J Neurosurg 73:291-295, 1990
Orbital varix with a pearly phlebolith Case report TOSHIHIKO KUBOTA, M.D., EIICHI KURODA, M.D., TOSHIHARU FUJII, M.D., HIROKAZU KAWANO, M.D., MASANORI KABUTO, M.D., AND MINORU HAYASHI, M.D. Department of Neurosurgery, Fukui Medical School, Fukui, and Department of Neurosurgery, Kanazawa University School of Medicine, Kanazawa, Japan A patient is described with an orbital varix arising from the fight superior ophthalmic vein, associated with ophthalmoplegia and severe pain, and without proptosis. The varix was detected using computerized tomography and orbital phlebography, and at surgery was verified as a venous aneurysm. During the operation, a pearly phlebolith was found. Histological examination of the varix revealed multiple ectatic venous channels. The etiology of this unusual clinical manifestation and the treatment of the patient are briefly discussed.
KEY WOADS
9 orbital varix
O
phlebolith
RBITAL vascular diseases are occasionally diagnosed and treated by specialists in the neuroophthalmological field. Among these entities are relatively c o m m o n disorders termed "orbital varices," which usually consist of congenital venous malformations. 16These abnormalities have been treated by ophthalmologists, 4'6'~4 neurosurgeons, 5'1j'~3 head and neck surgeons, ~7 and plastic surgeons. The classical clinical features of the orbital varix are intermittent proptosis which varies with the head position and which may be worsened during the Valsalva maneuver. 4-6~3 ~6 Instances of ophthalmoplegia and bruit are less common than carotid-cavernous fistulas and arteriovenous malformations. 6'j3'~4 Recent advances in neuroimaging techniques now make it possible to diagnose orbital lesions precisely 7'~5 and to select the best treatment procedures for these diseases. 6'1~'~4 Until recently, the treatment of orbital varices was unreliable because of their elusive clinical nature; 4-6'13'14however, the number of patients undergoing safe varix removal has increased due to the recent improvement of surgical techniques and instruments. 1'3-6'8'~1.13,14 We present the case of a female patient with a fight orbital varix and phlebolith who presented with ophthalmoplegia and pain but without proptosis. The varix was accurately diagnosed by computerized tomography (CT) and phlebography, and was safely removed through a superolateral orbital exposure. The varix was verified histologically as a venous channel. We
J. Neurosurg. / Volume 73/August, 1990
9 ophthalmoplegia
9 orbital pain
discuss the clinical manifestations of this orbital varix and phlebolith and describe the patient's treatment.
Case Report This 61-year-old woman was admitted to our hospital on March 7, 1985, complaining of double vision and a dull pain in the right orbit. She had first noticed double vision 1 week earlier while attempting to focus her eyes to the right. She complained of a dull pain in the upper part of her right eye. She immediately consulted an ophthalmologist who identified a right abducens palsy and referred her to our department for examination. She denied having had previous trauma, and the history provided by her family was noncontributory.
First Admission. The general physical examination showed no abnormalities; however, neurological examination revealed a mild disturbance of right eye movement with lateral gaze. The patient complained of dull pain in her right forehead, but no sensory disturbance was noted. There was no exophthalmos, conjunctival congestion, or eyelid swelling related to head position or Valsalva maneuver. Auscultation of the periorbital region was negative. Visual acuity was 0.6 in the fight eye and 0.7 in the left eye. Exophthalmometry performed by the Hertel technique gave a reading of 13 m m in both eyes. Perimetry, ocular tension, and funduscopic examination were normal. X-ray films of the skull and orbit were normal, as 291
T. Kubota, et al.
FIG. 1. Computerized tomography (CT) scans obtained when the patient suffered from the first attack of double vision and dull facial pain. A: Precontrast axial CT scan clearly showing a small retrobulbar mass on the right side (supine position). B: Postcontrast axial CT scan showing obvious enhancement of the lesion (supine position). C: Postcontrast coronal CT scan revealing a varix at the upper part of the right orbit (prone position).
was a routine laboratory examination. However, on March 11, a CT scan without contrast enhancement disclosed a moderately high-density mass, with a diameter of 5 x 5 m m , occupying the right orbital cavity (Fig. I A). The mass was believed to have the configuration of a vein stone. On a CT scan after intravenous injection of contrast material, performed while the patient was in the supine position, the mass was remarkably enlarged, measuring 10 • 10 m m in diameter (Fig. 1B). It was deep within the upper part of the orbit and appeared to lie under the levator palpebral muscle and the superior rectus muscle; the mass did not enlarge when the patient bent forward in the prone position (Fig. 1C). Right carotid angiography showed no abnor292
FIG. 2. A: Orbital phlebogram performed when the patient suffered from the first attack. A varix of the right superior ophthalmic vein is noted. B: Orbital phlebogram obtained during the patient's second attack. An enlarged varix is clearly seen. C: Postoperative phlebogram. No orbital varix is noted and the right superior ophthalmic vein is patent.
