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Varix of the Vortex Ampulla
VARIX OF THE VORTEX AMPULLA ROBERT H. OSHER, M.D., Miami, Florida, and Columbus, Ohio AND
GARY W. ABRAMS, M.D., DAVID YARIAN, AND DlANNE ARMAO, B.A. Miami, Florida
M.D.,
In five patients, extreme gaze resulted in the appearance and expansion of a lesion located at the site of a venous vortex ampulla. The dimensions and color of the lesion varied depending upon the direction and duration of gaze. The lesion expanded when the head was positioned below the level of the heart and when a Valsalva maneuver was performed, even when the primary position of gaze was main tained. The lesion rapidly detumesced and disappeared when the head was elevated, digital pressure was exerted on the globe, or gaze was directed elsewhere. Fluorescein angiography confirmed that this tumefaction originated from the venous vortex ampulla. These dynamic features observed ophthalmoscopically and demon strated by ultrasonography are characteristic of the vortex ampulla varix; the lesion should not be confused with a malignancy. The elevated fundus lesion is a poten tial danger for the patient and a diagnos tic challenge for the ophthalmologist. Recognition of certain benign character istics permits the physician to differenti ate the lesion from a malignancy. We describe herein an intermittent intraocu lar tumefaction that should be considered part of the differential diagnosis of the elevated fundus lesion. CASE REPORTS
Case 1—A 55-year-old woman, who had been deaf since a childhood episode of scarlet fever, underwent
From the Department of Ophthalmology, the Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida (Drs. Osher, Abrams, and Yarian and Ms. Armao); and the Depart ment of Ophthalmology, Ohio State University, Co lumbus, Ohio (Dr. Osher). Reprint requests to Robert H. Osher, M.D., 8040 Reading Rd., Cincinnati, OH 45237.
an uncomplicated intracapsular cataract extraction in her right eye. Her postoperative course was unevent ful and she had a visual acuity of 6/6 (20/20) with a + 11.25 +1.00 xl80 spectacle correction. Her visual acuity in the unoperated on eye was reduced to 6/15 (20/50), consistent with her cataract. The pupils, ocular motility, visual fields, anterior segments, and intraocular pressures were normal. Ophthalmoscopy performed with the eyes in the primary position disclosed a normal right fundus with one vortex vein present in each quadrant (Fig. 1). During upgaze, however, we observed a pigmented, smoothly elevated, tumefaction located at the superonasal vortex ampulla; during a five-second interval, it progressively enlarged to a lateral size of 2.5 x 2.5 disk diameters and a height of approximately 1.5 disk diameters (Fig. 2). On refixation to the primary position, the mass rapidly detumesced until no evi dence of the lesion remained and the fundus ap peared normal. Overlying the vortex ampulla, sever al small drusen were noted but the chorioretinal anatomy was otherwise unremarkable. The left fun dus was normal in the primary position and in all fields of gaze. Because this unusual sequence of events was re peatedly observed, an underlying neoplasm ap peared to be unlikely. The pathogenic mechanisms that could account for such dynamic behavior seemed limited to the following: (1) gaze-evoked compres-
AMERICAN JOURNAL OF OPHTHALMOLOGY 92:653-660, 1981
653
654
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1981
Fig. 1 (Osher and associates). Superonasal vortex ampulla in the primary position of gaze.
Fig. 2 (Osher and associates). On upgaze, tumefac tion appears and progressively expands.
