ISSN 1941-5923

© Am J Case Rep, 2017; 18: 1000-1004

DOI: 10.12659/AJCR.904663

Received: 2017.04.02
Accepted: 2017.06.21 A Case of Hepatorenal Syndrome and Abdominal
Published: 2017.09.18
Compartment Syndrome with High Renal
Congestion

Authors’ Contribution: AEF 1 Hiroteru Kamimura 1 Division of Gastroenterology and Hepatology, Niigata University Graduate School
Study Design  A B 1 Takayuki Watanabe of Medical and Dental Sciences, Niigata City, Niigata, Japan
Data Collection  B 2 Division of Molecular and Diagnostic Pathology, Niigata University Graduate

Statistical Analysis  C BC 1 Tomoyuki Sugano School of Medical and Dental Sciences, Niigata City, Niigata, Japan
Interpretation  D
Data B 1 Nao Nakajima
Manuscript
Preparation  E D 1 Junji Yokoyama
Literature Search  F

Funds Collection  G D 1 Kenya Kamimura
D 1 Atsunori Tsuchiya
D 1 Masaaki Takamura
D 1 Hirokazu Kawai
BD 2 Takashi Kato
B 2 Gen Watanabe
DE 1 Satoshi Yamagiwa
A 1 Shuji Terai

Corresponding Author: Hiroteru Kamimura, e-mail: [email protected]

Conflict of interest: None declared

Patient: Male, 40
Final Diagnosis: Hepatorenal syndrome
Symptoms: Abdominal distension
Medication: —
Clinical Procedure: —
Specialty: Nephrology

Objective: Rare co-existance of disease or pathology

Background: Hepatorenal syndrome (HRS) is a reversible renal impairment that occurs in patients with acute liver failure
and advanced liver cirrhosis. HRS is due to a renal vasoconstriction that results from extreme vasodilatation. It
is therefore a functional disorder, not associated with structural kidney damage. On the other hand, end-stage
liver diseases are often complicated by massive ascites. Massive ascites may cause abdominal compartment
syndrome (ACS), which includes impairment of renal blood flow, but there are no reports indicating that kid-
ney lesions caused by ACS may pathologically contribute to end-stage liver diseases.
Case Report: A 40-year-old man with acute liver failure was admitted to our hospital. He was diagnosed with type 1 HRS
and showed ACS at the same time. He died 30 days after admission. There were signs of congestion in the kid-
neys upon dissection and advanced erythroid fullness in the renal tubules.
Conclusions: We report an autopsy case with HRS and ACS diagnosed with a clinical and histopathological consideration of
liver and kidney. Further clinical studies are needed to improve management of renal failure in patients with
acute liver failure and advanced liver cirrhosis.

MeSH Keywords: Acute Kidney Injury • Hepatorenal Syndrome • Intra-Abdominal Hypertension

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Kamimura H. et al.:
HRS and ACS with high renal congestion
© Am J Case Rep, 2017; 18: 1000-1004

