The Neuroradiology Journal 20: 291-294, 2007
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Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis. A Case Report J.J.S. SHANKAR, S.G. SRIKANTH, J.M. E KOVOOR, M.K. VASUDEV, S. SINHA National Institute of Mental Health and Neuro-Sciences; Bangalore, Karnatka, India
Key words: cavernous sinus thrombosis, superior ophthalmic vein thrombosis, pituitary macroadenoma
SUMMARY – Superior ophthalmic vein thrombosis is uncommon, and bilateral superior ophthalmic vein thrombosis is rarer still. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and not reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution.
Introduction Superior ophthalmic vein thrombosis is an uncommon clinical entity. Bilateral superior ophthalmic vein thrombosis is rarer still and is usually associated with some local inflammatory condition or coagulation disorder 1. Superior ophthalmic vein thrombosis is managed with appropriate antibiotics, anticoagulants and steroids. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time 2. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and has not been reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution. Case Report A 59-year-old woman presented with a complaint of diminished vision in her right eye for three days. She had occasional headache and nausea not associated with fever or vomiting. One month previously she had sudden onset drooping of the right eyelid which was associated with right eye pain and mild headache.
There was painful restriction of right eyeball movement. There was no history of visual blurring or diplopia at that time. She sought medical service and was diagnosed to be diabetic. She was started on insulin injection. No antibiotics or anticoagulants were given. Eyelid drooping improved over the next two weeks. However, painful restriction of the right eyeball remained for about another month. On examination her vital parameters were normal. External ocular movements were restricted and painful in the right eye whereas they were full in the left eye. Her visual acuity was 6/9 and 6/12 in right and left eyes respectively. There was no other neurological deficit or history of weight loss, bladder and bowel disturbances, no cold intolerance or change in voice. Brain MRI examination using routine spin echo T1W and TSE T2W sequences performed one month previously demonstrated pituitary macroadenoma. That MRI scan focused only the sella and suprasellar portion and did not cover the orbit. Hormonal assessment including serum cortisol, FSH (follicular stimulating hormone), prolactin, T4 and TSH (thyroid stimulating hormone) were within normal limits. Repeat MRI performed at this time, also covering the orbit, showed T1 and T2 hyperintensity 291
Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis
A
J.J.S. Shankar
B
C D
E
Figure A-E T1 coronal and T2 coronal images showing hyperintensity and enlargement of superior ophthalmic vein (A,B). 4 days later T1 coronal and T2 coronal images showing normal size and signal intensity in superior ophthalmic vein (C,D). T2 W coronal image showing pituitary macroadenoma and right cavernous sinus thrombosis (E).
in the dilated superior ophthalmic veins of both orbits more prominent on the right side (figure 1 A,B). Contrast study showed no enhancement of the superior ophthalmic veins with prominent lateral wall of the right cavernous sinus. Paranasal sinuses were clear with no evidence of sinusitis. The imaging features were suggestive of cavernous sinus thrombosis extending into the bilateral superior ophthalamic vein. Four days later (i.e., one month after the start of the first symptom and institution of insulin therapy), the patient came for follow-up with a complete resolution of symptoms. Repeat MRI at this time revealed complete disappearance of the changes within the ophthalmic veins (figure 1 C,D). 292
Discussion Superior ophthalmic vein thrombosis (SOVT) is rare 1. Previous reports have described unilateral superior ophthalmic vein thrombosis associated with orbital cellulitis 3, ethmoidal rhinosinusitis 4 and the combined oral contraceptive pill 5. The other causes of superior ophthalmic vein thrombosis are presence of carotico-cavernous fistula and underlying hypercoagulable state like raised factor VIII 1. Other rare causes are epidural hematoma, epidural and subdural infections, acute angle-closure glaucoma, subarachnoid hemorrhage, subdural hematoma, carotid artery aneurysm or fistula, exophthalmic goiter and orbital neoplasm. Bi-
