Otogenic cavernous sinus thrombosis KAREN JO DOYLE, MD, PhD, and ROBERT K. JACKLER, MD, San Francisco, California
S e p t i c thrombosis of the dural venous sinuses was a fairly common complication of otitis media in the preantibiotic era. This dire complication frequently led to the patient’s death, either through overwhelming sepsis or intracranial complication. A small incidence of otogenic dural sinus thrombosis persists today, generally occurring in those patients whose ear infections have not been treated with specific antimicrobial therapy. The sigmoid sinus is the dural venous structure most frequently involved in otologic infection. Propagation of sigmoid sinus thrombosis (SST) via the petrosal sinuses to the cavernous sinus has been rarely reported in the literature. We present a recent case of otogenic cavernous sinus thrombosis (CST) that illustrates the contemporary diagnosis and management of this rare complication. CASE REPORT
A 19-year-old man with a submucous cleft palate and chronically draining ears was admitted to the hospital with a 48-hour history of fever and shaking chills, followed by headache, neck pain, and photophobia. Examination revealed bilateral purulent otorrhea emanating from a tympanic membrane perforation on the left and a right tympanostomy tube. Meningismus was detected and a lumbar puncture was performed that demonstrated 2500 WBCs per high-power field (90% polymorphonuclear leukocytes), a glucose of 60 mgldl, and a moderately elevated protein. Cerebrospinal fluid cultures were negative. Culture of the left ear drainage grew Pseudomonas aeruginosa and rare, coagulase-negative Staphylococcus. Blood cultures grew anaerobic gram-negative rods (not typed). Computed tomography (CT) of the head showed opacification of a hypopneumatic mastoid, changes interpreted as consistent with left mastoiditis. A regimen of intravenous vancomycin hydrochloride and aztreonam was begun to treat the cultured organisms, as recommended by the infectious disease specialists. Over the next 2 days, his fevers
From the Department of Otolaryngology -Head and Neck Surgery, University of California, San Francisco. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, New Orleans, La., Sept. 24-28, 1989. Received for publication July 6 , 1990; revision received Nov. 10, 1990; accepted Nov. 26, 1990. Reprint requests: Robert K . Jackler, MD, 350 Parnassus Ave., Suite 210, San Francisco, CA 941 17. 23 14 I26932
and meningismus persisted. On the third hospital day, the patient became obtunded, and subsequent to this a left proptosis, chemosis, sixth cranial nerve palsy, and partial third nerve palsy developed. A repeat CT scan revealed left orbital edema, engorgement of the superior orbital vein, and fluid in the sphenoid and ethmoid sinuses that had been clear on the previous study. Progressive thrombocytopenia to 37,000 occurred, and the patient was transferred to the Medical Center at the University of California, San Francisco. Shortly after admission the patient was taken to the operating room, where he underwent a complete left mastoidectomy and intranasal sphenoidotomy. At operation, hypopneumatized, purulent mastoid air cells were found, as was erosion of the bony plate overlying the sigmoid sinus, with granulations on the sinus wall. There was no cholesteatoma. A 25-gauge needle was introduced into the sigmoid sinus, and no blood was aspirated. The sigmoid sinus was incised longitudinally over a 1.5-centimeter length, revealing thrombus, which was partially removed and sent for culture. The cultures from the clot eventually grew coagulase-negative Staphylococcus. A strip of Surgicel was placed in the sigmoid sinus to control bleeding, and a drain was left in the mastoid antrum. Sphenoidotomy was performed under endoscopic control, with enlargement of the sphenoid ostium and removal of mucus from the sphenoid. The mucus was sent for culture and grew no organisms. The patient’s antibiotic regimen was changed postoperatively to intravenous ticarcillin with clavulanate potassium and tobramycin, to which all of the cultured organisms were sensitive. Postoperatively, the patient was given intravenous heparin to inhibit further propagation of the thrombosis. Chest radiograph revealed no evidence of septic emboli and the patient had no respiratory problems. Magnetic resonance imaging (MRI) of the head was performed that demonstrated changes consistent with meningitis, left cavernous sinus and sigmoid sinus thrombosis, and vasculitis of the internal carotid arteries (Figs. 1, 2, and 3). He was given oral anticoagulation with coumadin after placement of a Hickman catheter (Davol Inc., Cranston, R.I.) for home antibiotic administration. After a 3-week hospitalization, he was discharged home and intravenous antibiotics were administered for an additional 3 weeks and oral anticoagulation with coumadin for a total of 3 months. At latest followup, 1 year after his illness, he had returned entirely to his premorbid condition. His most lasting neurologic deficit was the sixth nerve palsy, which took several months to resolve.
