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Japanese Journal of Gastroenterology and Hepatology
Case Report Volume 5
ISSN 2435-1210
Hepatic Cysts with Cholesterol Crystal Formation: An Unusual Mimicker of
Cyst Infection in A Patient with Autosomal Dominant Polycystic Kidney Disease
Chou KC*
and Yen HH
Department of Gastroenterology and Hepatology, Changhua Christian Hospital, Changhua City 50006, Taiwan
*
Corresponding author:
Kun-Ching Chou,
Department of Gastroenterology and Hepatology,
Changhua Christian Hospital, Changhua City 50006,
Taiwan, Tel: +886-983901061,
E-mail: 84798@cch.org.tw
Received: 29 Jan 2021
Accepted: 11 Feb 2021
Published: 13 Feb 2021
Copyright:
©2021 Chou KC, This is an open access article distributed un-
der the terms of the Creative Commons Attribution License,
which permits unrestricted use, distribution, and build upon
your work non-commercially.
Citation:
Chou KC. Hepatic Cysts with Cholesterol Crystal Formation:
An Unusual Mimicker of Cyst Infection in A Patient with Au-
tosomal Dominant Polycystic Kidney Disease. Japanese J Gstro
Hepato. 2021; V5(12): 1-4
Keywords:
Hepatic cyst; Cholesterol crystal; Infection; Autoso-
mal dominant polycystic kidney disease; Polycystic
Liver disease
1. Abstract
Hyperechoic echogenicity in a hepatic cyst is a sign of cyst infection.
We report a case of autosomal dominant polycystic kidney disease
with polycystic liver disease where the hepatic cyst showed glittering
spots. Cyst fluid analysis revealed them as cholesterol crystals.
2. Introduction
The overall prevalence of hepatic cysts in patients with Autosomal
Dominant Polycystic Kidney Disease (ADPKD) has been reported
as 83% [1]. The complications of hepatic cysts include infection,
hemorrhage, rupture, and torsion of the cysts [2]. Computed To-
mography (CT) characteristics of infected hepatic cysts include peri-
cystic hyperemia and higher intracystic attenuation than normal cysts.
The ultrasound features of infected hepatic cysts include intracystic
hypoechoic or hyperechoic or heterogeneous echogenicity [3]. He-
patic cysts with cholesterol crystals is a rare condition. The ultra-
sound features of these cysts are hyperechoic sludge with a glittering
appearance that mimics cyst infection. Clinicians should be aware of
this rare association.
3. Case Report
We report a 50-year-old woman with ADPKD with Polycystic Liver
Disease (PLD). Written informed consent was obtained from the pa-
tient in all invasive procedures. She received alcohol sclerosing ther-
apy for liver cysts in 2016 and left lobe hepatic cyst aspiration due
to cyst infection in 2018. Upon admission, she had been suffering
from abdominal pain and distension for 2 weeks. A liver ultrasound
showed sludge in one of the cysts of the left lobe (Figure 1). The
sludge showed a glittering appearance when we tilted the probe (Vid-
eo 1). Because the ultrasound finding mimicked that of an infected
cyst, we performed aspiration. The fluid was cloudy brown (Figure 2)
and was sent for Gram stain, culture, total bilirubin, crystal analysis,
and total cholesterol. The Gram stain did not reveal the presence
of any organisms and no aerobes or anaerobes were isolated upon
culture of the cyst fluid. The total bilirubin of the cyst was 0.80mg/
dL and the total cholesterol was 168 mg/dL. Crystal analysis revealed
multiple cholesterol crystals showing typical rectangular-shaped crys-
tals with notched corners under light microscopy (Figure 3). The
crystals were birefringent under polarized light microscopy (Figure
4). The patient underwent right lobe hepatic cyst Transcatheter Ar-
terial Embolization (TAE) in 2019. Thereafter, her abdominal pain
improved and the abdominal distension subsided. The whole liver
cysts volume reduced by 10%.
4. Discussion
Hepatic involvement is the most common extrarenal manifestation
in ADPKD. The overall prevalence of hepatic cysts in patients with
ADPKD was reported as 83% with a prevalence of 85% in women
and 79% in men. The older group had the highest prevalence (94%)
and the younger group had the lowest prevalence (58%) [1]. A very
large polycystic liver may cause abdominal distension and abdomi-
nal pain. Symptoms related to adjacent organ compression include
easy satiety due to gastrointestinal compression, dyspnea due to dia-
phragm compression, and abdominal hernia or uterine prolapse due
1
to increased intra-abdominal pressure. The end stage of the disease is
characterized by malnutrition, disability, and, finally, physical exhaus-
tion [2]. The hepatic complications of ADPKD include infection,
hemorrhage, rupture, and torsion of the cysts. Multiple liver cysts
cause a mass effect, and complications include hepatic venous out-
flow obstruction, Budd–Chiari syndrome, inferior vena cava com-
pression, portal hypertension with variceal bleeding, and bile duct
compression with jaundice [2].
