Haemorrhagic rupture of hepatic simple cysts

Abstract

Haemorrhagic rupture is a life-threatening complication of a hepatic simple cyst. A 63-year-old man presented with severe acute abdominal pain and a massive haemoperitoneum resulting from haemorrhagic rupture of a large hepatic cyst. The haemorrhagic rupture was aggravated by an overdose of vitamin K-antagonist treatment. CT scans revealed a large hepatic simple cyst. The patient was successfully treated conservatively with resuscitation, transfusion therapy and administration of coagulation agents. To date, there is no clear evidence regarding optimal treatment of haemorrhagic hepatic cyst rupture. The risk of recurrent bleeding from the haemorrhagic hepatic simple cyst, and the need for final treatment to avoid rebleeding either by percutaneous sclerotherapy, endovascular embolisation, surgical cyst resection, or surgical deroofing, is discussed.

Background

Haemorrhagic rupture is a very rare and life-threatening complication of a hepatic simple cyst. Until now, very few cases have been described in the literature.¹ Hence, little is known about the aetiology, that is, why some hepatic cysts bleed and others do not, and there is no consensus on best treatment and follow-up for this uncommon complication.¹ The estimated prevalence of hepatic simple cysts varies from 2.5–4.1%. Hepatic cysts are usually asymptomatic.² They may become symptomatic when increasing in size, which occurs in 5% of cases.³ The symptoms include upper abdominal pain or discomfort, bloating, nausea, vomiting, decreased appetite and dyspnoea.

The patient described in this case presented at the emergency department with acute haemorrhagic rupture of a liver cyst. The critical presentation of the patient and the difficulties we encountered in the treatment process are presented below.

Case presentation

A 63-year-old man had a medical history of recurrent deep venous thrombosis for which he was treated with life-long acenocoumarol. He presented at the emergency department with acute onset of pain in the right hypochondrium. He had attended his general practitioner with abdominal complaints 1 week prior to the onset of the pain. The abdominal pain was attributed to constipation, and oral laxatives were prescribed.

Investigations

Because of persisting complaints he underwent a CT-scan of the abdomen in a private clinic. This CT-scan showed hepatic simple cysts with one large cyst of 13×14×14 cm in segment four of the liver (figure 1).

Figure 1.

Abdominal CT-scan showing a large hepatic simple cyst.

Physical examination revealed tenderness in the right upper quadrant of the abdomen and hepatomegaly. Laboratory results showed a haemoglobin level of 9.7 g/L, white cell count was 16.6×10/L, C reactive protein 8.2 mg/dL, international normalised ratio (INR) was 11.0 and partial thromboplastine time (PTT) indicated 54 s. At initial presentation, vital signs were normal, but during examination the blood pressure dropped and the patient developed tachycardia.

Treatment

The patient was immediately resuscitated with intravenous saline, after which he became haemodynamically stable. When the results of the earlier CT-scan became accessible, the patient was assumed to have hypovolemic shock caused by a ruptured haemorrhagic liver cyst. To confirm our differential diagnosis and to evaluate for ongoing bleeding, an intravenous contrast enhanced CT-scan of the abdomen was performed. This CT-scan showed haemorrhage within the large hepatic cyst and a rupture of the cyst, though without signs of active bleeding (figure 2). Nevertheless, the patient became haemodynamically unstable again after the CT-scan; the systolic blood pressure dropped and the patient developed tachycardia. Again the patient underwent an intravenous fluid challenge with saline, to which he responded. Given the prolonged coagulation time, and no indications for active bleeding seen on the CT-scan, the patient was treated conservatively. He received intravenous tranexamic acid, red blood cell transfusion and fresh frozen plasma transfusion. Initially, the patient became stable, and a haemoglobin level of 8.5 g/L was attained. Therapeutic low-molecular-weight heparin was started because of the medical history of recurrent deep venous thrombosis. Nevertheless, the patient subsequently developed dyspnoea; at that time the INR was 1.1. A CT-angiography of the thorax confirmed bilateral pulmonary embolism. Owing to the high risk of rebleeding, the patient was treated with continuous heparin infusion at a rate of 20 000 units per 24 h, with strictly haemodynamic and coagulant monitoring including daily control of the INR and PTT in the intensive care unit.

Figure 2.

Hepatic cyst with haemorrhage, and free intra-abdominal fluid around the liver and spleen.

When the INR was within the therapeutic range of 2–3, the heparin infusion was discontinued, and anticoagulation was further regulated with vitamin-K antagonists and strict INR monitoring.

Outcome and follow-up

The patient recovered uneventfully and was discharged after 31 days with lifelong anticoagulation treatment. CT-scan performed 6 months after the event showed that the hepatic cysts decreased in size and the hepatic cyst was without signs of malignancy. Eight months after the event the patient was without symptoms and remained event free.

Discussion

This case describes a rare and life-threatening complication of hepatic cysts. To date, only few cases of haemorrhagic hepatic cyst rupture have been reported in the literature. All these cases emphasise the difficulties encountered in the treatment process.

