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Phlegmasia cerulea dolens: A rare cause of compartment syndrome
Injury Extra 41 (2010) 51–52
Contents lists available at ScienceDirect
Injury Extra journal homepage: www.elsevier.com/locate/inext
Case report
Phlegmasia cerulea dolens: A rare cause of compartment syndrome Simon Hawkins *, Christopher Brown, Ranald Stuart, Andrew McAndrew Royal Berkshire Hospital, Reading, United Kingdom
A R T I C L E I N F O
Article history: Accepted 21 January 2010
1. Introduction The acutely painful limb without trauma provides a diagnostic conundrum for surgeons. We present such a case in a 54-year-old gentleman who presented emergently with severe left leg pain that had developed insidiously over 4 days, then rapidly worsened with a queried diagnosis of necrotizing fascitiis, ischaemia or compartment syndrome. 2. Case report The patient, who had Crohn’s disease, had undergone a right hemicolectomy of the terminal ileum 2 weeks previously for Dukes C adenocarcinoma. He was on warfarin for a pre-operative left subclavian venous thrombosis and had an inferior vena cava filter in situ, as he had developed a right popliteal venous thrombus whilst warfarinised. His international normalised ratio on the day of attendance was 2.6 and there was no history of trauma. Clinical examination found the patient to be pale, pyrexic and tachycardic with a warm, oedematous, exquisitely tender, swollen left lower leg. His pain was exacerbated by all movements, he had normal sensation and arterial pulses and no cellulitis or erythema was visible. Plain radiographs of the lower leg were normal and his blood tests demonstrated a white cell count 16; D-dimer 6000, C-reactive protein 228 with normal creatine kinase and lactate. A magnetic resonance imaging scan was performed demonstrating oedematous change within the musculature of the posterior compartment with interfascial and subcutaneous fluid and possible popliteal and proximal calf vein deep venous thrombosis. The differential diagnosis postulated was either compartment syndrome, myositis or necrotising fasciitis (Fig. 1). The patient was thought to have an impending compartment syndrome of unknown cause and underwent urgent lower leg exploration and fasciotomies. At surgery, all of the compartments of the limb were tense and bulging; most
* Corresponding author at: Department of Orthopaedic Surgery, Royal Berkshire Hospital, London Road, Reading RG1 5AN, United Kingdom. Tel.: +44 118 322 5111. E-mail address: [email protected] (S. Hawkins).
particularly the posterior ones. The muscle was dusky but viable; none was necrotic nor was there haematoma or pus. Biopsies taken at surgery did not demonstrate any abnormalities. Blood cultures were also negative. Post-operatively the patient’s pain fully resolved and the surgical wounds were subsequently closed, although one required skin grafting. Post-operatively, the patient had an ultrasound of his left thigh which demonstrated extensive thrombus in the popliteal vein extending into the iliac vessels, despite being on warfarin. He was diagnosed with phlegmasia cerulea dolens causing impending compartment syndrome and will now be on low molecular weight heparin therapy for life. 3. Discussion The acutely painful limb in the absence of trauma is a challenging conundrum. A possible diagnosis of necrotising fascititis requires all investigations and treatments to be performed expeditiously. MRI is believed to aid diagnosis when differentiating between causes of acutely swollen limbs and in particular necrotising fasciitis.5 In this case, however, the diagnosis remained in doubt and was ultimately only confirmed after
Fig. 1. Axial MRI STIR sequence of the lower leg. Diffuse increased signal intensity is seen throughout the posterior compartment muscle bellies, with fluid extending through the interfascial planes and over the deep fascia, with associated subcutaneous oedema. The proximal deep calf veins appear mildly distended, with absent flow-voids raising the possibility of venous thrombus.
