The Neuroradiology Journal 21: 77-80, 2008
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Dynamic Exophthalmos Secondary to an Orbital Varix U. ER*, D. BELEN*, M.H. OZTURK**, M. BAVBEK* * Second Neurosurgery, ** Radiology Clinics, Diskapi Yildirim Bayezit Education and Research Hospital; Ankara, Turkey
Key words: dynamic exophthalmos, exophthalmos, intermittent proptosis, orbital varix
SUMMARY – We present a case of dynamic exophthalmos secondary to an orbital varix. Objectives of this paper are to describe this rare entity and review its etiology, diagnosis and treatment options. A 21-year-old woman presented complaining of exophthalmos in the right eye on bending forward. Neuroimaging studies revealed an irregularly shaped mass located in the posterior intra-extraconal region adjacent to the orbital roof. Surgical and interventional endovascular therapies were considered, but due to the complex nature of the lesion the patient was followed-up conservatively. Orbital varix may cause dynamic exophthalmos and most patients may be managed conservatively. Patients harboring non-treatable orbital varix should be advised to avoid any strenuous exercises and postures which may increase intraorbital pressure.
Orbital varix is an uncommon hamartomatous venous complex of the orbit. Orbital varices consist of approximately 2% of all orbital lesions 1. The lesion is composed of either local dilated normal venous structures or abnormal venous channels 2,3,4,5. Patients harboring orbital varix usually present with retroorbital pain, intermittent exophthalmos and rarely orbital hemorrhages 4. CT, MRI, conventional or MR angiography and color flow imaging are the tools that may be employed in the diagnosis of orbital varix 6,7,8. The majority of patients need no treatment. In the remaining serious symptomatic group, intervention can be helpful, but is not always possible 9. Management options include surgical occlusion or removal, endovascular occlusion, electrothrombosis, injection of sclerosing agents and careful follow-up 3,4,10,11. Case Report A 21-year-old woman presented with the complaint of exophthalmos of the right eye on bending forward. She reported that the symptom had started a year ago following the birth of her second child. The exophthalmos was not evident in normal posture. On examination axial exophthalmos and periorbital swelling in
the right eye occurred during Valsalva maneuver and with compressing the jugular vein, and rapidly disappeared when provocative maneuvers were discontinued. Visual acuity, fundus and ocular movement examinations were found to be normal. There were no bruits or pulsation on auscultation and palpation. Systemic findings and routine laboratory investigations were normal. Orbital MRI revealed an irregularly shaped mass located at the right posterior intra and extraconal region, adjacent to the orbital roof which was hyperintense on T2-Weighted (T2-W) and hypointense on T1-Weighted (T1W) sequences. After contrast administration, the lesion enhanced strongly (figure 1). Selective catheter angiography without provocative maneuvers showed no abnormality either in arterial or venous phases. On the basis of physical examination and radiological findings the diagnosis of an orbital varix was made. Due to the location and the complex nature of the lesion, and lack of indication of threat to vision, intervention was deferred. We decided to follow up the patient conservatively after consultation with the neuro-ophthalmology and interventional neuroradiology departments. The patient was advised to avoid strenuous physical activities whenever possible to prevent constipation, and avoid conditions that 77
Dynamic Exophthalmos Secondary to an Orbital Varix
A
U. Er
B
C
may induce swelling in her right eye. During the two-year follow-up period the patient exhibited no deterioration. Discussion Orbital varix is the most common cause of dynamic exophthalmos 13. In the differential diagnosis of angiomas, lymphangiomas, cavernous hemangiomas, thrombosis of cavernous sinuses, angioneurotic type periodic orbital edema and intermittent ethmoiditis should be considered 12,13 . Orbital varices are classified as primary and secondary according to their underlying etiology. Primary orbital varix is regarded as a congenital venous malformation that contains 78
Figure 1 A-C) MRI sections of transverse and coronal FSET2W (A,B) and contrast enhanced sagittal T1-W (C). A posterior intra and extraconal mass hyperintense in T2-W and hypointense in T1-W images near to the orbital roof is seen. The lesion is irregular in shape and enhances strongly after contrast medium administration.