DISCUSSION Septic thrombophlebitis of the dural venous sinuses has long been recognized as a potential complication of ear infection. In the 1880s, Politzer’ described this
873 Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
OtolaryngologyHead and N e c k Surgery
874 Case Reports
Fig. 1. Sagittal TI-weighted MRi view demonstrates increased intensity of the left transverse sinus (7.S) consistent with thrombus. This structure normally would show flow void on TI-weighted images.
complication in considerable detail and suggested two pathogenic mechanisms: direct spread of infection along pathways created by erosive osteitis and retrograde thrombophlebitis. Little has been added to this thesis over the past 100 years. In the pre-antibiotic era, these infections were relatively common and almost invariably fatal. The patient usually succumbed from either systemic sepsis associated with widespread metastatic abscesses or as a consequence of intracranial suppuration. Since the advent of antibiotics, the frequency of otogenic dural sinus thrombosis has greatly diminished and in the rare cases seen today the morbidity and mortality have been greatly reduced.’.’ The initial nidus of thrombophlebitis that arises from otologic infection develops in the sigmoid sinus, which abuts the posterior aspect of the mastoid. In the early stages of the infectious process, a mural thrombus forms that enlarges and ultimately occludes the lumen. With prompt antibiotic treatment and mastoid drainage, the infection may be arrested at this stage. Delayed or incomplete therapy may permit propagation of the septic thrombus in either the anterograde or retrograde direction. This is fostered by stasis of blood proximal or distal to the thrombotic arrest in flow. The thrombotic
process tends to halt when the vein or sinus reaches a branch point at which flowing blood is encountered. Anterograde propagation results in progressive thrombosis of the jugular bulb or jugular vein and may even extend into the superior vena cava.’ In such cases, the internal jugular vein is ligated to prevent pulmonary complications. In the antibiotic era, routine ligation of the internal jugular vein is no longer performed, and complete removal of the sigmoid sinus thrombus is not considered necessary at the time of mastoidectomy.’ Downward spread of thrombus may be limited in the skull base by flowing blood entering the medial aspect of the jugular bulb via the inferior petrosal sinus or extracranially, at the junction of the internal jugular vein and the common facial or external jugular veins. Retrograde spread from the sigmoid sinus may extend posteriorly into the transverse sinus and potentially the superior sagittal sinus. Involvement of the superior sagittal sinus may impair the function of arachnoid granulations and result in otitic hydrocephalus.“ Retrograde propagation may be limited by flowing blood entering at the sigmoid/ transverse sinus junction (via the superior petrosal sinus) or, more posteriorly, by the torcular vein.
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Volume 104 Number 6 June 1991
Case Reports 875
(.'
Fig. 2. Axial gradient recalled echo MRI shows asymmetry of the right sigmoid sinus and jugular bulb (JB) compared with the left, with hyperintensity on the right. uninvolved side and hypointensity on the left. At surgev, the left SS was full of thrombus.
The focus of the present report is otogenic cavernous sinus thrombosis, an exceedingly rare-but nevertheless important-complication that may evolve from sigmoid sinus thrombosis. Two anatomic interconnections exist between the lateral venous sinus system and the cavernous sinus: the superior and inferior petrosal sinuses. The direction of blood flow in these sinuses is away from the cavernous sinus towards either the transverse/ sigmoid junction or the jugular bulb, respectively. Thus, this medial propagation of the thrombus to the cavernous sinus represents retrograde extension of the process. CST in the antibiotic era most frequently evolves from sinusitis, especially infections of the sphenoid or e t h m ~ i d In . ~ this case, sphenoid and ethmoid opacification did not appear until later in the course of the disease, as documented by CT scans, and we interpreted their presence as sympathetic effusions. This was confirmed by the intraoperative findings during sphenoidotomy. Infections of the mid-third of the face, which in the pre-antibiotic era were the most common
site of origin of CST, now constitute the second most common cause. The signs and symptoms of CST have been well-described. Fever and retro-orbital pain often precede the classic clinical signs of ptosis, proptosis, chemosis, and ophthalmoplegia. Without prompt therapy, eye findings are rapidly followed by drowsiness, confusion, inappropriate speech and behavior. and eventual death from overwhelming intracranial sepsis. In the pre-antibiotic era, otologic infections seldom gave rise to CST, despite the much higher frequency of otogenic intracranial complications at that time. In 1883, Politzerl described two cases of of otogenic CST. On post-mortem examination, both patients were found to have sigmoid sinus thrombosis with retrograde spread to the cavernous sinus-one via the superior petrosal sinus and the other by way of the inferior petrosal sinus. There are few contemporary reports of otogenic CST. Dawes'" included two cases of CST among 97 patients diagnosed with otogenic dural sinus thronibophlebitis
Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
OtolaryngologyHead and Neck Surgery
876 Case Reports
Fig. 3. Axial TI-weighted MRI shows thickening of the walls of the intracavernous carotid arteries (CAI. Hyperintensity (absence of flow void] of the left cavernous sinus [LCS)is consistent with thrombus in that sinus. Also note proptosis of the left orbit.
