Spontaneous muscle haematoma in a patient with cirrhosis

Abstract

Decompensated liver disease is associated with alterated haemostasis that can either lead to spontaneous bleeding or development of thrombosis. Alcohol consumption coupled with advanced liver disease favours spontaneous bleeding. There have been only few documented cases of spontaneous muscle haematoma (SMH) in patients with cirrhosis. The pathogenesis of SMH is hypothesised to be multifactorial and it has been seen in patients on anticoagulation or with haemostatic disorders. We report a case of alcohol-related cirrhosis presenting with an expanding, voluminous haematoma in the intermuscular plane between the trapezius and the teres major muscles. This patient also had a retroperitoneal haemorrhage, clinically evidenced by the Grey Turner’s and Cullen’s signs. Haemorrhage was confirmed radiologically by CT. The patient was managed in an intensive care facility and treated with multiple blood products, including packed red blood cells, fresh frozen plasma and cryoprecipitates. However, as his clinical condition deteriorated, he required surgical intervention by incision and drainage, followed by evacuation. Early identification of coagulopathy and aggressive treatment are essential in these cases of cirrhosis to avoid unfavourable outcomes.

Background

Cirrhosis can predispose to either a prothrombotic or an antithrombotic state. Possible mechanisms that explain increased bleeding in cirrhosis include thrombocytopaenia, low procoagulant factors, low antiplasmin and platelet dysfunction.¹ Alcohol consumption is known to inhibit platelet aggregation, causing premature activation of platelets and reduction of components such as von Willebrand factor (vWF), factor VII and fibrinogen.² In recent years, there have been few case reports on spontaneous muscle haematoma (SMH) in patients with advanced cirrhosis, mostly involving the iliopsoas muscle and the rectus sheath.³ SMH in patients with advanced cirrhosis is associated with a high mortality rate of up to 75%.⁴ Hence, early recognition and prompt treatment remain the cornerstone of management of these patients.

Most of the reported cases of SMH are secondary to the use of anticoagulants, suggesting a defect in the coagulation system. The mechanisms involved in spontaneous bleeding include hyperfibrinolysis and multifactorial microangiopathy.⁵ We hereby present a case of SMH in the intermuscular plane between the trapezius and the teres major muscles, along with retroperitoneal haemorrhage clinically evidenced by the Grey Turner’s and Cullen’s signs, in a patient with cirrhosis with a Child-Pugh Turcot score of B⁶ at the time of hospitalisation.

Case presentation

An adult man in his 50s diagnosed with cirrhosis with portal hypertension 4 months ago presented with progressive swelling in his right posterolateral chest wall of 8×2 cm in size for a week duration (figure 1). For the past 3 days, there had been bluish discolouration on his right flank that extends to the umbilicus (figures 2 and 3). The patient has been consuming around 6.1 ounces of brandy daily for the past 30 years (143 g of alcohol daily). He denies any history suggestive of bleeding tendencies, such as haematemesis, blood in urine or stools, epistaxis or bruising of joints, or any trauma. He does not take non-steroidal anti-inflammatory drugs, aspirin or anticoagulants, nor does he have a family history contributing to any bleeding disorders. The rest of his surgical and medical histories were unremarkable.

Figure 1.

Haematoma in the upper back.

Figure 2.

Haemorrhage around the umbilicus suggestive of Cullen’s sign.

Figure 3.

Haemorrhage in the retroperitoneum suggestive of Grey Turner’s sign.

On examination, he was conscious and afebrile, with a pulse rate of 100 per minute, blood pressure of 110/80 mm Hg and body mass index of 22. He looked pale and was icteric. There were no signs of hepatic encephalopathy. Local examination revealed a progressive swelling extending from the right posterolateral chest wall to the back, with dimensions of 8 cm (transverse) and 7 cm (anteroposterior), with warmth overlying the skin and with tenderness. Ecchymosis was seen in the right lumbar region, crossing past the umbilicus (figures 2 and 3). Systemic examination revealed a distended abdomen with evidence of ascites and an enlarged spleen with a smooth margin extending up to 6 cm below the costal margin.

Investigations

Laboratory parameters showed pancytopaenia with haemoglobin of 6.1 g/dL, thrombocytopaenia of 60 x 10⁹/L and leucopenia of 1.5 x 10⁹/L. Liver function test showed a total bilirubin of 2.23 mg/dL and direct bilirubin of 0.85 mg/dL, serum protein of 7.1 mg/dL, albumin of 2.8 mg/dL, aspartate transaminase of 54 IU/mL, alanine transaminase of 12 IU/mL and ammonia level of 48.7 mmol/L. His viral serology for hepatitis B and C was negative. His prothrombin time was 15.5 s, activated partial thromboplastin time was 35.6 s and the international normalised ratio (INR) was 1.34. His Model for End-Stage Liver Disease (MELD) score was 22 and his Child-Pugh score was 7, suggesting moderately severe liver disease.