thin walled, low-pressure, low-flow and distensible structures intermingled with normal orbital veins 2,13. There are rarely associated cranial defects such as encephalocele (2). The secondary type is associated with an arteriovenous shunt either intracranially or intraorbitally 13. In both forms, venous structures can be swollen as a result of rising venous pressure during bending forward or any kind of Valsalva maneuver, and may lead to temporary exophthalmos, as in our patient’s case. Although conventional angiography without provocative maneuvers did not show a venous abnormality in our patient, we considered the lesion to be a primary orbital varix because of the lack of arteriovenous shunt. Unrevealing angiography was likely due to lack of provocative maneu-
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vers. Computed tomography and color doppler imaging during and after provocative maneuvers were not performed. The relationship between the onset of our patient’s symptoms and childbirth may be coincidental. There are no reports in the literature linking pregnancy and delivery to the onset of symptomatic orbital varix. One could speculate that a venous thrombosis or venous fistula developed or became manifest due to hypercoagulable state or intense Valsalva of childbirth, but if this were the case, the association should be more common. Therefore, our patient’s varix was most likely pre-existing, and the association coincidental. Intermittent or dynamic exophthalmos should be differentiated from recurrent exophthalmos. Recurrent exophthalmos occurs periodically with exacerbation of certain disease processes such as lymphangioma or cavernous hemangioma 8. Typically these episodes are longer lasting; measured in days or weeks. Radiological findings of recurrent exophthalmos may show a mass lesion, but may be very similar to orbital varix. Accordingly, the medical history of the patient and physical examination play a crucial role in establishing the correct diagnosis. In the presented case, there were no such periodic exacerbations found in her history or medical records and radiological findings were not suggestive of a mass lesion. Management of orbital varices remains controversial, because of their benign course 4,9,11,14. Vision is usually unaffected and may remain so unless pressure within the orbit recurs frequently 12,15. Formation of enophthalmos due to atrophy of the orbital fat can be associated with development of visual impairment 16. In
The Neuroradiology Journal 21: 77-80, 2008
addition to this, silent orbital varix should be considered when selecting the method of anesthesia for ocular surgery, because venous congestion induced by retrobulber anesthesia may lead to rupture of the varix 17. Surgery, embolization, electrothrombosis or injection of sclerosing agents should be reserved for symptomatic patients with recurrent orbital hemorrhage, severe pain and thrombosis of the varix or increasing proptosis or enophthalmos. During surgical excision, bleeding tends to complicate the dissection and to mask the fine branches of the motor nerves to the extraocular muscles, which may unintentionally be cut, resulting in some degree of ophthalmoplegia 12,18. In addition, surgical resection is rarely complete and only moderately successful 11. Injection of sclerosing agents is difficult to manage even under fluoroscopy 11. Electrothrombosis cannot cure completely, but may facilitate surgical excision of the lesion 19. Embolization with microcoils is usually accepted as a non-invasive procedure and sufficient treatment can be performed after catheterization of the cavernous sinus via the jugular vein and the inferior petrosal sinus 4,11. Conclusion Orbital varices should be followed conservatively unless symptoms progress or hemorrhage occurs 3,13. Patients may be advised to avoid any strenuous exercises and postures which may increase intraorbital pressure. Interventional treatment should be considered if the lesion becomes more symptomatic. Endovascular treatment modalities are more appropriate than surgical intervention 3,4.
References 1 Shields JA, Shields JL, Scartozzi R: Survey of 1264 patients with orbital tumors and simulating lesions. The 2002 Montgomery lecture, part 1. Ophthalmology May 111: 997-1008, 2004. 2 Islam N, Mireskandari K, Burton BJ et Al: Orbital varices, cranial defects, and encephaloceles. Ophthalmology June 111: 1244-1247, 2004. 3 Rekhi GS, Dheer S, Kurian P: Orbital varix. JAPI 49: 1207, 2001. 4 Takechi A, Uozumi T, Kiya K et Al: Embolisation of orbital varix. Neuroradiology 36: 487-489, 1994. 5 White J, Fox AJ, Symons SP: Diagnosis and anatomic mapping of an orbital varix by computed tomographic angiography. Am J Ophthalmol 140: 945-947, 2005. 6 Secil M, Soylev M, Ada E et Al: Orbital varices: imaging findings and the role of color Doppler Sonography
7 8 9 10 11
in the diagnosis. Comput Med Imaging Graphics 25: 243-247, 2001. Shnier R, Parker GD, Hallinan JM et Al: Orbital varices: A new technique for noninvasive diagnosis. Am J Neuroradiol 12: 717-718, 1991. Wildenhain PM, Lehar SC, Dastur KJ et Al: Orbital varix: color flow imaging correlated with CT and MR studies. J Comput Assist Tomogr 15: 171-173, 1991. Yeatts RP, Driver PJ: Orbital varix. Arch Ophthalmol 111: 702-703, 1993. Ward PH: The treatment of orbital varicosities. Arch Otolaryngol Head Neck Surg March 113: 286-288, 1987. Weill A, Cognard C, Castaings L et Al: Embolisation of an orbital varix after surgical exposure. Am J Neuroradiol 19: 921-923, 1998.