sion of the vortex ampulla by an extraocular muscle; (2) gaze-evoked kinking of the intrascleral or episcleral vortex vein; (3) a hyperviscosity state contribut ing to vortex venous stasis; or (4) a varix involving the vortex ampulla. Specific maneuvers were de signed to distinguish between these hypothesized conditions. In order to eliminate the influence of extraocular muscle contraction, gaze was maintained in the pri mary position while head position was varied in relation to heart level. The mass gradually filled as the patient's head was lowered below the level of the table upon which she was reclining (Fig. 3), and it emptied when her head was elevated. A Valsalva maneuver, performed with the patient in either a recumbent or sitting position, resulted in slow filling of the lesion while gaze was maintained in the primary position. The mass rapidly collapsed if digital pressure was exerted against the globe regardless of gaze, position, or concurrence of forced expiration. The lesion did not expand with jugular vein compres sion, but a blood reflux immediately increased the width of the ampulla when the neck was compressed. When pressure on the jugular vein was released, the blood reflux disappeared. Applanation tonometry was performed in the pri mary position during upgaze. In the right eye, the intraocular pressure consistently measured 10 ± 2 mm Hg in the primary position, but increased to 20 ± 2 mm Hg during upgaze. In the left eye, the intraocular pressure consistently measured 10 ± 2 mm Hg in both the primary position and during upgaze. There was no spontaneous venous pulsation in the right eye in the primary position and during upgaze. Ophthalmodynamometry disclosed variable results (Table), although the lesion consistently collapsed
below diastole. The systemic blood pressure was recorded at 135/70 mm Hg. Fluorescein angiography confirmed that the lesion originated at the vortex ampulla. The large choroidal tributary vessels enter ing the vortex ampulla filled early, then dilated,
Fig. 3 (Osher and associates). Method of observing postural effect on lesion while gaze is maintained in primary position.
VOL. 92, NO. 5
VARIX OF THE VORTEX AMPULLA TABLE
OPHTHALMODYNAMOMETRY
Visit*
Systole
1 2 3
120 100 86
Pressure (mm Hg) Collapse of Varix Diastole 45 35 26
35 30 15
*Visits were approximately one month apart. implying venous congestion and intravascular stasis. This lesion did not stain (although a few overlying drusen did) and became hypofluorescent during the late phases of angiography. Ultrasonography documented the dynamic nature of the lesion. A thin-walled, dome-shaped, lesion of low acoustic reflectivity (Fig. 4) rapidly expanded on upgaze to a maximum height of 2.3 mm. During upgaze, the lesion detumesced when digital pressure was applied to the globe, but rapidly expanded as the external pressure was removed (Fig. 4). The lesion also detumesced and disappeared when gaze was directed elsewhere. Individual extraocular muscle size was unremarkable and appeared symmetric when compared to the rectus muscle in the fellow eye. A medical evaluation disclosed right atrial hyper trophy secondary to mild chronic obstructive pulmo nary disease. The results of screening hématologie and electrolyte studies were unremarkable. Two
655
years after the initial observation, the patient under went an uncomplicated intracapsular cataract extrac tion in the left eye. We did not find a mass similar to that in the right eye regardless of gaze direction or head position. Case 2—A 69-year-old man came to the outpatient clinic complaining of presbyopia. He was taking isoproterenol HC1 and digoxin for mild chronic ob structive pulmonary disease and asymptomatic ath erosclerotic cardiovascular disease. Visual acuity was correctable to 6/6 (20/20) with +2.25 +1.25 x 160 in the right eye and +3.00 +0.75 X020 in the left eye. The pupils, ocular motility, visual fields, anterior segments, and intraocular pres sures were unremarkable. Ophthalmoscopy in the primary position revealed a normal fondus. During upgaze, however, a nonpigmented, dome-shaped, elevated lesion located at the site of the superonasal vortex ampulla appeared simultaneously in the right (Fig. 5) and left eyes (Fig. 6). During a five-sec ond interval these lesions progressively enlarged to 2.5 x 3 disk diameters at the base of the lesion and nearly 2 disk diameters in height. When gaze was redirected to the primary position, the lesions rapid ly decreased in size until they were no longer visible and the overlying retina appeared flat. On each attempted upgaze, both lesions progressively ex panded and became elevated. They detumesced when gaze was shifted elsewhere or digital pressure was applied to the globe. Ultrasonography disclosed a dome-shaped elevat ed mass of low internal acoustic reflectivity in the superonasal quadrant of each eye that progressively appeared during upgaze (Fig. 7). The lesions detu mesced and disappeared when gaze was directed
Fig. 4 (Osher and associates). B-scan ultrasonography demon strates expansion of thin-walled, dome-shaped lesion of low acoustic reflectivity during upgaze (at left). Varix promptly collapses when digi tal pressure is applied to globe or gaze is redirected (at right).