There are different treatment options for patients presenting with bleeding from a hepatic simple cyst. These treatment options vary from observation or a conservative strategy with intravenous fluid and blood product administration to more invasive procedures such as radiological drainage and sclerotherapy, coiling or operative procedures.⁴

Fong and colleagues reviewed all publications reporting on haemorrhagic hepatic cysts until the year 2012. They identified 24 cases; 3 (12.5%) patients were treated conservatively. Seven (30%) were treated with transhepatic percutaneous drainage; two of these seven patients had recurrence of a ruptured hepatic cyst. Two (8%) patients were treated by open surgical deroofing of their haemorrhagic hepatic cyst and one patient (4%) underwent laparoscopic deroofing of the cyst. None of these patients developed recurrent bleeding of the hepatic cyst. Eight (33%) patients underwent hepatic resection; of these, in three cases the method was not specified, in one patient a left trisegmentectomy was performed, two underwent a left hemihepatectomy and the remaining two underwent a right hemihepatectomy. All these patients recovered uneventfully. Furthermore, 3 (12.5%) patients underwent enucleation of the haemorrhagic hepatic cyst.

The treatment options in the present case were limited. First of all, immediate surgery was contraindicated at that time because the INR was 11.0 at arrival in the emergency department and, besides, no active bleeding was observed on CT-angiography. The intracystic bleeding had most probably been aggravated due to an over-dosage of anti-vitamin K (INR 11.0). To date, only one other case has been described where haemorrhagic rupture within a hepatic cyst occurred in a patient receiving acenocoumarol with high INR (7.9).⁵ In this case an exploratory laparotomy was performed and omentum was placed over the ruptured cyst. Yet, the patient was persistently haemodynamically unstable and died 4 weeks after hospital admission.

Arterial embolisation of a branch of the hepatic artery was another possible treatment option in the present case. To date, arterial embolisation was performed for rupture of a hepatic cyst in only three patients described in the literature.^{6 7} In one case the patient underwent transhepatic drainage with concurrent transcatheter arterial embolisation with subsequent clinical improvement. However, the cyst had increased in size 3 weeks after the arterial embolisation procedure and a simple cystectomy was performed. The other two patients were treated by transcatheter hepatic arterial embolisation and were successfully managed non-operatively.⁷ The cases described in the literature emphasise the trade-offs within the different treatment options. No clear evidence exists on which treatment option to use. Our case was particularly difficult because of the dilemma between adequate anticoagulation in light of the patient's known history of deep venous thrombosis and the development of bilateral pulmonary embolisms, and the risk of rebleeding of the liver cyst. In case of life-threatening bleeding, current guidelines recommend stopping anticoagulation treatment and administering fresh frozen plasma, prothrombin complex concentrate, or recombinant factor VIIa supplemented with vitamin K (10 mg) by slow infusion. If necessary, vitamin K supplementation should be repeated. Nowadays, laparoscopic deroofing is a frequently applied treatment of symptomatic liver simple cysts and might remove symptoms of pain, abdominal discomfort, dyspnoea, vomiting and nausea. The treatment of simple hepatic cysts by laparoscopic deroofing provides a tissue sample for pathological confirmation of the lesion, low recurrence rates, complete relief of symptoms with minimal morbidity and a short hospital stay.⁸ However, laparoscopic deroofing should not be performed in case of active bleeding of a haemorrhagic cyst.

Percutaneous ethanol sclerotherapy is applied for patients with symptomatic hepatic cysts with good results.⁹ Similar to laparoscopic deroofing, percutaneous ethanol sclerotherapy is contraindicated in patients with active bleeding, though it might have a role in treatment of hepatic cysts with haemorrhagic content when the bleeding has stopped.

The role of tranexamic acid for bleeding of hepatic cysts is unclear, though a meta-analysis has shown that tranexamic acid is beneficial in reducing surgical bleeding.⁹

Hepatic simple cysts are by far the most common cystic lesions occurring in the liver. They should be differentiated from biliary cystadenomas and biliary cystadenocarcinomas.¹⁰

Biliary cystadenomas can transform to malignant lesions and should therefore be treated with surgical resection. Biliary cystadenomas often have septa within the cyst and a thicker wall than hepatic simple cysts.¹⁰

Learning points.

• Though extremely rare, rupture within a hepatic cyst should be considered in the differential diagnosis of acute abdomen, particularly in persons known to have hepatic cysts. Furthermore, patients with a known history of hepatic simple cysts presenting with abdominal pain and additional signs of hypovolemic shock, might suffer from haemorrhagic cyst rupture.

• To date, there is no clear evidence on optimal treatment for haemorrhagic hepatic cyst rupture. Patients might be treated conservatively with adequate fluid transfusion therapy, treated by selective endovascular hepatic artery embolisation, or treated by open surgery. If acute surgery is relatively contra-indicated, as in the present case, conservative treatment with active surveillance might be the most appropriate treatment method.

• The risk of recurrent bleeding from the haemorrhagic hepatic cyst and the need for final treatment to avoid rebleeding either by percutaneous sclerotherapy, endovascular arterial embolisation, laparoscopic or open surgical cyst resection or deroofing, is also unclear.

• Hepatic simple cysts are by far the most common cystic lesions in the liver and should be differentiated from biliary cystadenomas and biliary cystadenocarcinomas. Biliary cystadenomas can transform to malignant lesions and should therefore be treated with surgical resection.