between 1944 and 1960. No description of the clinical course or outcome of these patients was included. In 1967, Pasquet et al.” presented a case of otogenic CST complicating acute otitis media and meningitis. An autopsy revealed diffuse meningitis and thrombophlebitis of both the sigmoid and cavernous sinuses, with secondary involvement of the carotid artery and oculomotor nerve. In 1973, Seid and Sellars” included in their series of 13 cases of SST one patient with CST who died. This patient’s underlying otologic condition was chronic otitis media with cholesteatoma. In 1973. Biskupska-Wiecko and Zawisza” reported three cases of otogenic CST among 22 cases of CST. All three patients recovered, but one experienced permanent bilateral blindness. In the past, the diagnosis of CST was made by clinical evaluation or on post-mortem examination. Sigmoid sinus septic thrombosis, in contrast, was usually detected during mastoid surgery. More recently, the noninvasive diagnosis of venous sinus thrombosis has been
greatly facilitated by advances in diagnostic imaging techniques. CT and MRI scanning have become routine when intracranial complications of otitis are suspected. CT with intravenous contrast enhancement is an excellent tool for the diagnosis of epidural and parenchymal abscesses, subdural empyema, and hydrocephalus. IR sinus thrombosis, the so-called delta sign, a lowintensity central region surrounded by a high-intensity rim, may be seen.“ An empty triangle appearance is created by clot within the sinus surrounded by contrastenhanced dura. This sign has been used to establish the diagnosis of thromboses in the superior sagittal, transverse, and sigmoid sinuses.” A similar picture may be seen on MRI scanning, but only when accompanied by the administration of gadolinium-DTPA. I’ The tentorium may also demonstrate contrast enhancement in sinus thrombosis on both CT and MRI scans. The radiographic evaluation of the status of blood flow within a dural sinus no longer requires arteriography. On CT scanning. the appearance of contrast ma-
Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
Volume 104 Number 6 June 1991
Case Reports 877
terial in the lumen is evidence of flowing blood. However, the absence of intraluminal contrast enhancement does not necessarily imply thrombosis. MRI may be more helpful than CT in establishing the presence of blood flow or thrombosis. In non-contrast-enhanced MRI studies of sinus thrombosis-as in the present case-findings may be difficult to interpret.” The intraluminal signal may be hyper-, iso-, or hypointense compared to adjacent brain parenchyma and is dependent on a number of factors, such as the age of the thrombus and the rate of blood flow, if any. New techniques that involve special flow images (MRI angiography) may convincingly demonstrate high signal within flowing vessels and even indicate the direction of flow. ’* In a recent case of sigmoid sinus thrombosis (unreported), we used this technique and were able to demonstrate both sinus obstruction and reversal of flow in the transverse sinus proximal to the lesion. The use of CT and MRI in patients with SST represents an advance over the use of cerebral angiography, with its attendant risks of increasing intracranial pressure or dislodging thrombus. Treatment of otogenic sinus thrombosis requires prolonged intravenous antibiotics. l9 Anticoagulation is not recommended in sigmoid sinus thrombosis in the absence of embolic phenomena or progressive thrombophlebitis . 3 No studies have documented improved survival with the use of anticoagulants in typical sigmoid sinus thrombosis, and there is potential for hemorrhage within an intracerebral venous infarction or from the operative site. In contrast, anticoagulation is a mainstay of treatment in septic CST.” Early anticoagulation therapy, as was used in the present case, reduces the morbidity (blindness, stroke, ophthalmoplegia, seizures, vascular steal syndrome, hypopituitarism) associated with CST.20 In our case, we chose to anticoagulate, maintaining heparin for 12 days, preferring its reversibility in the face of possible bleeding, then continued therapy with coumadin for 3 months. In conclusion, the present case illustrates that survival in otogenic CST without lasting sequelae is possible with the combination of modern antibiotic therapy, anticoagulation, and prompt surgical intervention.
