Gastrointest Radiol 17:46-48 (1992)
Gastrointestinal
Radiology (c~ Springer-Verlag New York Inc. 1992
Polycystic Liver Disease with Obstructive Jaundice: Treatment with Ultrasound-Guided Cyst Aspiration M a r i l y n E. L e r n e r , I Julia E. R o s h k o w , I A m y S m i t h l i n e , 2 a n d C h r i s N g ~ Departments of ~Radiology and ZGastroenterology, Columbia-Presbyterian Medical Center, New York. New York, USA
Abstract. A 5 9 - y e a r - o l d w o m a n with p o l y c y s t i c liver ( P C L D ) a n d k i d n e y d i s e a s e ( P C K D ) p r e s e n t e d with j a u n d i c e , f e v e r , a n d a b d o m i n a l pain. A c o m p u t e r e d t o m o g r a p h i c (CT) s c a n p e r f o r m e d i m m e d i a t e l y a f t e r an e n d o s c o p i c r e t r o g r a d e c h o l a n g i o p a n c r e a t o g r a m (ERCP) clearly demonstrated a hepatic cyst obs t r u c t i n g the biliary s y s t e m . T h e c y s t was t h e n aspir a t e d u n d e r u l t r a s o u n d g u i d a n c e with c o m p l e t e relief o f o b s t r u c t i o n .
Key words: P o l y c y s t i c liver d i s e a s e , j a u n d i c e - Ultrasound cyst aspiration--CT--ERCP.
J a u n d i c e is a rare c o m p l i c a t i o n o f p o l y c y s t i c liver d i s e a s e ( P C L D ) [I]. W e p r e s e n t a c a s e o f biliary o b s t r u c t i o n d u e to a large h e p a t i c c y s t in a p a t i e n t with p o l y c y s t i c k i d n e y d i s e a s e ( P C K D ) a n d P C L D . T h i s w a s b e s t d e m o n s t r a t e d on a c o m p u t e d t o m o g r a p h i c (CT) s c a n o b t a i n e d i m m e d i a t e l y f o l l o w i n g an endoscopic retrograde cholangiopancreatogram (ERCP). To our knowledge, CT scan following E R C P w a s not p e r f o r m e d in a n y o f the o t h e r rep o r t e d c a s e s . In a d d i t i o n , the p a t i e n t was s u c c e s s fully t r e a t e d b y a s p i r a t i o n o f the c y s t u n d e r ultrasound guidance.
Case Report A 59-year-old woman with a history of autosomal dominanl polycystic kidney disease (PCKD) presented with jaundice, right upper quadrant pain, and fever. On physical examination, she had tender hepatomegaly.
Address ~ffprint requests to: Marilyn E. Lerner, M.D., Depart-
ment of Radiology, Columbia-Presbyterian Medical Center, 177 Ft. Washington Avenue, New York, NY 10032, USA
Abnormal laboratory data induced an elevated white count (17,000), alkaline phosphatase (2030 IU), total bilirubin (13 mg/dl), and direct bilirubin (10.6 mg/dl). The patient was started empirically on antibiotics and her temperature and white count returned to normal. An ultrasound was performed which showed enlarged kidneys containing numerous cysts consistent with the patient's known PCKD. There were multiple hepatic cysts, the largest of which was in the portal region and measured 6 cm in greatest dimension (Fig. IA and B). lntrahepatic biliary dilatation was present. The common bile duct was normal in caliber. An ERCP showed extrinsic compression of the right and left main hepatic ducts .just above the bifurcation, as well as dilated intrahepatic ducts and a normal common bile duct (Fig. 2). ACT scan obtained immediately following ERCP showed residual contrast filling the biliary tree. Compression of the right and left main hepatic ducts at the level of the bifurcation by the cyst was clearly demonstrated (Fig. 3A and B). Ultrasound-guided aspiration of the Cyst was performed with a 20-gauge spinal needle, and 130 ml of serosanguinous fluid were removed. The fluid cell count revealed a white blood cell count of 5000/dl and a red blood cell count of 46,000/di. All cultures were negative. In the week following the procedure, she had rapid resolution of her jaundice and improvement in her liver chemistries. CT and ultrasound 8 months later showed no significant cyst reaccumulation and no ductal dilatation. The patient felt well and had normal liver chemistries.
