- Email: [email protected]
Bilateral Peripapillary Staphyloma with Normal Vision
BILATERAL PERIPAPILLARY STAPHYLOMA
WITH NORMAL
VISION
J O H N B . H . CALDWELL, M . D . , M A R V I N L . SEARS, M . D . , A N D M I C H A E L G I L M A N , M . D .
New Hove», Connecticut Peripapillary staphyloma, or peripapillary scleral ectasia, has only infrequently been reported in the literature and remains somewhat of an ophthalmologic curiosity. DukeElder summarizes sporadic cases reported around the beginning of the twentieth century, mostly in the German literature ; in the English literature a case was reported as late as 1925. These examples were invariably unilateral, with markedly reduced vision in the affected eye, the fellow eye being normal. In 1966, Wise, MacLean, and Gass reported a further case which showed unilateral poor vision associated with such a peripapillary defect. In addition this was found to pulsate. That report also reviewed in more detail the previously reported cases referred to by Duke-Elder, indicated the likely embryology involved in such a defect, and proposed an explanation for the pulsatile nature of the staphyloma in their patient. Recently, Sugar and Beckman added a case in which the retina, separated from the outer coats of the staphyloma, appeared to move consequent to the respiratory cycle. François, Goes, and performed elaborate biométrie Yobbágyi studies on a patient with a unilateral defect and poor vision. 1
2
3
4
8
The purpose of this report is to tell about the occurrence of a bilateral staphyloma without disturbance of visual acuity. C A S E REPORT
A 15-year-old Caucasian girl came to the office for a checkup without any visual complaint. The patient was first seen at the age of nine, referred because she rated poorly on the visual acuity examination at school. In 1962 she had a normal exFrom the Section of Ophthalmology, Yale University School of Medicine, New Haven, Connecticut This work was supported in part by USPHS Grant EY 00002 and the Connecticut Lions Eye Research Foundation, Inc, Drs. Caldwell and Gilman were formerly USPHS clinical fellows. Reprint requests to Marvin L. Sears, M.D., Professor and Chairman, Section of Ophthalmology, Yale University School of Medicine, 333 Cedar Street, New Haven, Connecticut 06510.
ternal examination. Best corrected visual acuity, with a small astigmatic error, was RE : 20/30, LE : 20/25. Biomicroscopy was normal. Ophthalmoscopy was described as suggesting deep optic disks with broad pigmented choroidal streaks surrounding them. Because of the slightly suboptimal acuity and this unusual finding, the patient was advised to return at yearly intervals for re-examination. On a subsequent visit two years later, visual acuity was in the same range with a small astigmatic error ( R E : +0.50 X 90, L E : +0.50 X 180, at retinoscopy). It was remarked in the record, however, that the disks could be seen clearly only with the direct ophthalmoscope set at —8.00 D. The appearance of the disk and peripapillary region was unchanged from the previous examinations. It was felt that perhaps a posterior staphyloma was forming, though the retinoscopy did not suggest myopia The parents and two siblings were examined and found not to have a similar abnormality. At the recent office visit the findings were as follows: Externally, the eyelids, orbits, lacrimal apparatus were within normal limits. The extraocular muscles functioned normally. The pupils were round, equal at 7 mm in a dim room, and reacted briskly to light. Vision was RE: 20/70, LE: 20/30, uncorrected. Retinoscopy findings were RE: —0.50 sph. Z -2.00 cyl. ax. 90 20/25, L E : -0.50 sph. C —1.75 cyl. ax. 90 20/25. Near vision, with correction, was 20/20 in both eyes. Biomicroscopy showed normal anterior segments in both eyes. Tension was normal to touch in both eyes. Visual fields were full and normal to l/w/330 in both eyes, with slightly enlarged blind spots, the right being greater than left In both eyes, central color fields were normal to red and blue, and response to all Dvorine color plates was normal. With the direct ophthalmoscope, again it was remarkable that —8.00 D was required to focus clearly on the disk in each eye, while the surrounding retina was out of focus. With the indirect instrument, each normal-appearing disk lay at the bottom of a symmetrical, saucer-shaped depression at the posterior pole, not involving the macula. There was a ring of irregular choroidal atrophy at the peripheral edge of the depression, more prominent in the right eye than in the left eye. The retina was not separated in this area. Neither pulsation nor movement with respiration was noted. The macula and peripheral retina seemed normal. The patient now had five siblings, all of whom were examined and none was found to have a similar abnormality. The mother was re-examined; she had normal disks and peripapillary regions. Photographs were taken of this patient's defects (Fig. 1 ) . In addition, sodium fluorescein 5% was injected intravenously while rapid sequence photographs were taken through a blue filter with a Zeiss fundus camera. The dye was found to leak abnormally from the edges of the optic disk in both eyes 423
424
AMERICAN JOURNAL OF OPHTHALMOLOGY
JANUARY, 1971
Fig. 1 (Caldwell, Sears, and Gilman). Left: The right eye has a ring of mottled-appearing choroid in the depressed peripapillary area, with marked atrophy at the 9 o'clock position over the edge of the defect. Right: The left eye demonstrates a change in color at the edge of the defect, though the choroidal changes are less pronounced than the right eye. Arrows indicate edges of the staphyloma.
