CASE 10937 Published on 22.04.2013

Varied complications of multicystic liver

Section

Abdominal imaging

Case Type

Anatomy and Functional Imaging

Authors

"Luigi Sacco" University Hospital,
Radiology Department;
Via G.B. Grassi 74
20157 Milan, Italy;
Email:mtonolini@sirm.org

Patient

88 years, female

Categories

Area of Interest Liver, Kidney ; Imaging Technique Percutaneous, CT

Procedure Puncture, Diagnostic procedure, Dilation ; Special Focus Cysts, Haemorrhage ;

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Clinical History

Elderly woman with history of chronic congestive heart failure, diabetes, and previously treated pulmonary thromboembolism, hospitalised because of vomiting and reduced food intake for one week. Physical examination revealed non-tender mass at palpation in the right upper abdomen.
No significant abnormalities of routine laboratory tests including liver and renal function.

Imaging Findings

Two months earlier, CT (Fig. 1) performed to exclude inferior vena cava (IVC) thrombosis showed multiple, mostly large-sized simple liver cysts causing marked diaphragm elevation and moderate intrahepatic bile duct dilatation, with normal kidneys.
Currently, CT (Fig. 2) detected enlarged dominant hepatic cyst corresponding to physical findings, increased intrahepatic biliary dilatation, and IVC compression. Ultrasound-guided percutaneous drainage (Fig. 3) was effectively performed to relieve symptoms, biliary and vascular compression, yielding sterile serous fluid. She was discharged under long-term low-molecular-weight heparin anticoagulation.
Six months later, she presented to emergency with acute abdominal pain, hypotension, and signs of blood loss (6.5 g/dl haemoglobin). Unenhanced CT because of renal impairment (Fig. 4) disclosed enlarged dominant right lobe cyst with dependent hyperattenuating material from intracystic haemorrhage.
The next day, with normalised renal function following rehydration contrast-enhanced CT (Fig. 5) detected focal venous-phase contrast extravasation indicating active bleeding.
Considering comorbidities, conservative treatment with anticoagulant medications withdrawal and blood transfusions was chosen, ultimately resulting in recovery.

Discussion

Either sporadic or in hereditary polycystic conditions, hepatic cysts (HC) are commonly encountered in the general population, with an estimated 2.5-5% prevalence and a female predominance. Often incidentally detected on imaging studies performed for various reasons, in the vast majority of patients HC are asymptomatic, not associated with altered hepatic function, and do not require further workup nor treatment. Some patients, particularly those with polycystic liver disease (PLD), may complain of symptoms related to mass effect, such as hepatomegaly, abdominal distension, early satiety, intermittent or continuous abdominal pain, supine dyspnoea due to diaphragm elevation [1-3].
In most cases, simple HC with serous fluid content are confidently diagnosed on the basis of their characteristic anechoic sonographic appearance with posterior through-transmission, at CT as well-demarcated hypoattenuating lesions with homogeneous water-density, imperceptible walls, absent internal and mural enhancement [1, 2].
As this case exemplifies, in patients with PLD or large-sized HC serious complications may be encountered, including obstructive jaundice from mass effect, compression of portal vein and/or inferior vena cava, superinfection, haemorrhage, or rupture [1-3].
An uncommon occurrence, intracystic haemorrhage manifests with acute upper abdominal pain in 80% of patients, often accompanied by fever, and may represent a life-threatening condition. Moreover, the presence of giant SHC can be considered a relative contraindication for anticoagulation. Conversely, some patients with haemorrhagic HC are asymptomatic [4-9].
As in this patient under anticoagulant therapy, intracystic haemorrhage is suggested at imaging by cyst enlargement, the appearance of hyperattenuating dependent material at unenhanced CT during the first 24-72 hours, or by complex sonographic appearance with hyperechoic components. Furthermore, CT allows detecting or excluding contrast extravasation indicating ongoing bleeding, and associated haemoperitoneum indicating cyst rupture. The differential diagnosis of non-traumatic bleeding liver lesions includes mostly hepatic adenoma, hepatocellular carcinoma, metastases, and HELLP syndrome. Furthermore, the presence of mural thickening and/or irregularities may cause diagnostic uncertainty with the even more uncommon cystic hepatic tumours [2, 6, 10].
Whereas uncomplicated HC do not deserve treatment, enlarging or symptomatic cysts may be treated with aspiration, fenestration, or marsupialisation. Haemorrhagic HC may be managed conservatively, or otherwise require laparoscopic or open surgical treatment. In patients with PLD, partial hepatic resection or liver transplantation may be required [4-9].

Differential Diagnosis List

Polycystic liver disease with intrahepatic biliary obstruction, intracystic haemorrhage.

Simple liver cysts

Uncomplicated polycystic liver disease

Pyogenic liver abscess

Amoebic abscess

Hydatid cyst

Haemorrhagic hepatocellular adenoma

Bleeding hepatocellular carcinoma

Bleeding metastases

Liver cystadenoma / carcinoma

Final Diagnosis

Polycystic liver disease with intrahepatic biliary obstruction, intracystic haemorrhage.