Discussion P C L D o c c u r s in 30% o f p a t i e n t s with a u t o s o m a l d o m i n a n t P C K D [2, 3]. C o n v e r s e l y , 50% o f p a t i e n t s with P C L D h a v e P C K D [2, 3]. It has b e e n p r o p o s e d that the c y s t s in P C L D a r i s e f r o m a b e r r a n t bile d u c t s . It is b e l i e v e d that t h e s e d u c t s d o not c o n n e c t e m b r y o l o g i c a l l y with the int e r l o b u l a r bile d u c t s . T h e c u b o i d a l e p i t h e l i u m lining the c y s t is s i m i l a r to that lining the bile d u c t s [4]. T h e lining cells s e c r e t e fluid a n d t h u s d i l a t e and b e c o m e c y s t s [5]. In p a t i e n t s with b o t h P C L D and P C K D , t h e hepatic c y s t s a r e u s u a l l y a s y m p t o m a t i c and m o r b i d i t y
M.E. Lerner et al.: PCLD Jaundice and USG Cyst Aspiration
47
Fig. 1. A Ultrasound of the liver reveals multiple hepatic cysts and dilated intrahepatic ducts. B A large cyst compresses the common he~ patic duct in the portal region.
Fig. 2. Radiograph obtained during ERCP reveals a nondilated common bile duct. There is extrinsic compression at the bifurcation of the left and right ducts, lntrahepatic ductal dilatation is seen.
Fig. 3. A CT scan performed immediately after ERCP demonslrates compression of the left and right ducts by a iarge cyst. B Dilatation of intrahepatic ducts by the obstructing cyst is well seen.
48
and mortality is commonly associated with the renal disease [6]. Rupture of the associated circle of Willis aneurysms may also be the cause of death [6]. More recently, however, reported liver complications have increased [7]. This is probably due to the increased life span of these patients secondary to treatment of the renal failure with transplantation or dialysis [7]. The most common presenting symptom is abdominal pain, which was present in 89%, of symptomatic patients in one study [8]. Other presenting symptoms may be ascites, edema secondary to IVC obstruction, early satiety from hepatomegaly, fever and chills from infection, and jaundice from biliary obstruction [8]. Rarely, portal hypertension and bleeding esophageal varices may be encountered [10, I I]. Jaundice due to a cyst causing bile duct obstruction is a rare complication of PCLD [1]. Ultrasound, CT, and ERCP may all be helpful in establishing the diagnosis. In the previously reported cases, various modalities were used to evaluate the patients [9]. However, we found CT scan, immediately following ERCP when the biliary tree is still filled with contrast, to be the most helpful. In patients with PCLD, it can sometimes be difficult on both ultrasound and CT to separate multiple small cysts from intrahepatic biliary ductal dilation. ERCP can demonstrate compression of the bile ducts, but does not show the cause of the compression. The bile ducts on CT scan following ERCP were easily separated from the cysts, and the cyst compressing the right and left main hepatic ducts is well demonstrated. Surgical treatment has been generally advocated for symptomatic PCLD [8]. Resection of cysts, unroofing of cysts with or without drainage, and cyst fenestration have all been advocated [8, 12, 13]. Percutaneous cyst aspiration has been performed with variable success [12, 14, 15]. Recurrence of the cyst may occur, and rarely an infection may develop following aspiration [8, 15]. Treatment with sclerosing agents after cyst aspiration has also been advocated [16-18]. Both 95% alcohol [16, 17] and Pantopaque [13] have been used. Although recurrence of cysts after aspiration does occur, we feel that it is reasonable to perform ultrasound-guided aspiration with its minimal risks prior to surgical treatment.