and in addition from the edges of the areas of pronounced choroidal atrophy. This occurred rapidly in the choroidal arterial phase and remained for some time (at least 90 minutes) (Fig. 2 ) . DISCUSSION
This patient is of special interest because of the bilaterality of the defect and the essentially normal vision. Without exception, as far as we can determine, all previous cases reported had only one eye affected by the peripapillary defect, and invariably this was associated with quite poor visual acuity, though one patient had as good as 6 / 1 8 in the affected eye. There was a small anisometropia of — 1 . 0 0 D cylinder. Another patient showed a refractive difference of only 0 . 5 0 6
8
D with 6 / 2 0 0 in the abnormal eye. Young's
2
patient was emmetropic with best vision in the affected eye of 6 / 3 6 . On the other hand, the patient reported by Sugar and Beckman* showed a small hyperopic astigmatism in the eye with normal vision while the defective eye was esotropic and 7.25 D myopic with corrected vision of 1 0 / 4 0 0 . Stock and von
Szily did not mention the refraction of their 7
Fig. 2 (Caldwell, Sears, and Gilman). Fluorescein photo of right eye during retinal venous phase, emphasizing the boundary of the disk, with a ring of dye leaking from the choroid at peripheral edge of staphyloma, and a marked concentration at 9 o'clock corresponding to atrophic area noted in Fig. 1, left.
VOL. 71, NO. 1
PERIPAPILLARY STAPHYLOMA
patient's normal eye, while only a small convex cylinder somewhat improved acuity in the affected eye. Kayser mentioned only that the unaffected eye of his patient refracted at —1.25 D sph. Since our patient has such good acuity, we wondered if the amblyopia in unilateral cases might have been caused or aggravated by coincident anisometropia, but the evidence for this is inconclusive. 8
The appearance of the abnormality in our case seems much the same as those previously described. However, the drawings in the reports of Hancock and of Young show indication of much more choroidal atrophy, with bare sclera ringing the papilla. The fluorescein study simply confirms the impression of marked choroidopathy in the affected area and gives no clue to the etiologyW e can add nothing to the previous reports concerning the etiology and development of such defects, which appear clearly to be congenital. So far there is no evidence to suggest any hereditary factor. In light of our patient's normal visual acuity, it may be that the developmental anomaly is present more frequently than documented. There may be affected individuals who, having no visual complaint, do not come to our attention, or, having normal vision, do not receive complete ophthalmologic evaluation. 6
2
425 SUMMARY
An example of bilateral peripapillary staphyloma associated with probably normal vision was studied with fluorescein. This is a rare developmental defect, heretofore reported as unilateral and associated with decidedly subnormal visual acuity in the affected eye. Since the case reported shows probably normal visual acuity, it may be that the entity is more frequently present than now imagined, but, like so many rare anomalies, escapes us unless looked for. REFERENCES
1. Duke-Elder, S. : System of Ophthalmology, vol. 3. St. Louis, Mosby, 1963, part 2, p. 541. 2. Young, G. : Peripapillary ectasia. Tr. Ophth. Soc. U. K. 45:267, 1925. 3. Wise, J. B , MacLean, A. L , and Gass, J. D. M.: Contractile peripapillary staphyloma. Arch. Ophth. 75:626, 1966. 4. Sugar, H. S., and Beckman, H. : Peripapillary staphyloma with respiratory pulsation. Am. J. Ophth. 68:895, 1969. 5. François, J, Goes, F , and Yobbágyi, P. : Etude biométrique d'une ectasie sclérale péripapillaire congénitale (staphylome postérieur vrai). Ophthalmologica. 154:446, 1967. 6. Hancock, I.: Peripapillary ectasia with inclusion of the optic nerve. Tr. Ophth. Soc. U. K. 27 : 167, 1907. 7. Stock, W , and von Szily, A.: Eine Noch Nicht Beschriebene Kongenitale Anomalie des Augenhintergrundes. Klin. Mbl. Augenheilk. 44:48, 1906. 8. Kayser, B.: Ueber einem Fall von Tiefer Ektasie des Fundus am Sehnerveneintritt. Klin. Mbl. Augenheilk. 45:76, 1907.
