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Treatment of Total Retinal Detachment in Morning Glory Syndrome
TREATMENT OF TOTAL RETINAL DETACHMENT IN MORNING GLORY SYNDROME STANLEY CHANG, M.D., BARRETT G. HAIK, M.D., ROBERT M. ELLSWORTH, M.D., AND LESLIE ST. LOUIS, M.D. New York, New York
AND JOSE A. BERROCAL, M.D. San Juan, Puerto Rico
A I4-year-old girl had headaches and sudden loss of vision as a result of total retinal detachment in association with the morning glory optic nerve anomaly. Metrizamide eisternography with contrast dye introduced into the subarachnoid space disclosed migration of metrizamide with radiographic enhancement in the subretinal space. The demonstration of an abnormal communication between the subarachnoid and subretinal spaces suggested that the subretinal fluid is of cranial origin. The patient underwent surgical removal of a window of dura from the optic nerve sheath. This resulted in retinal reattachment and improvement of visual acuity to 20/200. The retina was still attached after a I5-month follow-up period. The morning glory syndrome is an unusual congenital anomaly of the optic nerve characterized by an enlarged optic disk with deep excavation of the optic cup resembling the flower after which the syndrome is named.l" The optic disk tissue frequently appears elevated. The retinal vessels appear to originate from deep within the optic disk excavations, travel along the peripheral optic disk tissue, and exit radially. Glial tissue may obscure or fill the anomalous optic cup. The optic disk may also appear to be elevated and there may be associated periretinal trac-
Accepted for publication Feb. 22, 1984. From the Departments of Ophthalmology (Drs. Chang, Haik, and Ellsworth) and Radiology (Dr. St. Louis), Cornell University Medical College/New YorkHospital, New York, New York, and the Department of Ophthalmology, University of Puerto Rico, San Juan, Puerto Rico (Dr. Berrocal). Reprint requests to Stanley Chang, M.D., Department of Ophthalmology, New York Hospital, 525 E. 68th St., New York, NY 10021. 596
tion folds. Alterations in the retinal pigment epithelium surrounding the optic disk are common. Visual acuity is generally poor and the condition may be bilateral. In unilateral cases, congenital anomalies such as optic disk coloboma, microphthalmos, and the anterior cleavage syndrome may be present in the contralateral eye."! Systemic abnormalities associated with this anomaly include encephalocele, cleft lip and palate, agenesis of the corpus callosum, and renal anomalies. 6,7 Commonly associated fundus findings are retinal vascular abnormalities and macular ectopia with displacement of the macula toward the optic disk. 4,8 Retinal detachment is less common and has been described as nonrhegmatogenous with subretinal fluid not extending to the ora serrata. 1,5,9,10 In a group of ten patients with this pattern of retinal detachment Haik and associates'? found that four had spontaneous resolution of the subretinal
©AMERICAN JOURNAL OF OPHTHALMOLOGY 97:596-600, 1984
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fluid and reattachment of the retina. In one patient with known morning glory syndrome without retinal detachment a totally organized fixed retinal detachment developed several years later. The cause of the retinal detachment in this syndrome is uncertain. We recently treated one patient with retinal detachment and bilateral morning glory syndrome. These findings may provide insight into the mechanisms for and treatment of retinal detachment in this syndrome. CASE REPORT The patient, a 14-year-old girl, was examined because of a retinal detachment in her left eye. She had had poor vision and nystagmus since birth. There was no history of prematurity. The right eye had been smaller since birth and vision was better in her left eye. Seven months earlier, the patient had developed headaches above both eyes and sudden loss of vision in her left eye. Four months later she was found to have a total retinal detachment with telangiectasis in the left eye. Her distance visual acuity was R. E.: 5/200 and L. E.: hand movements. Sensory nystagmus and a left exotropia of 30 prism diopters (Hirschberg) were present. The right eye and cornea appeared to be somewhat smaller than the left. Each pupil measured 3 mm and was reactive with the left pupil exhibiting an afferent pupillary defect. Slit-lamp findings were
597
unremarkable. Ophthalmoscopy of the right eye disclosed a morning glory appearance to the optic disk. The retina appeared to be atrophic and there were large radial folds extending from the optic disk temporally. These were associated with some peripapillary intraretinal or subretinal hard yellow exudates. The pigment epithelium was diffusely atrophic with widespread areas of pigment clumping suggestive of previous retinal detachment. Ophthalmoscopy of the left eye disclosed a total bullous retinal detachment with early funnel formation obscuring good visualization of the optic disk .(Fig. 1). Fixed folds were present temporally; these were associated with retinal telangiectasis in the temporal periphery. The retina appeared to be atrophic with mild preretinal fibrosis and subretinal strand formation. No retinal breaks were found and the subretinal fluid did not shift. Ultrasonography showed the axial length of the right eye to be approximately 18 mm. There was a localized elevation of the retina extending temporally from the optic disk. The choroid in this area was also acoustically dense, suggesting calcification. The optic nerve pattern demonstrated sonolucent areas in the region of the optic disk and parabulbar region, suggesting an orbital cyst or coloboma formation. The axial length of the left eye was 22 mm. The retina was totally detached. The optic nerve pattern disclosed deep excavation in the region of the optic nerve with irregularity of the optic nerve pattern in the parabulbar area. Computed tomography demonstrated a colobomatous area in the region of the optic nerve head in both eyes (Fig. 2). There was increased radiodensity in the region of the right optic disk, suggesting optic nerve drusen or calcification (Fig. 2). Computed tomography after metrizamide
Fig. 1 (Chang and associates). Left, Drawing of the left eye shows total retinal detachment with telangiectasis temporally. Right. The fundus photograph shows an abnormal optic disk that is almost obscured by the retinal detachment.