656
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1981
Fig. 5 (Osher and associates). Progressive expansion of superonasal vortex ampulla varix in right eye as fixation is shifted from the primary position (left) to upgaze (right).
elsewhere or digital pressure was applied to the globe (Fig. 7). The maximum height of either lesion was 2.6 mm. Cases 3 to 5—A single varix of the vortex ampulla has been identified in three additional middle-aged patients. The inferior fundus was the site of the varix in two eyes. The clinical appearance and dynamic behavior was identical to those lesions located in the superior quadrants, except that the gaze-evoked ex pansion occurred only in downgaze.
DISCUSSION
Knowledge of the characteristic behav ior displayed by the vortex ampulla varix should prevent misdiagnosis of this enti ty. The initial appearance of the lesion, detected most easily by indirect ophthalmoscopy, depends upon the direction
Fig. 6 (Osher and associates). Superonasal vortex ampulla in left eye demonstrates similar sequence in primary position (left) and upgaze (right).
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VARIX OF THE VORTEX AMPULLA
657
Fig. 7 (Osher and associates). B-scan ultrasonography demon strates expansion of thin-walled, dome-shaped lesion of low acoustic reflectivity during upgaze (at left). Varix promptly collapses when digi tal pressure is applied to globe or gaze is redirected (at right).
and duration of gaze at the time the vortex ampulla is observed. For example, when the lesion is located superiorly, examination of the ampulla in either early upgaze or during gaze directed else where may disclose only normal chorioretinal anatomy. As upgaze is sustained, a smoothly contoured, venous-colored (Case 1) or nonpigmented (Case 2) mass originates from the ampulla and may at tain a lateral size of 3 X 3 disk diameters and a height of 2 to 3 mm. As gaze is redirected the congestive state is re lieved and the mass detumesces. Expan sion of the lesion, by either a Valsalva maneuver or by positioning the head below heart level, can also be achieved while the primary position of gaze is maintained. The lesion rapidly empties and flattens when the head is elevated or digital pressure is applied against the globe. Jugular compression did not ex pand the lesion, but may produce a visi ble reflux of blood into the ampulla, which promptly drains when the neck is decompressed. In our first patient, upgaze was accom panied by a marked elevation of intraocu lar pressure in the involved eye. The
fellow eye had a stable intraocular pres sure when gaze was shifted between the primary position and upgaze. Possibly, contraction of the inferior oblique muscle during upgaze compressed the underly ing inferotemporal vortex vein, thereby placing an increased venous load upon the remaining three vortex veins. The consequent increase in intravenous vol ume increased venous pressure enough to expand the varix. Neither the com pressed inferotemporal vortex vein nor the congested superonasal varix could function effectively, leaving only the two remaining normal vortex veins for venous outflow. The choroidal blood volume in creased, and may thus have accounted for the increase in intraocular pressure. 1 Fluorescein angiography confirmed intravascular stasis and dilatation of the tributary vessels, in addition to demon strating that the origin of the lesion was the vortex ampulla. Ophthalmodynamometry (performed in our first patient) further supported a choroidal origin, be cause the lesion consistently collapsed below the observed diastole. Ultrasonography proved to be a reli able method of detecting the dynamic
658
AMERICAN JOURNAL OF OPHTHALMOLOGY
nature of this entity, and could provide a diagnosis in a patient with opaque media. Progressive expansion of the lesion dur ing a specific field of gaze was easily demonstrated, whereas detumescence was observed when either digital pres sure was applied to the globe or when gaze was shifted elsewhere. In order to determine the prevalence of this lesion, we examined 100 consecu tive patients undergoing routine eye evaluations. No similar lesion was identi fied. The pathogenesis of this lesion was challenging to establish. No significant predisposing systemic disease process contributing to venous sludging (such as congestive heart failure, superior vena cava syndrome, or polycythemia vera) was found. The involvement of the superonasal vortex ampulla in the first three eyes initially suggested that a specific local factor might be responsible for this phenomenon, but examination of other eyes disclosed that the lesion was present elsewhere. Extraocular muscle compression was considered as a possible cause of venous obstruction proximal to the vortex ampul la, because venous tamponade could occur during muscle contraction. This appears to be unlikely, however, because expansion of the lesion should not result from either passive head positioning or a Valsalva maneuver when the primary po sition of gaze is maintained. Further, the recti muscles are not in contact with the exit of the vortex veins, and only the oblique muscles can compress the two lateral veins (Fig. 8). 2 Nevertheless, we examined five patients with docu mented enlargement of extraocular mus cles secondary to severe thyroid ophthalmopathy as controls. We were unable to identify similar expanding lesions regard less of gaze direction or maneuvers per formed.