WITH NORMAL
VISION
J O H N B . H . CALDWELL, M . D . , M A R V I N L . SEARS, M . D . , A N D M I C H A E L G I L M A N , M . D .
New Hove», Connecticut Peripapillary staphyloma, or peripapillary scleral ectasia, has only infrequently been reported in the literature and remains somewhat of an ophthalmologic curiosity. DukeElder summarizes sporadic cases reported around the beginning of the twentieth century, mostly in the German literature ; in the English literature a case was reported as late as 1925. These examples were invariably unilateral, with markedly reduced vision in the affected eye, the fellow eye being normal. In 1966, Wise, MacLean, and Gass reported a further case which showed unilateral poor vision associated with such a peripapillary defect. In addition this was found to pulsate. That report also reviewed in more detail the previously reported cases referred to by Duke-Elder, indicated the likely embryology involved in such a defect, and proposed an explanation for the pulsatile nature of the staphyloma in their patient. Recently, Sugar and Beckman added a case in which the retina, separated from the outer coats of the staphyloma, appeared to move consequent to the respiratory cycle. François, Goes, and performed elaborate biométrie Yobbágyi studies on a patient with a unilateral defect and poor vision. 1
2
3
4
8
The purpose of this report is to tell about the occurrence of a bilateral staphyloma without disturbance of visual acuity. C A S E REPORT
A 15-year-old Caucasian girl came to the office for a checkup without any visual complaint. The patient was first seen at the age of nine, referred because she rated poorly on the visual acuity examination at school. In 1962 she had a normal exFrom the Section of Ophthalmology, Yale University School of Medicine, New Haven, Connecticut This work was supported in part by USPHS Grant EY 00002 and the Connecticut Lions Eye Research Foundation, Inc, Drs. Caldwell and Gilman were formerly USPHS clinical fellows. Reprint requests to Marvin L. Sears, M.D., Professor and Chairman, Section of Ophthalmology, Yale University School of Medicine, 333 Cedar Street, New Haven, Connecticut 06510.
ternal examination. Best corrected visual acuity, with a small astigmatic error, was RE : 20/30, LE : 20/25. Biomicroscopy was normal. Ophthalmoscopy was described as suggesting deep optic disks with broad pigmented choroidal streaks surrounding them. Because of the slightly suboptimal acuity and this unusual finding, the patient was advised to return at yearly intervals for re-examination. On a subsequent visit two years later, visual acuity was in the same range with a small astigmatic error ( R E : +0.50 X 90, L E : +0.50 X 180, at retinoscopy). It was remarked in the record, however, that the disks could be seen clearly only with the direct ophthalmoscope set at —8.00 D. The appearance of the disk and peripapillary region was unchanged from the previous examinations. It was felt that perhaps a posterior staphyloma was forming, though the retinoscopy did not suggest myopia The parents and two siblings were examined and found not to have a similar abnormality. At the recent office visit the findings were as follows: Externally, the eyelids, orbits, lacrimal apparatus were within normal limits. The extraocular muscles functioned normally. The pupils were round, equal at 7 mm in a dim room, and reacted briskly to light. Vision was RE: 20/70, LE: 20/30, uncorrected. Retinoscopy findings were RE: —0.50 sph. Z -2.00 cyl. ax. 90 20/25, L E : -0.50 sph. C —1.75 cyl. ax. 90 20/25. Near vision, with correction, was 20/20 in both eyes. Biomicroscopy showed normal anterior segments in both eyes. Tension was normal to touch in both eyes. Visual fields were full and normal to l/w/330 in both eyes, with slightly enlarged blind spots, the right being greater than left In both eyes, central color fields were normal to red and blue, and response to all Dvorine color plates was normal. With the direct ophthalmoscope, again it was remarkable that —8.00 D was required to focus clearly on the disk in each eye, while the surrounding retina was out of focus. With the indirect instrument, each normal-appearing disk lay at the bottom of a symmetrical, saucer-shaped depression at the posterior pole, not involving the macula. There was a ring of irregular choroidal atrophy at the peripheral edge of the depression, more prominent in the right eye than in the left eye. The retina was not separated in this area. Neither pulsation nor movement with respiration was noted. The macula and peripheral retina seemed normal. The patient now had five siblings, all of whom were examined and none was found to have a similar abnormality. The mother was re-examined; she had normal disks and peripapillary regions. Photographs were taken of this patient's defects (Fig. 1 ) . In addition, sodium fluorescein 5% was injected intravenously while rapid sequence photographs were taken through a blue filter with a Zeiss fundus camera. The dye was found to leak abnormally from the edges of the optic disk in both eyes 423
424
AMERICAN JOURNAL OF OPHTHALMOLOGY
JANUARY, 1971
Fig. 1 (Caldwell, Sears, and Gilman). Left: The right eye has a ring of mottled-appearing choroid in the depressed peripapillary area, with marked atrophy at the 9 o'clock position over the edge of the defect. Right: The left eye demonstrates a change in color at the edge of the defect, though the choroidal changes are less pronounced than the right eye. Arrows indicate edges of the staphyloma.