mality. Orbital phlebography was performed with the injection of contrast medium into a frontal vein. On the right side, the varix (measuring 10 • 10 x 10 m m ) was noted to be connected to the third segment of the superior ophthalmic vein (Fig. 2A). The patient received 5 mg prednisolone per day for 3 weeks and her double vision and dull pain gradually subsided. On March 27, 1985, she had no complaints related to the eye and was discharged; however, a postcontrast CT scan on April 2 showed the enhancing mass similar in size to that shown on Fig. lB. The patient was monitored on a regular basis in the outpatient clinic for about 7 months. On November 1, 1985, she again complained of a dull pain in her right upper orbit and forehead. The pain gradually increased and became intractable over the next 7 days, and she was unable to eat properly. She again complained of double vision, and was readmitted on November 8. J. Neurosurg. / Volume 73/August, 1990
Orbital varix with a pearly phlebolith
FIG. 4. Operative photograph showing removal of a pearly phlebolith.
FIG. 3. A: Postcontrast computerized tomography (CT) scan during the patient's second attack of severe facial pain and ophthalmoplegia (supine position). The varix is clearly seen in the right orbit. B: Postoperative contrast-enhanced CT scan showing no varix in the right orbit.
Second Admission. On the day of admission, an incomplete right oculomotor nerve palsy was noted. The patient had ptosis which showed a maximum levator excursion of 2 mm on the right compared to 8 mm on the left. In addition, she had a 4-mm outward displacement of the right eye with a marked ocular restriction (0" elevation, 20 ~ abduction, 0 ~ adduction, and 5* depression). Both pupils were circular, but the right pupil was 3 m m while the left pupil was 2.5 mm. Reaction to light was sluggish in the right pupil but prompt in the left. Measurements with the Hertel exophthalmometer revealed no proptosis related to position or to a Valsalva maneuver. Ocular tension and funduscopic examination were normal. The patient complained of severe pain in the area of her right frontal nerve, but examination of facial sensation and a corneal reflex test produced normal results. A postcontrast CT scan showed a varix measuring 10 x 10 mm in diameter (Fig. 3A), the same size as on the CT scan obtained on March 11, 1985. A postcontrast CT scan in the coronal plane again indicated a similarsized varix when the patient bent forward in the prone position. However, orbital venography on November 14 showed that the varix had enlarged to 15 x 17 x 15 mm (Fig. 2B). Operation. Right transcranial superolateral orbitotomy was performed through a coronal incision on November 28, 1985. Three separate bone flaps were J. Neurosurg. / Volume 73/August, 1990
removed: namely, a craniotomy flap of the frontal bone, a small bone flap including the superior, lateral orbital rim, and the lateral wall of the orbit, and a small bone flap of the orbital roof. After incision of the periorbita, the superior levator palpebral muscle was retracted laterally. Then the orbital fat was removed and a major portion of the varix was exposed using a microdissection technique. The wall of the varix was elastic and hard, allowing the complete varix to be exposed in a bloodless field. It was confirmed that the varix arose from the third segment of the superior ophthalmic vein. Repeated coagulation reduced the size of the varix, and a pearly phlebolith was extracted from the neck of the varix at the last stage of coagulation (Fig. 4). Finally, the coagulated varix neck was cut. It was ascertained that the bleeding from the vein and venous engorgement were not elicited by compression of the neck. Following this, the three bone flaps were repositioned to reconstruct the orbital roof and the orbital rim including the lateral wall. Postoperative Course. The postoperative course was uneventful except for the occurrence of incomplete oculomotor nerve palsy and dull pain in the operative field. The patient noted disappearance of the pain l week following the operation. Orbital venography obtained 21 days after the operation showed complete disappearance of the varix with patency of the superior ophthalmic vein (Fig. 2C). A postcontrast CT scan also showed no mass in the right orbit (Fig. 3B). Within I year postoperatively, the fight oculomotor palsy had completely subsided; the patient had returned to perfect health and was able to resume her regular work. She had no functional or cosmetic deficits when last examined in June, 1989, approximately 3 89years postoperatively. 293
T. Kubota, et al. orbital symptoms indicating a possible varix, such as diplopia and pain, are relatively u n u s u a l . 6'13'14 Ophthalmoplegia and pain become conspicuous during severe episodic attacks and may last for periods ranging from a few seconds to several days. 6'13 In the case presented here, the clinical manifestations of the orbital varix were unusual. Pain and diplopia developed gradually over several days and persisted after the second attack. On no occasion were they accompanied by exophthalmos induced by postural changes. These clinical features are difficult to interpret; however, it is reasonable to assume that they were due to a thrombosis formed by longstanding blood collections. 16'19The thrombosis may have attached to the vascular wall, causing first lamellar fibrosis then central necrosis. Finally, this resuited in the formation of a stone that was isolated from the vascular wall. 19 The phlebolith lodged at the neck of the varix, presumably occluding the venous channel; eventully the varix enlarged and, in turn, compressed the surrounding intraorbital structure. The pain and ophthalmoplegia probably occurred as a result of the nerves being compressed by the engorged varix. Intermittent or varied proptosis failed to appear, presumably due to the persistent size of the varix which was composed of the thick vessel wall. This type of varix can be referred to as a "venous aneurysm." FIG. 5. A: Photomicrograph of part of the orbital varix showing venous channels with a thick wall of vessels. H & E, x 60. B: Photomicrograph of the orbital phlebolith exhibiting a peripheral concentric lamellar structure encompassing a central amorphous material. Van Gieson, x 60.
