intrabiliary rupture of liver hydatid cysts - MedIND

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INTRABILIARY RUPTURE OF LIVER HYDATID CYSTS: DIAGNOSTIC ACCURACY OF ENDOSCOPIC ULTRASOUND AND TREATMENT BY ENDOTHERAPY Abid Shoukat, Malay Sharma, Vijendra Kirnake, Piyush Somani

Abid Shoukat, MD, DM,Vijendra Kimake,Piyush Smani :Department of Gastroenterology; Malay Sharma MD,DM (Gastroenterologist) ,Director , Jaswant Rai Speciality Hospital, Saket, Meerut, PIN- 250001, India Received Accepted

August November

2015 2015

Correspondence:-Dr.Abid Shoukat Department of Gastroenterology,JaswantRaiSpeciality Hospital, Saket, Meerut, PIN- 250001, India . Email: [email protected]

Abstract Intrabiliary rupture (IBR) is a serious and common complication of liver hydatid cyst (LHC). Different imaging modalities have been used to diagnose IBR of LHC with varied success rates. We evaluated whether imaging features on endoultrasound (EUS) are characteristic enough for its diagnosis. The role of ERCP in its treatment is also described. Between 2004–2012,77 LHC were seen by us; of them 5 (3 males, mean age 33 ± 10.2 years) had IBR of LHC. Cysts were in right (N=4) and left lobe in (N=1) with mean size of 7.5 ± 1.9 cm. Abdominal ultrasound (US) missed diagnosis in 2 cases. Clinical presentations were: cholangitis, 4; pyogenic cholangitis, 1 and pancreatitis, 1. EUS findings were: visible communication between biliary tree and cyst, (4); deformation or reduction in cyst size, (4); floating membranes in cyst, (3); ill-defined, irregular or leaf-like structures in the bile duct, (5); with changing their shapes in (3); dilatation (5) and extrinsic compression (2) of bile duct. Four of 5 patients were treated successfully with combination of endoscopic sphincterotomy, and clearance of the duct and cyst material with basket and balloon, followed by saline irrigation via nasobiliary-cystic drain.One required surgery. EUS should to be used early in the diagnostic work-up IBR of LHC when US is equivocal or inconclusive. The presence of cysto-biliary communication, and /or hydatid debris with their changing shape in the biliary tract in presence of LHC on EUS is diagnostic of IBR. Keywords:

Endoscopic Ultrasound, ERCP, Intrabiliary rupture of hydatid cyst. JK-Practitioner2015;20(3-4):7-13 Introduction Hydatid disease is a public health problem worldwide. Itis endemic in areas where dogs are used to help raise livestock. It is caused by the parasite Echinococcusgranulosus and predominantly involves the liver in 70% of cases1. Biliary rupture of LHC is the most common complication of LHC and is of two types – occult and frank. Whereas the

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occult communication is usually asymptomatic but frank rupture is a serious complication and occurs in 5% - 15% of cases 2 Although, liver hydatid cyst (LHC) is usually asymptomatic and diagnosed accidentally, the intrabiliary rupture (IBR) is a serious complication presenting with abdominal pain, cholangitis, cholestasis, and pancreatitis. Although diagnosis can be suspected in presence of clinical features,

