Diabetic muscle infarction with pelvic vein thrombosis: An uncommon presentation to the emergency department

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1 OPEN ACCESS Case study Diabetic muscle infarction with pelvic vein thrombosis: An uncommon presentation to the emergency department Suresh Varadarajulu, Sameer A. Pathan* Emergency Department, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar * ABSTRACT Diabetic muscle infarction (DMI) occurs as a rare complication associated with long-standing, poorly controlled diabetes mellitus (DM) and typically presents as acute painful muscle swelling in the absence of trauma. It resolves spontaneously over a few weeks to months in most patients and generally has a good prognosis. However, the coexistence of deep vein thrombosis (DVT) with DMI may have an additional risk of complications such as pulmonary embolism, recurrent DVT, and postthrombotic syndrome (PTS). Although the treatment for DMI is mainly analgesia, rest, and control of diabetes, the coexistence of extensive DVT and diabetic ketoacidosis (DKA) may demand anticoagulation and a multidisciplinary team approach. Its management may also involve the endocrinologist, hematologist, and occasionally an interventional radiologist or vascular surgeon. We report two cases of patients with DMI with extensive DVT that presented to our emergency department (ED) as acute non-traumatic swelling of the lower limb. The diagnosis of DMI in both the cases was based on the presence of characteristic clinical features and typical magnetic resonance imaging (MRI) findings. The patients were treated with anticoagulation therapy in addition to the management of DKA, and discharged with good recovery. Keywords: Venous thromboembolism, emergency medicine, diabetes endocrinology, general practice, family medicine /jemtac Submitted: 31 May 2016 Accepted: 18 July 2016 ª 2016 Varadarajulu, Pathan, licensee HBKU Press. This is an open access article distributed under the terms of the Creative Commons Attribution license CC BY 4.0, which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited. Cite this article as: Varadarajulu S, Pathan SA. Diabetic muscle infarction with pelvic vein thrombosis: An uncommon presentation to the emergency department, Journal of Emergency Medicine, Trauma & Acute Care 2016:7

2 Page 2 of 7 INTRODUCTION Diabetic muscle infarction (DMI) is a rare complication of poorly controlled, long-standing diabetes. It presents as non-traumatic acute onset of muscle swelling, sometimes palpable, with mild to moderate pain. 1 Though first reported in 1965, less than 200 cases have been reported in the last 50 years. 2,3 However, the presentation of DMI with extensive deep vein thrombosis (DVT), involving femoral and iliac veins, has not been previously reported. Lower limb vastus group muscles and calf muscles are commonly involved in DMI. 4 It may also affect upper limb muscles in patients with end-stage renal disease along with long-standing diabetes. 5 DMI affects women more than men, and up to two-thirds of cases have type 1 diabetes for a mean duration of 18.9 years. 6 Bilateral involvement may occur in nearly one-third of cases, and half of patients may experience recurrence at the same or different sites. 7 Important differential diagnoses to be considered include cellulitis, pyomyositis, spontaneous gangrenous myositis, necrotizing fasciitis, venous thrombosis, intramuscular hematoma, and neoplasm. CASE PRESENTATIONS Case 1 A 40-year-old woman presented to the emergency department (ED) with pain in the left thigh and swelling. The pain was gradual in onset and there was no history of trauma or prolonged immobilization. She suffered with type 2 diabetes and was on oral hypoglycemic treatment for the past eight years. She denied having any other medical problem or similar complaint in the past. She was a nonsmoker, a busy homemaker, and lived with her husband. There were no constitutional symptoms and apart from tachycardia, her blood pressure, respiratory rate, and oxygen saturation were within normal limits on presentation. Her bedside random blood glucose reading was 22 mmol/l (normal value mmol/l). She experienced a reduction in pain after administration of intravenous (IV) morphine. Compared to the right side, the left lower limb was swollen, erythematous, yet cool to the touch. The presence of significant edema made it difficult to palpate the left popliteal, posterior tibialis, and dorsalis pedis artery pulsations. However, her left femoral pulse and distal capillary refill were normal (,2 seconds). A bedside Doppler ultrasound confirmed the presence of weak pulsation in the dorsalis pedis and posterior tibial arteries on the left side and normal pulsations on the right side. The muscles of the left calf were tender on palpation, and passive movements did not exaggerate the pain. There was no sensory or motor deficit on neurological examination in both the lower limbs. A clinical diagnosis of DVT was made, and to exclude this possible diagnosis, a formal Doppler scan of the left lower limb was requested. Blood test revealed an elevated total white cell count (WBC /L; normal /L). Considering the possibility of infection, she was treated with IV ceftriaxone (2 g). To manage her hyperglycemia (22 mmol/l), short-acting insulin was administered, and insulin infusion was initiated later to treat her ketoacidosis (serum B-hydroxybutyrate 3.4; normal 0 1 mmol/l; venous blood ph 7.30). The HbA1C level was 13.1% (normal %), reflecting poorly controlled diabetes. The D-dimer was elevated (9.99 mg/l; normal up to 0.55 mg/l) and so was serum myoglobin (311 ng/ml; reference range ng/ml). The Doppler ultrasound scan showed thrombosis of superficial and common femoral veins, swollen muscles in the thigh and calf region along with the compressed arteries on the left side. The scan of the pelvic veins was suboptimal due to the gaseous abdomen. Considering the persistent nature of the pain despite repeated IV analgesia, on-call orthopedic and vascular surgeons were consulted for a presumptive diagnosis of compartment syndrome. A computed tomography (CT) angiogram was requested for pelvic vessels examination. Additional information on the CT angiogram report revealed a filling defect in the left femoral and iliac veins with a normal arterial flow (Figure 1). Low-molecular-weight heparin (enoxaparin 1 mg/kg subcutaneously every 12 hours) was initiated, and the patient was admitted under the medical team for further workup and management. Blood cultures were later reported negative, and antibiotics were discontinued. Following admission to the medical ward, a magnetic resonance imaging (MRI) with contrast examination was performed. The MRI images showed a significant subcutaneous tissue edema, a marked muscle swelling, and contrast enhancement from the left thigh muscles compared to the right side (Figure 2). In addition, a magnetic resonance venography (MRV) was also performed. The MRV revealed thrombosis of the left femoral and iliac veins with extension thrombus into the inferior vena cava up to the renal vein. The patient was continued on medical management for two weeks and

