Bilateral cavernous sinus and superior ophthalmic vein thrombosis in the setting of facial cellulitis Almas Syed, MD, Bruce Bell, MD, Joseph Hise, MD, Joseph Philip, MD, Cedric Spak, MD, MPH, and Michael J. Opatowsky, MD, MBA
Cavernous sinus thrombosis is a rare, potentially fatal cause of cerebral venous thrombosis. Infectious causes typically arise from the mid face, orbit, or sinonasal region. We present a case of bilateral cavernous sinus and superior ophthalmic thrombosis secondary to an extreme case of facial cellulitis.
CASE REPORT A 25-year-old woman initially presented with complaints of a headache and had reportedly injected methamphetamine into her left cheek. She was diagnosed with left facial cellulitis with an open ulceration, sinusitis, and methicillin-resistant Staphylococcus aureus (MRSA) bacteremia, for which she was being treated. On presentation to our facility, she was obtunded with left greater than right chemosis and anisocoria. The left maxillary and periorbital regions were swollen. A chest radiograph revealed a multifocal nodular and patchy infiltrate. She was intubated due to worsening respiratory distress and obtundation. Laboratory analysis revealed a neutrophilic-predominant leukocytosis (21.5 K/uL), metabolic acidosis (pH 7.2), and substantial electrolyte derangements, including a serum sodium level of 160 mEq/L. Repeat blood cultures showed MRSA. (1,3)-Beta-D-glucan (Fungitell) assay was negative for associated fungal infections. An unenhanced computed tomography (CT) scan of the brain and contrast-enhanced CT angiogram demonstrated multiple abnormal findings, including multiple acute infarcts in the bilateral frontal and parietal lobes and left superior cerebellum. Thrombus was present in the left internal jugular vein, left sigmoid and transverse sinuses, cavernous sinuses bilaterally, and the superior ophthalmic veins bilaterally, as confirmed on magnetic resonance imaging (MRI) (Figure 1). Substantial vasospasm was noted involving the cavernous segment of the left internal carotid artery (Figure 1a). Extensive bilateral acute cerebral infarcts were apparent within the bilateral anterior circulation as well as developing ischemic injury to the pons, cerebellum, hypothalamus, and mammillary bodies (Figure 2a). The MRI examination also showed diffuse left facial cellulitis in conjunction with sites of venous thrombosis within the bilateral cavernous sinuses, superior ophthalmic veins, left transverse sinus, sigmoid sinus, and left internal jugular vein (Figure 2b, 2c). 36
Attempts were made to hemodynamically stabilize the patient and to gradually correct the hypernatremia. She was initially placed on broad-spectrum antibiotic coverage that was later refined to vancomycin and rifampin based on positive blood cultures for MRSA. Heparin was started. The patient rapidly succumbed. DISCUSSION While not rare, thrombosis of the cerebral veins is a relatively uncommon clinical presentation. Venous thrombosis is thought to be associated with 0.5% to 1% of all strokes (1). Cavernous sinus thrombosis (CST) is a potentially devastating condition associated with a mortality rate approaching 30% (2). When precipitated by highly aggressive infections, CST can result in rapid neurologic deterioration, permanent impairment, or death. Prompt diagnosis and emergent treatment are crucial to ensure survival and to prevent permanent disability. Patients with infection-induced CST will present with a nonspecific fever and headache. Often, accompanying proptosis, chemosis, periorbital swelling, and cranial nerve palsies will be encountered (2). An understanding of the underlying venous anatomy allows for a better appreciation of the pathophysiology and clinical presentation of CST. The cavernous sinuses reside bilaterally adjacent to the pituitary-containing sella turcica. They are formed by a separation of the dura mater with multiple trabeculae forming the “cavernous” appearance of these venous spaces. Unique to the cavernous sinuses is the intimate association of flowing venous channels with a number of critical cranial nerves as well as a robust anastomotic vascular network. The horizontal segment of the internal carotid artery and the abducens nerve (CNVI) are enveloped by and traverse this venous sinus. The oculomotor nerve (CN III), trochlear nerve (CN IV), and the first and second divisions of the trigeminal nerve (CN V) are intimately associated with the lateral walls of the cavernous sinuses (3). Intracavernous pathology can alter ocular motility by preferentially affecting CNVI function. This might From the Department of Radiology (Syed, Bell, Hise, Philip, Opatowsky) and the Division of Infectious Diseases (Spak), Baylor University Medical Center at Dallas. Corresponding author: Almas Syed, MD, Department of Radiology, Baylor University Medical Center at Dallas, 3500 Gaston Avenue, Dallas, TX 75246 (e-mail: [email protected]). Proc (Bayl Univ Med Cent) 2016;29(1):36–38
a
b
c
d
Figure 1. (a) CT angiogram demonstrating the attenuated appearance of the left supraclinoid internal carotid artery secondary to inflammatory vasospasm (arrow). (b) Contrast-enhanced MR revealing lack of enhancement and enlargement of the left cavernous sinus with a convex margin (arrow). (c) T2-weighted MR sequence showing enlarged bilateral superior ophthalmic veins with lack of flow-related signal due to thromboses (arrows). (d) Contrast-enhanced MR also showing a lack of expected flow-related signal in the setting of bilateral superior ophthalmic venous thromboses (arrows).