NOVEMBER, 1981
We also considered kinking of the vor tex vein as a possible cause of gazeevoked venous obstruction. For example, if the intrascleral portion of the superonasal vortex vein pursued an exaggerated oblique course posteriorly toward the optic nerve, extreme gaze could kink the vessel where it emerges from the globe (Fig. 9). Rutnin 3 has found a variable in trascleral length (3.5 to 6 mm) and course of the vortex vein. Upgaze could similarly kink the vortex vein if a posterior episcleral adhesion were present (Fig. 9). However, the expansion of the lesion by maneuvers performed while the eye is maintained in the primary position ar gues strongly against gaze-evoked kink ing. The most plausible explanation is that the lesion itself represents a varix of the vortex ampulla. All maneuvers (head depression, jugular vein compression, forced expiration) that are independent of gaze direction and increase intravenous pressure can result in variable distention of the varix. Detumescence can be achieved by either facilitating venous outflow (head elevation) or by superceding the venous pressure and directly col lapsing the varix (digital pressure). Local
Fig. 8 (Osher and associates). Globe viewed from retrobulbar space illustrating anatomic relationship between extraocular muscles and vortex veins as they emerge from their intrascleral canals.
VOL. 92, NO. 5
VARIX OF THE VORTEX AMPULLA
659
Fig. 9 (Osher and associates). Theoretic basis for vortex vein kinking during upgaze secondary to oblique intrascleral canal (top) and episcleral adhesion (bottom).
factors causing gaze-evoked distention of the varix remain unknown, although we have hypothesized that contraction of the oblique extraocular muscles may obstruct venous outflow in the underlying vortex
system. The remaining vortex veins must assume an additional venous flow, in creasing intravenous pressure and caus ing an expansion of the varix. Regardless of the actual pathogenesis,
660
AMERICAN JOURNAL OF OPHTHALMOLOGY
this dynamic tumefaction should be easily recognized by its distinct clinical behav ior and not confused with a malignancy. ACKNOWLEDGMENT
Randy Campo, M.D., and Mark Daily, M.D., referred Patients 3, 4, and 5.
Fifty years ago this month in
NOVEMBER, 1981
REFERENCES 1. Hayreh, S. S., and Baines, J. A. B.: Occlusion of the vortex veins. Br. J. Ophthalmol. 57:217, 1973. 2. Wolff, E.: Anatomy of the Eye and Orbit, 2nd ed. Philadelphia, Blakiston Co., 1940, p. 60. 3. Rutnin, U.: Fundus appearance in normal eyes. 1. The choroid. Am. J. Ophthalmol. 64:821, 1967.