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lateral ophthalamic vein thrombosis is an even rarer entity. Bilateral SOVT has only been reported in one patient in association with septic cavernous sinus thrombosis 6 and another young woman who was found to have raised factor VIII level and was on combined oral contraceptive pills 1. Superior ophthalmic vein thrombsosis is usually associated with cavernous sinus thrombosis (CST). This entity is usually a late complication of an infection of the central face or paranasal sinuses. Other causes include bacteremia, trauma and infections of the ear or maxillary teeth. This is generally a fulminant process with high rates of morbidity and mortality. Fortunately, the incidence of CST has been decreased greatly with the advent of effective antimicrobial agents 2. Staphylococcus aureus accounts for approximately 70% of all infections. Streptococcus pneumoniae, gram-negative bacilli, and anaerobes can also be seen. Fungi are a less common pathogen and may include Aspergillus and Rhizopus species. In a setting of diabetic and immunocompromised state fungal infection takes precedence. Earlier the mortality rate from CST was effectively 100%. Typically, death is due to sepsis or central nervous system (CNS) infection. With aggressive management, the mortality rate is now less than 30%. Morbidity, however, remains high, and complete recovery is rare. Roughly one sixth of patients are left with some degree of visual impairment, and one half have cranial nerve deficits 2. Imaging by plain CT scan shows the thrombosis as increased density. On post contrast CT there may be a filling defects in the cavernous sinus as well as thickening of the superior ophthalmic vein. However CT findings may be subtle and a negative CT scan cannot rule out CST reliably when the clinical suspicion is high. MRI is a more sensitive modality for superior ophthalmic vein thrombosis. It will show T1 and T2 hyperintensity in the superior ophthalmic vein associated with thickening. Carotid angiography can demonstrate narrowing or obstruction of the intercavernous segment of the carotid artery or presence of carotico-cavernous fistula. The mainstay of therapy is early and aggressive antibiotic therapy. Regarding anticoagulation for superior ophthalmic vein and cavernous sinus thrombosis there is controversy. Because of the rarity of the entity control trials are lacking. Corticosteroids are used as an
The Neuroradiology Journal 20: 291-294, 2007
adjuvant therapy to reduce inflammation and edema. Spontaneous resolution is unimaginable and not known so far. In our case the patient clearly presented with features of bilateral superior ophthalmic vein thrombosis and cavernous sinus thrombosis both clinically and on imaging. She was not at all septic as there were no constitutional features. Another differential diagnosis can be carotico-cavernous fistula. In our opinion, CCF is an unlikely possibility in our case as there was no history of trauma or hypertension and also because of the gradual onset of symptoms. Even MRI and MR venography of the patient did not show any evidence of the same. Although we do agree that DSA was not done in this case but based on the clinical and MRI picture, the possibility of CCF remains remote. A fungal infection is also unlikely in view of the absence of sinusitis. Moreover it was ruled out by the patient’s spontaneous recovery. Pituitary macroadenomas are known to become clinically significant only when they reach a size large enough to cause mass effect as most but not all are nonfunctioning tumors. Due to very thin medial wall of the cavernous sinus it is difficult to assess cavernous sinus invasion by the pituitary macroadenoma 7. However the association of pituitary macroadenoma which appeared to be confined within the sella on MRI (figure 1 E) with cavernous sinus or superior ophthalmic vein thrombosis is not known. Case reports have described pituitary apoplexy associated with painful ophthalmoplegia which has resolved on treatment but those were associated with unilateral ophthalmoplegia 8. Even in those cases there was no evidence of cavernous sinus or superior ophthalmic vein thrombosis. Moreover these cases show a large suprasellar mass, whereas in our patient there was no evidence of pituitary apoplexy and the pituitary macroadenoma was very much confined to the sella. Since all other local and systemic causes of superior ophthalmic vein thrombosis and its resolution have been ruled out, we think diabetes itself and its control may be responsible for these events. It is known that diabetes itself due to hyperglycemia by different mechanisms can cause thrombosis and with the control of diabetes it can also cause a resolution of the thrombosis 9. However such events have not been reported in cases of superior ophthalmic vein thrombosis. In our case although it appears that the 293
Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis
thrombosis resolved in four days, it was not so as the patient had symptoms of superior ophthalmic vein thrombosis for one month. MRI done one month before at the beginning of symptoms did not cover the orbit, so thrombo-
J.J.S. Shankar
sis of the bilateral superior ophthalmic vein resolved over a period of one month. To conclude, spontaneous resolution of idiopathic bilateral superior ophthalmic vein thrombosis is possible, but awaits an explanation.