Figure 1: Liver ultrasound of an ADPKD patient with PLD. A cyst 4.2cm at left lobe showed hyperechoic sludge with glittering spots.
Figure 2: Cloudy brown cyst fluid was aspirated from the cyst with sludge and glittering spots.
2
2021, V5(12): 1-2
Figure 3: Crystal analysis of the cystic fluid revealed typical rectangular-shaped crystals with notched corners under light microscopy, indicating cholesterol
crystals.
Figure 4: Under polarized light microscopy, the cholesterol crystals showed bifringence.
The initial treatment for PLD and ADPKD includes the termination
of oral anticonceptives, conservative treatment for asymptomatic pa-
tients, and analgesic administration for patients with acute or chronic
pain or tenderness. Other invasive treatments include percutaneous
aspiration combined with sclerotherapy, installation of a stent for
compression syndrome, laparoscopic fenestration, liver resection,
and liver transplantation [2].In several case series studies have report-
ed TAE for treatment of the PLD as an acceptable treatment option
for ADPKD patients [4]. Furthermore, 83% of patients experience
an improvement in symptoms like abdominal distension and pain
2–6 months after TAE. The mean liver cystic volumes were reported
as reduced by 33%, and liver parenchyma volumes increased by 35%
at 36 months after TAE. TAE is a safe procedure without major
complications. Post-treatment syndrome is self-limiting and includes
3
2021, V5(12): 1-3
the development of abdominal pain, low-grade fever, and loss of
appetite within 1 week of TAE.
A complication in ADPKD with PLD is hepatic cyst infection,
which occurs in approximately 1% of all patients with hepatic cysts.
The most common organisms isolated from blood culture or aspi-
rated hepatic cysts fluid are Escherichia coli and Klebsiella sp., indicating
that the bacteria have translocated across the intestinal barrier [5].
The symptoms of hepatic cyst infection include right upper-quad-
rant pain, fever, and malaise. Sepsis may occur without adequate
treatment. CT shows infected hepatic cysts with pericystic hyperemia
and higher intracystic attenuation than normal cysts (median, 19.0
Hounsfield unit (HU) vs. 8.5 HU). The ultrasound features of infect-
ed hepatic cyst lose their anechoic property and present intracystic
hypoechoic or hyperechoic or heterogeneous echogenicity [3].
Cholesterol crystals in hepatic cyst is a rare condition, and has not
been reported in the literature. The crystals showed hyperechoic
echogenicity that mimicked cyst infection. The glittering appearance
under ultrasound may be related to the flat, flaky crystal structure of
cholesterol. Clinicians should be aware of this rare association.
5. Acknowledgements
We thank Medical Examiner Hsiu-Chun Lin for taking photos of the
cholesterol crystals.
References
1. Bae KT, Zhu F, Chapman AB, Torres VE, Grantham JJ, Guay-Wood-
ford LM, Baumgarten DA et al. Consortium for Radiologic Imaging
Studies of Polycystic Kidney Disease (CRISP) Magnetic resonance
imaging evaluation of hepatic cysts in early autosomal-dominant poly-
cystic kidney disease: the Consortium for Radiologic Imaging Studies
of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2006;
1: 64-69.
2. Chauveau D1, Fakhouri F, Grünfeld JP. Liver involvement in autoso-
mal-dominant polycystic kidney disease: therapeutic dilemma. J Am
Soc Nephrol. 2000; 11: 1767-75.
3. Oh J, Shin CI, Kim SY. Infected cyst in patients with autosomal domi-
nant polycystic kidney disease: Analysis of computed tomographic and
ultrasonographic imaging features. PLoS One. 2018; 13: e0207880.
4. Zhang JL, Yuan K, Wang MQ, Yan JY, Xin HN, Wang Y et al. Tran-
sarterial Embolization for Treatment of Symptomatic Polycystic Liver
Disease: More than 2-year Follow-up. Chin Med J (Engl). 2017; 130:
1938-44.