and in addition from the edges of the areas of pronounced choroidal atrophy. This occurred rapidly in the choroidal arterial phase and remained for some time (at least 90 minutes) (Fig. 2 ) . DISCUSSION
This patient is of special interest because of the bilaterality of the defect and the essentially normal vision. Without exception, as far as we can determine, all previous cases reported had only one eye affected by the peripapillary defect, and invariably this was associated with quite poor visual acuity, though one patient had as good as 6 / 1 8 in the affected eye. There was a small anisometropia of — 1 . 0 0 D cylinder. Another patient showed a refractive difference of only 0 . 5 0 6
8
D with 6 / 2 0 0 in the abnormal eye. Young's
2
patient was emmetropic with best vision in the affected eye of 6 / 3 6 . On the other hand, the patient reported by Sugar and Beckman* showed a small hyperopic astigmatism in the eye with normal vision while the defective eye was esotropic and 7.25 D myopic with corrected vision of 1 0 / 4 0 0 . Stock and von
Szily did not mention the refraction of their 7
Fig. 2 (Caldwell, Sears, and Gilman). Fluorescein photo of right eye during retinal venous phase, emphasizing the boundary of the disk, with a ring of dye leaking from the choroid at peripheral edge of staphyloma, and a marked concentration at 9 o'clock corresponding to atrophic area noted in Fig. 1, left.
VOL. 71, NO. 1
PERIPAPILLARY STAPHYLOMA
patient's normal eye, while only a small convex cylinder somewhat improved acuity in the affected eye. Kayser mentioned only that the unaffected eye of his patient refracted at —1.25 D sph. Since our patient has such good acuity, we wondered if the amblyopia in unilateral cases might have been caused or aggravated by coincident anisometropia, but the evidence for this is inconclusive. 8
The appearance of the abnormality in our case seems much the same as those previously described. However, the drawings in the reports of Hancock and of Young show indication of much more choroidal atrophy, with bare sclera ringing the papilla. The fluorescein study simply confirms the impression of marked choroidopathy in the affected area and gives no clue to the etiologyW e can add nothing to the previous reports concerning the etiology and development of such defects, which appear clearly to be congenital. So far there is no evidence to suggest any hereditary factor. In light of our patient's normal visual acuity, it may be that the developmental anomaly is present more frequently than documented. There may be affected individuals who, having no visual complaint, do not come to our attention, or, having normal vision, do not receive complete ophthalmologic evaluation. 6
2
425 SUMMARY
An example of bilateral peripapillary staphyloma associated with probably normal vision was studied with fluorescein. This is a rare developmental defect, heretofore reported as unilateral and associated with decidedly subnormal visual acuity in the affected eye. Since the case reported shows probably normal visual acuity, it may be that the entity is more frequently present than now imagined, but, like so many rare anomalies, escapes us unless looked for. REFERENCES
1. Duke-Elder, S. : System of Ophthalmology, vol. 3. St. Louis, Mosby, 1963, part 2, p. 541. 2. Young, G. : Peripapillary ectasia. Tr. Ophth. Soc. U. K. 45:267, 1925. 3. Wise, J. B , MacLean, A. L , and Gass, J. D. M.: Contractile peripapillary staphyloma. Arch. Ophth. 75:626, 1966. 4. Sugar, H. S., and Beckman, H. : Peripapillary staphyloma with respiratory pulsation. Am. J. Ophth. 68:895, 1969. 5. François, J, Goes, F , and Yobbágyi, P. : Etude biométrique d'une ectasie sclérale péripapillaire congénitale (staphylome postérieur vrai). Ophthalmologica. 154:446, 1967. 6. Hancock, I.: Peripapillary ectasia with inclusion of the optic nerve. Tr. Ophth. Soc. U. K. 27 : 167, 1907. 7. Stock, W , and von Szily, A.: Eine Noch Nicht Beschriebene Kongenitale Anomalie des Augenhintergrundes. Klin. Mbl. Augenheilk. 44:48, 1906. 8. Kayser, B.: Ueber einem Fall von Tiefer Ektasie des Fundus am Sehnerveneintritt. Klin. Mbl. Augenheilk. 45:76, 1907.