79
Dynamic Exophthalmos Secondary to an Orbital Varix
12 Menon SV, Shome D, Mahesh L et Al: Trombosed orbital varix- a correlation between imaging studies and histopathology 23: 13-18, 2004. 13 Phanthumchinda K, Locharernkul C, Hemachudha T: Intermittent exophtalmos. J Med Assoc Tai June 72: 351-354, 1989. 14 Srinivasan S, Gaskell A, McWhinnie H et Al: Conservative management of globe luxation associated with congenital orbital venous anomaly. J Pediatr Ophthalmology Strabismus 40: 170-171, 2003. 15 Yazici B, Yazici Z, Gelisken O: An unusual case: Bilateral orbital varices. Acta Ophthalmol Scand 77: 453455, 1999. 16 Haritoglou C, Hintschich C: Progressive enophthalmos in association with an orbital varix. Klin Monatsbl Augenheilkd 220: 268-71, 2003. 17 Yoshimoto M, Matsumoto S: Orbital varix rupture during cataract surgery. J Cataract Refract Surg March 30: 722-725, 2004.
80
U. Er
18 Henderson JW: Orbital Tumors. 3rd ed. New York: Raven Press 1993: 128-133. 19 Handa H, Mori K: Large varix of the superior ophthalmic vein: demonstration by angular phlebography and removal by electrically induced thrombosis. J Neurosurg 29: 202-205, 1968.
Uygur ER, MD Sogutozu C., 4. Sk., No: 22-7 06470 Ankara, Turkey Tel.: 00903122841151 Fax: 00903123162929 E-mail: [email protected]
www. centauro. it
Dynamic Exophthalmos Secondary to an Orbital Varix U. ER*, D. BELEN*, M.H. OZTURK**, M. BAVBEK* * Second Neurosurgery, ** Radiology Clinics, Diskapi Yildirim Bayezit Education and Research Hospital; Ankara, Turkey
Key words: dynamic exophthalmos, exophthalmos, intermittent proptosis, orbital varix
SUMMARY – We present a case of dynamic exophthalmos secondary to an orbital varix. Objectives of this paper are to describe this rare entity and review its etiology, diagnosis and treatment options. A 21-year-old woman presented complaining of exophthalmos in the right eye on bending forward. Neuroimaging studies revealed an irregularly shaped mass located in the posterior intra-extraconal region adjacent to the orbital roof. Surgical and interventional endovascular therapies were considered, but due to the complex nature of the lesion the patient was followed-up conservatively. Orbital varix may cause dynamic exophthalmos and most patients may be managed conservatively. Patients harboring non-treatable orbital varix should be advised to avoid any strenuous exercises and postures which may increase intraorbital pressure.