1572-3461/$ – see front matter . Crown Copyright ß 2010 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.injury.2010.01.111
52
S. Hawkins et al. / Injury Extra 41 (2010) 51–52
exploration and ultrasound confirmation of thrombus in the popliteal, femoral and iliac veins. Phlegmasia cerulea dolens is a rare condition that presents as an acutely painful limb with the clinical triad of oedema, severe pain and cyanosis as a result of massive acute venous thrombosis of the lower extremity resulting in obstruction of the venous drainage. The thrombus extends to collateral veins and 40–60% also have capillary involvement. It results in venous hypertension and fluid sequestration that causes increased intramuscular tissue pressure, ischaemia and gangrene of the limb. It is differentiated from the other form of fulminant venous thrombosis, phlegmasia alba dolens, by the presence of ischaemia.1 Malignancy is the most common triggering factor. Radiological investigation includes ultrasonography, venography and magnetic resonance venography. Treatment options for this type of extensive DVT are anticoagulation, thrombectomy,2 thrombolysis3 and fasciotomy, although fasciotomy is usually in combination with thrombectomy. Historically, surgical thrombectomy was the procedure of
choice for PCD refractory to medical therapy. The degree of increase in intramuscular compartment pressures has been showed to be associated with the extent of thrombus,4 and IVC filters may act as a thrombogenic nidus. Its rarity and presentation can lead to initial misdiagnosis and confusion with alternative pathology. We advise clinicians to be wary of its presentation, even in fully anti-coagulated patients. References 1. Gregoire R. La Phlebite bleue Phlegmasia Cerulea Dolens1938;48:1313–5. 2. Lord RS, Chen FC, Devine TJ, Benn IV. Surgical treatment of acute deep venous thrombosis. World J Surg 1990;14:694–702. 3. Paquet KJ, Popov S, Egli H. Guidelines and results of consequent fibrinolytic therapy in phlegmasia cerulea dolens. Dtsch Med Wochenschr 1970;95(April(16)):903–4. 4. Qvarfordt P, Eklof B, Ohlin P. Intramuscular pressure in the lower leg in deep vein thrombosis and phlegmasia cerulea dolens. Anmi Surg 1983;197:450–3. 5. Re´velon G, Rahmouni A, Jazaerli N, Godeau B, Chosidow O, Authier J, et al. Acute swelling of the limbs: magnetic resonance pictorial review of fascial and muscle signal changes. Eur J Radiol 1999;30(April(1)):11–21.
Contents lists available at ScienceDirect
Injury Extra journal homepage: www.elsevier.com/locate/inext
Case report
Phlegmasia cerulea dolens: A rare cause of compartment syndrome Simon Hawkins *, Christopher Brown, Ranald Stuart, Andrew McAndrew Royal Berkshire Hospital, Reading, United Kingdom
A R T I C L E I N F O
Article history: Accepted 21 January 2010
1. Introduction The acutely painful limb without trauma provides a diagnostic conundrum for surgeons. We present such a case in a 54-year-old gentleman who presented emergently with severe left leg pain that had developed insidiously over 4 days, then rapidly worsened with a queried diagnosis of necrotizing fascitiis, ischaemia or compartment syndrome. 2. Case report The patient, who had Crohn’s disease, had undergone a right hemicolectomy of the terminal ileum 2 weeks previously for Dukes C adenocarcinoma. He was on warfarin for a pre-operative left subclavian venous thrombosis and had an inferior vena cava filter in situ, as he had developed a right popliteal venous thrombus whilst warfarinised. His international normalised ratio on the day of attendance was 2.6 and there was no history of trauma. Clinical examination found the patient to be pale, pyrexic and tachycardic with a warm, oedematous, exquisitely tender, swollen left lower leg. His pain was exacerbated by all movements, he had normal sensation and arterial pulses and no cellulitis or erythema was visible. Plain radiographs of the lower leg were normal and his blood tests demonstrated a white cell count 16; D-dimer 6000, C-reactive protein 228 with normal creatine kinase and lactate. A magnetic resonance imaging scan was performed demonstrating oedematous change within the musculature of the posterior compartment with interfascial and subcutaneous fluid and possible popliteal and proximal calf vein deep venous thrombosis. The differential diagnosis postulated was either compartment syndrome, myositis or necrotising fasciitis (Fig. 1). The patient was thought to have an impending compartment syndrome of unknown cause and underwent urgent lower leg exploration and fasciotomies. At surgery, all of the compartments of the limb were tense and bulging; most
* Corresponding author at: Department of Orthopaedic Surgery, Royal Berkshire Hospital, London Road, Reading RG1 5AN, United Kingdom. Tel.: +44 118 322 5111. E-mail address: [email protected] (S. Hawkins).
