Hindawi Publishing Corporation Case Reports in Emergency Medicine Volume 2014, Article ID 208960, 3 pages http://dx.doi.org/10.1155/2014/208960
Case Report A Case of Lemierre Syndrome Secondary to Otitis Media and Mastoiditis Aynur Turan,1 Harun Cam,1 Yeliz Dadali,2 Serdar Korkmaz,1 Ali Özdek,3 and Baki HekimoLlu1 1
Department of Radiology, Diskapi Yildirim Beyazit Training and Research Hospital, Etlik, 06010 Ankara, Turkey Department of Radiology, Faculty of Medicine, Ahi Evran University, Kirs¸ehir, Turkey 3 Department of Otolaryngology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey 2
Correspondence should be addressed to Aynur Turan; aynur [email protected] Received 4 May 2014; Accepted 16 October 2014; Published 6 November 2014 Academic Editor: Aristomenis K. Exadaktylos Copyright © 2014 Aynur Turan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Lemierre’s syndrome is a rare clinical condition that generally develops secondary to oropharyngeal infection caused by Fusobacterium necrophorum, which is an anaerobic bacteria. A 62-year-old patient with diabetes mellitus presented with internal jugular vein and sigmoid sinus-transverse sinus thrombophlebitis, accompanying otitis media and mastoiditis that developed after an upper airway infection. Interestingly, there were air bubbles in both the internal jugular vein and transverse sinus. Vancomycin and meropenem were started and a right radical mastoidectomy was performed. The patient’s clinical picture completely resolved in 14 days. High mortality and morbidity may be prevented with a prompt diagnosis of Lemierre’s syndrome.
1. Introduction In 90% of cases, the cause of Lemierre’s syndrome (LS) is the Fusobacterium necrophorum, which is an anaerobic, gram negative bacteria. On the other hand, anaerobic Streptococci and other gram negative anaerobic bacteria are responsible for the remaining 10% of cases [1]. The disease has been described in detail by Lemierre in 1936 [2]. Mortality rates were as high as 90% in its first described periods and decreased to 4–18% with the widespread use of antibiotics [2, 3]. The current study aimed to draw attention to this disease described as the “forgotten disease” in the literature [4] and present the radiological findings of a case of internal jugular vein (IJV) thrombophlebitis and accompanying sigmoidtransverse sinus thrombosis that was believed to develop secondary to otitis media (OM) and mastoiditis.
2. Case A 62-year-old male patient was on cefuroxime axetil treatment for an upper airway infection that persisted for 15 days. However, he was admitted to the emergency department with complaints of redness, swelling, and pain increasing
with motion in the neck region, leakage from the right ear, headache, shivering, and fever. His body temperature was 39∘ C upon physical examination. There was grade I tonsillar hypertrophy in the oropharyngeal examination. The right external ear way was edematous and the tympanic membrane was macerated. There was also tenderness in the neck region. Any abnormalities related to the lungs were not present in the physical examination and the neurological examination was normal. No other additional findings were detected in the physical examination. The patient had a history of diabetes mellitus type 2 (T2DM) for 15 years. His blood glucose was 317 mg/dL and hemoglobin A1c was 10.2%, indicating poorly controlled T2DM. His white cell count was 18,000/mm3 and other pertinent laboratory results were unremarkable. With the clinical suspicion of deep neck infection, brain and neck computed tomography (CT) was performed. The neck CT revealed internal jugular venous distention with a thickened enhancing wall, filling defects in the lumen, and air bubbles. In the brain CT, there were aeration defects and effusion on mastoid cellules and the middle ear and filling defects and air bubbles in the right sigmoid and transvers sinuses (Figure 1). In order to determine the extent and other
2
Figure 1: Air bubbles as seen in the brain CT of the right sigmoid sinus.
accompanying complications, contrasted brain and neck MRI were performed, which revealed that there was signal void loss in T2WI on the right IJV (Figure 2), right sigmoid, and transverse sinus; internal jugular venous distention with a thickened enhancing wall and filling defects in the IJV and sigmoid-transverse sinus lumens (Figures 3 and 4). Thrombosis of other intracerebral veins was not observed. Moreover, there were inflammatory signal changes on the right mastoid cellules and the middle ear cavity. These findings were evaluated as compatible with right OM, mastoiditis, IJV, sigmoid, and transverse sinus thrombophlebitis. Air bubbles observed both in the IJV and transverse sinus alluded to the fact that the infection was caused by anaerobe bacteria. The blood culture was negative. The patient was diagnosed with Lemierre’s syndrome, owing to the OM and mastoiditis and accompanying sigmoid and transverse sinus thrombosis. Vancomycin 2 gr/day IV and Meropenem 3 × 2 gr/day IV treatments were administered for two weeks and a right radical mastoidectomy was performed. The patient improved rapidly and the clinical picture of the patient resolved completely in 14 days. A control imaging for the resolution of thrombosis could not be performed because the patient was lost during follow-up.
3. Discussion The causative agent of Lemierre’s syndrome is commonly Fusobacterium necrophorum, which is an anaerobic bacteria present in the oral cavity, gastrointestinal system (GIS), and female genital system flora. In the last 20 years, an increase in its incidence has been reported owing to various reasons. The disease may be seen in any age group; however, more than 70% of cases are healthy adolescents and young adults. It has been reported more commonly in males than females [4, 5]. An association between Lemierre’s syndrome and diabetes mellitus has been reported [6] in the literature as well.
Case Reports in Emergency Medicine
Figure 2: Axial T2-weighted MRI revealed signal void loss on the right sigmoid and transverse sinuses.
Figure 3: Neck MRI with contrast showed internal jugular venous distention with a thickened enhancing wall and filling defects.