REFERENCES
1. Politzer A. Suppuration of the middle ear ending fatally from phlebitis of the cerebral sinuses. In: Diseases of the ear and adjacent organs. Philadelphia: Henry C. Lea’s Son & Co, 1883536.41. 2. Teichgraeber JF, Per-Lee JH, Turner JS. Lateral sinus thrombosis: a modern perspective. Laryngoscope 1982;92:744-5 1. 3. Sneed WF. Lateral sinus thrombosis. Am J Otol 1983;4:258-62. 4. Debmyne F. Lateral sinus thrombosis in the eighties. J Laryngol Otol 1985;99:91-3. 5 . Venezio FR, Naidich TP, Shulman ST. Complications of mastoiditis with special emphasis on venous sinus thrombosis. J Pediatr 1982;101:509-13. 6. Amirmajdi NM. Sigmoid sinus involvement in middle ear infection. Laryngoscope 1988;98:310-2. 7. Tovi F, Hirsch M, Gatot A. Superior vena cava syndrome: presenting symptom of silent otitis media. J Laryngol Otol 1988;102:623-5. 8. Lenz RP, McDonald GA. Otitic hydrocephalus. Laryngoscope 1984;94: 1451-4. 9. Southwick FS, Richardson EP, Swartz MN. Septic thrombosis of the dural venous sinuses. Medicine 1986;65:82-106. 10. Dawes JDK. Discussion on intracranial complications of otogenic origin. Proc Roy SOCMed 1961;54:315-20. 11. Pasquet EG, Medoc J , Purriel JA. Meningitis aguda purulenta otogena complicada de thromboplebitis del sen0 cavernoso. Acta Neurol Latinoamer 1967;13:140-4. 12. Seid AB, Sellars SL. The management of otogenic lateral sinus disease at Groote Schuur Hospital. Laryngoscope 1973;83:397403. 13. Biskupska-Wiecko J, Zawisza E. Otogenic thrombophlebitis of the cavernous sinus. Otolaryngol Pol 1973;27:149-55. 14. Buonanno FS, Moody DM, Ball MR, Laster DW. Computed cranial tomographic findings in cerebral sinovenous occlusion. J Comput Assist Tomogr 1978;2:281-90. 15. Goldberg AL, Rosenbaum AE, Wang H, Kim WS, Lewis VL, Hanley DF. Computed tomography of dural sinus thrombosis. J Comput Assist Tomogr 1986;lO:16-20. 16. Harris TM, Smith RR, Koch KJ. Gadolinium-DTPA enhanced MR imaging of septic dural sinus thrombosis. J Comput Assist Tomogr 1989;13:682-4. 17. Macchi PJ, Grossman RI, Gomori JM, Goldberg HI, Zimmerman RA, Bilaniuk LT. High field MR imaging of cerebral venous thrombosis. J Comp Assist Tomogr 1986;lO: 10-5. 18. Masaryk TJ, Modic MT, Ross JS, et al. Intracranial circulation: preliminary clinical results with three-dimensional (volume) MR angiography. Radiology 1989;171:793-9. 19. Mathews TJ. Lateral sinus pathology (22 cases managed at Groote Schuur Hospital). J Laryngol Otol 1988;102:118-20. 20. Levine SR, Twyman RE, Gilman S. The role of anticoagulation in cavernous sinus thrombosis. Neurology 1988;38:517-22.