M.E. Lerner et al.: PCLD Jaundice and USG Cyst Aspiration
References 1. Melnick DJ. Polycystic liver. Arch Pathol 1955;59:162-172 2. Milutinovic J, Frallsen PJ, Rudd TG, et al. Liver cysts in patients with autosomal dominant polycystic kidney disease. Am J Med 1980:68:741-744 3. Dalgaard OZ. Bilateral polycystic disease of the kidneys: a follow-up of two hundred and eighty four patients and their families. Acta Med Stand 1957; 158(Suppl): 1-255 4. Hartnett M, Bennett W. Extrarenal manifestations of cystic kidney disease. In: Gardner KD, ed. Cystic diseases o f the kidney. New York, Wiley, 1976:201-219 5. Patterson M, Gonzalez-Vilale JC, Fagan CJ. Polycystic liver disease: a study of cyst fluid constituents. Hepatolo~,,y 1982;2: 475-478 6. Grantham JJ. Polycystic renal disease. In: Earley LE, Gottschalls CW, eds. Strauss and Well's diseases ~ff"the kidney, 3rd ed. Boston: Little Brown, 1979:1123-1146 7. Grunfeld JP, Albonze G, Jungers P, el al. Liver changes and complications in adult polycystic kidney disease. Ado Neptual 1985:14:1-20 8. Turnage RH, Eckhauser FE, Kaol JA, Thompson NW. Therapeutic dilemmas in patients with symptomatic polycystic liver disease. Am Sttrg 1988:54:365-372 9. Levine E, Cools L, Grantham J. Liver cysts in autosomal dominant polycystic kidney disease: clinical and computed tomographic study. A JR 1985;145:229-233 10. DelGuercia E, Greco J, Kim KE, et al. Esophageal varices in adult patients with polycystic kidney and liver disease. N Engl J Med 1973:289:678-679 I1. Ratcliffe PJ, Reeders S, Thealsey JM. Bleeding esophagela varices and hepatic dysfunction in adult polycystic kidney disease. Br Med J 1984:288:1330-133 I 12. Pettolsaillia V. Non-parasitic cysts of the liver: a clinical study of 117 cases. Ann Clin Gynecol 1970:59:1-58 13. Armitage NC, Blungart LH. Partial resection and fenestration in the treatment of polycystic liver disease. Br J SttrL, 1984;71: 242-245 14. Jones WL, Mountain JC, Warren KW. Symptomatic nonparasitic cysts of the liver. Br J Sttrg, 1974:61 : 118-123 15. Saini S, Mueller PR, Ferrucci JT Jr, et al. Percutaneous aspiration of hepatic cysts does not provide definitive therapy. A JR 1983;141:559-560 16. Trinbal W, Sassaris M, Hunter FM. Nonsurgical treatment for symptomatic liver cyst. Am J Gastroenterol 1985:81): 907-911 17. Bean WJ, Rodan BA. Hepatic cysts: treatment with alcohol. A JR 1985;144:237-241 18. Goldstein HM, Carlyle DR, Nelson RS. Treatment of symptomatic hepatic cyst by percutaneous installation of pantopaque. A JR 1976:127:850-853
Received: June 13, 1991; accepted: July 19, 1991
Gastrointestinal
Radiology (c~ Springer-Verlag New York Inc. 1992
Polycystic Liver Disease with Obstructive Jaundice: Treatment with Ultrasound-Guided Cyst Aspiration M a r i l y n E. L e r n e r , I Julia E. R o s h k o w , I A m y S m i t h l i n e , 2 a n d C h r i s N g ~ Departments of ~Radiology and ZGastroenterology, Columbia-Presbyterian Medical Center, New York. New York, USA
Abstract. A 5 9 - y e a r - o l d w o m a n with p o l y c y s t i c liver ( P C L D ) a n d k i d n e y d i s e a s e ( P C K D ) p r e s e n t e d with j a u n d i c e , f e v e r , a n d a b d o m i n a l pain. A c o m p u t e r e d t o m o g r a p h i c (CT) s c a n p e r f o r m e d i m m e d i a t e l y a f t e r an e n d o s c o p i c r e t r o g r a d e c h o l a n g i o p a n c r e a t o g r a m (ERCP) clearly demonstrated a hepatic cyst obs t r u c t i n g the biliary s y s t e m . T h e c y s t was t h e n aspir a t e d u n d e r u l t r a s o u n d g u i d a n c e with c o m p l e t e relief o f o b s t r u c t i o n .
Key words: P o l y c y s t i c liver d i s e a s e , j a u n d i c e - Ultrasound cyst aspiration--CT--ERCP.
J a u n d i c e is a rare c o m p l i c a t i o n o f p o l y c y s t i c liver d i s e a s e ( P C L D ) [I]. W e p r e s e n t a c a s e o f biliary o b s t r u c t i o n d u e to a large h e p a t i c c y s t in a p a t i e n t with p o l y c y s t i c k i d n e y d i s e a s e ( P C K D ) a n d P C L D . T h i s w a s b e s t d e m o n s t r a t e d on a c o m p u t e d t o m o g r a p h i c (CT) s c a n o b t a i n e d i m m e d i a t e l y f o l l o w i n g an endoscopic retrograde cholangiopancreatogram (ERCP). To our knowledge, CT scan following E R C P w a s not p e r f o r m e d in a n y o f the o t h e r rep o r t e d c a s e s . In a d d i t i o n , the p a t i e n t was s u c c e s s fully t r e a t e d b y a s p i r a t i o n o f the c y s t u n d e r ultrasound guidance.