Pathological Examination. The specimens obtained from the operation were stained with hematoxylin and eosin, and van Gieson for elastic fibers. The angiomatous portion consisted of sinusoid channels of varied shapes with a thick wall of vessels (Fig. 5A). A single layer of endothelium was aligned in some places at the inner aspect of the sinusoids. There were a moderate number of fine elastic fibers within the vessel walls. A round vein stone exhibited concentric laminations containing amorphous material in the center (Fig. 5B). The stone consisted of stout collagen fibers containing fine elastic fibers. No calcified area was observed. Discussion
Clinical Features The typical clinical symptom of an orbital varix is dramatic intermittent unilateral proptosis which depends upon the patient's postural changes. 4-6'13'14Rapid protrusion of one eye may be induced by stooping, coughing, holding the breath, or jugular vein compression, and may be particularly worsened by the Valsalva maneuver. The proptosis may soon disappear when venous congestion is relieved, commonly resulting in enophthalmos when the head is erect. Other ocular and 294
Diagnosis Diagnosis of an orbital varix has been much improved through recent advances in neuroimaging techniques. 2"4'73H538 Enlargement of the superior orbital fissure, dilated vascular markings on the frontal bone, and phleboliths can be detected on plain x-ray films.X~ These findings suggest various vascular lesions such as venous angioma, arteriovenous fistula, and arteriovenous malformation, 6'1~which can provide indirect evidence for the occurrence of an orbital varix. With the increased use of CT and magnetic resonance imaging, the size and position of an orbital varix and related anatomical conditions have been more easily detected and correctly diagnosed. 4'7'1z-15'18 A noncontrast CT scan obtained in basal conditions of the varix usually shows no abnormalities within the o r b i t ; 7'12'13'15 bilateral jugular compression or the Valsalva maneuver is often necessary to demonstrate the location and the extent of the varix, which may disappear when venous pressure is diminished. 7'12-15'~8 Mass density may be seen on a noncontrast CT scan while the patient is undergoing a severe episode of proptosis.13 A phlebolith, 2'7"15hemorrhage, 14 or an acutely thrombosed varix also can be detected on plain CT. A postcontrast CT scan may be more helpful in precisely identifying the status of the varix. Orbital phlebography has been widely recognized as the most reliable method of detecting an orbital varix. 5-7'9'14 The size and location of the varix can be commonly demonstrated when proptosis is provoked by increasing venous pressure. An orbital varix consists of either irregular segmental dilatation of the venous outJ. Neurosurg. / Volume 73~August, 1990
Orbital varix with a pearly phlebolith flow system or a local saccular dilatation, ~4 as seen in the present case. The information acquired from orbital phlebography may be necessary in planning surgical removal of the orbital varix. In this study, the preoperative size of the varix was identified more accurately on the phlebogram than on the postcontrast CT scan; however, phlebography may result only in partial filling of the varix and in such cases the CT findings may be more highly sensitive in detecting the lesion. ,3 Treatment There is no consensus concerning treatment for patients suffering from an orbital varix. 6'13A4 Treatment depends on the size of the varix, the duration of the proptosis, and the degree of disease progression. 6'14 Surgical intervention should be recommended when repeated episodes ofexophthalmos threaten visual function or cause intractable pain. 6'~3'~4 Surgery may be required when thrombosis or hemorrhage suddenly occLlr. 6'13A4 In the case presented here, acute thrombosis presumably took place and deteriorated to cause ocular and orbital symptoms. Surgical treatment may also be recommended if the patient suffers from continuous ophthalmoplegia and intractable pain. Injection of sclerosing agents into the orbit or ligation of the ophthalmic vein in the orbit may cause damage to the ocular structureJ 3 In our opinion, the best method of treatment is the radical excision of the varix. 