Prior to the disease, in general as in the current case, an oropharyngeal disease history of about one week is present. At the beginning of the disease, the physical examination findings may be very slight [4, 7]. Typically, the clinical course shows a gradual, step-by-step pattern in LS. The preliminary period includes findings such as fever, pharyngitis, otitis media, mastoiditis, and parotitis. Later, local microbial invasion to the lateral pharyngeal area and extension to the IJV by the way of infected peritonsillary veins and lymphatics may be determined. Owing to the involvement of the posterior compartment, cranial nerve X and XII palsies and Horner syndrome may also develop. Lastly, bacteremia and septic emboli may be seen in the other organs like lungs, bones, brain, and liver [4, 7, 8]. In the present case, transition to
Case Reports in Emergency Medicine
3 Since the incidence of Lemierre’s syndrome has increased over the last 20 years and the disease has high mortality and morbidity rates, prompt diagnosis of the disease is important. Using radiological instruments in the diagnosis, such as Doppler US, contrasted CT, and/or MRI should not be delayed in suspected cases.
Conflict of Interests The authors declare that there is no conflict of interests regarding the publication of this paper.
References
Figure 4: Axial brain MRI with contrast showed filling defects in right sigmoid and transverse sinuses.
the last step was not yet present, but OM, mastoiditis, and thrombophlebitis of the IJV and sigmoid-transverse sinus were present. Central nervous system involvement is extremely rare in LS, but purulent meningitis, cerebral abscess, and extension of IJV thrombophlebitis to the sigmoid and cavernous sinuses in a retrograde manner have been reported [5, 9]. Though few in number, similar to the current case, IJV thrombophlebitis and sigmoid and transverse sinus thrombosis cases secondary to OM and mastoiditis have been reported in the literature [7]. In the diagnosis of LS, radiology is tremendously important. Doppler US considerably enables the demonstration of the thrombus in IJV without ionizing radiation. In regions to which US cannot extend, such as the skull base or below the clavicle, contrasted CT and MRI warrant complete IJV visualization and moreover enable the visualization of anterograde and retrograde extensions and accompanying complications, as in the current case [8]. Since we have thought of retropharyngeal abscess in our case, contrasted CT and MRI investigations were performed. The CT and MRI revealed right IJV thrombophlebitis findings, and filling defects in sigmoid-transvers sinus and air bubbles in IJV and transverse sinus were thought to be due to anaerobic infection. Although many LS cases were defined in the literature, air bubbles were not reported in the cranial sinuses or IJV in any of the cases. Interestingly, in the present case, there were air bubbles both in the transverse sinus and in the IJV. Radiology is also effective in determination of metastatic infections [8]. Satisfactory results are obtained in most cases with antimicrobial treatment and surgical drainage. The use of anticoagulants in LS is controversial due to a lack of controlled studies [10]. We did not use anticoagulants in the present study. We could not obtain control imaging and, therefore, cannot comment. However, with radical mastoidectomy and appropriate antibiotic treatment, the patient completely recovered.
[1] P. D. Karkos, S. Asrani, C. D. Karkos et al., “Lemierre’s syndrome: a systematic review,” Laryngoscope, vol. 119, no. 8, pp. 1552–1559, 2009. [2] A. Lemierre, “On certain septicaemias due to anaerobic organisms,” The Lancet, vol. 227, no. 5874, pp. 701–703, 1936. [3] S. K. Aggarwal, A. Nath, R. Singh, and A. Keshri, “Lemierre’s syndrome presenting with neurological and pulmonary symptoms: case report and review of the literature,” Annals of Indian Academy of Neurology, vol. 16, no. 2, pp. 259–263, 2013. [4] J. Moore Gillon, T. H. Lee, S. J. Eykyn, and I. Phillips, “Necrobacillosis: a forgotten disease,” British Medical Journal, vol. 288, no. 6429, pp. 1526–1527, 1984. [5] T. Riordan and M. Wilson, “Lemierre’s syndrome: more than a historical curiosa,” Postgraduate Medical Journal, vol. 80, no. 944, pp. 328–334, 2004. [6] D. Nguyen, Y. Yaacob, H. Hamid, and S. Muda, “Necrotizing fasciitis on the right side of the neck with internal jugular vein thrombophlebitis and septic emboli: a case of Lemierre’s-like syndrome,” Malaysian Journal of Medical Sciences, vol. 20, no. 5, pp. 70–78, 2013. [7] C. M. Harris, M. Johnikin, H. Rhodes et al., “Lemierre’s syndrome resulting from streptococcal induced otitis media and mastoiditis: a case report,” Journal of Medical Case Reports, vol. 3, article 6658, 2009. [8] N. J. Screaton, J. G. Ravenel, P. J. Lehner, E. R. Heitzman, and C. D. Flower, “Lemierre syndrome: forgotten but not extinct— report of four cases,” Radiology, vol. 213, no. 2, pp. 369–374, 1999. [9] C. Repanos, N. K. Chadha, and M. V. Griffiths, “Sigmoid sinus thrombosis secondary to Lemierre’s syndrome,” Ear, Nose and Throat Journal, vol. 85, no. 2, pp. 98–101, 2006. [10] C. K. Phua, V. M. Chadachan, and R. Acharya, “Lemierre syndrome—should we anticoagulate? A case report and review of the literature,” International Journal of Angiology, vol. 22, no. 2, pp. 137–142, 2013.