Background protein (CRP) (4.1 mg/dL), and NH3 (121 µg/dL) (Table 1).
Computed tomography of the abdomen showed massive as-
Hepatorenal syndrome (HRS) was originally described in 1863 cites (Figure 1A), and the intraperitoneal and retroperitone-
by Flint as an association between liver disease and oligu- al organ, especially right renal vein, were compressed by the
ric renal failure in the absence of significant renal histologi- ascites (Figure 1B). We diagnosed him with sepsis-associat-
cal change [1]. It is characterized by marked reduction in glo- ed late-onset hepatic failure and AKIN stage 2. We appropri-
merular filtration rate (GFR) and renal plasma flow in patients ately performed general care and maintained proper circula-
with liver cirrhosis and hepatic failure in the absence of other tion, breathing, and fluids. Moreover, we performed plasma
causes of renal failure [2]. Renal failure is caused by vasocon- exchange and administered fresh frozen plasma and albu-
striction resulting from systematic vasodilation, and HRS pa- min to support his liver function. However, his liver dysfunc-
tients are thought to recover from renal failure after receiv- tion did not recover and the kidney function worsened. Serum
ing a liver transplant [3]. However, the mechanisms governing creatinine increased to 4.1 mg/dl and urine volume gradual-
HRS pathology are not completely understood. Abdominal as- ly decreased. Then, we started noradrenaline for HRS 20 days
cites occurs typically at the end stage of liver failure. Massive after admission. We palliatively performed large-volume para-
ascites also influences intraabdominal pressure (IAP) patho- centesis (LVP) with an upper limit of 5 L, and urine volume in-
genesis [4]. Abdominal compartment syndrome (ACS) results creased after the LVP. However, IAP was continuously high at
in organ failure, including renal failure. Although elevation of 20 mmHg even after LVP, indicating he had severe ACS. He
renal parenchymal and renal vein pressure are likely mecha- died 30 days after admission.
nisms of renal impairment in ACS patients, oliguria and acute
kidney injury (AKI) are early and frequent consequences of ACS We performed an autopsy after obtaining consent from his fam-
and can be present at relatively low levels of IAP [5]. That is- ily. At the time of death, the amount of abdominal ascites had
sue has not been taken into account in the definition of HRS. reached 10 L, even though LVP was performed a few days be-
Song et al. described the role of urea transporter protein with fore. The liver weighed 1973 g with no signs of severe atrophy.
in the state of HRS and ACS by successfully making ACS mu- There was significant accumulation of neutrophils and severe
rine model [6]. Chang et al. suggested that renal pathologic cholestasis in the bile duct. In the portal area, there was bridging
changes occur due to massive abdominal ascites as gleaned fibrosis and aggregation of Kupffer cells. The primary cause of
from studying a murine model of cirrhosis [7]. However, there hepatic failure was thought to be sepsis-associated liver injury.
are no reports indicating that kidney lesions with massive ab-
dominal ascites due to subacute liver failure may pathological- There were signs of congestion in the kidneys – weight 301 g
ly contribute to HRS. This autopsy case report serves to high- in the right kidney and 345 g in the left kidney – upon dis-
light kidney pathology when HRS and massive ascites occur. section (Figure 2). Regarding bilateral renal congestion, there
were bile casts and advanced erythroid fullness in the renal
tubules. There was also swelling in the renal tubules. There
Case Report was no change in the glomerulus and collecting tubule and no
renal fibrosis (Figure 3). When we measured bladder pressure
A 40-year-old man with acute liver failure was admitted to after LVP, it was 20 mmHg, which is in the range of intraab-
our hospital. He had undergone coronary artery bypass graft- dominal hypertension (IAH). IAH was due to massive ascites.
ing because of angina pectoris 3 years ago. He had no pre- Additionally, we thought that the observed renal congestion
vious history of renal and liver disease. He had no-smoking was due to renal vein compression, which was caused by a
history and drank socially. He was previously hospitalized else- large quantity of abdominal ascites. Thus, we concluded that
where for septic shock due to acute epiglottitis. He recovered this patient had HRS-1 and ACS at the same time.
from septic shock, but he had unrecoverable liver dysfunc-
tion and his hepatic spare ability had decreased. Therefore,
he was transferred to our hospital 30 days after the disease Discussion
onset. On admission, he had severe jaundice and massive as-
cites and showed flapping tremor; however, his conscious- Although liver diseases cause irreversible renal dysfunction,
ness was lucid. He was severely obese (body weight: 120 kg, such as hepatitis C virus-induced cryoglobulinemia, membra-
body mass index: 41.5). Physical examination showed a high noproliferative glomerulonephritis, HRS causes reversible re-
fever of 38.2°C, pulse of 72 beats/min, and blood pressure of nal failure [8,9]. HRS is difficult to clinically distinguish from
116/70 mm/Hg. Laboratory results showed decreases of pro- primary renal failure. According to the definition of HRS from
thrombin time (38%), albumin (2.1 g/dL), and cholinesterase the International Ascites Club (IAC) proposed in 2007, HRS is
(49 IU/L), and increases of white blood cell count (22 300/µL), divided into 2 types (1 and 2) based on prognosis and clini-
total bilirubin (26.4 mg/dL), creatinine (2.1 mg/dL), C-reactive cal characteristics [2].

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HRS and ACS with high renal congestion
© Am J Case Rep, 2017; 18: 1000-1004

Table 1. Laboratory data on admission.

(CBC)

WBC 22300/μl (Chemistry) ANA <40

Neut 87% T-bil 26.4 mg/dl AMA (–)

Eo 2.90% D-bil 20.5 mg/dl HBsAg (–)

Baso 0.40% AST 107 IU/l anti-HBs (–)

Mon 3.20% ALT 46 IU/l anti-HCV (–)

Lymph 37.40% ALP 446 IU/l (Urine examination)

RBC 352×104/μl LDH 621 IU/L Specific gravity 1.018

Hb 12.2 g/dl CHE 256 IU/l pH 6

Ht 40.30% g-GT 83 IU/l Protein 30 mg/dl

Plt 12.4×10 /μl4

TP 7.1 g/dl Occult blood 10–19/HP

Alb 2.1 g/dl Ketone (–)

(Coagulation) CRP 4.14 mg/dl

PT 38% T-Cho 145 mg/dl

APTT 60.0 sec TG 107 mg/dl

fibrinogen 227 mg/dl BUN 65 mg/dl

Cr 2.09 mg/dl

NH3 121 µg/dl

Alb – albumin; ALP – alkaline phosphatase; ALT – alanine aminotransferase; ANA – antinuclear antibody; AMA – antimitochondrial
antibody; APTT – activated partial thromboplastin time; AST – aspartate aminotransferase; BUN – blood urea nitrogen; CBC – complete
blood count; CHE – cholinesterase; Cr – creatinine; CRP – C-reactive protein; D-bil – direct bilirubin; Hb – hemoglobin; Ht – hematocrit;
LDH – lactate dehydrogenase; Plt – platelets; PT – prothrombin time; RBC – red blood cells; T-bil – total bilirubin; T-chol – total
cholesterol; TP – total protein; WBC – white blood cells; g-GT – g-glutamyltransferase.