5.6.J3.t4 Rem oval of a very large varix may be facilitated by electrical thrombosis with shrinkage of the mass, 5 making it easier to identify and ligate or clip the limits of the varix. Three main surgical approaches to the orbital lesions have been described: namely, the transcranial supraorbital, lateral (Kr6nlein), and medial approaches? ~ It may be most suitable to remove the varix via the transcranial approach if the varix is confined to the posterior orbit. 5'6'11'13 We chose an orbital approach involving a superolateral exposure ~'~'~ and an intraorbital lateral approach. 3 This exposure offered a wide operative field disclosing the complete varix. The varix with its thick aneurysmal wall (as shown histologically) was safely removed without profuse bleeding or damage to the surrounding anatomical structures. Following bone-flap reconstruction, the patient exhibited no functional or cosmetic deficits. References
1. A1-MeftyO, Fox JL: Superolateral orbital exposure and reconstruction. Surg Neurol 23:609-613, 1985 2. Beltramello A, Perini S, Marchini G, et al: Orbital varix with unusually large phlebolith. J Beige Radiol 67: 321-323, 1984
J. Neurosurg. / Volume 73/August, 1990
3. Blinkov SM, Gabibov GA, Tcherekayev VA: Transcranial surgical approaches to the orbital part of the optic nerve: an anatomical study. J Neurosurg 65:44-47, 1986 4. Bullock JD, Bartley GB: Dynamic proptosis. Am J Ophthalmol 102:104-110, 1986 5. Handa H, Moil K: Large varix of the superior ophthalmic vein: demonstration by angular phlebography and removal by electrically induced thrombosis. Case report. J Neurosurg 29:202-205, 1968 6. Henderson JW: Vascular malformations, in Henderson JW (ed): Orbital Tumors, ed 2. New York: ThiemeStratton, 1980, pp 154-176 7. Jacobs L: Vascular diseases of the orbit, in Jacobs L, Weisberg LA, Kinnkel WR (eds): Computerized Tomography of the Orbit and Sella Turcica. New York: Raven Press, 1980, pp 179-194 8. Jane JA, Park TS, Pobereskin LH, et al: The supraorbital approach: technical note. Neurosnrgery 11:537-542, 1982 9. Lloyd GAS: Pathological veins in the orbit. Br J Radiol 47:570-578, 1974 10. Lloyd GAS: Phleboliths in the orbit. Clio Radiol 16: 339-346, 1965 11. Maroon JC, Kennerdell JS, Abla A: The diagnosis and treatment of orbital tumors. Clin Neurosurg 34: 485-498, 1988 12. Osborn RE, DeWitt JD, Lester PD, et al: Magnetic resonance imaging of an orbital varix with CT and ultrasound correlation. Comput Radiol 10:155-159, 1986 13. Rivas JJ, Lobato RD, Cordob6s F, et al: Intermittent exophthalmos studied with computerized tomography. Report of two cases. J Neurosurg 57:290-294, 1982 14. Rootman J, Graeb DA.: Vascular lesions, in Rootman J (ed): Disease of the Orbit. Philadephia: JB Lippincott, 1988, pp 525-568 15. Salvolini U, Menichelli F, Pasquini U: Computer assisted tomography in 90 cases of exophthalmos. J Comput Assist Tomogr 1:81-100, 1977 16. Spencer WH: Vascular tumor and malformations, in Spencer WH (ed): Ophthalmic Pathology, ed 3. Philadelphia: WB Saunders, 1986, Vol 3, pp 2525-2554 17. Ward PH: The treatment of orbital varicosities. Arch Otolaryngol Head Neck Surg 113:286-288, 1987 18. Winter J, Centeno RS, Benton JR: Manuever to aid diagnosis of orbital varix by computed tomography. AJNR 3:39-40, 1982 19. Yamamoto H, Sch~ifer H, Sakae T, et al: Phlebolithiasis associated with intramuscular hemangioma. X-ray diffractometric, x-ray microanalytical and scanning electron microscopic investigations. Pathol Res Pract 181:55-59, 1986 Manuscript received November 7, 1989. Accepted in final form February 7, 1990. Address for Drs. Kuroda and Fujii: Department of Neurosurgery, Kanazawa University School of Medicine, Kanazawa, Japan. Address reprint requests to: Toshihiko Kubota, M.D., Department of Neurosurgery, Fukui Medical School, Matsuoka, Fukui 910-11, Japan.