Hindawi Publishing Corporation Case Reports in Emergency Medicine Volume 2014, Article ID 210146, 3 pages http://dx.doi.org/10.1155/2014/210146
Case Report Spontaneous Cervical Epidural Hematoma with Hemiparesis Mimicking Cerebral Stroke Mehmet Tiryaki,1 Recep Basaran,1 Serdar Onur Aydin,1 Mustafa Efendioglu,1 Ece Balkuv,2 and Naci Balak3 1
Department of Neurosurgery, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Kartal, 34890 Istanbul, Turkey Department of Neurology, Istanbul Medeniyet University Goztepe Education and Research Hospital, 34730 Istanbul, Turkey 3 Department of Neurosurgery, Istanbul Medeniyet University Goztepe Education and Research Hospital, 34730 Istanbul, Turkey 2
Correspondence should be addressed to Recep Basaran; [email protected] Received 20 July 2014; Revised 20 September 2014; Accepted 22 September 2014; Published 2 October 2014 Academic Editor: Kazuhito Imanaka Copyright © 2014 Mehmet Tiryaki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Aim. Spontaneous cervical epidural hematoma (SCEH) is defined as an epidural hematoma that does not have an etiological explanation. The most common site for SCEH is cervicothoracic area. Early diagnosis and treatment are important for prognosis and good results. In this paper, we aimed to present a case who complains of sudden weakness on right extremities imitating cerebral stroke and that neuroimaging reveals spontaneous cervical epidural hematoma. Case. A 72-year-old woman was admitted to our hospital with acute neck pain and loss of strength on right extremities. On neurological examination, the patient had right hemiparesis. PT, aPTT, and INR results were 50.5, 42.8, and 4.8, respectively. Cranial MRI was in normal limits. Spinal MRI revealed a lesion that extends from C4 to C7 located on the right side and compatible with epidural hematoma. The patient was operated after normalization of INR values. Conclusion. Even though SCEH is a rare condition, it can cause severe morbidity and mortality. Early diagnosis and treatment are quiet important for prognosis. SCEH can easily be mistaken for stroke as with other pathologies and this diagnosis should come to mind especially in patients who have diathesis of bleeding.
1. Introduction
2. Case Report
Spontaneous cervical epidural hematoma (SCEH) is defined as an epidural hematoma that does not have a known etiological reason [1]. Although arteriovenous malformations, tumors, trauma, or postoperative complications are blamed for causes of this situation, the most common site of SCEH is cervicothoracic area [2]. Patients usually complain of acute neck pain or interscapular pain. As a result of spinal cord pressure, sensory and motor loss can be seen [1]. SCEH is an important and urgent cause of spinal cord pressure. Its incidence is estimated as 0,1/100000 patients [3]. Male/female ratio is 1,4/1 [4]. Early diagnosis and treatment are essential for a good prognosis. We aimed to present a patient with right hemiparesis initially mistaken for a cerebrovascular disease. Further tests revealed cervical epidural hemorrhage and the patient has been operated on.
A 72-year-old female patient was brought to the emergency department with a sudden onset of severe sharp neck pain, upper back pain, and weakness on right arm and leg. Approximately 5 years ago, the patient had a mitral valve replacement and uses warfarin. On neurological examination, her strength was 2-3/5 in right upper limb and 2/5 in right lower limb. In laboratory test results PT value was 50, 5, APTT value was 42, 8, and INR value was 4, 8. Cranial CT scan results were in normal limits and there was no sign of bleeding (Figure 1). Cranial magnetic resonance imaging (MRI) was also within normal limits and there were no evidence pathologies such as tumor or ischemia (Figure 2). Spinal MRI revealed a rightly localized lesion compatible with epidural hematoma that extended from C4 to C7 (Figure 3). Following the infusion of three units of fresh frozen plasma (FFP), INR value was 1.8 and the patient underwent surgery. Right partial C4–C7
2
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Figure 3: MRI of spine showed epidural hematoma lining right lateral to spinal cord in cervical region.
Figure 1: CT scan showed no sign of hemorrhage.
Figure 2: MRI of brain was normal; there is no sign of ischemic or tumor lesion.
and C5-C6 total laminectomy was performed. Thrombosed hematoma has been discharged. On postoperative cervical MRI hematoma was totally emptied. On postoperative neurological examination of the right upper and lower extremity, muscle strength on the right side was 4/5. The patient was able to walk without aid after three months of physical therapy and rehabilitation program.
3. Discussion SCEH was first described by Jackson in 1869 [5]. The first surgery was realized by Bain in 1897 [6]. The annual incidence
is estimated as 0,1/100000 [3]. SCEH is defined as the accumulation of blood in the epidural space in the absence of trauma or vertebral iatrogenic interference. Some authors included coagulopathy, vascular malformation bleeding, or hemorrhagic tumor in this recognition; by some authors, only idiopathic bleeding was evaluated in this definition. Idiopathic SCEH constitutes 40–61% of the cases [1]. Most common localization sites are C6 and T12 levels [2]. Lo et al. listed some factors that cause SCEH such as the use of anticoagulants, thrombolytic therapy, uncontrolled hypertension, long-term use of antiplatelet, factor XI deficiency, and congenital disorders such as hemophilia B [7]. There are some controversies whether the source of bleeding is venous or arterial. Many authors claim that the source of bleeding is venous and it is due to a lack of tissue cover in the epidural venous plexus. A sudden pressure increase in the abdominal cavity or thoracic venous pressure is thought to cause tearing and bleeding [8]. After all, according to some authors, because the arterial pressure in the epidural space is higher than the venous pressure and because of the rapid progression of neurological deterioration, the bleeding that causes SCEH is an arterial bleeding not a venous one [1]. In our case, we did not observe any arterial bleeding during surgery. Still, there is a need for more studies in order to clarify the pathogenesis of SCEH. The most common initial symptom of SCEH is sudden