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S e p t i c thrombosis of the dural venous sinuses was a fairly common complication of otitis media in the preantibiotic era. This dire complication frequently led to the patient’s death, either through overwhelming sepsis or intracranial complication. A small incidence of otogenic dural sinus thrombosis persists today, generally occurring in those patients whose ear infections have not been treated with specific antimicrobial therapy. The sigmoid sinus is the dural venous structure most frequently involved in otologic infection. Propagation of sigmoid sinus thrombosis (SST) via the petrosal sinuses to the cavernous sinus has been rarely reported in the literature. We present a recent case of otogenic cavernous sinus thrombosis (CST) that illustrates the contemporary diagnosis and management of this rare complication. CASE REPORT
A 19-year-old man with a submucous cleft palate and chronically draining ears was admitted to the hospital with a 48-hour history of fever and shaking chills, followed by headache, neck pain, and photophobia. Examination revealed bilateral purulent otorrhea emanating from a tympanic membrane perforation on the left and a right tympanostomy tube. Meningismus was detected and a lumbar puncture was performed that demonstrated 2500 WBCs per high-power field (90% polymorphonuclear leukocytes), a glucose of 60 mgldl, and a moderately elevated protein. Cerebrospinal fluid cultures were negative. Culture of the left ear drainage grew Pseudomonas aeruginosa and rare, coagulase-negative Staphylococcus. Blood cultures grew anaerobic gram-negative rods (not typed). Computed tomography (CT) of the head showed opacification of a hypopneumatic mastoid, changes interpreted as consistent with left mastoiditis. A regimen of intravenous vancomycin hydrochloride and aztreonam was begun to treat the cultured organisms, as recommended by the infectious disease specialists. Over the next 2 days, his fevers
From the Department of Otolaryngology -Head and Neck Surgery, University of California, San Francisco. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, New Orleans, La., Sept. 24-28, 1989. Received for publication July 6 , 1990; revision received Nov. 10, 1990; accepted Nov. 26, 1990. Reprint requests: Robert K . Jackler, MD, 350 Parnassus Ave., Suite 210, San Francisco, CA 941 17. 23 14 I26932
and meningismus persisted. On the third hospital day, the patient became obtunded, and subsequent to this a left proptosis, chemosis, sixth cranial nerve palsy, and partial third nerve palsy developed. A repeat CT scan revealed left orbital edema, engorgement of the superior orbital vein, and fluid in the sphenoid and ethmoid sinuses that had been clear on the previous study. Progressive thrombocytopenia to 37,000 occurred, and the patient was transferred to the Medical Center at the University of California, San Francisco. Shortly after admission the patient was taken to the operating room, where he underwent a complete left mastoidectomy and intranasal sphenoidotomy. At operation, hypopneumatized, purulent mastoid air cells were found, as was erosion of the bony plate overlying the sigmoid sinus, with granulations on the sinus wall. There was no cholesteatoma. A 25-gauge needle was introduced into the sigmoid sinus, and no blood was aspirated. The sigmoid sinus was incised longitudinally over a 1.5-centimeter length, revealing thrombus, which was partially removed and sent for culture. The cultures from the clot eventually grew coagulase-negative Staphylococcus. A strip of Surgicel was placed in the sigmoid sinus to control bleeding, and a drain was left in the mastoid antrum. Sphenoidotomy was performed under endoscopic control, with enlargement of the sphenoid ostium and removal of mucus from the sphenoid. The mucus was sent for culture and grew no organisms. The patient’s antibiotic regimen was changed postoperatively to intravenous ticarcillin with clavulanate potassium and tobramycin, to which all of the cultured organisms were sensitive. Postoperatively, the patient was given intravenous heparin to inhibit further propagation of the thrombosis. Chest radiograph revealed no evidence of septic emboli and the patient had no respiratory problems. Magnetic resonance imaging (MRI) of the head was performed that demonstrated changes consistent with meningitis, left cavernous sinus and sigmoid sinus thrombosis, and vasculitis of the internal carotid arteries (Figs. 1, 2, and 3). He was given oral anticoagulation with coumadin after placement of a Hickman catheter (Davol Inc., Cranston, R.I.) for home antibiotic administration. After a 3-week hospitalization, he was discharged home and intravenous antibiotics were administered for an additional 3 weeks and oral anticoagulation with coumadin for a total of 3 months. At latest followup, 1 year after his illness, he had returned entirely to his premorbid condition. His most lasting neurologic deficit was the sixth nerve palsy, which took several months to resolve.