THE
JOURNAL:
Like the dispute, now almost forgotten, concerning the relative merits of limbal extraction (as introduced by Daviel in the eight eenth century) and of the couching operation which had been used for thousands of years all over the world, the controversy as to the advantages and disadvantages of intracapsular as compared with extracapsular extraction of cataract may rage for a hundred years. It may even happen that the two methods will persist side by side for many centuries to come. W. H. Crisp: Intracapsular versus extracapsular Am. J. Ophthalmol. 14:1170, 1931
GARY W. ABRAMS, M.D., DAVID YARIAN, AND DlANNE ARMAO, B.A. Miami, Florida
M.D.,
In five patients, extreme gaze resulted in the appearance and expansion of a lesion located at the site of a venous vortex ampulla. The dimensions and color of the lesion varied depending upon the direction and duration of gaze. The lesion expanded when the head was positioned below the level of the heart and when a Valsalva maneuver was performed, even when the primary position of gaze was main tained. The lesion rapidly detumesced and disappeared when the head was elevated, digital pressure was exerted on the globe, or gaze was directed elsewhere. Fluorescein angiography confirmed that this tumefaction originated from the venous vortex ampulla. These dynamic features observed ophthalmoscopically and demon strated by ultrasonography are characteristic of the vortex ampulla varix; the lesion should not be confused with a malignancy. The elevated fundus lesion is a poten tial danger for the patient and a diagnos tic challenge for the ophthalmologist. Recognition of certain benign character istics permits the physician to differenti ate the lesion from a malignancy. We describe herein an intermittent intraocu lar tumefaction that should be considered part of the differential diagnosis of the elevated fundus lesion. CASE REPORTS
Case 1—A 55-year-old woman, who had been deaf since a childhood episode of scarlet fever, underwent
From the Department of Ophthalmology, the Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida (Drs. Osher, Abrams, and Yarian and Ms. Armao); and the Depart ment of Ophthalmology, Ohio State University, Co lumbus, Ohio (Dr. Osher). Reprint requests to Robert H. Osher, M.D., 8040 Reading Rd., Cincinnati, OH 45237.
an uncomplicated intracapsular cataract extraction in her right eye. Her postoperative course was unevent ful and she had a visual acuity of 6/6 (20/20) with a + 11.25 +1.00 xl80 spectacle correction. Her visual acuity in the unoperated on eye was reduced to 6/15 (20/50), consistent with her cataract. The pupils, ocular motility, visual fields, anterior segments, and intraocular pressures were normal. Ophthalmoscopy performed with the eyes in the primary position disclosed a normal right fundus with one vortex vein present in each quadrant (Fig. 1). During upgaze, however, we observed a pigmented, smoothly elevated, tumefaction located at the superonasal vortex ampulla; during a five-second interval, it progressively enlarged to a lateral size of 2.5 x 2.5 disk diameters and a height of approximately 1.5 disk diameters (Fig. 2). On refixation to the primary position, the mass rapidly detumesced until no evi dence of the lesion remained and the fundus ap peared normal. Overlying the vortex ampulla, sever al small drusen were noted but the chorioretinal anatomy was otherwise unremarkable. The left fun dus was normal in the primary position and in all fields of gaze. Because this unusual sequence of events was re peatedly observed, an underlying neoplasm ap peared to be unlikely. The pathogenic mechanisms that could account for such dynamic behavior seemed limited to the following: (1) gaze-evoked compres-
AMERICAN JOURNAL OF OPHTHALMOLOGY 92:653-660, 1981
653
654
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1981
Fig. 1 (Osher and associates). Superonasal vortex ampulla in the primary position of gaze.
Fig. 2 (Osher and associates). On upgaze, tumefac tion appears and progressively expands.