References 1 Michaelides M, Aclimandos W: Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 81: 88-90, 2003. 2 Sharma R, Bessman E: Cavernous sinus thrombosis. eMedicine; May 2006. 3 Luxenberg MN: Colour doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 109: 582-583, 1991. 4 Berenholz B, Kessler A, Shlomkovitz N: Superior ophthalmic vein thrombosis – complication of ethmoidal rhinosinusitis. Arch Otolaryngol Head Neck Surg 1: 95-97, 1998. 5 Jaais F, Habib ZA: Unilateral superior ophthalmic vein thrombosis in user of oral contraceptives. Med J Malaysia 49: 416-418, 1994. 6 Schuknecht B, Simmen D, Yü ksel C et Al: Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings. Am J Neuroradiol 19: 617626, 1998. 7 Atlas SW: The sella turcica and parasellar region. In Magnetic resonance imaging of the brain and spine.
294
3rd ed. Lippincott Williams and Wilkins: 1307, 2002. 8 Chen Z, Murray AW, Quinlan JJ: Pituitary Apoplexy Presenting as Unilateral Third Cranial Nerve Palsy After Coronary Artery Bypass. Surgery Anesth Analg 98: 46-48, 2004. 9 Ceriello A: Coagulation activation in diabetes mellitus: the role of hyperglycaemia and therapeutic prospects. Diabetologia 36: 1119-25, 1993.
Jai Jai Shiva Shankar, MD D.M. Senior resident Neuro-Imaging and Interventional Radiology Department National Institute of Mental Health and Neuro-Sciences Room No G25, Kabini Hostel, Nimhans Bangalore, 560029 Karnataka India Tel.: 91-9448958907 Fax: 91-80-26564830 E-mail: [email protected]
www. centauro. it
Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis. A Case Report J.J.S. SHANKAR, S.G. SRIKANTH, J.M. E KOVOOR, M.K. VASUDEV, S. SINHA National Institute of Mental Health and Neuro-Sciences; Bangalore, Karnatka, India
Key words: cavernous sinus thrombosis, superior ophthalmic vein thrombosis, pituitary macroadenoma
SUMMARY – Superior ophthalmic vein thrombosis is uncommon, and bilateral superior ophthalmic vein thrombosis is rarer still. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and not reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution.
Introduction Superior ophthalmic vein thrombosis is an uncommon clinical entity. Bilateral superior ophthalmic vein thrombosis is rarer still and is usually associated with some local inflammatory condition or coagulation disorder 1. Superior ophthalmic vein thrombosis is managed with appropriate antibiotics, anticoagulants and steroids. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time 2. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and has not been reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution. Case Report A 59-year-old woman presented with a complaint of diminished vision in her right eye for three days. She had occasional headache and nausea not associated with fever or vomiting. One month previously she had sudden onset drooping of the right eyelid which was associated with right eye pain and mild headache.
There was painful restriction of right eyeball movement. There was no history of visual blurring or diplopia at that time. She sought medical service and was diagnosed to be diabetic. She was started on insulin injection. No antibiotics or anticoagulants were given. Eyelid drooping improved over the next two weeks. However, painful restriction of the right eyeball remained for about another month. On examination her vital parameters were normal. External ocular movements were restricted and painful in the right eye whereas they were full in the left eye. Her visual acuity was 6/9 and 6/12 in right and left eyes respectively. There was no other neurological deficit or history of weight loss, bladder and bowel disturbances, no cold intolerance or change in voice. Brain MRI examination using routine spin echo T1W and TSE T2W sequences performed one month previously demonstrated pituitary macroadenoma. That MRI scan focused only the sella and suprasellar portion and did not cover the orbit. Hormonal assessment including serum cortisol, FSH (follicular stimulating hormone), prolactin, T4 and TSH (thyroid stimulating hormone) were within normal limits. Repeat MRI performed at this time, also covering the orbit, showed T1 and T2 hyperintensity 291
Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis
A
J.J.S. Shankar
B
C D
E
Figure A-E T1 coronal and T2 coronal images showing hyperintensity and enlargement of superior ophthalmic vein (A,B). 4 days later T1 coronal and T2 coronal images showing normal size and signal intensity in superior ophthalmic vein (C,D). T2 W coronal image showing pituitary macroadenoma and right cavernous sinus thrombosis (E).