DISCUSSION Septic thrombophlebitis of the dural venous sinuses has long been recognized as a potential complication of ear infection. In the 1880s, Politzer’ described this
873 Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
OtolaryngologyHead and N e c k Surgery
874 Case Reports
Fig. 1. Sagittal TI-weighted MRi view demonstrates increased intensity of the left transverse sinus (7.S) consistent with thrombus. This structure normally would show flow void on TI-weighted images.
complication in considerable detail and suggested two pathogenic mechanisms: direct spread of infection along pathways created by erosive osteitis and retrograde thrombophlebitis. Little has been added to this thesis over the past 100 years. In the pre-antibiotic era, these infections were relatively common and almost invariably fatal. The patient usually succumbed from either systemic sepsis associated with widespread metastatic abscesses or as a consequence of intracranial suppuration. Since the advent of antibiotics, the frequency of otogenic dural sinus thrombosis has greatly diminished and in the rare cases seen today the morbidity and mortality have been greatly reduced.’.’ The initial nidus of thrombophlebitis that arises from otologic infection develops in the sigmoid sinus, which abuts the posterior aspect of the mastoid. In the early stages of the infectious process, a mural thrombus forms that enlarges and ultimately occludes the lumen. With prompt antibiotic treatment and mastoid drainage, the infection may be arrested at this stage. Delayed or incomplete therapy may permit propagation of the septic thrombus in either the anterograde or retrograde direction. This is fostered by stasis of blood proximal or distal to the thrombotic arrest in flow. The thrombotic
process tends to halt when the vein or sinus reaches a branch point at which flowing blood is encountered. Anterograde propagation results in progressive thrombosis of the jugular bulb or jugular vein and may even extend into the superior vena cava.’ In such cases, the internal jugular vein is ligated to prevent pulmonary complications. In the antibiotic era, routine ligation of the internal jugular vein is no longer performed, and complete removal of the sigmoid sinus thrombus is not considered necessary at the time of mastoidectomy.’ Downward spread of thrombus may be limited in the skull base by flowing blood entering the medial aspect of the jugular bulb via the inferior petrosal sinus or extracranially, at the junction of the internal jugular vein and the common facial or external jugular veins. Retrograde spread from the sigmoid sinus may extend posteriorly into the transverse sinus and potentially the superior sagittal sinus. Involvement of the superior sagittal sinus may impair the function of arachnoid granulations and result in otitic hydrocephalus.“ Retrograde propagation may be limited by flowing blood entering at the sigmoid/ transverse sinus junction (via the superior petrosal sinus) or, more posteriorly, by the torcular vein.
Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
Volume 104 Number 6 June 1991
Case Reports 875
(.'
Fig. 2. Axial gradient recalled echo MRI shows asymmetry of the right sigmoid sinus and jugular bulb (JB) compared with the left, with hyperintensity on the right. uninvolved side and hypointensity on the left. At surgev, the left SS was full of thrombus.
The focus of the present report is otogenic cavernous sinus thrombosis, an exceedingly rare-but nevertheless important-complication that may evolve from sigmoid sinus thrombosis. Two anatomic interconnections exist between the lateral venous sinus system and the cavernous sinus: the superior and inferior petrosal sinuses. The direction of blood flow in these sinuses is away from the cavernous sinus towards either the transverse/ sigmoid junction or the jugular bulb, respectively. Thus, this medial propagation of the thrombus to the cavernous sinus represents retrograde extension of the process. CST in the antibiotic era most frequently evolves from sinusitis, especially infections of the sphenoid or e t h m ~ i d In . ~ this case, sphenoid and ethmoid opacification did not appear until later in the course of the disease, as documented by CT scans, and we interpreted their presence as sympathetic effusions. This was confirmed by the intraoperative findings during sphenoidotomy. Infections of the mid-third of the face, which in the pre-antibiotic era were the most common
site of origin of CST, now constitute the second most common cause. The signs and symptoms of CST have been well-described. Fever and retro-orbital pain often precede the classic clinical signs of ptosis, proptosis, chemosis, and ophthalmoplegia. Without prompt therapy, eye findings are rapidly followed by drowsiness, confusion, inappropriate speech and behavior. and eventual death from overwhelming intracranial sepsis. In the pre-antibiotic era, otologic infections seldom gave rise to CST, despite the much higher frequency of otogenic intracranial complications at that time. In 1883, Politzerl described two cases of of otogenic CST. On post-mortem examination, both patients were found to have sigmoid sinus thrombosis with retrograde spread to the cavernous sinus-one via the superior petrosal sinus and the other by way of the inferior petrosal sinus. There are few contemporary reports of otogenic CST. Dawes'" included two cases of CST among 97 patients diagnosed with otogenic dural sinus thronibophlebitis
Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
OtolaryngologyHead and Neck Surgery
876 Case Reports
Fig. 3. Axial TI-weighted MRI shows thickening of the walls of the intracavernous carotid arteries (CAI. Hyperintensity (absence of flow void] of the left cavernous sinus [LCS)is consistent with thrombus in that sinus. Also note proptosis of the left orbit.