a
b
Figure 2. (a) Diffusion-weighted MR sequence demonstrating bilateral frontoparietal areas of diffusion restriction (arrows) consistent with acute infarctions (confirmatory ADC maps not shown). (b) Enhanced MR demonstrating lack of enhancement of the left sigmoid sinus consistent with dural venous sinus thrombosis (arrow). January 2016
be seen clinically as an inability of the affected globe to achieve normal conjugate temporal gaze. The veins from the middle third of the face are interconnected to the cavernous sinus through the pterygoid plexus and ophthalmic veins. With progressive CST, venous engorgement and stasis often will be appreciated clinically by developing proptosis and chemosis (4). It is thought that this interconnected vascular network, with its unique pattern of venous drainage accompanied by valveless veins, leads to the propensity for infections from the middle third of the face to spread to the cavernous sinuses. Th e contribution of valveless veins as a risk factor for the dissemination of facial or orbital infections into the cavernous sinuses has been debated in the literature. At least one report in the literature indicated finding valves in the facial and superior ophthalmic veins (5). The cavernous sinuses can also communicate with one another across the anatomic midline via the anterior and posterior intercavernous veins, potentially serving as a source for bilateral seeding of thrombophlebitis. The cavernous sinuses drain into the petrosal sinuses with eventual downstream drainage into the internal jugular veins, placing them at risk for thrombosis as well (4). Diagnosis of CST is best established on clinical grounds and confirmed by radiographic studies. Radiologic findings include expansion of a cavernous sinus with reversal of its typical concave outer margin, asymmetrically diminished enhancement with filling defects, and narrowing or occlusion of the cavernous portion of the internal carotid artery (Figure 1). Due to obstruction or marked narrowing and compromise, upstream venous congestion can be seen in the ophthalmic veins with resultant extraocular muscle enlargement and induration of the retrobulbar fat (5–7).
Bilateral cavernous sinus and superior ophthalmic vein thrombosis in the setting of facial cellulitis
37
1.
2. 3.
4.
38
Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M, deVeber G, Ferro JM, Tsai FY; American Heart Association Stroke Council and the Council on Epidemiology and Prevention. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011;42(4):1158–1192. DiNubile MJ. Septic thrombosis of the cavernous sinuses. Arch Neurol 1988;45(5):567–572. Lucarilli MJ, Kim N. Applied anatomy of the orbit. In Mallajosyula S, ed. Surgical Atlas of Orbital Disease, 1st ed. Maryland Heights, MO: Jaypee Brothers, 2009:8–9. Ebright JR, Pace MT, Niazi AF. Septic thrombosis of the cavernous sinuses. Arch Intern Med 2001;161(22):2671–2676.
Zhang J, Stringer MD. Ophthalmic and facial veins are not valveless. Clin Experiment Ophthalmol 2010;38(5):502–510. 6. Roth E, Wiant A, Sperling V. Cavernous sinus thrombosis [American College of Radiology Case in Point, 2012]. Retrieved from https://3s.acr. org/CIP/ArchiveCaseView.aspx?CaseId=MuJzbBK185U%253d; accessed October 26, 2015. 7. Komatsu H, Matsumoto F, Kasai M, Kurano K, Sasaki D, Ikeda K. Cavernous sinus thrombosis caused by contralateral sphenoid sinusitis: a case report. Head Face Med 2013;9:9. 8. Coutinho J, de Bruijn SF, Deveber G, Stam J. Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database Syst Rev 2011;(8):CD002005. 5.