Case Report A 59-year-old woman with a history of autosomal dominanl polycystic kidney disease (PCKD) presented with jaundice, right upper quadrant pain, and fever. On physical examination, she had tender hepatomegaly.
Address ~ffprint requests to: Marilyn E. Lerner, M.D., Depart-
ment of Radiology, Columbia-Presbyterian Medical Center, 177 Ft. Washington Avenue, New York, NY 10032, USA
Abnormal laboratory data induced an elevated white count (17,000), alkaline phosphatase (2030 IU), total bilirubin (13 mg/dl), and direct bilirubin (10.6 mg/dl). The patient was started empirically on antibiotics and her temperature and white count returned to normal. An ultrasound was performed which showed enlarged kidneys containing numerous cysts consistent with the patient's known PCKD. There were multiple hepatic cysts, the largest of which was in the portal region and measured 6 cm in greatest dimension (Fig. IA and B). lntrahepatic biliary dilatation was present. The common bile duct was normal in caliber. An ERCP showed extrinsic compression of the right and left main hepatic ducts .just above the bifurcation, as well as dilated intrahepatic ducts and a normal common bile duct (Fig. 2). ACT scan obtained immediately following ERCP showed residual contrast filling the biliary tree. Compression of the right and left main hepatic ducts at the level of the bifurcation by the cyst was clearly demonstrated (Fig. 3A and B). Ultrasound-guided aspiration of the Cyst was performed with a 20-gauge spinal needle, and 130 ml of serosanguinous fluid were removed. The fluid cell count revealed a white blood cell count of 5000/dl and a red blood cell count of 46,000/di. All cultures were negative. In the week following the procedure, she had rapid resolution of her jaundice and improvement in her liver chemistries. CT and ultrasound 8 months later showed no significant cyst reaccumulation and no ductal dilatation. The patient felt well and had normal liver chemistries.
Discussion P C L D o c c u r s in 30% o f p a t i e n t s with a u t o s o m a l d o m i n a n t P C K D [2, 3]. C o n v e r s e l y , 50% o f p a t i e n t s with P C L D h a v e P C K D [2, 3]. It has b e e n p r o p o s e d that the c y s t s in P C L D a r i s e f r o m a b e r r a n t bile d u c t s . It is b e l i e v e d that t h e s e d u c t s d o not c o n n e c t e m b r y o l o g i c a l l y with the int e r l o b u l a r bile d u c t s . T h e c u b o i d a l e p i t h e l i u m lining the c y s t is s i m i l a r to that lining the bile d u c t s [4]. T h e lining cells s e c r e t e fluid a n d t h u s d i l a t e and b e c o m e c y s t s [5]. In p a t i e n t s with b o t h P C L D and P C K D , t h e hepatic c y s t s a r e u s u a l l y a s y m p t o m a t i c and m o r b i d i t y
M.E. Lerner et al.: PCLD Jaundice and USG Cyst Aspiration
47
Fig. 1. A Ultrasound of the liver reveals multiple hepatic cysts and dilated intrahepatic ducts. B A large cyst compresses the common he~ patic duct in the portal region.
Fig. 2. Radiograph obtained during ERCP reveals a nondilated common bile duct. There is extrinsic compression at the bifurcation of the left and right ducts, lntrahepatic ductal dilatation is seen.
Fig. 3. A CT scan performed immediately after ERCP demonslrates compression of the left and right ducts by a iarge cyst. B Dilatation of intrahepatic ducts by the obstructing cyst is well seen.