JK-Practitioner Vol.20, No (3-4) July - December 2015

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imaging is essential for prompt detection of cyst and localization of rupture, and early treatment to avoid fatal complications 2, 35 .The intrabiliary rupture of hepatic hydatidosis (IBRH) is a serious complication of LHC with morbidity and mortality of 19-43% and 1.8-4.5% respectively 6. Imaging tools like abdominal ultrasonography (US), computed tomography [CT) and magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) are useful for preoperative diagnosis of IBRLHC but they lack high sensitivity and specificity 2,3,5,7. The endoscopic retrograde cholangiography (ERC)has higher sensitivity than US, CT and MR imaging for preoperative diagnosis of IBR-LHC and can demonstrate the communication in more detail but is invasive and associated with complications. However, ERC in conjunction with sphincterotomy permits clearance of hydatid materials from obstructed biliary tree and plays a temporizing role in stabilizing sick patient so that definitive procedure can be performed on electivebasis 6,8-10,11. Endoscopic ultrasound (EUS) is rapidly replacing diagnostic ERC in the diagnosis of biliary tract disorders. EUS has a higher sensitivity for visualization of biliary tree than other imaging modalities and should have a promising role to play in clinching diagnosis of IBRH. A meta-analysis compared diagnostic accuracy of EUS with US, ERCP/MRCP and CT in patients with biliary obstruction, reported the sensitivity of 85%-91% and specificity of 87%-93% which is at par with ERCP and highly superior to US and CT 11,12. EUS features of IBRHhave been described in case reports. Hence, the present study aims to assess the role of EUS in the diagnosis of IBRH and to study as to whether imaging features of the biliary abnormalities seen at EUS are characteristic enough for the diagnosis of IBR-LHC. Furthermore, the role of ERCP

in the management of IBR-LHC will also be described. Material & Methods: Study Group Between 2004 and 2012, a total of 77 patients with liver hydatid diseases were diagnosed based on the findings of serology and abdominal US; of them five had suspicion of biliary involvement on clinical and laboratory findings. Routine investigations included hemogram, serum biochemistry, coagulogram, hydatid serology, US and others. Thereafter all cases underwent EUS with Pentax scope (Pentax 3630 UR and 3870 UTK, Pentax Corp., Tokyo, Japan). After initial stabilization with IV fluids, antibiotics, analgesics, and vitamin K, ERCP was performed under iv propofol. After confirmation of IBRH, endoscopic sphincterotomy was done with clearance of biliary tree and cyst cavity followed by placement of endoscopic nasobiliary drain (ENBD) into the cyst. The EUS diagnosis of IBR of LHC was based upon demonstration of the LHC together with intrabiliaryhydatid contents (vesicles, membranes, and sand) and/or visible communication between LHC and biliary tree. The cholangiograms available at ERCP were used the reference standards for comparison with EUS findings. The diagnosis was confirmed by ERCP and/or surgery Statistical Analysis: Summary statistics for quantitative data were expressed as mean ± standard deviation. All statistical analysis was performed with the statistical package for social sciences(SPSS 10; Chicago, IL) software program. Results Patient characteristics and clinical features (Table 1) There were 3 males and 2 females with


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original article

Table 1: Patient characteristics Case 1

Case 2

Case 3

Case 4

Case 5

Age in yrs

35

22

37

24

47

Sex

Female

Male

Male

Female

Male

Presentation

Cholangitis

Cholangitis

Cholangitis

Cholangitis

Pyogenic

Pancreatitis

cholangitis

Cyst size, cm

7.2

6.5

5.5

7.8

10.3

TLC cells/µL

13300

12210

11450

14325

13270

Bilirubin,

3.6

3.1

5.4

6.5

7.4

ALT, IU/L

89

111

81

78

122

ALP, IU/L

330

546

455

685

534

mg/dL

Abbreviations used: RL, right lobe; LL, left lobe; TLC, total leucocyte count; ALT, serum alanine transferase; ALP, serum alkaline phosphatase; mg/dl (milligram/decilitre) Table 2: Endo-ultrasonographic features Endo-ultrasonographic features

No of cases

Site of cyst – right/left

4/1

Gharbi cyst type – 1/2/3/5

3/1/1

Size of cyst – mean (range) cm

7.5 ± 1.9 (5.5 – 10.3)

Visible communication between biliary

5

tree and cyst seen as a rent in cyst cavity Deformation or reduction in cyst size

4

Floating membranes in cyst

3

Ill-defined, oval or nodular anechoic

5

defects or irregular or leaf -like echogenic structures in the bile duct without posterior shadowing echogenic structures in the bile duct with