Baylor University Medical Center Proceedings
Volume 29, Number 1
Cavernous sinus thrombosis is a rare, potentially fatal cause of cerebral venous thrombosis. Infectious causes typically arise from the mid face, orbit, or sinonasal region. We present a case of bilateral cavernous sinus and superior ophthalmic thrombosis secondary to an extreme case of facial cellulitis.
CASE REPORT A 25-year-old woman initially presented with complaints of a headache and had reportedly injected methamphetamine into her left cheek. She was diagnosed with left facial cellulitis with an open ulceration, sinusitis, and methicillin-resistant Staphylococcus aureus (MRSA) bacteremia, for which she was being treated. On presentation to our facility, she was obtunded with left greater than right chemosis and anisocoria. The left maxillary and periorbital regions were swollen. A chest radiograph revealed a multifocal nodular and patchy infiltrate. She was intubated due to worsening respiratory distress and obtundation. Laboratory analysis revealed a neutrophilic-predominant leukocytosis (21.5 K/uL), metabolic acidosis (pH 7.2), and substantial electrolyte derangements, including a serum sodium level of 160 mEq/L. Repeat blood cultures showed MRSA. (1,3)-Beta-D-glucan (Fungitell) assay was negative for associated fungal infections. An unenhanced computed tomography (CT) scan of the brain and contrast-enhanced CT angiogram demonstrated multiple abnormal findings, including multiple acute infarcts in the bilateral frontal and parietal lobes and left superior cerebellum. Thrombus was present in the left internal jugular vein, left sigmoid and transverse sinuses, cavernous sinuses bilaterally, and the superior ophthalmic veins bilaterally, as confirmed on magnetic resonance imaging (MRI) (Figure 1). Substantial vasospasm was noted involving the cavernous segment of the left internal carotid artery (Figure 1a). Extensive bilateral acute cerebral infarcts were apparent within the bilateral anterior circulation as well as developing ischemic injury to the pons, cerebellum, hypothalamus, and mammillary bodies (Figure 2a). The MRI examination also showed diffuse left facial cellulitis in conjunction with sites of venous thrombosis within the bilateral cavernous sinuses, superior ophthalmic veins, left transverse sinus, sigmoid sinus, and left internal jugular vein (Figure 2b, 2c). 36
Attempts were made to hemodynamically stabilize the patient and to gradually correct the hypernatremia. She was initially placed on broad-spectrum antibiotic coverage that was later refined to vancomycin and rifampin based on positive blood cultures for MRSA. Heparin was started. The patient rapidly succumbed. DISCUSSION While not rare, thrombosis of the cerebral veins is a relatively uncommon clinical presentation. Venous thrombosis is thought to be associated with 0.5% to 1% of all strokes (1). Cavernous sinus thrombosis (CST) is a potentially devastating condition associated with a mortality rate approaching 30% (2). When precipitated by highly aggressive infections, CST can result in rapid neurologic deterioration, permanent impairment, or death. Prompt diagnosis and emergent treatment are crucial to ensure survival and to prevent permanent disability. Patients with infection-induced CST will present with a nonspecific fever and headache. Often, accompanying proptosis, chemosis, periorbital swelling, and cranial nerve palsies will be encountered (2). An understanding of the underlying venous anatomy allows for a better appreciation of the pathophysiology and clinical presentation of CST. The cavernous sinuses reside bilaterally adjacent to the pituitary-containing sella turcica. They are formed by a separation of the dura mater with multiple trabeculae forming the “cavernous” appearance of these venous spaces. Unique to the cavernous sinuses is the intimate association of flowing venous channels with a number of critical cranial nerves as well as a robust anastomotic vascular network. The horizontal segment of the internal carotid artery and the abducens nerve (CNVI) are enveloped by and traverse this venous sinus. The oculomotor nerve (CN III), trochlear nerve (CN IV), and the first and second divisions of the trigeminal nerve (CN V) are intimately associated with the lateral walls of the cavernous sinuses (3). Intracavernous pathology can alter ocular motility by preferentially affecting CNVI function. This might From the Department of Radiology (Syed, Bell, Hise, Philip, Opatowsky) and the Division of Infectious Diseases (Spak), Baylor University Medical Center at Dallas. Corresponding author: Almas Syed, MD, Department of Radiology, Baylor University Medical Center at Dallas, 3500 Gaston Avenue, Dallas, TX 75246 (e-mail: [email protected]). Proc (Bayl Univ Med Cent) 2016;29(1):36–38
a
b
c
d
Figure 1. (a) CT angiogram demonstrating the attenuated appearance of the left supraclinoid internal carotid artery secondary to inflammatory vasospasm (arrow). (b) Contrast-enhanced MR revealing lack of enhancement and enlargement of the left cavernous sinus with a convex margin (arrow). (c) T2-weighted MR sequence showing enlarged bilateral superior ophthalmic veins with lack of flow-related signal due to thromboses (arrows). (d) Contrast-enhanced MR also showing a lack of expected flow-related signal in the setting of bilateral superior ophthalmic venous thromboses (arrows).