in the dilated superior ophthalmic veins of both orbits more prominent on the right side (figure 1 A,B). Contrast study showed no enhancement of the superior ophthalmic veins with prominent lateral wall of the right cavernous sinus. Paranasal sinuses were clear with no evidence of sinusitis. The imaging features were suggestive of cavernous sinus thrombosis extending into the bilateral superior ophthalamic vein. Four days later (i.e., one month after the start of the first symptom and institution of insulin therapy), the patient came for follow-up with a complete resolution of symptoms. Repeat MRI at this time revealed complete disappearance of the changes within the ophthalmic veins (figure 1 C,D). 292
Discussion Superior ophthalmic vein thrombosis (SOVT) is rare 1. Previous reports have described unilateral superior ophthalmic vein thrombosis associated with orbital cellulitis 3, ethmoidal rhinosinusitis 4 and the combined oral contraceptive pill 5. The other causes of superior ophthalmic vein thrombosis are presence of carotico-cavernous fistula and underlying hypercoagulable state like raised factor VIII 1. Other rare causes are epidural hematoma, epidural and subdural infections, acute angle-closure glaucoma, subarachnoid hemorrhage, subdural hematoma, carotid artery aneurysm or fistula, exophthalmic goiter and orbital neoplasm. Bi-
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lateral ophthalamic vein thrombosis is an even rarer entity. Bilateral SOVT has only been reported in one patient in association with septic cavernous sinus thrombosis 6 and another young woman who was found to have raised factor VIII level and was on combined oral contraceptive pills 1. Superior ophthalmic vein thrombsosis is usually associated with cavernous sinus thrombosis (CST). This entity is usually a late complication of an infection of the central face or paranasal sinuses. Other causes include bacteremia, trauma and infections of the ear or maxillary teeth. This is generally a fulminant process with high rates of morbidity and mortality. Fortunately, the incidence of CST has been decreased greatly with the advent of effective antimicrobial agents 2. Staphylococcus aureus accounts for approximately 70% of all infections. Streptococcus pneumoniae, gram-negative bacilli, and anaerobes can also be seen. Fungi are a less common pathogen and may include Aspergillus and Rhizopus species. In a setting of diabetic and immunocompromised state fungal infection takes precedence. Earlier the mortality rate from CST was effectively 100%. Typically, death is due to sepsis or central nervous system (CNS) infection. With aggressive management, the mortality rate is now less than 30%. Morbidity, however, remains high, and complete recovery is rare. Roughly one sixth of patients are left with some degree of visual impairment, and one half have cranial nerve deficits 2. Imaging by plain CT scan shows the thrombosis as increased density. On post contrast CT there may be a filling defects in the cavernous sinus as well as thickening of the superior ophthalmic vein. However CT findings may be subtle and a negative CT scan cannot rule out CST reliably when the clinical suspicion is high. MRI is a more sensitive modality for superior ophthalmic vein thrombosis. It will show T1 and T2 hyperintensity in the superior ophthalmic vein associated with thickening. Carotid angiography can demonstrate narrowing or obstruction of the intercavernous segment of the carotid artery or presence of carotico-cavernous fistula. The mainstay of therapy is early and aggressive antibiotic therapy. Regarding anticoagulation for superior ophthalmic vein and cavernous sinus thrombosis there is controversy. Because of the rarity of the entity control trials are lacking. Corticosteroids are used as an
The Neuroradiology Journal 20: 291-294, 2007