3 Page 3 of 7 Figure 1. Filling defect in the left femoral vein indicating thrombosis (red arrow) on CT scan. Figure 2. On T2-weighted axial image, significant subcutaneous tissue edema (black star), marked muscle swelling (red diamond) and contrast enhancement on the left thigh compared with the right side. subsequently discharged on warfarin (5 mg once a day) with INR of 3.1, resulting in resolution of pain and reduction in the swelling. Case 2 A 58-year-old man presented to our ED for the first time with swelling in the left lower limb and pain for 12 hours. The pain was gradual in onset and moderate to severe in intensity. There was no history of trauma or prolonged immobilization. Although he was never formally diagnosed as having diabetes, his blood sugar level was 33.5 mmol/l (normal mmol/l) on the index visit. He denied having any other medical problems or previous such episodes. He was a smoker who worked as a crane operator. There were no constitutional symptoms on presentation. He also reported intermittent

4 Page 4 of 7 Figure 3. MRI T2-weighted coronal image with hyper-intense signal in the adductor (white asterisk) and the hamstring muscles (red diamond) group on the left side. cramps like pain in both lower limbs for the past four days without any particular disability or limitation of movement. The pain in the left lower limb became worse during the night before presentation. His initial vital signs and electrocardiogram (ECG) were normal. Physical examination revealed extensive swelling of the left thigh and leg, which was hard in consistency. No sensory or motor deficits were observed, and capillary refill time was normal (,2 seconds). The pain did not worsen on passive movements. Moreover, compartment syndrome was excluded by measuring bedside compartment pressure. He received IV morphine for the pain and short-acting insulin for the treatment of hyperglycemia. Results of the blood tests revealed leukocytosis (WBC /L; normal /L) with neutrophilia (83%), high blood glucose (33.5 mmol/l), elevated lactate levels (3.1 mmol/l; normal for venous blood mmol/l), raised levels of creatinine kinase (33,904 unit/l; normal unit/l), and impaired kidney function (creatinine 211 mmol/l; normal mmol/l). The other blood test results reported a HbA1C of 8.0% (normal %), CRP of 22 mg/l (normal,5 mg/l), serum B-hydroxybutyrate of 2.2 mmol/l (normal 0 1 mmol/l), and venous blood ph of 7.21 (normal ). A Doppler ultrasound scan of the left lower limb was performed to exclude DVT. The examination was suboptimal due to extensive swelling in the muscles of the anterior compartment of the thigh. However, this excluded the presence of thrombosis of the calf veins. The diabetic ketoacidosis (DKA) was treated according to hospital protocol with insulin infusion. The patient was treated with IV antibiotics (2 g piperacillin and 0.25 g tazobactam) to cover for possible infection. After discussion with the radiologist, a CT angiogram of the abdomen, pelvis, and lower limbs was performed. The CT angiogram showed thrombosis of the left common, external, and internal iliac veins with grossly edematous left thigh muscles and subcutaneous tissues. After admission to the medical ward, an MRI with contrast was performed. The MRI showed high-intensity signals from the lower limb muscles in the adductor and hamstring groups associated with edema in the fascia and subcutaneous tissue (Figure 3). The high-intensity signals on T2 images were prominent in the vastus, adductors, and hamstring muscle groups compatible with the presentation of DMI (Figure 4). The DVT was treated with low-molecular-weight heparin (enoxaparin 1 mg/kg subcutaneously every 12 hours). During the hospital admission, oral warfarin was initiated, overlapping with heparin injections, and the treatment was aimed for the optimum INR of The muscle swelling increased with bullae