sion of the vortex ampulla by an extraocular muscle; (2) gaze-evoked kinking of the intrascleral or episcleral vortex vein; (3) a hyperviscosity state contribut ing to vortex venous stasis; or (4) a varix involving the vortex ampulla. Specific maneuvers were de signed to distinguish between these hypothesized conditions. In order to eliminate the influence of extraocular muscle contraction, gaze was maintained in the pri mary position while head position was varied in relation to heart level. The mass gradually filled as the patient's head was lowered below the level of the table upon which she was reclining (Fig. 3), and it emptied when her head was elevated. A Valsalva maneuver, performed with the patient in either a recumbent or sitting position, resulted in slow filling of the lesion while gaze was maintained in the primary position. The mass rapidly collapsed if digital pressure was exerted against the globe regardless of gaze, position, or concurrence of forced expiration. The lesion did not expand with jugular vein compres sion, but a blood reflux immediately increased the width of the ampulla when the neck was compressed. When pressure on the jugular vein was released, the blood reflux disappeared. Applanation tonometry was performed in the pri mary position during upgaze. In the right eye, the intraocular pressure consistently measured 10 ± 2 mm Hg in the primary position, but increased to 20 ± 2 mm Hg during upgaze. In the left eye, the intraocular pressure consistently measured 10 ± 2 mm Hg in both the primary position and during upgaze. There was no spontaneous venous pulsation in the right eye in the primary position and during upgaze. Ophthalmodynamometry disclosed variable results (Table), although the lesion consistently collapsed
below diastole. The systemic blood pressure was recorded at 135/70 mm Hg. Fluorescein angiography confirmed that the lesion originated at the vortex ampulla. The large choroidal tributary vessels enter ing the vortex ampulla filled early, then dilated,
Fig. 3 (Osher and associates). Method of observing postural effect on lesion while gaze is maintained in primary position.
VOL. 92, NO. 5
VARIX OF THE VORTEX AMPULLA TABLE
OPHTHALMODYNAMOMETRY
Visit*
Systole
1 2 3
120 100 86
Pressure (mm Hg) Collapse of Varix Diastole 45 35 26
35 30 15
*Visits were approximately one month apart. implying venous congestion and intravascular stasis. This lesion did not stain (although a few overlying drusen did) and became hypofluorescent during the late phases of angiography. Ultrasonography documented the dynamic nature of the lesion. A thin-walled, dome-shaped, lesion of low acoustic reflectivity (Fig. 4) rapidly expanded on upgaze to a maximum height of 2.3 mm. During upgaze, the lesion detumesced when digital pressure was applied to the globe, but rapidly expanded as the external pressure was removed (Fig. 4). The lesion also detumesced and disappeared when gaze was directed elsewhere. Individual extraocular muscle size was unremarkable and appeared symmetric when compared to the rectus muscle in the fellow eye. A medical evaluation disclosed right atrial hyper trophy secondary to mild chronic obstructive pulmo nary disease. The results of screening hématologie and electrolyte studies were unremarkable. Two
655
years after the initial observation, the patient under went an uncomplicated intracapsular cataract extrac tion in the left eye. We did not find a mass similar to that in the right eye regardless of gaze direction or head position. Case 2—A 69-year-old man came to the outpatient clinic complaining of presbyopia. He was taking isoproterenol HC1 and digoxin for mild chronic ob structive pulmonary disease and asymptomatic ath erosclerotic cardiovascular disease. Visual acuity was correctable to 6/6 (20/20) with +2.25 +1.25 x 160 in the right eye and +3.00 +0.75 X020 in the left eye. The pupils, ocular motility, visual fields, anterior segments, and intraocular pres sures were unremarkable. Ophthalmoscopy in the primary position revealed a normal fondus. During upgaze, however, a nonpigmented, dome-shaped, elevated lesion located at the site of the superonasal vortex ampulla appeared simultaneously in the right (Fig. 5) and left eyes (Fig. 6). During a five-sec ond interval these lesions progressively enlarged to 2.5 x 3 disk diameters at the base of the lesion and nearly 2 disk diameters in height. When gaze was redirected to the primary position, the lesions rapid ly decreased in size until they were no longer visible and the overlying retina appeared flat. On each attempted upgaze, both lesions progressively ex panded and became elevated. They detumesced when gaze was shifted elsewhere or digital pressure was applied to the globe. Ultrasonography disclosed a dome-shaped elevat ed mass of low internal acoustic reflectivity in the superonasal quadrant of each eye that progressively appeared during upgaze (Fig. 7). The lesions detu mesced and disappeared when gaze was directed
Fig. 4 (Osher and associates). B-scan ultrasonography demon strates expansion of thin-walled, dome-shaped lesion of low acoustic reflectivity during upgaze (at left). Varix promptly collapses when digi tal pressure is applied to globe or gaze is redirected (at right).