3

changing shape Beak-like projection

3

Dilated common bile

5/5/1

duct/intrahepatic/pancreatic ducts

9

Distension of gallbladder

1

Extrinsic compression of bile duct

2

mean age of 33 ± 10.2years (range 22–47 years). Three patients were known to have hepatic hydatidosis and 1 had a previous history of surgery for hydatid cyst. Single cysts were detected in 4 cases and multiple in 1. Symptoms included abdominal pain (5 patients); fever(4) and jaundice (4). The patient characteristics are described in Table 1. Imaging findings US showed (i) LHC (5 patients); (ii) dilated intrahepatic duct (4), and common bile duct (CBD) (3); (iii)site of cysto-biliary communications (2); (iv) filling defects of varying sizes shapes in the CBD without acoustic findings (3); and distended gallbladder (1). The CBD was normal in 1 and could not be seen in 1 patient. The EUS findings are summarized in Table 2. The CBD was dilated ranging from 9 mm – 15 mm (N=5) and compressed extrinsically in 2 cases. The filling defects in the CBD represented hydatid material such as daughter vesicles, membranes and sand (N=5) which characteristically changed shape and size in 3 cases and without posterior acoustic shadows. Treatment Treatment and success After initial stabilization, all patients underwent emergency ERCP. Bile stained hydatid material was seen extruding at the ampullary orifice in 2 patients (Table 3). The CBD was dilated containing hydatid membranes in all the patients. The cholangiograms revealed the communication between the cyst and the biliary tree in 5 patients.The endoscopic sphincterotomy was done followed by removal of daughter cysts, hydatid membranes and sand with occlusion balloon and Dormia basket within biliary tree and cyst cavity followed by placement of endoscopic naso-biliary drain (ENBD) into the cyst cavity with or without stenting. Patients were discharged after they had recovered with ENBD in situ. Patients were advised to take albendazole 10/kg mg daily


original article

and flush the NBD tube with 50 ml of saline twice daily. A repeat ERCP was performed 3 weekly till cure was achieved. 2 patients achieved full cure after 2 sessions of ERCP while 2 recovered after the third session. The ERCP findings of IBRH are depicted in Table 3. There were no complications. The mean time to fistula closure was 12.5 days. Surgery was done in 1 patient; cystectomy with choledochotomy and T-tube drainage and he recovered. Follow up All patients were followed up with serial US and hydatid serology initially 4 weeklyfor 3 months and then 8 weekly for the next 6 months. Patients received oral albendazole 10 mg/kg daily for 6 months.The cysto-biliary communication sealed in all the patients. After 9 – 12 months, the cyst cavity disappeared in 2 patients and regressed in 2. The patient managed surgically was lost on follow up. Discussion The present study showed that EUS has significantly higher sensitivity than US for diagnosing IBR-LHC.US missed the diagnosis of IBR in 2 patients but EUS correctly identified IBR of LHC in all cases. The characteristicfindings of visualization of cyst-biliary communication was seen in 4 patients and/or presence of hydatid material within biliary tract was seen in all cases. The hydatid material visualized in the bile duct was oval or nodularand anechoic representing daughter vesicles and linear, irregular or leaf-like echogenic structures represented hydatid membranes. On realtime EUS, these structures changed their size and shapes which is again typical of hydatid material. Again unlike stones, hydatid materialsare not associated with posterior acoustic shadowing.Dilatation of biliary tree is an indirect sign and may be present due to compression of LHC on bile duct also. The other imaging findings such as deformation of cyst, and beak-like projection also aid in the diagnosis. Imaging tools like US, CT and MRI/MRCP

Table 3: Endoscopic retrograde cholangiopancreatography (ERCP) findings Patulous papilla