5 Page 5 of 7 Figure 4. MRI T2W axial section: the left thigh showing extensive heterogeneous signals (red diamond) from the vastus muscle group, hyper-intensity signals (red star) from the adductor muscles, and the unaffected bicep femoris muscle (white asterisk). formation during day four of the admission and spontaneously resolved over the subsequent two weeks (Figure 5). The patient was finally discharged after three weeks on warfarin 8 mg once a day and INR of 2.8. DISCUSSION DMI is a rare complication of long-standing diabetes. It is of serious concern when it is associated with further complications such as compartment syndrome, DVT, or superadded infection with staphylococcal sepsis. 8,9 To the best of our knowledge, DVT of pelvic veins with DMI is an unusual co-presentation and has not been previously reported. The exact pathogenesis of DMI is uncertain. However, based on the histopathological findings and MRI, micro- and macrovascular fibrin degeneration is proposed as one of the possible mechanisms underlying DMI. 10 The mean duration of diabetes mellitus (DM) at the time of DMI diagnosis has been reported as 18.9 years for type 1 DM, and 11.0 years for type 2 DM. 6 However, the duration of DM at the time of diagnosis in both cases in our study was shorter. Like other cases of DMI, both the patients were afebrile and had typical acute onset of pain in the thigh with localized tenderness and swelling. 6 Definitive diagnosis requires tissue biopsy to show ischemic necrosis of the affected muscles and culture to exclude infection. 11 However, this is reserved for patients who have failed conservative treatment or with clinical suspicion of muscle or fascia infection. In the rest of cases, diagnosis of DMI can be made in patients with long-standing DM presenting with acute, non-traumatic, painful, muscle swelling. This diagnosis is supported by typical MRI findings such as diffuse edema and swelling of multiple thigh or calf muscles, with high-intensity signals from the affected muscles on T2-weighted images. 6,12 14 MRI with contrast is also useful in excluding other differential diagnoses, such as DVT, pyomyositis, and primary or secondary muscle tumors. The calf muscles are involved in up to 20% of cases in DMI mimicking DVT therefore, Doppler ultrasound is indicated as a part of initial workup. 1 The management of DMI is mostly conservative with rest, analgesia, and control of hyperglycemia. Physiotherapy in the acute recovery period should be avoided as it may worsen the condition. 6,10 Invasive diagnostic tests and surgical interventions are not recommended due to the risk of procedure-associated complications. 11 Surgical management is reserved only for cases of conservative

6 Page 6 of 7 Figure 5. Bleb and bulla formation in the left lower limb. treatment failure. Short-term prognosis is usually good with spontaneous resolution of symptoms within a few weeks to months with conservative management. 15 However, long-term prognosis is generally poor due to widespread vascular complications of DM and recurrence of the disease. 10 CONCLUSION In summary, DMI is uncommon, and its co-presentation with DVT is rare. The diagnosis of DMI is highly suggestive in the presence of characteristic clinical features and typical MRI findings. MRI examination can also help to exclude other complications and diagnoses, thereby avoiding unnecessary biopsy. The management of DMI is mostly conservative, and surgical treatment is reserved for treatment failure, compartment syndrome, or on presence of tissue infection. REFERENCES [1] Trujillo-Santos AJ. Diabetic muscle infarction: an underdiagnosed complication of long-standing diabetes. Diabetes Care. 2003;26(1): [2] Angervall L, Stener B. Tumoriform focal muscular degeneration in two diabetic patients. Diabetologia. 1965;1(1): [3] Iyer SN, Drake AJ III, West RL, Tanenberg RJ. Diabetic muscle infarction: a rare complication of long-standing and poorly controlled diabetes mellitus. Case Rep Med. 2011;2011:Article ID , 4 pp. [4] Rashidi A, Bahrani O. Diabetic myonecrosis of the thigh. J Clin Endocrinol Metab. 2011;96(8): [5] Joshi R, Reen B, Sheehan H. Upper extremity diabetic muscle infarction in three patients with end-stage renal disease: a case series and review. J Clin Rheumatol. 2009;15(2): [6] Horton WB, Taylor JS, Ragland TJ, Subauste AR. Diabetic muscle infarction: a systematic review. BMJ Open Diabetes Res Care. 2015;3(1):e [7] Kapur S, Brunet JA, McKendry RJ. Diabetic muscle infarction: case report and review. J Rheumatol. 2004;31(1): [8] Woolley SL, Smith DR. Acute compartment syndrome secondary to diabetic muscle infarction: case report and literature review. Eur J Emerg Med. 2006;13(2):

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