5. van Aerts RMM, van de Laarschot LFM, Banales JM, Drenth JPH.
Clinical management of polycystic liver disease. J Hepatol. 2017; 68:
827-837.
4
2021, V5(12): 1-4

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Hepatic Cysts with Cholesterol Crystal Formation: An Unusual Mimicker of Cyst Infection in A Patient with Autosomal Dominant Polycystic Kidney Disease

  • 1. Japanese Journal of Gastroenterology and Hepatology Case Report Volume 5 ISSN 2435-1210 Hepatic Cysts with Cholesterol Crystal Formation: An Unusual Mimicker of Cyst Infection in A Patient with Autosomal Dominant Polycystic Kidney Disease Chou KC* and Yen HH Department of Gastroenterology and Hepatology, Changhua Christian Hospital, Changhua City 50006, Taiwan * Corresponding author: Kun-Ching Chou, Department of Gastroenterology and Hepatology, Changhua Christian Hospital, Changhua City 50006, Taiwan, Tel: +886-983901061, E-mail: 84798@cch.org.tw Received: 29 Jan 2021 Accepted: 11 Feb 2021 Published: 13 Feb 2021 Copyright: ©2021 Chou KC, This is an open access article distributed un- der the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and build upon your work non-commercially. Citation: Chou KC. Hepatic Cysts with Cholesterol Crystal Formation: An Unusual Mimicker of Cyst Infection in A Patient with Au- tosomal Dominant Polycystic Kidney Disease. Japanese J Gstro Hepato. 2021; V5(12): 1-4 Keywords: Hepatic cyst; Cholesterol crystal; Infection; Autoso- mal dominant polycystic kidney disease; Polycystic Liver disease 1. Abstract Hyperechoic echogenicity in a hepatic cyst is a sign of cyst infection. We report a case of autosomal dominant polycystic kidney disease with polycystic liver disease where the hepatic cyst showed glittering spots. Cyst fluid analysis revealed them as cholesterol crystals. 2. Introduction The overall prevalence of hepatic cysts in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD) has been reported as 83% [1]. The complications of hepatic cysts include infection, hemorrhage, rupture, and torsion of the cysts [2]. Computed To- mography (CT) characteristics of infected hepatic cysts include peri- cystic hyperemia and higher intracystic attenuation than normal cysts. The ultrasound features of infected hepatic cysts include intracystic hypoechoic or hyperechoic or heterogeneous echogenicity [3]. He- patic cysts with cholesterol crystals is a rare condition. The ultra- sound features of these cysts are hyperechoic sludge with a glittering appearance that mimics cyst infection. Clinicians should be aware of this rare association. 3. Case Report We report a 50-year-old woman with ADPKD with Polycystic Liver Disease (PLD). Written informed consent was obtained from the pa- tient in all invasive procedures. She received alcohol sclerosing ther- apy for liver cysts in 2016 and left lobe hepatic cyst aspiration due to cyst infection in 2018. Upon admission, she had been suffering from abdominal pain and distension for 2 weeks. A liver ultrasound showed sludge in one of the cysts of the left lobe (Figure 1). The sludge showed a glittering appearance when we tilted the probe (Vid- eo 1). Because the ultrasound finding mimicked that of an infected cyst, we performed aspiration. The fluid was cloudy brown (Figure 2) and was sent for Gram stain, culture, total bilirubin, crystal analysis, and total cholesterol. The Gram stain did not reveal the presence of any organisms and no aerobes or anaerobes were isolated upon culture of the cyst fluid. The total bilirubin of the cyst was 0.80mg/ dL and the total cholesterol was 168 mg/dL. Crystal analysis revealed multiple cholesterol crystals showing typical rectangular-shaped crys- tals with notched corners under light microscopy (Figure 3). The crystals were birefringent under polarized light microscopy (Figure 4). The patient underwent right lobe hepatic cyst Transcatheter Ar- terial Embolization (TAE) in 2019. Thereafter, her abdominal pain improved and the abdominal distension subsided. The whole liver cysts volume reduced by 10%. 4. Discussion Hepatic involvement is the most common extrarenal manifestation in ADPKD. The overall prevalence of hepatic cysts in patients with ADPKD was reported as 83% with a prevalence of 85% in women and 79% in men. The older group had the highest prevalence (94%) and the younger group had the lowest prevalence (58%) [1]. A very large polycystic liver may cause abdominal distension and abdomi- nal pain. Symptoms related to adjacent organ compression include easy satiety due to gastrointestinal compression, dyspnea due to dia- phragm compression, and abdominal hernia or uterine prolapse due 1