a
b
Figure 2. (a) Diffusion-weighted MR sequence demonstrating bilateral frontoparietal areas of diffusion restriction (arrows) consistent with acute infarctions (confirmatory ADC maps not shown). (b) Enhanced MR demonstrating lack of enhancement of the left sigmoid sinus consistent with dural venous sinus thrombosis (arrow). January 2016
be seen clinically as an inability of the affected globe to achieve normal conjugate temporal gaze. The veins from the middle third of the face are interconnected to the cavernous sinus through the pterygoid plexus and ophthalmic veins. With progressive CST, venous engorgement and stasis often will be appreciated clinically by developing proptosis and chemosis (4). It is thought that this interconnected vascular network, with its unique pattern of venous drainage accompanied by valveless veins, leads to the propensity for infections from the middle third of the face to spread to the cavernous sinuses. Th e contribution of valveless veins as a risk factor for the dissemination of facial or orbital infections into the cavernous sinuses has been debated in the literature. At least one report in the literature indicated finding valves in the facial and superior ophthalmic veins (5). The cavernous sinuses can also communicate with one another across the anatomic midline via the anterior and posterior intercavernous veins, potentially serving as a source for bilateral seeding of thrombophlebitis. The cavernous sinuses drain into the petrosal sinuses with eventual downstream drainage into the internal jugular veins, placing them at risk for thrombosis as well (4). Diagnosis of CST is best established on clinical grounds and confirmed by radiographic studies. Radiologic findings include expansion of a cavernous sinus with reversal of its typical concave outer margin, asymmetrically diminished enhancement with filling defects, and narrowing or occlusion of the cavernous portion of the internal carotid artery (Figure 1). Due to obstruction or marked narrowing and compromise, upstream venous congestion can be seen in the ophthalmic veins with resultant extraocular muscle enlargement and induration of the retrobulbar fat (5–7).
Bilateral cavernous sinus and superior ophthalmic vein thrombosis in the setting of facial cellulitis
37
1.
2. 3.
4.
38
Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M, deVeber G, Ferro JM, Tsai FY; American Heart Association Stroke Council and the Council on Epidemiology and Prevention. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011;42(4):1158–1192. DiNubile MJ. Septic thrombosis of the cavernous sinuses. Arch Neurol 1988;45(5):567–572. Lucarilli MJ, Kim N. Applied anatomy of the orbit. In Mallajosyula S, ed. Surgical Atlas of Orbital Disease, 1st ed. Maryland Heights, MO: Jaypee Brothers, 2009:8–9. Ebright JR, Pace MT, Niazi AF. Septic thrombosis of the cavernous sinuses. Arch Intern Med 2001;161(22):2671–2676.
Zhang J, Stringer MD. Ophthalmic and facial veins are not valveless. Clin Experiment Ophthalmol 2010;38(5):502–510. 6. Roth E, Wiant A, Sperling V. Cavernous sinus thrombosis [American College of Radiology Case in Point, 2012]. Retrieved from https://3s.acr. org/CIP/ArchiveCaseView.aspx?CaseId=MuJzbBK185U%253d; accessed October 26, 2015. 7. Komatsu H, Matsumoto F, Kasai M, Kurano K, Sasaki D, Ikeda K. Cavernous sinus thrombosis caused by contralateral sphenoid sinusitis: a case report. Head Face Med 2013;9:9. 8. Coutinho J, de Bruijn SF, Deveber G, Stam J. Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database Syst Rev 2011;(8):CD002005. 5.
Baylor University Medical Center Proceedings
Volume 29, Number 1