A B

Figure 1. C
 omputed tomography of the abdomen. (A) Massive ascites and obesity was observed (arrow). (B) The pelvic viscera was
compressed by the ascites (arrow).

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HRS and ACS with high renal congestion
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Figure 2. M
 acroscopic findings of the kidney. Macroscopic Figure 3. Microscopic findings of the kidney. Microscopic
findings showed bilateral renal congestion. findings showed swelling in the renal tubules. There
was no change in the glomerulus and collecting
tubule and no renal fibrosis. There were bile casts and
The patient had no reaction to rehydration therapy and albu-
advanced erythroid fullness in the renal tubules.
min administration, and he had no previous history of renal dys-
function. He had recovered from bacteremia and his blood pres-
sure was maintained. Clinically, he was originally diagnosed as vein pressure and renal vascular resistance are significantly el-
HRS-1. We did not find major evidence of renal tubule necro- evated [16]. It is suggested that compression of renal vein is
sis in the autopsy; otherwise, we found some bile casts caused vital in the development renal dysfunction [17]. IAH is the sig-
by elevation of serum bilirubin, which affect renal function. To nificant pathological mechanism and independent risk factor in
maintain effective plasma circulation, an albumin infusion is the occurrence and development of HRS.
first performed. In addition, albumin treatment is combined with
terlipressin as the first-line treatment, or midodrine and nor-
adrenaline are used as alternatives [10]. We administrated nor- Conclusions
adrenaline, which was permitted by health insurance. However,
regardless of treatment choice, the effect is temporary, and a liv- There are effective treatments of ascites to control IAP accord-
er transplant is the only treatment that can extend patient sur- ing to pathophysiology. Currently, tolvaptan effectively treats
vival. The 3-year survival rate after a liver transplant of an HRS- hepatic edema and ascites-related clinical symptoms in liver
1 patient is reported to be 65% [11]. Various factors concern cirrhosis patients who do not respond sufficiently to conven-
the etiology of HRS, and the definition is corrected as needed. tional diuretics [18]. In the case of acute liver failure and a ter-
Wong et al. reported new diagnostic criteria of HRS in cirrhosis minal cirrhosis patient, the final lifesaving effort is a liver trans-
patients, jointly proposed by the IAC and Acute Dialysis Quality plant, but while the patient waits for the opportunity, renal
Initiative. In the new criteria, HRS-1 and HRS-2 are both incor- dysfunction becomes a high-risk factor affecting survival [19].
porated in AKI and chronic kidney disease (CKD) [12]. AKI is a
relatively frequent problem, occurring in approximately 20% of In liver cirrhosis, because of the dynamic change of blood flow
hospitalized patients with cirrhosis [13]. In the autopsy of this that may cause rupture of esophageal varices and fall in blood
case, both kidneys showed severe renal congestion. ACS is de- pressure, LVP is often avoided. Renal function is reversible for
fined as hypoperfusion and ischemia of intraabdominal viscera HRS patients as aforementioned, but in the case of liver cirrho-
and structures caused by raised IAP. It causes lethal conditions, sis, there may be advanced renal insufficiency when massive
with acute renal, acute respiratory, and acute circulatory failure. ascites exists. Thus, we have to take into consideration ACS
Cade et al. reported a significant increase in urine flow rate and and ACS-mediated renal dysfunction. For HRS patients, main-
Ccr after reduction in IAP, from 22 to 10 mmHg, with paracente- tenance of renal function is critical to improve the MELD score
sis in patients with cirrhosis [14]. ACS is diagnosed upon mea- and patient survival. Currently, ACS is not mentioned in the def-
suring IAP by the pressure transducer and is often found in de- inition of HRS, but we think, from this case, that the preven-
creased eGFR in the presence of ascites in the early stage [15]. tion of ACS at an early stage is crucial for prevention of HRS.
IAP in a normal individual ranges from slightly sub-atmospheric
to approximately 6.5 mmHg. Recent studies demonstrated that Conflict of interest
even at the relatively low IAP of 10–15 mmHg, significant alter-
ations in organ function are still observed. In this state, renal None declared.

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HRS and ACS with high renal congestion
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