neck or back pain that spreads to a dermatome depending on hematoma’s localization area. Due to the compression of the spinal cord and nerve roots, sensation and motor deficits may be seen in the patients. Mostly, paraparesis or quadriparesis is seen depending on the level of compression of the spinal cord. Hemiparesis is a rare clinical feature [9]. Hemiparesis may be produced by anything that interrupts the corticospinal tract from its origin down to the cervical spine. Etiologies include lesions of the cerebral hemisphere as tumor, traumatic brain pathologies, vascular, and infection or lesions of internal capsule, brain stem, and unilateral spinal cord above C5. Hypoglycemia can sometimes be associated with hemiparesis that clears after the administration of glucose [10]. In 2012, Matsumoto et al. reported cases of SCEH that reveal hemiparesis similarly to our case [11]. Unlike cerebral infarction, pain exists in SCEH. Depending on the size of the lesion, the pain may be followed by loss of
Case Reports in Emergency Medicine sensory or motor deficits and motor deficits are seen more frequently [11]. Computed tomography (CT) is the first choice of imaging in the suspicion of cerebral hemorrhage. In the presence of ischemic lesions, diffusion weighted MRI is a better choice for imaging than CT. Cerebral infarction can be detected in diffusion weighted MRI in a couple of hours after the onset of complaints [12]. For the assessment of spinal lesions, MRI gives detailed information about the localization and size of hematomas, spinal cord edema, and severity of the pressure. In the early stages, SCEH is seen iso or hypointense in T1-weighted imaging and hyperintense in T2-weighted imaging [7]. SCEH is usually a surgical emergency. The most effective treatment is to perform a decompressive laminectomy and hematoma evacuation quickly [13]. Conservative treatment may be preferred in patients with no neurological deterioration or cases with serious high surgical risk or regressive complaints. Recently, studies showed that conservative treatment of cervical lesions is associated with poor outcomes [14]. In SCEH, postoperative mortality rate is around 3–6% [8]. The prognosis of SCEH is closely related to the level and size of the hematoma, the degree of preoperative neurological deficit, and the time between the onset of symptoms and surgery. Recent studies showed that hematomas extending between 2 and 10 spinal segments are associated with poor outcomes [15]. Also, surgery performed in the first 36 hours on patients with severe deficits and surgery performed in the first 48 hours on patients with mild deficits increase the possibility of recovery [8]. In a study realized by Shin et al., the surgical recovery rates were found as 83% for the patients operated in the first 12 hours after the onset of initial symptoms, 63.6% for the patients operated between the 12th and the 24th hours after the onset of initial symptoms, and 46.7% for the patients operated 24 hours following the onset of initial symptoms [16].
4. Conclusion Although SCEH is a rare condition, it can cause severe morbidity and mortality. Early diagnosis and treatment are crucial for the best outcomes. SCEH can imitate different pathologies such as a stroke and this diagnosis should come to mind especially in patients with bleeding diathesis.
Conflict of Interests The authors declare that there is no conflict of interests regarding the publication of this paper.
References [1] R. M. Beatty and K. R. Winston, “Spontaneous cervical epidural hematoma: a consideration of etiology,” Journal of Neurosurgery, vol. 61, no. 1, pp. 143–148, 1984. [2] R. J. M. Groen and J. Goffin, “Non-operative treatment of spontaneous spinal epidural hematomas: a review of the literature and a comparison with operative cases,” Acta Neurochirurgica, vol. 146, no. 2, pp. 103–110, 2004.
3 [3] S. Holt˚as, M. Heiling, and M. L¨onntoft, “Spontaneous spinal epidural hematoma: findings at MR imaging and clinical correlation,” Radiology, vol. 199, no. 2, pp. 409–413, 1996. [4] M. M. C. Lonjon, P. Paquis, S. Chanalet, and P. Grellier, “Nontraumatic spinal epidural hematoma: report of four cases and review of the literature,” Neurosurgery, vol. 41, no. 2, pp. 483–487, 1997. [5] R. Jackson, “Case of spinal apoplexy,” The Lancet, vol. 94, no. 2392, pp. 5–6, 1869. [6] W. Bain, “A case of haematorrachis,” British Medical Journal, vol. 2, article 455, 1912. [7] C.-C. Lo, J.-Y. Chen, Y.-K. Lo, P.-H. Lai, and Y.-T. Lin, “Spontaneous spinal epidural hematoma: a case report and review of the literatures,” Acta Neurologica Taiwanica, vol. 21, no. 1, pp. 31–34, 2012. [8] C.-C. Liao, S.-T. Lee, W.-C. Hsu, L.-R. Chen, T.-N. Lui, and S.C. Lee, “Experience in the surgical management of spontaneous spinal epidural hematoma,” Journal of Neurosurgery, vol. 100, no. 1, pp. 38–45, 2004. [9] S. Riaz, H. Jiang, R. Fox, M. Lavoie, and J. K. Mahood, “Spontaneous spinal epidural hematoma causing Brown- Sequard syndrome: case report and review of the literature,” Journal of Emergency Medicine, vol. 33, no. 3, pp. 241–244, 2007. [10] M. S. Greenberg, Handbook of Neurosurgery, vol. 914, Georg Thieme, New York, NY, USA, 2006. [11] H. Matsumoto, T. Miki, Y. Miyaji et al., “Spontaneous spinal epidural hematoma with hemiparesis mimicking acute cerebral infarction: two case reports,” Journal of Spinal Cord Medicine, vol. 35, no. 4, pp. 262–266, 2012. [12] J. A. Chalela, C. S. Kidwell, L. M. Nentwich et al., “Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison,” The Lancet, vol. 369, no. 9558, pp. 293–298, 2007. [13] C.-C. Liao, P.-C. Hsieh, T.-K. Lin, C.-L. Lin, Y.-L. Lo, and S.-C. Lee, “Surgical treatment of spontaneous spinal epidural hematoma: a 5-year experience: clinical article,” Journal of Neurosurgery: Spine, vol. 11, no. 4, pp. 480–486, 2009. [14] S. J. Hentschel, A. R. Woolfenden, and D. J. Fairholm, “Resolution of spontaneous spinal epidural hematoma without surgery: report of two cases.,” Spine, vol. 26, no. 22, pp. E525–527, 2001. [15] Z. Liu, Q. Jiao, J. Xu, X. Wang, S. Li, and C. You, “Spontaneous spinal epidural hematoma: analysis of 23 cases,” Surgical Neurology, vol. 69, no. 3, pp. 253–260, 2008. [16] J.-J. Shin, S.-U. Kuh, and Y.-E. Cho, “Surgical management of spontaneous spinal epidural hematoma,” European Spine Journal, vol. 15, no. 6, pp. 998–1004, 2006.