References

[1] Vachha B, Sun MRM, Siewert B, et al. (2011) Cystic lesions of the liver. AJR Am J Roentgenol 196:W355-W366 (PMID: 21427297)

[2] Mortelè KJ, Ros PR (2001) Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics 21:895-910. (PMID: 11452064)

[3] Morgan DE, Lockhart ME, Canon CL, et al. (2006) Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics 26:1655-1668 (PMID: 17102042)

[4] Agrawal S, Khurana J, Sahu M (2012) Hemorrhagic liver cyst. J Gastrointest Surg 16:1629-1631 (PMID: 22350722)

[5] Carels RA, van Bommel EF (2002) Ruptured giant liver cyst: a rare cause of acute abdomen in a haemodialysis patient with autosomal dominant polycystic kidney disease. Neth J Med 60:363-365 (PMID: 12572709)

[6] Fong ZV, Wolf AM, Doria C, et al. (2012) Hemorrhagic hepatic cyst: report of a case and review of the literature with emphasis on clinical approach and management. J Gastrointest Surg 16:1782-1789 (PMID: 22688416)

[7] Kawano Y, Yoshida H, Mamada Y, et al. (2011) Intracystic hemorrhage required no treatment from one of multiple hepatic cysts.. J Nippon Med Sch 78:312-316 (PMID: 22041878)

[8] Takahashi G, Yoshida H, Mamada Y, et al. (2008) Intracystic hemorrhage of a large simple hepatic cyst. J Nippon Med Sch 75:302-305 (PMID: 19023172)

[9] Yoshida H, Onda M, Tajiri T, et al. (2002) Intracystic hemorrhage of a simple hepatic cyst. Hepatogastroenterology 49:1095-1097 (PMID: 12143211)

[10] Casillas VJ, Amendola MA, Gascue A, et al. (2000) Imaging of nontraumatic hemorrhagic hepatic lesions. Radiographics 20:367-378 (PMID: 10715337)

Case information

URL:	https://www.eurorad.org/case/10937
DOI:	10.1594/EURORAD/CASE.10937
ISSN:	1563-4086

Figure 1

Initial contrast-enhanced CT including multiplanar image reformations

Two months earlier, contrast-enhanced axial (a..d, in cranio-caudal order), coronal (e) and sagittal (f) reformatted images showed several, mostly large-sized simple liver cysts with fluid attenuation.

Moderate dilatation of some intrahepatic bile ducts (arrowheads) was noted, caused by compression from one of the largest cysts. The inferior vena cava is patent and not significantly compressed.

Several, mostly large-sized simple liver cysts with fluid attenuation are present throughout the liver. The inferior vena cava is patent and not significantly compressed. No polycystic kidney changes were noted.

Coronal (d) and sagittal (f) reformatted images effectively show occupation of the liver by several, confluent giant hepatic cysts, causing marked right diaphragm elevation.

Coronal (d) and sagittal (f) reformatted images effectively show occupation of the liver by several, confluent giant hepatic cysts, causing marked right diaphragm elevation. Note moderate dilatation of intrahepatic bile ducts (arrowhead).

Unenhanced (f,g), arterial-dominant (h) and venous (i) phase images show both kidneys with normal size, parenchymal thickness and contrast enhancement for age, without cysts and hydronephrosis.

Figure 2

Repeat contrast-enhanced CT

Currently, axial images (a..c) show enlargement of the dominant simple hepatic cyst (compared to Fig.1b) corresponding to physical finding, and increased dilatation of intrahepatic bile ducts (arrowheads).

The enlarged dominant simple hepatic cyst causes increased dilatation of intrahepatic bile ducts (arrowheads), and compression of the inferior vena cava.

Coronal reformatted images (d,e) better depict increased dilatation of intrahepatic bile ducts (arrowheads), caused by compression of the dominant cyst on the liver hilum.

Figure 3

Percutaneous ultrasound-guided drainage & Post-drainage follow-up contrast-enhanced CT

Ultrasound-guided percutaneous puncture (a) and drainage positioning (arrowhead in b) was performed on the dominant hepatic cyst, yielding sterile serous fluid.

Days later, post-procedural follow-up contrast-enhanced CT showed dominant hepatic cyst partially collapsed with drainage tube in place (arrowhead).

Figure 4

Urgent unenhanced CT at acute presentation

Six months after hospital discharge, urgent unenhanced acquisition because of impaired renal function showed enlarged dominant hepatic cyst, with hyperattenuating material (*) consistent of fresh blood in its dependent part. Note moderate non-haemorrhagic peritoneal effusion.

Urgent unenhanced acquisition due to impaired renal function showed enlarged dominant hepatic cyst, filled by hyperattenuating material (*) consistent of fresh blood. Note moderate non-haemorrhagic peritoneal effusion.

Coronal reformatted unenhanced images (c, detail in d) confirm enlarged dominant hepatic cyst, with hyperattenuating material (*) consistent with fresh blood in its dependent part. Note moderate non-haemorrhagic peritoneal effusion.

Figure 5

Repeat contrast-enhanced CT 24 hours later

With improved renal function, 24 hours later contrast-enhanced CT including arterial (a,b) and venous (c..e)-phase acquisitions shows stable hemorrhagic (*) giant liver cyst, persistent non-haemorrhagic peritoneal effusion.

In the arterial phase acquisition, stable haemorrhagic (*) giant liver cyst is seen without appreciable contrast extravasation indicating active bleeding.

Axial (c) and coronal (d, detail in e) venous phase images show giant haemorrhagic (*) hepatic cyst with small foci (arrows) of contrast extravasation indicating active bleeding. Persistent non-haemorrhagic peritoneal effusion.