295
Orbital varix with a pearly phlebolith Case report TOSHIHIKO KUBOTA, M.D., EIICHI KURODA, M.D., TOSHIHARU FUJII, M.D., HIROKAZU KAWANO, M.D., MASANORI KABUTO, M.D., AND MINORU HAYASHI, M.D. Department of Neurosurgery, Fukui Medical School, Fukui, and Department of Neurosurgery, Kanazawa University School of Medicine, Kanazawa, Japan A patient is described with an orbital varix arising from the fight superior ophthalmic vein, associated with ophthalmoplegia and severe pain, and without proptosis. The varix was detected using computerized tomography and orbital phlebography, and at surgery was verified as a venous aneurysm. During the operation, a pearly phlebolith was found. Histological examination of the varix revealed multiple ectatic venous channels. The etiology of this unusual clinical manifestation and the treatment of the patient are briefly discussed.
KEY WOADS
9 orbital varix
O
phlebolith
RBITAL vascular diseases are occasionally diagnosed and treated by specialists in the neuroophthalmological field. Among these entities are relatively c o m m o n disorders termed "orbital varices," which usually consist of congenital venous malformations. 16These abnormalities have been treated by ophthalmologists, 4'6'~4 neurosurgeons, 5'1j'~3 head and neck surgeons, ~7 and plastic surgeons. The classical clinical features of the orbital varix are intermittent proptosis which varies with the head position and which may be worsened during the Valsalva maneuver. 4-6~3 ~6 Instances of ophthalmoplegia and bruit are less common than carotid-cavernous fistulas and arteriovenous malformations. 6'j3'~4 Recent advances in neuroimaging techniques now make it possible to diagnose orbital lesions precisely 7'~5 and to select the best treatment procedures for these diseases. 6'1~'~4 Until recently, the treatment of orbital varices was unreliable because of their elusive clinical nature; 4-6'13'14however, the number of patients undergoing safe varix removal has increased due to the recent improvement of surgical techniques and instruments. 1'3-6'8'~1.13,14 We present the case of a female patient with a fight orbital varix and phlebolith who presented with ophthalmoplegia and pain but without proptosis. The varix was accurately diagnosed by computerized tomography (CT) and phlebography, and was safely removed through a superolateral orbital exposure. The varix was verified histologically as a venous channel. We
J. Neurosurg. / Volume 73/August, 1990
9 ophthalmoplegia
9 orbital pain
discuss the clinical manifestations of this orbital varix and phlebolith and describe the patient's treatment.
Case Report This 61-year-old woman was admitted to our hospital on March 7, 1985, complaining of double vision and a dull pain in the right orbit. She had first noticed double vision 1 week earlier while attempting to focus her eyes to the right. She complained of a dull pain in the upper part of her right eye. She immediately consulted an ophthalmologist who identified a right abducens palsy and referred her to our department for examination. She denied having had previous trauma, and the history provided by her family was noncontributory.
First Admission. The general physical examination showed no abnormalities; however, neurological examination revealed a mild disturbance of right eye movement with lateral gaze. The patient complained of dull pain in her right forehead, but no sensory disturbance was noted. There was no exophthalmos, conjunctival congestion, or eyelid swelling related to head position or Valsalva maneuver. Auscultation of the periorbital region was negative. Visual acuity was 0.6 in the fight eye and 0.7 in the left eye. Exophthalmometry performed by the Hertel technique gave a reading of 13 m m in both eyes. Perimetry, ocular tension, and funduscopic examination were normal. X-ray films of the skull and orbit were normal, as 291
T. Kubota, et al.
FIG. 1. Computerized tomography (CT) scans obtained when the patient suffered from the first attack of double vision and dull facial pain. A: Precontrast axial CT scan clearly showing a small retrobulbar mass on the right side (supine position). B: Postcontrast axial CT scan showing obvious enhancement of the lesion (supine position). C: Postcontrast coronal CT scan revealing a varix at the upper part of the right orbit (prone position).
was a routine laboratory examination. However, on March 11, a CT scan without contrast enhancement disclosed a moderately high-density mass, with a diameter of 5 x 5 m m , occupying the right orbital cavity (Fig. I A). The mass was believed to have the configuration of a vein stone. On a CT scan after intravenous injection of contrast material, performed while the patient was in the supine position, the mass was remarkably enlarged, measuring 10 • 10 m m in diameter (Fig. 1B). It was deep within the upper part of the orbit and appeared to lie under the levator palpebral muscle and the superior rectus muscle; the mass did not enlarge when the patient bent forward in the prone position (Fig. 1C). Right carotid angiography showed no abnor292
FIG. 2. A: Orbital phlebogram performed when the patient suffered from the first attack. A varix of the right superior ophthalmic vein is noted. B: Orbital phlebogram obtained during the patient's second attack. An enlarged varix is clearly seen. C: Postoperative phlebogram. No orbital varix is noted and the right superior ophthalmic vein is patent.