  • 2. to increased intra-abdominal pressure. The end stage of the disease is characterized by malnutrition, disability, and, finally, physical exhaus- tion [2]. The hepatic complications of ADPKD include infection, hemorrhage, rupture, and torsion of the cysts. Multiple liver cysts cause a mass effect, and complications include hepatic venous out- flow obstruction, Budd–Chiari syndrome, inferior vena cava com- pression, portal hypertension with variceal bleeding, and bile duct compression with jaundice [2]. Figure 1: Liver ultrasound of an ADPKD patient with PLD. A cyst 4.2cm at left lobe showed hyperechoic sludge with glittering spots. Figure 2: Cloudy brown cyst fluid was aspirated from the cyst with sludge and glittering spots. 2 2021, V5(12): 1-2
  • 3. Figure 3: Crystal analysis of the cystic fluid revealed typical rectangular-shaped crystals with notched corners under light microscopy, indicating cholesterol crystals. Figure 4: Under polarized light microscopy, the cholesterol crystals showed bifringence. The initial treatment for PLD and ADPKD includes the termination of oral anticonceptives, conservative treatment for asymptomatic pa- tients, and analgesic administration for patients with acute or chronic pain or tenderness. Other invasive treatments include percutaneous aspiration combined with sclerotherapy, installation of a stent for compression syndrome, laparoscopic fenestration, liver resection, and liver transplantation [2].In several case series studies have report- ed TAE for treatment of the PLD as an acceptable treatment option for ADPKD patients [4]. Furthermore, 83% of patients experience an improvement in symptoms like abdominal distension and pain 2–6 months after TAE. The mean liver cystic volumes were reported as reduced by 33%, and liver parenchyma volumes increased by 35% at 36 months after TAE. TAE is a safe procedure without major complications. Post-treatment syndrome is self-limiting and includes 3 2021, V5(12): 1-3
  • 4. the development of abdominal pain, low-grade fever, and loss of appetite within 1 week of TAE. A complication in ADPKD with PLD is hepatic cyst infection, which occurs in approximately 1% of all patients with hepatic cysts. The most common organisms isolated from blood culture or aspi- rated hepatic cysts fluid are Escherichia coli and Klebsiella sp., indicating that the bacteria have translocated across the intestinal barrier [5]. The symptoms of hepatic cyst infection include right upper-quad- rant pain, fever, and malaise. Sepsis may occur without adequate treatment. CT shows infected hepatic cysts with pericystic hyperemia and higher intracystic attenuation than normal cysts (median, 19.0 Hounsfield unit (HU) vs. 8.5 HU). The ultrasound features of infect- ed hepatic cyst lose their anechoic property and present intracystic hypoechoic or hyperechoic or heterogeneous echogenicity [3]. Cholesterol crystals in hepatic cyst is a rare condition, and has not been reported in the literature. The crystals showed hyperechoic echogenicity that mimicked cyst infection. The glittering appearance under ultrasound may be related to the flat, flaky crystal structure of cholesterol. Clinicians should be aware of this rare association. 5. Acknowledgements We thank Medical Examiner Hsiu-Chun Lin for taking photos of the cholesterol crystals. References 1. Bae KT, Zhu F, Chapman AB, Torres VE, Grantham JJ, Guay-Wood- ford LM, Baumgarten DA et al. Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) Magnetic resonance imaging evaluation of hepatic cysts in early autosomal-dominant poly- cystic kidney disease: the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2006; 1: 64-69. 2. Chauveau D1, Fakhouri F, Grünfeld JP. Liver involvement in autoso- mal-dominant polycystic kidney disease: therapeutic dilemma. J Am Soc Nephrol. 2000; 11: 1767-75. 3. Oh J, Shin CI, Kim SY. Infected cyst in patients with autosomal domi- nant polycystic kidney disease: Analysis of computed tomographic and ultrasonographic imaging features. PLoS One. 2018; 13: e0207880. 4. Zhang JL, Yuan K, Wang MQ, Yan JY, Xin HN, Wang Y et al. Tran- sarterial Embolization for Treatment of Symptomatic Polycystic Liver Disease: More than 2-year Follow-up. Chin Med J (Engl). 2017; 130: 1938-44. 5. van Aerts RMM, van de Laarschot LFM, Banales JM, Drenth JPH. Clinical management of polycystic liver disease. J Hepatol. 2017; 68: 827-837. 4 2021, V5(12): 1-4