincteric evacuation of the CBD and sphincteroplasty could be applied. Nasobiliary drainage could also be done. Usually, the majority of biliary fistulae could be closed in a few weeks[2,8,27,30,32].

The usage of endoprostheses in biliary fistula was not common. This can be considered in either high output bile leakage or for intractable fistulae[31].

The morbidity and mortality rates of all patients were 19.44-43.03% and 1.8-4.5% respectively in literature. The most common causes of deaths were sepsis and hepatic failure[3,4,9,22,23].

Hospital stay was the longest in the T-tube group. The patients undergoing choledochoduodenostomy had longer hospital stay than those undergoing simple orifice suturing[3].

In conclusion, IBR has an algorithm in the diagnosis and treatment. If it is not detected pre- or intra-operatively, a biliary fistula is common, its morbidity and mortality rates are high. Detecting and suturing orifices in cystic wall are the best methods of treatment. When cystic content is found in choledochus or when biliary fistula occurs, more complex procedures are necessary.