mality. Orbital phlebography was performed with the injection of contrast medium into a frontal vein. On the right side, the varix (measuring 10 • 10 x 10 m m ) was noted to be connected to the third segment of the superior ophthalmic vein (Fig. 2A). The patient received 5 mg prednisolone per day for 3 weeks and her double vision and dull pain gradually subsided. On March 27, 1985, she had no complaints related to the eye and was discharged; however, a postcontrast CT scan on April 2 showed the enhancing mass similar in size to that shown on Fig. lB. The patient was monitored on a regular basis in the outpatient clinic for about 7 months. On November 1, 1985, she again complained of a dull pain in her right upper orbit and forehead. The pain gradually increased and became intractable over the next 7 days, and she was unable to eat properly. She again complained of double vision, and was readmitted on November 8. J. Neurosurg. / Volume 73/August, 1990
Orbital varix with a pearly phlebolith
FIG. 4. Operative photograph showing removal of a pearly phlebolith.
FIG. 3. A: Postcontrast computerized tomography (CT) scan during the patient's second attack of severe facial pain and ophthalmoplegia (supine position). The varix is clearly seen in the right orbit. B: Postoperative contrast-enhanced CT scan showing no varix in the right orbit.
Second Admission. On the day of admission, an incomplete right oculomotor nerve palsy was noted. The patient had ptosis which showed a maximum levator excursion of 2 mm on the right compared to 8 mm on the left. In addition, she had a 4-mm outward displacement of the right eye with a marked ocular restriction (0" elevation, 20 ~ abduction, 0 ~ adduction, and 5* depression). Both pupils were circular, but the right pupil was 3 m m while the left pupil was 2.5 mm. Reaction to light was sluggish in the right pupil but prompt in the left. Measurements with the Hertel exophthalmometer revealed no proptosis related to position or to a Valsalva maneuver. Ocular tension and funduscopic examination were normal. The patient complained of severe pain in the area of her right frontal nerve, but examination of facial sensation and a corneal reflex test produced normal results. A postcontrast CT scan showed a varix measuring 10 x 10 mm in diameter (Fig. 3A), the same size as on the CT scan obtained on March 11, 1985. A postcontrast CT scan in the coronal plane again indicated a similarsized varix when the patient bent forward in the prone position. However, orbital venography on November 14 showed that the varix had enlarged to 15 x 17 x 15 mm (Fig. 2B). Operation. Right transcranial superolateral orbitotomy was performed through a coronal incision on November 28, 1985. Three separate bone flaps were J. Neurosurg. / Volume 73/August, 1990
removed: namely, a craniotomy flap of the frontal bone, a small bone flap including the superior, lateral orbital rim, and the lateral wall of the orbit, and a small bone flap of the orbital roof. After incision of the periorbita, the superior levator palpebral muscle was retracted laterally. Then the orbital fat was removed and a major portion of the varix was exposed using a microdissection technique. The wall of the varix was elastic and hard, allowing the complete varix to be exposed in a bloodless field. It was confirmed that the varix arose from the third segment of the superior ophthalmic vein. Repeated coagulation reduced the size of the varix, and a pearly phlebolith was extracted from the neck of the varix at the last stage of coagulation (Fig. 4). Finally, the coagulated varix neck was cut. It was ascertained that the bleeding from the vein and venous engorgement were not elicited by compression of the neck. Following this, the three bone flaps were repositioned to reconstruct the orbital roof and the orbital rim including the lateral wall. Postoperative Course. The postoperative course was uneventful except for the occurrence of incomplete oculomotor nerve palsy and dull pain in the operative field. The patient noted disappearance of the pain l week following the operation. Orbital venography obtained 21 days after the operation showed complete disappearance of the varix with patency of the superior ophthalmic vein (Fig. 2C). A postcontrast CT scan also showed no mass in the right orbit (Fig. 3B). Within I year postoperatively, the fight oculomotor palsy had completely subsided; the patient had returned to perfect health and was able to resume her regular work. She had no functional or cosmetic deficits when last examined in June, 1989, approximately 3 89years postoperatively. 293
T. Kubota, et al. orbital symptoms indicating a possible varix, such as diplopia and pain, are relatively u n u s u a l . 6'13'14 Ophthalmoplegia and pain become conspicuous during severe episodic attacks and may last for periods ranging from a few seconds to several days. 6'13 In the case presented here, the clinical manifestations of the orbital varix were unusual. Pain and diplopia developed gradually over several days and persisted after the second attack. On no occasion were they accompanied by exophthalmos induced by postural changes. These clinical features are difficult to interpret; however, it is reasonable to assume that they were due to a thrombosis formed by longstanding blood collections. 16'19The thrombosis may have attached to the vascular wall, causing first lamellar fibrosis then central necrosis. Finally, this resuited in the formation of a stone that was isolated from the vascular wall. 19 The phlebolith lodged at the neck of the varix, presumably occluding the venous channel; eventully the varix enlarged and, in turn, compressed the surrounding intraorbital structure. The pain and ophthalmoplegia probably occurred as a result of the nerves being compressed by the engorged varix. Intermittent or varied proptosis failed to appear, presumably due to the persistent size of the varix which was composed of the thick vessel wall. This type of varix can be referred to as a "venous aneurysm." FIG. 5. A: Photomicrograph of part of the orbital varix showing venous channels with a thick wall of vessels. H & E, x 60. B: Photomicrograph of the orbital phlebolith exhibiting a peripheral concentric lamellar structure encompassing a central amorphous material. Van Gieson, x 60.