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Case Report A Case of Lemierre Syndrome Secondary to Otitis Media and Mastoiditis Aynur Turan,1 Harun Cam,1 Yeliz Dadali,2 Serdar Korkmaz,1 Ali Özdek,3 and Baki HekimoLlu1 1
Department of Radiology, Diskapi Yildirim Beyazit Training and Research Hospital, Etlik, 06010 Ankara, Turkey Department of Radiology, Faculty of Medicine, Ahi Evran University, Kirs¸ehir, Turkey 3 Department of Otolaryngology, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey 2
Correspondence should be addressed to Aynur Turan; aynur [email protected] Received 4 May 2014; Accepted 16 October 2014; Published 6 November 2014 Academic Editor: Aristomenis K. Exadaktylos Copyright © 2014 Aynur Turan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Lemierre’s syndrome is a rare clinical condition that generally develops secondary to oropharyngeal infection caused by Fusobacterium necrophorum, which is an anaerobic bacteria. A 62-year-old patient with diabetes mellitus presented with internal jugular vein and sigmoid sinus-transverse sinus thrombophlebitis, accompanying otitis media and mastoiditis that developed after an upper airway infection. Interestingly, there were air bubbles in both the internal jugular vein and transverse sinus. Vancomycin and meropenem were started and a right radical mastoidectomy was performed. The patient’s clinical picture completely resolved in 14 days. High mortality and morbidity may be prevented with a prompt diagnosis of Lemierre’s syndrome.
1. Introduction In 90% of cases, the cause of Lemierre’s syndrome (LS) is the Fusobacterium necrophorum, which is an anaerobic, gram negative bacteria. On the other hand, anaerobic Streptococci and other gram negative anaerobic bacteria are responsible for the remaining 10% of cases [1]. The disease has been described in detail by Lemierre in 1936 [2]. Mortality rates were as high as 90% in its first described periods and decreased to 4–18% with the widespread use of antibiotics [2, 3]. The current study aimed to draw attention to this disease described as the “forgotten disease” in the literature [4] and present the radiological findings of a case of internal jugular vein (IJV) thrombophlebitis and accompanying sigmoidtransverse sinus thrombosis that was believed to develop secondary to otitis media (OM) and mastoiditis.
2. Case A 62-year-old male patient was on cefuroxime axetil treatment for an upper airway infection that persisted for 15 days. However, he was admitted to the emergency department with complaints of redness, swelling, and pain increasing
with motion in the neck region, leakage from the right ear, headache, shivering, and fever. His body temperature was 39∘ C upon physical examination. There was grade I tonsillar hypertrophy in the oropharyngeal examination. The right external ear way was edematous and the tympanic membrane was macerated. There was also tenderness in the neck region. Any abnormalities related to the lungs were not present in the physical examination and the neurological examination was normal. No other additional findings were detected in the physical examination. The patient had a history of diabetes mellitus type 2 (T2DM) for 15 years. His blood glucose was 317 mg/dL and hemoglobin A1c was 10.2%, indicating poorly controlled T2DM. His white cell count was 18,000/mm3 and other pertinent laboratory results were unremarkable. With the clinical suspicion of deep neck infection, brain and neck computed tomography (CT) was performed. The neck CT revealed internal jugular venous distention with a thickened enhancing wall, filling defects in the lumen, and air bubbles. In the brain CT, there were aeration defects and effusion on mastoid cellules and the middle ear and filling defects and air bubbles in the right sigmoid and transvers sinuses (Figure 1). In order to determine the extent and other
2
Figure 1: Air bubbles as seen in the brain CT of the right sigmoid sinus.
accompanying complications, contrasted brain and neck MRI were performed, which revealed that there was signal void loss in T2WI on the right IJV (Figure 2), right sigmoid, and transverse sinus; internal jugular venous distention with a thickened enhancing wall and filling defects in the IJV and sigmoid-transverse sinus lumens (Figures 3 and 4). Thrombosis of other intracerebral veins was not observed. Moreover, there were inflammatory signal changes on the right mastoid cellules and the middle ear cavity. These findings were evaluated as compatible with right OM, mastoiditis, IJV, sigmoid, and transverse sinus thrombophlebitis. Air bubbles observed both in the IJV and transverse sinus alluded to the fact that the infection was caused by anaerobe bacteria. The blood culture was negative. The patient was diagnosed with Lemierre’s syndrome, owing to the OM and mastoiditis and accompanying sigmoid and transverse sinus thrombosis. Vancomycin 2 gr/day IV and Meropenem 3 × 2 gr/day IV treatments were administered for two weeks and a right radical mastoidectomy was performed. The patient improved rapidly and the clinical picture of the patient resolved completely in 14 days. A control imaging for the resolution of thrombosis could not be performed because the patient was lost during follow-up.
3. Discussion The causative agent of Lemierre’s syndrome is commonly Fusobacterium necrophorum, which is an anaerobic bacteria present in the oral cavity, gastrointestinal system (GIS), and female genital system flora. In the last 20 years, an increase in its incidence has been reported owing to various reasons. The disease may be seen in any age group; however, more than 70% of cases are healthy adolescents and young adults. It has been reported more commonly in males than females [4, 5]. An association between Lemierre’s syndrome and diabetes mellitus has been reported [6] in the literature as well.
Case Reports in Emergency Medicine
Figure 2: Axial T2-weighted MRI revealed signal void loss on the right sigmoid and transverse sinuses.
Figure 3: Neck MRI with contrast showed internal jugular venous distention with a thickened enhancing wall and filling defects.