3

Whitish/yellow bile stained membranes protruding through

2

papilla Dilatation of the common bile and

5

intrahepatic ducts Irregular and linear filling defects of

5

varying sizes and shapes Extravasation of contrast into the

4

cyst cavity Endoscopic sphincterotomy

5

ENBD established

5

Stent deployment

3

Abbreviations used: ENBD, endoscopic naso biliary drainage are useful for preoperative diagnosis of IBRH but they lack high sensitivity and specificity. The two direct signs of IBRH include visualization of cysto-biliary communication or loss of continuity of cyst wall and intrabiliary hydatid contents (hydatid vesicles, membranes and sand). The former represents the site of perforation between cyst and biliary tree and is an unequivocal sign of IBR of LHC. The latter sign i.e., intrabiliaryhydatid material is also characteristic of IBR. The US which is a simple, rapid, cheap and noninvasive toolis rightly used asan initial imaging tool. On US, hydatid material in biliary tract is seen as anechoic rounded or linear or leaf-like echogenic structures. The overall sensitivity of US variesfrom 46% 2 to 75% 3. Other modalities (CT and MRI/MRCP), although, have higher


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1a & b 1c 1d & e 1f

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: : : :

Hydatid membranes seen the dilated common bile duct. Endoscopic image showing hydatid membranes being ballooned out. Cholangiograms showing cystobiliary communication and hydatid membranes in the biliary tree. Cholangiograms showing placement of Endoscopic nasobiliary drain and stent.


original article

sensitivity than US for demonstration of IBRH but have inherent drawbacks.The CT detects cyst wall discontinuity and hydatid materials in the biliary tract in 77% 2 to 83% 4 . Though MRCP is an excellent tool for visualizing the biliary tree and has sensitivity and specificity of 91.% and 82.8% respectively for detection of IBRH but visible communication between the LHC and the biliary tract – direct sign of rupture – was present only in 33% of cases 7 . Furthermore, MRCP has potential disadvantages including image artifacts, patient compliance, time consumption and lower sensitivity for the biliary tree in a non-dilated common bile duct. Furthermore, since most of these patients tend to be quite sick due to associated cholangitis or sepsis, it may not be possible to do MRI/MRCP as it may require breath holding or the patient may have some contraindication to the procedure or may be claustrophobic In the light of our findings and available literature, we believe, US should be the initial tool for diagnosis IBRH, and EUS should be next diagnostic tool in cases in whom US is negative or equivocal. The centers without EUS facilities may use CT or MRCP for preoperative diagnosis of IBR-LHC. In the present era, EUS seems the modality of choice for visualizing the biliary tree in other disease processes since it gives additional information which may be missed on US and has a superior diagnostic accuracy for the biliary tree than MRCP especially in the setting of nondilated ductal systems. The role of EUS for diagnosing IBRH is evolving and has been reported in sporadic case reports. Different modalities have been used for treating IBRH. Surgery has been the mainstay treatment modality 13-16. Since these patients have associated sepsis and cholangitis,early surgical intervention is associated with higher morbidity, mortality prolonged hospitalization and higher recurrence. The goal is to immediately


decompress the biliary tree by performing an emergency ERCP in conjunction with sphincterotomy followed by clearance of hydatid material within obstructed biliary tree. Finally, ENBD or stent is placed till complete clearance is achieved; ENBD is preferred as it permits irrigation of biliary tree and cyst cavity with saline. The cyst as well as biliary tree can be evacuated completely in 2 – 3 session. If complete evacuation is not manageable, an elective surgery can be planned. We achieved complete cure in 4 of 5 (80%) of cases as reported by others 6,8,9. Due to the noninvasive nature of ERCP in comparison to surgery, there is an increasing optimism for ERCP as a definitive curative therapy for IBRH. Endoscopic sphincterotomy reduces the high intra-biliary pressure and promotes early closure of these fistulae even in the absence of distal biliary obstruction. Most of the reports which have evaluated the role of ERCP for IBRH are merely restricted to case reports. Conclusion Diagnosis and treatment of IBRH has to be prompt since these patients are quite sick due to the associated cholangitis and septicemia. Different modalities have been used to diagnose IBRH but EUS seems to be superior to US, CT and MRCP. Also, EUS is more feasible than CT and MRCP. We advocate that EUS should be used in early work-up of IBRH especially if US is equivocal and negative. ERCP has both temporizing role in biliary sepsis as well as can offer a definitive therapy. Surgery must be reserved for those who do not respond to endoscopic therapy. References 1. McManus DP, Zhang W, Li J, Bartley PB. Echinococcosis. Lancet 2003;362:1295304 2. Marti-Bonmati L, Menor Serrano F. Complications of hepatic hydatid cysts: ultrasound, computed tomography, and magnetic resonance diagnosis. GastrointestRadiol. 1990;15:119-25.