Pathological Examination. The specimens obtained from the operation were stained with hematoxylin and eosin, and van Gieson for elastic fibers. The angiomatous portion consisted of sinusoid channels of varied shapes with a thick wall of vessels (Fig. 5A). A single layer of endothelium was aligned in some places at the inner aspect of the sinusoids. There were a moderate number of fine elastic fibers within the vessel walls. A round vein stone exhibited concentric laminations containing amorphous material in the center (Fig. 5B). The stone consisted of stout collagen fibers containing fine elastic fibers. No calcified area was observed. Discussion
Clinical Features The typical clinical symptom of an orbital varix is dramatic intermittent unilateral proptosis which depends upon the patient's postural changes. 4-6'13'14Rapid protrusion of one eye may be induced by stooping, coughing, holding the breath, or jugular vein compression, and may be particularly worsened by the Valsalva maneuver. The proptosis may soon disappear when venous congestion is relieved, commonly resulting in enophthalmos when the head is erect. Other ocular and 294
Diagnosis Diagnosis of an orbital varix has been much improved through recent advances in neuroimaging techniques. 2"4'73H538 Enlargement of the superior orbital fissure, dilated vascular markings on the frontal bone, and phleboliths can be detected on plain x-ray films.X~ These findings suggest various vascular lesions such as venous angioma, arteriovenous fistula, and arteriovenous malformation, 6'1~which can provide indirect evidence for the occurrence of an orbital varix. With the increased use of CT and magnetic resonance imaging, the size and position of an orbital varix and related anatomical conditions have been more easily detected and correctly diagnosed. 4'7'1z-15'18 A noncontrast CT scan obtained in basal conditions of the varix usually shows no abnormalities within the o r b i t ; 7'12'13'15 bilateral jugular compression or the Valsalva maneuver is often necessary to demonstrate the location and the extent of the varix, which may disappear when venous pressure is diminished. 7'12-15'~8 Mass density may be seen on a noncontrast CT scan while the patient is undergoing a severe episode of proptosis.13 A phlebolith, 2'7"15hemorrhage, 14 or an acutely thrombosed varix also can be detected on plain CT. A postcontrast CT scan may be more helpful in precisely identifying the status of the varix. Orbital phlebography has been widely recognized as the most reliable method of detecting an orbital varix. 5-7'9'14 The size and location of the varix can be commonly demonstrated when proptosis is provoked by increasing venous pressure. An orbital varix consists of either irregular segmental dilatation of the venous outJ. Neurosurg. / Volume 73~August, 1990
Orbital varix with a pearly phlebolith flow system or a local saccular dilatation, ~4 as seen in the present case. The information acquired from orbital phlebography may be necessary in planning surgical removal of the orbital varix. In this study, the preoperative size of the varix was identified more accurately on the phlebogram than on the postcontrast CT scan; however, phlebography may result only in partial filling of the varix and in such cases the CT findings may be more highly sensitive in detecting the lesion. ,3 Treatment There is no consensus concerning treatment for patients suffering from an orbital varix. 6'13A4 Treatment depends on the size of the varix, the duration of the proptosis, and the degree of disease progression. 6'14 Surgical intervention should be recommended when repeated episodes ofexophthalmos threaten visual function or cause intractable pain. 6'~3'~4 Surgery may be required when thrombosis or hemorrhage suddenly occLlr. 6'13A4 In the case presented here, acute thrombosis presumably took place and deteriorated to cause ocular and orbital symptoms. Surgical treatment may also be recommended if the patient suffers from continuous ophthalmoplegia and intractable pain. Injection of sclerosing agents into the orbit or ligation of the ophthalmic vein in the orbit may cause damage to the ocular structureJ 3 In our opinion, the best method of treatment is the radical excision of the varix. 