Prior to the disease, in general as in the current case, an oropharyngeal disease history of about one week is present. At the beginning of the disease, the physical examination findings may be very slight [4, 7]. Typically, the clinical course shows a gradual, step-by-step pattern in LS. The preliminary period includes findings such as fever, pharyngitis, otitis media, mastoiditis, and parotitis. Later, local microbial invasion to the lateral pharyngeal area and extension to the IJV by the way of infected peritonsillary veins and lymphatics may be determined. Owing to the involvement of the posterior compartment, cranial nerve X and XII palsies and Horner syndrome may also develop. Lastly, bacteremia and septic emboli may be seen in the other organs like lungs, bones, brain, and liver [4, 7, 8]. In the present case, transition to
Case Reports in Emergency Medicine
3 Since the incidence of Lemierre’s syndrome has increased over the last 20 years and the disease has high mortality and morbidity rates, prompt diagnosis of the disease is important. Using radiological instruments in the diagnosis, such as Doppler US, contrasted CT, and/or MRI should not be delayed in suspected cases.
Conflict of Interests The authors declare that there is no conflict of interests regarding the publication of this paper.
References
Figure 4: Axial brain MRI with contrast showed filling defects in right sigmoid and transverse sinuses.
the last step was not yet present, but OM, mastoiditis, and thrombophlebitis of the IJV and sigmoid-transverse sinus were present. Central nervous system involvement is extremely rare in LS, but purulent meningitis, cerebral abscess, and extension of IJV thrombophlebitis to the sigmoid and cavernous sinuses in a retrograde manner have been reported [5, 9]. Though few in number, similar to the current case, IJV thrombophlebitis and sigmoid and transverse sinus thrombosis cases secondary to OM and mastoiditis have been reported in the literature [7]. In the diagnosis of LS, radiology is tremendously important. Doppler US considerably enables the demonstration of the thrombus in IJV without ionizing radiation. In regions to which US cannot extend, such as the skull base or below the clavicle, contrasted CT and MRI warrant complete IJV visualization and moreover enable the visualization of anterograde and retrograde extensions and accompanying complications, as in the current case [8]. Since we have thought of retropharyngeal abscess in our case, contrasted CT and MRI investigations were performed. The CT and MRI revealed right IJV thrombophlebitis findings, and filling defects in sigmoid-transvers sinus and air bubbles in IJV and transverse sinus were thought to be due to anaerobic infection. Although many LS cases were defined in the literature, air bubbles were not reported in the cranial sinuses or IJV in any of the cases. Interestingly, in the present case, there were air bubbles both in the transverse sinus and in the IJV. Radiology is also effective in determination of metastatic infections [8]. Satisfactory results are obtained in most cases with antimicrobial treatment and surgical drainage. The use of anticoagulants in LS is controversial due to a lack of controlled studies [10]. We did not use anticoagulants in the present study. We could not obtain control imaging and, therefore, cannot comment. However, with radical mastoidectomy and appropriate antibiotic treatment, the patient completely recovered.
[1] P. D. Karkos, S. Asrani, C. D. Karkos et al., “Lemierre’s syndrome: a systematic review,” Laryngoscope, vol. 119, no. 8, pp. 1552–1559, 2009. [2] A. Lemierre, “On certain septicaemias due to anaerobic organisms,” The Lancet, vol. 227, no. 5874, pp. 701–703, 1936. [3] S. K. Aggarwal, A. Nath, R. Singh, and A. Keshri, “Lemierre’s syndrome presenting with neurological and pulmonary symptoms: case report and review of the literature,” Annals of Indian Academy of Neurology, vol. 16, no. 2, pp. 259–263, 2013. [4] J. Moore Gillon, T. H. Lee, S. J. Eykyn, and I. Phillips, “Necrobacillosis: a forgotten disease,” British Medical Journal, vol. 288, no. 6429, pp. 1526–1527, 1984. [5] T. Riordan and M. Wilson, “Lemierre’s syndrome: more than a historical curiosa,” Postgraduate Medical Journal, vol. 80, no. 944, pp. 328–334, 2004. [6] D. Nguyen, Y. Yaacob, H. Hamid, and S. Muda, “Necrotizing fasciitis on the right side of the neck with internal jugular vein thrombophlebitis and septic emboli: a case of Lemierre’s-like syndrome,” Malaysian Journal of Medical Sciences, vol. 20, no. 5, pp. 70–78, 2013. [7] C. M. Harris, M. Johnikin, H. Rhodes et al., “Lemierre’s syndrome resulting from streptococcal induced otitis media and mastoiditis: a case report,” Journal of Medical Case Reports, vol. 3, article 6658, 2009. [8] N. J. Screaton, J. G. Ravenel, P. J. Lehner, E. R. Heitzman, and C. D. Flower, “Lemierre syndrome: forgotten but not extinct— report of four cases,” Radiology, vol. 213, no. 2, pp. 369–374, 1999. [9] C. Repanos, N. K. Chadha, and M. V. Griffiths, “Sigmoid sinus thrombosis secondary to Lemierre’s syndrome,” Ear, Nose and Throat Journal, vol. 85, no. 2, pp. 98–101, 2006. [10] C. K. Phua, V. M. Chadachan, and R. Acharya, “Lemierre syndrome—should we anticoagulate? A case report and review of the literature,” International Journal of Angiology, vol. 22, no. 2, pp. 137–142, 2013.