656
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1981
Fig. 5 (Osher and associates). Progressive expansion of superonasal vortex ampulla varix in right eye as fixation is shifted from the primary position (left) to upgaze (right).
elsewhere or digital pressure was applied to the globe (Fig. 7). The maximum height of either lesion was 2.6 mm. Cases 3 to 5—A single varix of the vortex ampulla has been identified in three additional middle-aged patients. The inferior fundus was the site of the varix in two eyes. The clinical appearance and dynamic behavior was identical to those lesions located in the superior quadrants, except that the gaze-evoked ex pansion occurred only in downgaze.
DISCUSSION
Knowledge of the characteristic behav ior displayed by the vortex ampulla varix should prevent misdiagnosis of this enti ty. The initial appearance of the lesion, detected most easily by indirect ophthalmoscopy, depends upon the direction
Fig. 6 (Osher and associates). Superonasal vortex ampulla in left eye demonstrates similar sequence in primary position (left) and upgaze (right).
VOL. 92, NO. 5
VARIX OF THE VORTEX AMPULLA
657
Fig. 7 (Osher and associates). B-scan ultrasonography demon strates expansion of thin-walled, dome-shaped lesion of low acoustic reflectivity during upgaze (at left). Varix promptly collapses when digi tal pressure is applied to globe or gaze is redirected (at right).
and duration of gaze at the time the vortex ampulla is observed. For example, when the lesion is located superiorly, examination of the ampulla in either early upgaze or during gaze directed else where may disclose only normal chorioretinal anatomy. As upgaze is sustained, a smoothly contoured, venous-colored (Case 1) or nonpigmented (Case 2) mass originates from the ampulla and may at tain a lateral size of 3 X 3 disk diameters and a height of 2 to 3 mm. As gaze is redirected the congestive state is re lieved and the mass detumesces. Expan sion of the lesion, by either a Valsalva maneuver or by positioning the head below heart level, can also be achieved while the primary position of gaze is maintained. The lesion rapidly empties and flattens when the head is elevated or digital pressure is applied against the globe. Jugular compression did not ex pand the lesion, but may produce a visi ble reflux of blood into the ampulla, which promptly drains when the neck is decompressed. In our first patient, upgaze was accom panied by a marked elevation of intraocu lar pressure in the involved eye. The
fellow eye had a stable intraocular pres sure when gaze was shifted between the primary position and upgaze. Possibly, contraction of the inferior oblique muscle during upgaze compressed the underly ing inferotemporal vortex vein, thereby placing an increased venous load upon the remaining three vortex veins. The consequent increase in intravenous vol ume increased venous pressure enough to expand the varix. Neither the com pressed inferotemporal vortex vein nor the congested superonasal varix could function effectively, leaving only the two remaining normal vortex veins for venous outflow. The choroidal blood volume in creased, and may thus have accounted for the increase in intraocular pressure. 1 Fluorescein angiography confirmed intravascular stasis and dilatation of the tributary vessels, in addition to demon strating that the origin of the lesion was the vortex ampulla. Ophthalmodynamometry (performed in our first patient) further supported a choroidal origin, be cause the lesion consistently collapsed below the observed diastole. Ultrasonography proved to be a reli able method of detecting the dynamic
658
AMERICAN JOURNAL OF OPHTHALMOLOGY
nature of this entity, and could provide a diagnosis in a patient with opaque media. Progressive expansion of the lesion dur ing a specific field of gaze was easily demonstrated, whereas detumescence was observed when either digital pres sure was applied to the globe or when gaze was shifted elsewhere. In order to determine the prevalence of this lesion, we examined 100 consecu tive patients undergoing routine eye evaluations. No similar lesion was identi fied. The pathogenesis of this lesion was challenging to establish. No significant predisposing systemic disease process contributing to venous sludging (such as congestive heart failure, superior vena cava syndrome, or polycythemia vera) was found. The involvement of the superonasal vortex ampulla in the first three eyes initially suggested that a specific local factor might be responsible for this phenomenon, but examination of other eyes disclosed that the lesion was present elsewhere. Extraocular muscle compression was considered as a possible cause of venous obstruction proximal to the vortex ampul la, because venous tamponade could occur during muscle contraction. This appears to be unlikely, however, because expansion of the lesion should not result from either passive head positioning or a Valsalva maneuver when the primary po sition of gaze is maintained. Further, the recti muscles are not in contact with the exit of the vortex veins, and only the oblique muscles can compress the two lateral veins (Fig. 8). 2 Nevertheless, we examined five patients with docu mented enlargement of extraocular mus cles secondary to severe thyroid ophthalmopathy as controls. We were unable to identify similar expanding lesions regard less of gaze direction or maneuvers per formed.