between 1944 and 1960. No description of the clinical course or outcome of these patients was included. In 1967, Pasquet et al.” presented a case of otogenic CST complicating acute otitis media and meningitis. An autopsy revealed diffuse meningitis and thrombophlebitis of both the sigmoid and cavernous sinuses, with secondary involvement of the carotid artery and oculomotor nerve. In 1973, Seid and Sellars” included in their series of 13 cases of SST one patient with CST who died. This patient’s underlying otologic condition was chronic otitis media with cholesteatoma. In 1973. Biskupska-Wiecko and Zawisza” reported three cases of otogenic CST among 22 cases of CST. All three patients recovered, but one experienced permanent bilateral blindness. In the past, the diagnosis of CST was made by clinical evaluation or on post-mortem examination. Sigmoid sinus septic thrombosis, in contrast, was usually detected during mastoid surgery. More recently, the noninvasive diagnosis of venous sinus thrombosis has been
greatly facilitated by advances in diagnostic imaging techniques. CT and MRI scanning have become routine when intracranial complications of otitis are suspected. CT with intravenous contrast enhancement is an excellent tool for the diagnosis of epidural and parenchymal abscesses, subdural empyema, and hydrocephalus. IR sinus thrombosis, the so-called delta sign, a lowintensity central region surrounded by a high-intensity rim, may be seen.“ An empty triangle appearance is created by clot within the sinus surrounded by contrastenhanced dura. This sign has been used to establish the diagnosis of thromboses in the superior sagittal, transverse, and sigmoid sinuses.” A similar picture may be seen on MRI scanning, but only when accompanied by the administration of gadolinium-DTPA. I’ The tentorium may also demonstrate contrast enhancement in sinus thrombosis on both CT and MRI scans. The radiographic evaluation of the status of blood flow within a dural sinus no longer requires arteriography. On CT scanning. the appearance of contrast ma-
Downloaded from oto.sagepub.com at CAMBRIDGE UNIV LIBRARY on June 4, 2016
Volume 104 Number 6 June 1991
Case Reports 877
terial in the lumen is evidence of flowing blood. However, the absence of intraluminal contrast enhancement does not necessarily imply thrombosis. MRI may be more helpful than CT in establishing the presence of blood flow or thrombosis. In non-contrast-enhanced MRI studies of sinus thrombosis-as in the present case-findings may be difficult to interpret.” The intraluminal signal may be hyper-, iso-, or hypointense compared to adjacent brain parenchyma and is dependent on a number of factors, such as the age of the thrombus and the rate of blood flow, if any. New techniques that involve special flow images (MRI angiography) may convincingly demonstrate high signal within flowing vessels and even indicate the direction of flow. ’* In a recent case of sigmoid sinus thrombosis (unreported), we used this technique and were able to demonstrate both sinus obstruction and reversal of flow in the transverse sinus proximal to the lesion. The use of CT and MRI in patients with SST represents an advance over the use of cerebral angiography, with its attendant risks of increasing intracranial pressure or dislodging thrombus. Treatment of otogenic sinus thrombosis requires prolonged intravenous antibiotics. l9 Anticoagulation is not recommended in sigmoid sinus thrombosis in the absence of embolic phenomena or progressive thrombophlebitis . 3 No studies have documented improved survival with the use of anticoagulants in typical sigmoid sinus thrombosis, and there is potential for hemorrhage within an intracerebral venous infarction or from the operative site. In contrast, anticoagulation is a mainstay of treatment in septic CST.” Early anticoagulation therapy, as was used in the present case, reduces the morbidity (blindness, stroke, ophthalmoplegia, seizures, vascular steal syndrome, hypopituitarism) associated with CST.20 In our case, we chose to anticoagulate, maintaining heparin for 12 days, preferring its reversibility in the face of possible bleeding, then continued therapy with coumadin for 3 months. In conclusion, the present case illustrates that survival in otogenic CST without lasting sequelae is possible with the combination of modern antibiotic therapy, anticoagulation, and prompt surgical intervention.
REFERENCES
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