Orbital varix is an uncommon hamartomatous venous complex of the orbit. Orbital varices consist of approximately 2% of all orbital lesions 1. The lesion is composed of either local dilated normal venous structures or abnormal venous channels 2,3,4,5. Patients harboring orbital varix usually present with retroorbital pain, intermittent exophthalmos and rarely orbital hemorrhages 4. CT, MRI, conventional or MR angiography and color flow imaging are the tools that may be employed in the diagnosis of orbital varix 6,7,8. The majority of patients need no treatment. In the remaining serious symptomatic group, intervention can be helpful, but is not always possible 9. Management options include surgical occlusion or removal, endovascular occlusion, electrothrombosis, injection of sclerosing agents and careful follow-up 3,4,10,11. Case Report A 21-year-old woman presented with the complaint of exophthalmos of the right eye on bending forward. She reported that the symptom had started a year ago following the birth of her second child. The exophthalmos was not evident in normal posture. On examination axial exophthalmos and periorbital swelling in
the right eye occurred during Valsalva maneuver and with compressing the jugular vein, and rapidly disappeared when provocative maneuvers were discontinued. Visual acuity, fundus and ocular movement examinations were found to be normal. There were no bruits or pulsation on auscultation and palpation. Systemic findings and routine laboratory investigations were normal. Orbital MRI revealed an irregularly shaped mass located at the right posterior intra and extraconal region, adjacent to the orbital roof which was hyperintense on T2-Weighted (T2-W) and hypointense on T1-Weighted (T1W) sequences. After contrast administration, the lesion enhanced strongly (figure 1). Selective catheter angiography without provocative maneuvers showed no abnormality either in arterial or venous phases. On the basis of physical examination and radiological findings the diagnosis of an orbital varix was made. Due to the location and the complex nature of the lesion, and lack of indication of threat to vision, intervention was deferred. We decided to follow up the patient conservatively after consultation with the neuro-ophthalmology and interventional neuroradiology departments. The patient was advised to avoid strenuous physical activities whenever possible to prevent constipation, and avoid conditions that 77
Dynamic Exophthalmos Secondary to an Orbital Varix
A
U. Er
B
C
may induce swelling in her right eye. During the two-year follow-up period the patient exhibited no deterioration. Discussion Orbital varix is the most common cause of dynamic exophthalmos 13. In the differential diagnosis of angiomas, lymphangiomas, cavernous hemangiomas, thrombosis of cavernous sinuses, angioneurotic type periodic orbital edema and intermittent ethmoiditis should be considered 12,13 . Orbital varices are classified as primary and secondary according to their underlying etiology. Primary orbital varix is regarded as a congenital venous malformation that contains 78
Figure 1 A-C) MRI sections of transverse and coronal FSET2W (A,B) and contrast enhanced sagittal T1-W (C). A posterior intra and extraconal mass hyperintense in T2-W and hypointense in T1-W images near to the orbital roof is seen. The lesion is irregular in shape and enhances strongly after contrast medium administration.
thin walled, low-pressure, low-flow and distensible structures intermingled with normal orbital veins 2,13. There are rarely associated cranial defects such as encephalocele (2). The secondary type is associated with an arteriovenous shunt either intracranially or intraorbitally 13. In both forms, venous structures can be swollen as a result of rising venous pressure during bending forward or any kind of Valsalva maneuver, and may lead to temporary exophthalmos, as in our patient’s case. Although conventional angiography without provocative maneuvers did not show a venous abnormality in our patient, we considered the lesion to be a primary orbital varix because of the lack of arteriovenous shunt. Unrevealing angiography was likely due to lack of provocative maneu-
www. centauro. it
vers. Computed tomography and color doppler imaging during and after provocative maneuvers were not performed. The relationship between the onset of our patient’s symptoms and childbirth may be coincidental. There are no reports in the literature linking pregnancy and delivery to the onset of symptomatic orbital varix. One could speculate that a venous thrombosis or venous fistula developed or became manifest due to hypercoagulable state or intense Valsalva of childbirth, but if this were the case, the association should be more common. Therefore, our patient’s varix was most likely pre-existing, and the association coincidental. Intermittent or dynamic exophthalmos should be differentiated from recurrent exophthalmos. Recurrent exophthalmos occurs periodically with exacerbation of certain disease processes such as lymphangioma or cavernous hemangioma 8. Typically these episodes are longer lasting; measured in days or weeks. Radiological findings of recurrent exophthalmos may show a mass lesion, but may be very similar to orbital varix. Accordingly, the medical history of the patient and physical examination play a crucial role in establishing the correct diagnosis. In the presented case, there were no such periodic exacerbations found in her history or medical records and radiological findings were not suggestive of a mass lesion. Management of orbital varices remains controversial, because of their benign course 4,9,11,14. Vision is usually unaffected and may remain so unless pressure within the orbit recurs frequently 12,15. Formation of enophthalmos due to atrophy of the orbital fat can be associated with development of visual impairment 16. In
The Neuroradiology Journal 21: 77-80, 2008
addition to this, silent orbital varix should be considered when selecting the method of anesthesia for ocular surgery, because venous congestion induced by retrobulber anesthesia may lead to rupture of the varix 17. Surgery, embolization, electrothrombosis or injection of sclerosing agents should be reserved for symptomatic patients with recurrent orbital hemorrhage, severe pain and thrombosis of the varix or increasing proptosis or enophthalmos. During surgical excision, bleeding tends to complicate the dissection and to mask the fine branches of the motor nerves to the extraocular muscles, which may unintentionally be cut, resulting in some degree of ophthalmoplegia 12,18. In addition, surgical resection is rarely complete and only moderately successful 11. Injection of sclerosing agents is difficult to manage even under fluoroscopy 11. Electrothrombosis cannot cure completely, but may facilitate surgical excision of the lesion 19. Embolization with microcoils is usually accepted as a non-invasive procedure and sufficient treatment can be performed after catheterization of the cavernous sinus via the jugular vein and the inferior petrosal sinus 4,11. Conclusion Orbital varices should be followed conservatively unless symptoms progress or hemorrhage occurs 3,13. Patients may be advised to avoid any strenuous exercises and postures which may increase intraorbital pressure. Interventional treatment should be considered if the lesion becomes more symptomatic. Endovascular treatment modalities are more appropriate than surgical intervention 3,4.