48
and mortality is commonly associated with the renal disease [6]. Rupture of the associated circle of Willis aneurysms may also be the cause of death [6]. More recently, however, reported liver complications have increased [7]. This is probably due to the increased life span of these patients secondary to treatment of the renal failure with transplantation or dialysis [7]. The most common presenting symptom is abdominal pain, which was present in 89%, of symptomatic patients in one study [8]. Other presenting symptoms may be ascites, edema secondary to IVC obstruction, early satiety from hepatomegaly, fever and chills from infection, and jaundice from biliary obstruction [8]. Rarely, portal hypertension and bleeding esophageal varices may be encountered [10, I I]. Jaundice due to a cyst causing bile duct obstruction is a rare complication of PCLD [1]. Ultrasound, CT, and ERCP may all be helpful in establishing the diagnosis. In the previously reported cases, various modalities were used to evaluate the patients [9]. However, we found CT scan, immediately following ERCP when the biliary tree is still filled with contrast, to be the most helpful. In patients with PCLD, it can sometimes be difficult on both ultrasound and CT to separate multiple small cysts from intrahepatic biliary ductal dilation. ERCP can demonstrate compression of the bile ducts, but does not show the cause of the compression. The bile ducts on CT scan following ERCP were easily separated from the cysts, and the cyst compressing the right and left main hepatic ducts is well demonstrated. Surgical treatment has been generally advocated for symptomatic PCLD [8]. Resection of cysts, unroofing of cysts with or without drainage, and cyst fenestration have all been advocated [8, 12, 13]. Percutaneous cyst aspiration has been performed with variable success [12, 14, 15]. Recurrence of the cyst may occur, and rarely an infection may develop following aspiration [8, 15]. Treatment with sclerosing agents after cyst aspiration has also been advocated [16-18]. Both 95% alcohol [16, 17] and Pantopaque [13] have been used. Although recurrence of cysts after aspiration does occur, we feel that it is reasonable to perform ultrasound-guided aspiration with its minimal risks prior to surgical treatment.
M.E. Lerner et al.: PCLD Jaundice and USG Cyst Aspiration
References 1. Melnick DJ. Polycystic liver. Arch Pathol 1955;59:162-172 2. Milutinovic J, Frallsen PJ, Rudd TG, et al. Liver cysts in patients with autosomal dominant polycystic kidney disease. Am J Med 1980:68:741-744 3. Dalgaard OZ. Bilateral polycystic disease of the kidneys: a follow-up of two hundred and eighty four patients and their families. Acta Med Stand 1957; 158(Suppl): 1-255 4. Hartnett M, Bennett W. Extrarenal manifestations of cystic kidney disease. In: Gardner KD, ed. Cystic diseases o f the kidney. New York, Wiley, 1976:201-219 5. Patterson M, Gonzalez-Vilale JC, Fagan CJ. Polycystic liver disease: a study of cyst fluid constituents. Hepatolo~,,y 1982;2: 475-478 6. Grantham JJ. Polycystic renal disease. In: Earley LE, Gottschalls CW, eds. Strauss and Well's diseases ~ff"the kidney, 3rd ed. Boston: Little Brown, 1979:1123-1146 7. Grunfeld JP, Albonze G, Jungers P, el al. Liver changes and complications in adult polycystic kidney disease. Ado Neptual 1985:14:1-20 8. Turnage RH, Eckhauser FE, Kaol JA, Thompson NW. Therapeutic dilemmas in patients with symptomatic polycystic liver disease. Am Sttrg 1988:54:365-372 9. Levine E, Cools L, Grantham J. Liver cysts in autosomal dominant polycystic kidney disease: clinical and computed tomographic study. A JR 1985;145:229-233 10. DelGuercia E, Greco J, Kim KE, et al. Esophageal varices in adult patients with polycystic kidney and liver disease. N Engl J Med 1973:289:678-679 I1. Ratcliffe PJ, Reeders S, Thealsey JM. Bleeding esophagela varices and hepatic dysfunction in adult polycystic kidney disease. Br Med J 1984:288:1330-133 I 12. Pettolsaillia V. Non-parasitic cysts of the liver: a clinical study of 117 cases. Ann Clin Gynecol 1970:59:1-58 13. Armitage NC, Blungart LH. Partial resection and fenestration in the treatment of polycystic liver disease. Br J SttrL, 1984;71: 242-245 14. Jones WL, Mountain JC, Warren KW. Symptomatic nonparasitic cysts of the liver. Br J Sttrg, 1974:61 : 118-123 15. Saini S, Mueller PR, Ferrucci JT Jr, et al. Percutaneous aspiration of hepatic cysts does not provide definitive therapy. A JR 1983;141:559-560 16. Trinbal W, Sassaris M, Hunter FM. Nonsurgical treatment for symptomatic liver cyst. Am J Gastroenterol 1985:81): 907-911 17. Bean WJ, Rodan BA. Hepatic cysts: treatment with alcohol. A JR 1985;144:237-241 18. Goldstein HM, Carlyle DR, Nelson RS. Treatment of symptomatic hepatic cyst by percutaneous installation of pantopaque. A JR 1976:127:850-853
Received: June 13, 1991; accepted: July 19, 1991