Hindawi Publishing Corporation Case Reports in Emergency Medicine Volume 2014, Article ID 210146, 3 pages http://dx.doi.org/10.1155/2014/210146
Case Report Spontaneous Cervical Epidural Hematoma with Hemiparesis Mimicking Cerebral Stroke Mehmet Tiryaki,1 Recep Basaran,1 Serdar Onur Aydin,1 Mustafa Efendioglu,1 Ece Balkuv,2 and Naci Balak3 1
Department of Neurosurgery, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Kartal, 34890 Istanbul, Turkey Department of Neurology, Istanbul Medeniyet University Goztepe Education and Research Hospital, 34730 Istanbul, Turkey 3 Department of Neurosurgery, Istanbul Medeniyet University Goztepe Education and Research Hospital, 34730 Istanbul, Turkey 2
Correspondence should be addressed to Recep Basaran; [email protected] Received 20 July 2014; Revised 20 September 2014; Accepted 22 September 2014; Published 2 October 2014 Academic Editor: Kazuhito Imanaka Copyright © 2014 Mehmet Tiryaki et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Aim. Spontaneous cervical epidural hematoma (SCEH) is defined as an epidural hematoma that does not have an etiological explanation. The most common site for SCEH is cervicothoracic area. Early diagnosis and treatment are important for prognosis and good results. In this paper, we aimed to present a case who complains of sudden weakness on right extremities imitating cerebral stroke and that neuroimaging reveals spontaneous cervical epidural hematoma. Case. A 72-year-old woman was admitted to our hospital with acute neck pain and loss of strength on right extremities. On neurological examination, the patient had right hemiparesis. PT, aPTT, and INR results were 50.5, 42.8, and 4.8, respectively. Cranial MRI was in normal limits. Spinal MRI revealed a lesion that extends from C4 to C7 located on the right side and compatible with epidural hematoma. The patient was operated after normalization of INR values. Conclusion. Even though SCEH is a rare condition, it can cause severe morbidity and mortality. Early diagnosis and treatment are quiet important for prognosis. SCEH can easily be mistaken for stroke as with other pathologies and this diagnosis should come to mind especially in patients who have diathesis of bleeding.
1. Introduction
2. Case Report
Spontaneous cervical epidural hematoma (SCEH) is defined as an epidural hematoma that does not have a known etiological reason [1]. Although arteriovenous malformations, tumors, trauma, or postoperative complications are blamed for causes of this situation, the most common site of SCEH is cervicothoracic area [2]. Patients usually complain of acute neck pain or interscapular pain. As a result of spinal cord pressure, sensory and motor loss can be seen [1]. SCEH is an important and urgent cause of spinal cord pressure. Its incidence is estimated as 0,1/100000 patients [3]. Male/female ratio is 1,4/1 [4]. Early diagnosis and treatment are essential for a good prognosis. We aimed to present a patient with right hemiparesis initially mistaken for a cerebrovascular disease. Further tests revealed cervical epidural hemorrhage and the patient has been operated on.
A 72-year-old female patient was brought to the emergency department with a sudden onset of severe sharp neck pain, upper back pain, and weakness on right arm and leg. Approximately 5 years ago, the patient had a mitral valve replacement and uses warfarin. On neurological examination, her strength was 2-3/5 in right upper limb and 2/5 in right lower limb. In laboratory test results PT value was 50, 5, APTT value was 42, 8, and INR value was 4, 8. Cranial CT scan results were in normal limits and there was no sign of bleeding (Figure 1). Cranial magnetic resonance imaging (MRI) was also within normal limits and there were no evidence pathologies such as tumor or ischemia (Figure 2). Spinal MRI revealed a rightly localized lesion compatible with epidural hematoma that extended from C4 to C7 (Figure 3). Following the infusion of three units of fresh frozen plasma (FFP), INR value was 1.8 and the patient underwent surgery. Right partial C4–C7
2
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Figure 3: MRI of spine showed epidural hematoma lining right lateral to spinal cord in cervical region.
Figure 1: CT scan showed no sign of hemorrhage.
Figure 2: MRI of brain was normal; there is no sign of ischemic or tumor lesion.
and C5-C6 total laminectomy was performed. Thrombosed hematoma has been discharged. On postoperative cervical MRI hematoma was totally emptied. On postoperative neurological examination of the right upper and lower extremity, muscle strength on the right side was 4/5. The patient was able to walk without aid after three months of physical therapy and rehabilitation program.
3. Discussion SCEH was first described by Jackson in 1869 [5]. The first surgery was realized by Bain in 1897 [6]. The annual incidence
is estimated as 0,1/100000 [3]. SCEH is defined as the accumulation of blood in the epidural space in the absence of trauma or vertebral iatrogenic interference. Some authors included coagulopathy, vascular malformation bleeding, or hemorrhagic tumor in this recognition; by some authors, only idiopathic bleeding was evaluated in this definition. Idiopathic SCEH constitutes 40–61% of the cases [1]. Most common localization sites are C6 and T12 levels [2]. Lo et al. listed some factors that cause SCEH such as the use of anticoagulants, thrombolytic therapy, uncontrolled hypertension, long-term use of antiplatelet, factor XI deficiency, and congenital disorders such as hemophilia B [7]. There are some controversies whether the source of bleeding is venous or arterial. Many authors claim that the source of bleeding is venous and it is due to a lack of tissue cover in the epidural venous plexus. A sudden pressure increase in the abdominal cavity or thoracic venous pressure is thought to cause tearing and bleeding [8]. After all, according to some authors, because the arterial pressure in the epidural space is higher than the venous pressure and because of the rapid progression of neurological deterioration, the bleeding that causes SCEH is an arterial bleeding not a venous one [1]. In our case, we did not observe any arterial bleeding during surgery. Still, there is a need for more studies in order to clarify the pathogenesis of SCEH. The most common initial symptom of SCEH is sudden neck or back pain that spreads to a dermatome depending on hematoma’s localization area. Due to the compression of the spinal cord and nerve roots, sensation and motor deficits may be seen in the patients. Mostly, paraparesis or quadriparesis is seen depending on the level of compression of the spinal cord. Hemiparesis is a rare clinical feature [9]. Hemiparesis may be produced by anything that interrupts the corticospinal tract from its origin down to the cervical spine. Etiologies include lesions of the cerebral hemisphere as tumor, traumatic brain pathologies, vascular, and infection or lesions of internal capsule, brain stem, and unilateral spinal cord above C5. Hypoglycemia can sometimes be associated with hemiparesis that clears after the administration of glucose [10]. In 2012, Matsumoto et al. reported cases of SCEH that reveal hemiparesis similarly to our case [11]. Unlike cerebral infarction, pain exists in SCEH. Depending on the size of the lesion, the pain may be followed by loss of
Case Reports in Emergency Medicine sensory or motor deficits and motor deficits are seen more frequently [11]. Computed tomography (CT) is the first choice of imaging in the suspicion of cerebral hemorrhage. In the presence of ischemic lesions, diffusion weighted MRI is a better choice for imaging than CT. Cerebral infarction can be detected in diffusion weighted MRI in a couple of hours after the onset of complaints [12]. For the assessment of spinal lesions, MRI gives detailed information about the localization and size of hematomas, spinal cord edema, and severity of the pressure. In the early stages, SCEH is seen iso or hypointense in T1-weighted imaging and hyperintense in T2-weighted imaging [7]. SCEH is usually a surgical emergency. The most effective treatment is to perform a decompressive laminectomy and hematoma evacuation quickly [13]. Conservative treatment may be preferred in patients with no neurological deterioration or cases with serious high surgical risk or regressive complaints. Recently, studies showed that conservative treatment of cervical lesions is associated with poor outcomes [14]. In SCEH, postoperative mortality rate is around 3–6% [8]. The prognosis of SCEH is closely related to the level and size of the hematoma, the degree of preoperative neurological deficit, and the time between the onset of symptoms and surgery. Recent studies showed that hematomas extending between 2 and 10 spinal segments are associated with poor outcomes [15]. Also, surgery performed in the first 36 hours on patients with severe deficits and surgery performed in the first 48 hours on patients with mild deficits increase the possibility of recovery [8]. In a study realized by Shin et al., the surgical recovery rates were found as 83% for the patients operated in the first 12 hours after the onset of initial symptoms, 63.6% for the patients operated between the 12th and the 24th hours after the onset of initial symptoms, and 46.7% for the patients operated 24 hours following the onset of initial symptoms [16].