5.6.J3.t4 Rem oval of a very large varix may be facilitated by electrical thrombosis with shrinkage of the mass, 5 making it easier to identify and ligate or clip the limits of the varix. Three main surgical approaches to the orbital lesions have been described: namely, the transcranial supraorbital, lateral (Kr6nlein), and medial approaches? ~ It may be most suitable to remove the varix via the transcranial approach if the varix is confined to the posterior orbit. 5'6'11'13 We chose an orbital approach involving a superolateral exposure ~'~'~ and an intraorbital lateral approach. 3 This exposure offered a wide operative field disclosing the complete varix. The varix with its thick aneurysmal wall (as shown histologically) was safely removed without profuse bleeding or damage to the surrounding anatomical structures. Following bone-flap reconstruction, the patient exhibited no functional or cosmetic deficits. References
1. A1-MeftyO, Fox JL: Superolateral orbital exposure and reconstruction. Surg Neurol 23:609-613, 1985 2. Beltramello A, Perini S, Marchini G, et al: Orbital varix with unusually large phlebolith. J Beige Radiol 67: 321-323, 1984
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3. Blinkov SM, Gabibov GA, Tcherekayev VA: Transcranial surgical approaches to the orbital part of the optic nerve: an anatomical study. J Neurosurg 65:44-47, 1986 4. Bullock JD, Bartley GB: Dynamic proptosis. Am J Ophthalmol 102:104-110, 1986 5. Handa H, Moil K: Large varix of the superior ophthalmic vein: demonstration by angular phlebography and removal by electrically induced thrombosis. Case report. J Neurosurg 29:202-205, 1968 6. Henderson JW: Vascular malformations, in Henderson JW (ed): Orbital Tumors, ed 2. New York: ThiemeStratton, 1980, pp 154-176 7. Jacobs L: Vascular diseases of the orbit, in Jacobs L, Weisberg LA, Kinnkel WR (eds): Computerized Tomography of the Orbit and Sella Turcica. New York: Raven Press, 1980, pp 179-194 8. Jane JA, Park TS, Pobereskin LH, et al: The supraorbital approach: technical note. Neurosnrgery 11:537-542, 1982 9. Lloyd GAS: Pathological veins in the orbit. Br J Radiol 47:570-578, 1974 10. Lloyd GAS: Phleboliths in the orbit. Clio Radiol 16: 339-346, 1965 11. Maroon JC, Kennerdell JS, Abla A: The diagnosis and treatment of orbital tumors. Clin Neurosurg 34: 485-498, 1988 12. Osborn RE, DeWitt JD, Lester PD, et al: Magnetic resonance imaging of an orbital varix with CT and ultrasound correlation. Comput Radiol 10:155-159, 1986 13. Rivas JJ, Lobato RD, Cordob6s F, et al: Intermittent exophthalmos studied with computerized tomography. Report of two cases. J Neurosurg 57:290-294, 1982 14. Rootman J, Graeb DA.: Vascular lesions, in Rootman J (ed): Disease of the Orbit. Philadephia: JB Lippincott, 1988, pp 525-568 15. Salvolini U, Menichelli F, Pasquini U: Computer assisted tomography in 90 cases of exophthalmos. J Comput Assist Tomogr 1:81-100, 1977 16. Spencer WH: Vascular tumor and malformations, in Spencer WH (ed): Ophthalmic Pathology, ed 3. Philadelphia: WB Saunders, 1986, Vol 3, pp 2525-2554 17. Ward PH: The treatment of orbital varicosities. Arch Otolaryngol Head Neck Surg 113:286-288, 1987 18. Winter J, Centeno RS, Benton JR: Manuever to aid diagnosis of orbital varix by computed tomography. AJNR 3:39-40, 1982 19. Yamamoto H, Sch~ifer H, Sakae T, et al: Phlebolithiasis associated with intramuscular hemangioma. X-ray diffractometric, x-ray microanalytical and scanning electron microscopic investigations. Pathol Res Pract 181:55-59, 1986 Manuscript received November 7, 1989. Accepted in final form February 7, 1990. Address for Drs. Kuroda and Fujii: Department of Neurosurgery, Kanazawa University School of Medicine, Kanazawa, Japan. Address reprint requests to: Toshihiko Kubota, M.D., Department of Neurosurgery, Fukui Medical School, Matsuoka, Fukui 910-11, Japan.
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