adjuvant therapy to reduce inflammation and edema. Spontaneous resolution is unimaginable and not known so far. In our case the patient clearly presented with features of bilateral superior ophthalmic vein thrombosis and cavernous sinus thrombosis both clinically and on imaging. She was not at all septic as there were no constitutional features. Another differential diagnosis can be carotico-cavernous fistula. In our opinion, CCF is an unlikely possibility in our case as there was no history of trauma or hypertension and also because of the gradual onset of symptoms. Even MRI and MR venography of the patient did not show any evidence of the same. Although we do agree that DSA was not done in this case but based on the clinical and MRI picture, the possibility of CCF remains remote. A fungal infection is also unlikely in view of the absence of sinusitis. Moreover it was ruled out by the patient’s spontaneous recovery. Pituitary macroadenomas are known to become clinically significant only when they reach a size large enough to cause mass effect as most but not all are nonfunctioning tumors. Due to very thin medial wall of the cavernous sinus it is difficult to assess cavernous sinus invasion by the pituitary macroadenoma 7. However the association of pituitary macroadenoma which appeared to be confined within the sella on MRI (figure 1 E) with cavernous sinus or superior ophthalmic vein thrombosis is not known. Case reports have described pituitary apoplexy associated with painful ophthalmoplegia which has resolved on treatment but those were associated with unilateral ophthalmoplegia 8. Even in those cases there was no evidence of cavernous sinus or superior ophthalmic vein thrombosis. Moreover these cases show a large suprasellar mass, whereas in our patient there was no evidence of pituitary apoplexy and the pituitary macroadenoma was very much confined to the sella. Since all other local and systemic causes of superior ophthalmic vein thrombosis and its resolution have been ruled out, we think diabetes itself and its control may be responsible for these events. It is known that diabetes itself due to hyperglycemia by different mechanisms can cause thrombosis and with the control of diabetes it can also cause a resolution of the thrombosis 9. However such events have not been reported in cases of superior ophthalmic vein thrombosis. In our case although it appears that the 293
Spontaneous Resolution of Bilateral Superior Ophthalmic Vein and Cavernous Sinus Thrombosis
thrombosis resolved in four days, it was not so as the patient had symptoms of superior ophthalmic vein thrombosis for one month. MRI done one month before at the beginning of symptoms did not cover the orbit, so thrombo-
J.J.S. Shankar
sis of the bilateral superior ophthalmic vein resolved over a period of one month. To conclude, spontaneous resolution of idiopathic bilateral superior ophthalmic vein thrombosis is possible, but awaits an explanation.
References 1 Michaelides M, Aclimandos W: Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 81: 88-90, 2003. 2 Sharma R, Bessman E: Cavernous sinus thrombosis. eMedicine; May 2006. 3 Luxenberg MN: Colour doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 109: 582-583, 1991. 4 Berenholz B, Kessler A, Shlomkovitz N: Superior ophthalmic vein thrombosis – complication of ethmoidal rhinosinusitis. Arch Otolaryngol Head Neck Surg 1: 95-97, 1998. 5 Jaais F, Habib ZA: Unilateral superior ophthalmic vein thrombosis in user of oral contraceptives. Med J Malaysia 49: 416-418, 1994. 6 Schuknecht B, Simmen D, Yü ksel C et Al: Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings. Am J Neuroradiol 19: 617626, 1998. 7 Atlas SW: The sella turcica and parasellar region. In Magnetic resonance imaging of the brain and spine.
294
3rd ed. Lippincott Williams and Wilkins: 1307, 2002. 8 Chen Z, Murray AW, Quinlan JJ: Pituitary Apoplexy Presenting as Unilateral Third Cranial Nerve Palsy After Coronary Artery Bypass. Surgery Anesth Analg 98: 46-48, 2004. 9 Ceriello A: Coagulation activation in diabetes mellitus: the role of hyperglycaemia and therapeutic prospects. Diabetologia 36: 1119-25, 1993.
Jai Jai Shiva Shankar, MD D.M. Senior resident Neuro-Imaging and Interventional Radiology Department National Institute of Mental Health and Neuro-Sciences Room No G25, Kabini Hostel, Nimhans Bangalore, 560029 Karnataka India Tel.: 91-9448958907 Fax: 91-80-26564830 E-mail: [email protected]