NOVEMBER, 1981
We also considered kinking of the vor tex vein as a possible cause of gazeevoked venous obstruction. For example, if the intrascleral portion of the superonasal vortex vein pursued an exaggerated oblique course posteriorly toward the optic nerve, extreme gaze could kink the vessel where it emerges from the globe (Fig. 9). Rutnin 3 has found a variable in trascleral length (3.5 to 6 mm) and course of the vortex vein. Upgaze could similarly kink the vortex vein if a posterior episcleral adhesion were present (Fig. 9). However, the expansion of the lesion by maneuvers performed while the eye is maintained in the primary position ar gues strongly against gaze-evoked kink ing. The most plausible explanation is that the lesion itself represents a varix of the vortex ampulla. All maneuvers (head depression, jugular vein compression, forced expiration) that are independent of gaze direction and increase intravenous pressure can result in variable distention of the varix. Detumescence can be achieved by either facilitating venous outflow (head elevation) or by superceding the venous pressure and directly col lapsing the varix (digital pressure). Local
Fig. 8 (Osher and associates). Globe viewed from retrobulbar space illustrating anatomic relationship between extraocular muscles and vortex veins as they emerge from their intrascleral canals.
VOL. 92, NO. 5
VARIX OF THE VORTEX AMPULLA
659
Fig. 9 (Osher and associates). Theoretic basis for vortex vein kinking during upgaze secondary to oblique intrascleral canal (top) and episcleral adhesion (bottom).
factors causing gaze-evoked distention of the varix remain unknown, although we have hypothesized that contraction of the oblique extraocular muscles may obstruct venous outflow in the underlying vortex
system. The remaining vortex veins must assume an additional venous flow, in creasing intravenous pressure and caus ing an expansion of the varix. Regardless of the actual pathogenesis,
660
AMERICAN JOURNAL OF OPHTHALMOLOGY
this dynamic tumefaction should be easily recognized by its distinct clinical behav ior and not confused with a malignancy. ACKNOWLEDGMENT
Randy Campo, M.D., and Mark Daily, M.D., referred Patients 3, 4, and 5.
Fifty years ago this month in
NOVEMBER, 1981
REFERENCES 1. Hayreh, S. S., and Baines, J. A. B.: Occlusion of the vortex veins. Br. J. Ophthalmol. 57:217, 1973. 2. Wolff, E.: Anatomy of the Eye and Orbit, 2nd ed. Philadelphia, Blakiston Co., 1940, p. 60. 3. Rutnin, U.: Fundus appearance in normal eyes. 1. The choroid. Am. J. Ophthalmol. 64:821, 1967.
THE
JOURNAL:
Like the dispute, now almost forgotten, concerning the relative merits of limbal extraction (as introduced by Daviel in the eight eenth century) and of the couching operation which had been used for thousands of years all over the world, the controversy as to the advantages and disadvantages of intracapsular as compared with extracapsular extraction of cataract may rage for a hundred years. It may even happen that the two methods will persist side by side for many centuries to come. W. H. Crisp: Intracapsular versus extracapsular Am. J. Ophthalmol. 14:1170, 1931