References 1 Shields JA, Shields JL, Scartozzi R: Survey of 1264 patients with orbital tumors and simulating lesions. The 2002 Montgomery lecture, part 1. Ophthalmology May 111: 997-1008, 2004. 2 Islam N, Mireskandari K, Burton BJ et Al: Orbital varices, cranial defects, and encephaloceles. Ophthalmology June 111: 1244-1247, 2004. 3 Rekhi GS, Dheer S, Kurian P: Orbital varix. JAPI 49: 1207, 2001. 4 Takechi A, Uozumi T, Kiya K et Al: Embolisation of orbital varix. Neuroradiology 36: 487-489, 1994. 5 White J, Fox AJ, Symons SP: Diagnosis and anatomic mapping of an orbital varix by computed tomographic angiography. Am J Ophthalmol 140: 945-947, 2005. 6 Secil M, Soylev M, Ada E et Al: Orbital varices: imaging findings and the role of color Doppler Sonography
7 8 9 10 11
in the diagnosis. Comput Med Imaging Graphics 25: 243-247, 2001. Shnier R, Parker GD, Hallinan JM et Al: Orbital varices: A new technique for noninvasive diagnosis. Am J Neuroradiol 12: 717-718, 1991. Wildenhain PM, Lehar SC, Dastur KJ et Al: Orbital varix: color flow imaging correlated with CT and MR studies. J Comput Assist Tomogr 15: 171-173, 1991. Yeatts RP, Driver PJ: Orbital varix. Arch Ophthalmol 111: 702-703, 1993. Ward PH: The treatment of orbital varicosities. Arch Otolaryngol Head Neck Surg March 113: 286-288, 1987. Weill A, Cognard C, Castaings L et Al: Embolisation of an orbital varix after surgical exposure. Am J Neuroradiol 19: 921-923, 1998.
79
Dynamic Exophthalmos Secondary to an Orbital Varix
12 Menon SV, Shome D, Mahesh L et Al: Trombosed orbital varix- a correlation between imaging studies and histopathology 23: 13-18, 2004. 13 Phanthumchinda K, Locharernkul C, Hemachudha T: Intermittent exophtalmos. J Med Assoc Tai June 72: 351-354, 1989. 14 Srinivasan S, Gaskell A, McWhinnie H et Al: Conservative management of globe luxation associated with congenital orbital venous anomaly. J Pediatr Ophthalmology Strabismus 40: 170-171, 2003. 15 Yazici B, Yazici Z, Gelisken O: An unusual case: Bilateral orbital varices. Acta Ophthalmol Scand 77: 453455, 1999. 16 Haritoglou C, Hintschich C: Progressive enophthalmos in association with an orbital varix. Klin Monatsbl Augenheilkd 220: 268-71, 2003. 17 Yoshimoto M, Matsumoto S: Orbital varix rupture during cataract surgery. J Cataract Refract Surg March 30: 722-725, 2004.
80
U. Er
18 Henderson JW: Orbital Tumors. 3rd ed. New York: Raven Press 1993: 128-133. 19 Handa H, Mori K: Large varix of the superior ophthalmic vein: demonstration by angular phlebography and removal by electrically induced thrombosis. J Neurosurg 29: 202-205, 1968.
Uygur ER, MD Sogutozu C., 4. Sk., No: 22-7 06470 Ankara, Turkey Tel.: 00903122841151 Fax: 00903123162929 E-mail: [email protected]