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original article 3. Zargar SA, Khuroo MS, Khan BA, Dar MY, Alai MS, Koul P. Intrabiliary rupture of hepatic hydatid cyst: sonographic and cholangiographic appearances. GastrointestRadiol. 1992;17:41-5. 4. Wani NA, Kosar T, Gojwari T, Robbani I, Choh NA, Shah AI, et.al. Intrabiliary rupture of hepatic hydatid cyst: multidetector-row CT demonstration. Abdom Imaging. 2011;36:433-7. 5. Pedrosa I, Saz A, Arrazola J, Ferreirs J, Pedrosa CS. Hydatid disease: radiologic and pathologic features and complications. Radiographics2000; 20:795-817. 6. Singh V, Reddy DC, Vernma GR, Singh G. Endoscopic management of intrabiliaryruptured hepatic hydatid cyst. Liver Int2006; 26:621-4. 7. Erden A, Ormeci N, Fitoz S, Erden I, Tanju S, Genç Y. Intrabiliary rupture of hepatic hydatid cysts: diagnostic accuracy of MR cholangiopancreatography. AJR Am J Roentgenol2007;189: W84-9. 8. Simesk H, Ozaslan E, Savek I, Savas C, Abbasoglu O, Soylu AR. Diagnostic and therapeutic ERCP in hepatic hydatid disease. Gastrointest Endosc 2003; 58:3849. 9. Goumas K, Poulou A, Dandakis D, Tyrmpas I, Georgouli A, Sgourakis G, et al. Role of endoscopic intervention in biliary complications of hepatic hydatid cyst disease. Scand J Gastroenterol2007; 42:1113-9. 10. Zhan X, Guo X, Chen Y, Dong Y, Yu Q, Wang K.et al. EUS in exploring the etiology of mild acute biliary pancreatitis with a negative ﬁnding of biliary origin by conventional radiological methods. J Gastroenterol Hepatol. 2011; 26:1500–1503.

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11. Ozaslan E, Bayraktar Y. Endoscopic Therapy in the Management of HepatobiliaryHydatid Disease. Journal of Clinical Gastroenterology 2002;35:160-74 12. , et al. Endoscopic ultrasound: a metaanalysis of test performance in suspected biliary obstruction. Clin Gastroenterol Hepatol 2007; 5:616-23. 13. Prousalidis J, Kosmidis C, Kapoutzis K, Fachantidis E, Harlaftis N, Aletras H. Intrabiliary rupture of hydatid cysts of the liver. Am J Surg. 2009;197:193-8. 14. Chautems R, Bühler LH, Gold B, Giostra E, Poletti P, Chilcott M, et.al.Surgical management and long-term outcome of complicated liver hydatid cysts caused by E c h i n o c o c c u s g r a n u l o s u s . S u rg e r y 2005;137:312-16 15. Elbir O, Gundogdu H, Caglikulekci M, Kaysalp C, Atalay F, Savkilioglu M, et.al. Surgical treatment of intrabiliary rupture of hydatid cysts of the liver: comparsion of choledochoduodenostomy with T-tube drainage. Dig Surg 2001;18:289-293 16. Taçyildiz I, Aldemir M, Aban N, Keles C. Diagnosis and surgical treatment of intrabiliary ruptured hydatid disease of the liver. S Afr J Surg. 2004;42:43-6. Figure Legends: 1a & b: Hydatid membranes seen the dilated common bile duct. 1c: Endoscopic image showing hydatid membranes being ballooned out. 1d & e: Cholangiograms showing cystobiliary communication and hydatid membranes in the biliary tree. 1f: Cholangiograms showing placement of Endoscopic nasobiliary drain and stent.