particularly the posterior ones. The muscle was dusky but viable; none was necrotic nor was there haematoma or pus. Biopsies taken at surgery did not demonstrate any abnormalities. Blood cultures were also negative. Post-operatively the patient’s pain fully resolved and the surgical wounds were subsequently closed, although one required skin grafting. Post-operatively, the patient had an ultrasound of his left thigh which demonstrated extensive thrombus in the popliteal vein extending into the iliac vessels, despite being on warfarin. He was diagnosed with phlegmasia cerulea dolens causing impending compartment syndrome and will now be on low molecular weight heparin therapy for life. 3. Discussion The acutely painful limb in the absence of trauma is a challenging conundrum. A possible diagnosis of necrotising fascititis requires all investigations and treatments to be performed expeditiously. MRI is believed to aid diagnosis when differentiating between causes of acutely swollen limbs and in particular necrotising fasciitis.5 In this case, however, the diagnosis remained in doubt and was ultimately only confirmed after
Fig. 1. Axial MRI STIR sequence of the lower leg. Diffuse increased signal intensity is seen throughout the posterior compartment muscle bellies, with fluid extending through the interfascial planes and over the deep fascia, with associated subcutaneous oedema. The proximal deep calf veins appear mildly distended, with absent flow-voids raising the possibility of venous thrombus.
1572-3461/$ – see front matter . Crown Copyright ß 2010 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.injury.2010.01.111
52
S. Hawkins et al. / Injury Extra 41 (2010) 51–52
exploration and ultrasound confirmation of thrombus in the popliteal, femoral and iliac veins. Phlegmasia cerulea dolens is a rare condition that presents as an acutely painful limb with the clinical triad of oedema, severe pain and cyanosis as a result of massive acute venous thrombosis of the lower extremity resulting in obstruction of the venous drainage. The thrombus extends to collateral veins and 40–60% also have capillary involvement. It results in venous hypertension and fluid sequestration that causes increased intramuscular tissue pressure, ischaemia and gangrene of the limb. It is differentiated from the other form of fulminant venous thrombosis, phlegmasia alba dolens, by the presence of ischaemia.1 Malignancy is the most common triggering factor. Radiological investigation includes ultrasonography, venography and magnetic resonance venography. Treatment options for this type of extensive DVT are anticoagulation, thrombectomy,2 thrombolysis3 and fasciotomy, although fasciotomy is usually in combination with thrombectomy. Historically, surgical thrombectomy was the procedure of
choice for PCD refractory to medical therapy. The degree of increase in intramuscular compartment pressures has been showed to be associated with the extent of thrombus,4 and IVC filters may act as a thrombogenic nidus. Its rarity and presentation can lead to initial misdiagnosis and confusion with alternative pathology. We advise clinicians to be wary of its presentation, even in fully anti-coagulated patients. References 1. Gregoire R. La Phlebite bleue Phlegmasia Cerulea Dolens1938;48:1313–5. 2. Lord RS, Chen FC, Devine TJ, Benn IV. Surgical treatment of acute deep venous thrombosis. World J Surg 1990;14:694–702. 3. Paquet KJ, Popov S, Egli H. Guidelines and results of consequent fibrinolytic therapy in phlegmasia cerulea dolens. Dtsch Med Wochenschr 1970;95(April(16)):903–4. 4. Qvarfordt P, Eklof B, Ohlin P. Intramuscular pressure in the lower leg in deep vein thrombosis and phlegmasia cerulea dolens. Anmi Surg 1983;197:450–3. 5. Re´velon G, Rahmouni A, Jazaerli N, Godeau B, Chosidow O, Authier J, et al. Acute swelling of the limbs: magnetic resonance pictorial review of fascial and muscle signal changes. Eur J Radiol 1999;30(April(1)):11–21.