4. Conclusion Although SCEH is a rare condition, it can cause severe morbidity and mortality. Early diagnosis and treatment are crucial for the best outcomes. SCEH can imitate different pathologies such as a stroke and this diagnosis should come to mind especially in patients with bleeding diathesis.
Conflict of Interests The authors declare that there is no conflict of interests regarding the publication of this paper.
References [1] R. M. Beatty and K. R. Winston, “Spontaneous cervical epidural hematoma: a consideration of etiology,” Journal of Neurosurgery, vol. 61, no. 1, pp. 143–148, 1984. [2] R. J. M. Groen and J. Goffin, “Non-operative treatment of spontaneous spinal epidural hematomas: a review of the literature and a comparison with operative cases,” Acta Neurochirurgica, vol. 146, no. 2, pp. 103–110, 2004.
3 [3] S. Holt˚as, M. Heiling, and M. L¨onntoft, “Spontaneous spinal epidural hematoma: findings at MR imaging and clinical correlation,” Radiology, vol. 199, no. 2, pp. 409–413, 1996. [4] M. M. C. Lonjon, P. Paquis, S. Chanalet, and P. Grellier, “Nontraumatic spinal epidural hematoma: report of four cases and review of the literature,” Neurosurgery, vol. 41, no. 2, pp. 483–487, 1997. [5] R. Jackson, “Case of spinal apoplexy,” The Lancet, vol. 94, no. 2392, pp. 5–6, 1869. [6] W. Bain, “A case of haematorrachis,” British Medical Journal, vol. 2, article 455, 1912. [7] C.-C. Lo, J.-Y. Chen, Y.-K. Lo, P.-H. Lai, and Y.-T. Lin, “Spontaneous spinal epidural hematoma: a case report and review of the literatures,” Acta Neurologica Taiwanica, vol. 21, no. 1, pp. 31–34, 2012. [8] C.-C. Liao, S.-T. Lee, W.-C. Hsu, L.-R. Chen, T.-N. Lui, and S.C. Lee, “Experience in the surgical management of spontaneous spinal epidural hematoma,” Journal of Neurosurgery, vol. 100, no. 1, pp. 38–45, 2004. [9] S. Riaz, H. Jiang, R. Fox, M. Lavoie, and J. K. Mahood, “Spontaneous spinal epidural hematoma causing Brown- Sequard syndrome: case report and review of the literature,” Journal of Emergency Medicine, vol. 33, no. 3, pp. 241–244, 2007. [10] M. S. Greenberg, Handbook of Neurosurgery, vol. 914, Georg Thieme, New York, NY, USA, 2006. [11] H. Matsumoto, T. Miki, Y. Miyaji et al., “Spontaneous spinal epidural hematoma with hemiparesis mimicking acute cerebral infarction: two case reports,” Journal of Spinal Cord Medicine, vol. 35, no. 4, pp. 262–266, 2012. [12] J. A. Chalela, C. S. Kidwell, L. M. Nentwich et al., “Magnetic resonance imaging and computed tomography in emergency assessment of patients with suspected acute stroke: a prospective comparison,” The Lancet, vol. 369, no. 9558, pp. 293–298, 2007. [13] C.-C. Liao, P.-C. Hsieh, T.-K. Lin, C.-L. Lin, Y.-L. Lo, and S.-C. Lee, “Surgical treatment of spontaneous spinal epidural hematoma: a 5-year experience: clinical article,” Journal of Neurosurgery: Spine, vol. 11, no. 4, pp. 480–486, 2009. [14] S. J. Hentschel, A. R. Woolfenden, and D. J. Fairholm, “Resolution of spontaneous spinal epidural hematoma without surgery: report of two cases.,” Spine, vol. 26, no. 22, pp. E525–527, 2001. [15] Z. Liu, Q. Jiao, J. Xu, X. Wang, S. Li, and C. You, “Spontaneous spinal epidural hematoma: analysis of 23 cases,” Surgical Neurology, vol. 69, no. 3, pp. 253–260, 2008. [16] J.-J. Shin, S.-U. Kuh, and Y.-E. Cho, “Surgical management of spontaneous spinal epidural hematoma,” European Spine Journal, vol. 15, no. 6, pp. 998–1004, 2006.
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