Abdominal ultrasound revealed a coarse echo texture in the liver with moderate splenomegaly and mild ascites. Upper gastroduodenoscopy showed grade I oesophageal varix. Ultrasonogram of the upper back showed a heteroechoic collection 7.6×1×1.1 cm in size with a volume of 10 mL approximately noted in the right infrascapular region. As the swelling progressively increased, a repeat imaging showed that the collection had increased to 17×8×3 cm, extending from the fourth to the ninth rib. A contrast-enhanced CT of the chest revealed a well-defined heterogeneous collection with multiple hyperdense areas within the intramuscular plane, suggestive of intramuscular haematoma (figures 4 and 5). A CT arteriogram of the abdomen showed no active bleeding vessels.

Figure 4.

Contrast CT showing the muscle haematoma (arrow): arterial phase.

Figure 5.

Contrast CT showing the muscle haematoma (arrow): venous phase.

Differential diagnosis

Muscle haematoma due to a bleed from a feeding artery was ruled out with the help of a CT arteriogram. Concomitant use of drugs that might predispose to bleeding was also ruled out based on our patient’s history. Underlying bleeding disorders were also ruled out as there were no previous episodes of bleeding diathesis in childhood. Intense contraction of the recti muscles, as in the Valsalva manoeuvre, vomiting, straining or severe retching can cause bleeding, which were not applicable to our patient. Strangulated hernia, torsion of the ovarian cyst in females, perforation of the intestine, abdominal neoplasm and appendicitis, which are differentials for muscle haematoma, were ruled out in our patient based on imaging of the abdomen.

Treatment

On admission, due to the underlying coagulopathy evidenced by a deranged INR, the patient was started on intravenous injection of vitamin K, which was given for 3 days, and multiple units of fresh frozen plasma were transfused as per serial measurements of coagulation parameters. Packed red blood cells (PRBCs) were transfused and his haemoglobin increased to 8.6 g/L. As the haematoma increased in size due to active bleeding within the intermuscular plane, cryoprecipitates were transfused on successive days as per the requirements, which are high concentrates of fibrinogen. He did not require any platelet transfusion since his platelet values were always above 50 x 10⁹ /L . Despite the above conservative measures, the patient’s condition spiralled downward, and given the expanding haematoma he was transferred to intensive care. After explaining to the patient and the attendants his deteriorating clinical status despite the above measures and the limited evidence of surgical interventions in this subset of patients, consent was obtained and surgical evacuation of the haematoma proceeded under local anaesthesia. About 350 mL of blood drained. Simultaneously fresh frozen plasma and PRBCs were transfused to prevent haemodynamic instability. The haematoma stabilised and the patient did not have any further bleeding and was subsequently discharged.

Outcome and follow-up

The patient was hospitalised for 18 days. After the surgical intervention, there was no further increase in haematoma and he was discharged in a stable condition. Health education about the deleterious effects of alcohol was given. On follow-up in the outpatient clinic, the haematoma had resolved.

Discussion

Chronic liver disease is associated with a myriad of complications. One such vital complication is alterations in haemostasis, such as bleeding or thrombosis. SMH in cirrhosis is unusual and there is limited information available in the literature. Muscle haematoma may be contained within the muscle fascia or can enlarge and diffuse into the retroperitoneal or peritoneal spaces.⁷ SMH occurs in three anatomical regions: the anterior abdominal musculature, the posterior abdominal musculature and the muscles in the gluteal region. Abdominal haematomas may be intra-abdominal or abdominal wall haematomas. Abdominal wall haematoma results from bleeding into the muscle layers of the abdominal wall, most commonly involving the rectus abdominis muscle. Bleeding occurs due to the involvement of the inferior or superior epigastric arteries, veins or tributaries. Rectus sheath haematoma most commonly occurs on the right side. Haemorrhage that occurs above the arcuate line remains confined but displaces the rectus sheath to the opposite side. If the bleeding is below the arcuate line, the haemorrhage extends laterally deep to the oblique muscle aponeurosis,⁸ involving the long and proximal epigastric branches. About 5 cm below the umbilicus, all the aponeuroses pass in front of the rectus abdominis muscle and fascia transversalis into the prevesical space due to the lack of tamponade effect. As the inferior epigastric artery penetrates the rectus muscle at the level of the arcuate line, the blood vessels are relatively fixed and are more prone to injury (figure 6).

Figure 6.

(A) Cross-section of the rectus sheath and (B) sagittal section through the abdominal wall. Image provided by Nanthakumar Logithasan.

Some of the causes of spontaneous haematomas include haemorrhagic disease, neoplasms and arterial disease. Spontaneous muscular haematomas have the potential to develop into life-threatening complications. The incidence of SMH in patients who receive anticoagulation is 5%, with an annual mortality rate of 0.65%.⁹ The iliopsoas and rectus muscles are the most common sites for SMH,^{10 11} and risk factors include haemodynamic disorders, elderly population, atheromatous lesions, vascular lesions from chronic arterial hypertension and diabetes.

The pathogenesis of SMH is complex, based on multifactorial microangiopathy,⁵ and is anecdotal. Cirrhosis can produce thrombocytopaenia, a defect in coagulation factors and an alteration in the fibrinolytic pathways (figure 7).

Figure 7.

