Phlegmasia cerulea dolens: a swelling, cyanosis and discolouration of the extremity in the ICU

Background

Phlegmasia cerulea dolens (PCD) is characterised by an extensive deep vein thrombosis (DVT) that compromises the arterial flow of the superficial and collateral circulation and progressively leads to venous gangrene. Given the scarcity of cases, the precise incidence of PCD is unknown. However, clinicians, especially those who work in the intensive care unit (ICU), should be aware of PCD. ICU patients may be at greater risk of PCD due to the complexity of these patients and their susceptibility to frequent complications, such as severe congestion and fluid sequestration of the limb, leading to venous hypertension, circulatory shock, arterial insufficiency or other thromboembolic events.1 2 The typical clinical presentation of PCD is a cold, swollen, painful and cyanotic lower extremity.3 The dorsal foot and posterior tibial pulses are not palpable. Vascular ultrasound can enhance the absence of the pulse to confirm the diagnosis. Initial treatment usually involves medical thrombolysis and conservative management methods.

In patients with contraindications to medical treatment or therapeutic failure, there are two other treatment options, namely, endovascular or surgical. Catheter-directed thrombolysis is the recommended endovascular treatment for restoring venous flow. Surgical thrombectomy is an emergent treatment.4 Given the high risk of complications that can lead to the patient’s death, PCD requires rapid diagnosis and immediate treatment.

Case presentation

An otherwise healthy man in his 50s suffered multiple trauma. He was admitted to the ICU with traumatic brain injury (TBI) with foci of contusion, subarachnoid haemorrhage (SAH) and diffuse oedema. Intracranial pressure sensor monitoring was put in place, and antioedematous treatment was provided. Chest trauma was also present with fracture of three ribs on the right. Hypovolaemic shock secondary to the rupture of the spleen required emergent splenectomy. Thromboprophylaxis with compression stockings was commenced immediately, and low molecular weight heparin (LMWH) was started from day 6 of hospital admission.

On day 11 of hospitalisation, the right lower limb showed oedema, skin tension and paleness, and the popliteal and pedal pulses were undetectable (figure 1). Bedside vascular ultrasound showed the absence of vascular flow in the femoral and iliac territories. CT angiography indicated extensive DVT of the external iliac and femoral veins, constricted by a central venous catheter, and PCD was diagnosed (figure 2). Emergency venous thrombectomy of the right lower limb was performed via the femoral route (figure 3) resulting in a drastic reduction in lower limb tension at the end of surgery (figure 4). Hence, fasciotomy was not performed.

Figure 1

Patient’s lower limb in the acute phase of thrombus presentation.

Figure 2

Thrombosis of the right femoral vein and right external iliac vein, with marked thickening of the musculocutaneous muscle planes.

Figure 3

Thrombus identified intraoperatively.

Figure 4

Patient’s lower limb with total limb reperfusion postoperation.

Perioperatively, the patient was haemodynamically stable and did not require vasopressor support. Hyperlactataemia was not evident (maximum value=0.98 mmol/L), and no signs of acute kidney damage were observed. Postoperatively, haemoglobin was 64 g/L, and erythrocyte concentrate was transfused. The maximum peak creatine kinase was 886 U/L.

On day 14 of ICU admission, the patient developed a thrombotic event in the cephalic vein of the left upper limb. The embolus measured 3 cm. The radial pulse was palpable, and no external signs of phlebitis were observed. Specific interventions were not indicated. Prophylactic LMWH was maintained. The clinical course progressed favourably, and the patient was transferred to the ward on day 24. The Glasgow Coma Scale score of the patient was 14 (E4, M6 and V4). The LMWH dosage was monitored, with anti-Xa measurements during the hospital stay, and the values were maintained within the prophylactic range.

Treatment

There is no consensus for PCD management; however, several options may be considered, including limb elevation, heparin and intravenous administration of a thrombolytic agent. In critically ill patients hospitalised mainly for trauma or hypovolaemic shock, patient mobilisation may be affected, and adequate antithrombotic prophylaxis or therapeutic anticoagulation may not be possible. If these conservative options are contraindicated, surgical venous thrombectomy is recommended as the first-line treatment.5

In the present case, elastic compression bandages were applied to the patient following thrombectomy, and the LMWH was maintained at a prophylactic dose due to a high bleeding risk.

Outcome and follow-up

Our patient was discharged to the neurosurgery department after 24 days in the ICU. After a further 29 days in the ward, the patient was transferred to a rehabilitation centre. The patient was followed up for 9 months after being discharged from the hospital. The 9-month follow-up ultrasound doppler confirmed deep venous system permeability (figure 5). Clinically, the patient reported pain, and oedema was evident in the right lower limb. Improvements were achieved with compression stockings. Anticoagulation with 2.5 mg apixaban was maintained for another 3 months to prevent post-thrombotic syndrome.

Figure 5

Doppler of the affected lower limb at the 9-month follow-up after hospital discharge.