Trends of prohaemostatic and antihaemostatic drives in different phases of haemostasis in chronic liver disease. Image provided by Nanthakumar Logithasan. AT -III, antithrombin III; PAI, plasminogen activator inhibitor; TAFI, thrombin-activatable fibrinolysis inhibitor; TFPI, tissue factor pathway inhibitor; t-PA, tissue plasminogen activator; vWF, von Willebrand factor.

Thrombocytopaenia in chronic liver disease is multifactorial. Congestive splenomegaly secondary to portal hypertension causes a redistribution of platelets to the splenic pool, causing thrombocytopaenia.^{12 13} Consumptive reduction in the platelets occurs secondary to hypercoagulability, leading to thrombocytopaenia in cirrhosis.¹⁴ There may be a defect in the physiological regulation of platelet production, as evidenced by low levels of serum thrombopoietin in patients with cirrhosis.¹⁵ Immune-mediated destruction of platelets happens in hepatitis C and primary biliary cirrhosis, causing thrombocytopaenia. In liver disease, due to proteolysis of the platelet receptor by plasmin, there may be impaired interaction of the platelets with the vessel wall.¹⁶ Other mechanisms for thrombocytopaenia include bone marrow suppression secondary to the effects of chronic alcoholism, folate deficiency or antiviral therapy in patients with hepatitis B and C infection.

Chronic liver disease is characterised by reduced synthesis of procoagulant proteins (Factors I, V, VII, IX, X and XI). Coagulopathy occurs secondary to alterations in the procoagulant or anticoagulant pathways or a disorder in the fibrinolytic system. Clotting factor levels fall in parallel to the severity and progression of liver disease, although the levels vary considerably.¹⁷ Hyperfibrinolysis has been proposed as the mechanism of coagulopathy in chronic liver disease. Under normal circumstances, as fibrin deposits in the vascular system, the proenzyme plasminogen is converted into plasmin, which is propagated by factors such as tissue plasminogen activator (t-PA). Factors like thrombin-activatable fibrinolysis inhibitors (TAFI), t-PA inhibitors like plasminogen activator inhibitors, and plasmin inhibitors act as antifibrinolytic. Any perturbation of this delicate balance will result in hyperfibrinolysis. Fibrinogen, the precursor of fibrin, is reduced in advanced cirrhosis or acute liver failure due to endothelial dysfunction, vasodilation mediated by nitric oxide and decreased tissue factor production, which contribute to antihaemostatic effects.^{17 18} Reduced levels of plasminogen, antiplasmin, TAFI and factor XIII are observed in cirrhosis, as evidenced by various studies,^19–21 causing an imbalance in the profibrinolytic and antifibrinolytic drives, leading to coagulopathy.

Liver disease coupled with alcohol consumption favours a synergistic risk of bleeding as haemostasis is precariously balanced in advanced cirrhosis, and any minor alteration favours bleeding in these patients. Alcohol consumption inhibits platelet aggregation in response to collagen and ADP, decreases intracellular cyclic AMP levels, increases intracellular inositol-1,4,5-trisphosphate concentration, leads to poor activation of functional platelets, and decreases vWF, factor VII and fibrinogen levels.²²

A review of the literature shows that, in a case series of 18 patients with SMH with alcoholic cirrhosis involving the iliopsoas or rectus muscles, 15 patients died, making early diagnosis and aggressive therapy necessary.²³ CT is reliable and accurate in diagnosing the condition and defining the anatomy of the haematoma. Patients with SMH can be managed conservatively with analgesics. Drugs that precipitate bleeding have to be withheld. If the patient has an underlying coagulopathy, blood products such as fresh frozen plasma, recombinant clotting factors, vitamin K or protamine sulfate may be required. Blood transfusion needs to be done if there is haemodynamic instability. Interventional radiological procedures such as angiography and digital subtraction angiography with embolisation can be done if active bleeding persists.^24–26 Surgical procedures such as evacuation of haematomas or ligation of vessels can be done in cases of haemodynamic instability, failure of angioembolisation or underlying sepsis.²⁷ Haemostasis can be achieved by removing the haematoma and packing the cavity.

We treated the patient with blood products, which led to clinical improvement. The treatment approach can be conservative, surgical or combined. Conservative management of hyperfibrinolysis in cirrhosis includes fresh frozen plasma, vitamin K, cryoprecipitate and use of antifibrinolytic agents. Some radical approaches include transcatheter arterial embolisation (TAE).

In another case report, 19 of 26 patients with haematoma secondary to anticoagulants recovered with TAE.¹⁴ Since our patient did not have clinical improvement with blood and blood products, he required minor surgical intervention involving the evacuation of the haematoma. SMH is a very rare complication secondary to coagulopathy in cirrhosis and it is essential to make a prompt diagnosis and start an appropriate management, without which most patients will have a terminal course.

Learning points.

• Spontaneous muscle haematoma (SMH) is also a complication of cirrhosis.

• Primary hyperfibrinolysis is proposed to be the mechanism behind SMH in cirrhosis.

• Although rare, SMH is a fatal complication of cirrhosis and hence early recognition and aggressive treatment are necessary to avoid mortality.

Ethics statements

Patient consent for publication

Obtained.