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Fig. 2 (Chang and associates). Left, Computed axial tomogram with contrast demonstrates a colobomatous area in the region of the optic nerve head in both eyes. Right, Increased radiodensity suggests optic nerve drusen or calcification in the right eye.
contrast dye was injected into the subarachnoid space via a lumbar puncture in the IA-L5 space showed that the metrizamide diffused around both optic nerves. After the metrizamide injection a radiographic enhancement of the subretinal space in the left eye appeared (Fig. 3) but there was no radiographic enhancement in the vitreous space, suggesting that the sub retinal fluid was in communication with the cerebrospinal fluid. Because of these findings, the optic nerve sheath was surgically opened by a medial approach described by Galbraith and Sullivan. 11 The medial rectus muscle was disinserted to expose the optic nerve sheath. Clear fluid gushed out after a window of dura was removed from the nerve sheath. On the first postoperative day, the retina had flattened dramatically with complete flattening superonasally and opening of the funnel. Postoperatively, visual acuity improved to 5/200. A shallow retinal detachment persisted in the temporal and inferonasal quadrants (Fig. 4). Several observers were unable to find a retinal break and persistent vitreous traction was considered. Seven weeks post-
Fig. 3 (Chang and associates). Computed axial tomogram after metrizamide injection shows contrast enhancement of the left optic nerve sheath and subretinal space.
operatively, subretinal fluid was drained at the equator in the 3:30 o'clock meridian. The subretinal fluid was yellow and viscous. The retina flattened completely and visual acuity improved to 20/200 (Fig. 5). The retina was still completely flat at the most recent follow-up examination 15 months later. DISCUSSION
Retinal detachment is an intriguing finding in association with the morning glory optic disk anomaly. 9,10 Of 32 patients described by Haik and associates," 11 had retinal detachments. Ten of these detachments were nonrhegmatogenous;
Fig. 4. (Chang and associates). Fundus drawing of retinal detachment before external drainage of subretinal fluid. No retinal breaks were found.
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Fig. 5 (Chang and associates). Two 45-degree fundus photographs taken two months after drainage of subretinal fluid. The optic cup is covered by glial tissue (left). The retina is entirely flat. There is no evidence of the macula (right).
they usually appeared as an accumulation of subretinal fluid originating around the optic disk. When the fluid extends to the ora serrata, a retinal break usually cannot be found. Spontaneous resolution of the retinal detachment has been observed in some cases. In other cases there have been pigmentary changes in the retinal pigment epithelium suggesting previous retinal detachment. These clinical observations suggest that the accumulation of subretinal fluid is related to the defect in the optic disk. Possible sources of the fluid include liquid vitreous passing through the optic cup and into the subretinal space, leakage of the peripapillary retinal vascular abnormalities into the subretinal space, or a communicating defect from the subarachnoid space allowing cerebrospinal fluid to enter the subretinal space. The findings in this case strongly suggested a communicating defect between the subarachnoid and subretinal spaces. Because the intraocular pressure normally exceeds the cerebrospinal pressure, we hypothesize that either progressive vitreoretinal traction or peripapillary traction from fibroglial proliferation over the
optic disk initiated the development of subretinal fluid. Metrizamide cisternography demonstrated that contrast dye could enter the subretinal space from the subarachnoid spaces of the optic nerve sheath. Creating a window in the dural sheath of the optic nerve probably fused the dural sheath to the optic nerve, thereby interrupting the pathway by which the cerebrospinal fluid progressed anteriorly. The residual subretinal fluid that required a secondary drainage procedure can probably be explained by residual vitreous traction or by the presence of viscous subretinal fluid. The cerebrospinal fluid normally has a higher protein content than the subretinal fluid found in recent rhegmatogenous detachments. 12 The protein content of subretinal fluid increases as the duration of retinal detachment increases, 13 resulting in delayed retinal reattachment. Histopathologic observations in the few reported cases supported our hypothesis concerning this case. 14-16 Connective tissue in the region of the optic cup is associated with redundant peripapillary retinal folds that may contribute to traction retinal detachment. Also, the his to-
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pathologic finding of a large scleral canal with defective fusion of the sclera to the optic nerve and its sheath, reported in all cases, could result in a communicating pathway from the subarachnoid space to the subretinal space in some patients. The surgical intervention in our case was justified because the only eye with useful vision had been involved for at least four months. It is probably advisable to observe patients in whom the opposite eye is normal because in many cases the retinal detachment resolves spontaneously. Additionally, the visual potential is usually lower in, the involved eye than in the normal eye. In theory, an alternative therapy may be considered. External drainage of subretinal fluid and peripapillary krypton laser photocoagulation after the flattening of the retina might be possible in cases of extensive retinal detachment. However, optic nerve sheath surgery appears to be the best method of interrupting the flow of cerebrospinal fluid, particularly in cases in which the subretinal fluid is restricted to the posterior pole. This procedure also preserves the most retinal function in an already compromised retina. REFERENCES 1. Kindler, P.: Morning glory syndrome. Unusual congenital optic disk anomaly. Am. J. Ophthalmol. 69:376, 1970. 2. Krause, U.: Three cases of the morning glory syndrome. Acta OphthalmoL 50:188, 1972.
MAY, 1984
3. Steinkuller, P. G.: The morning glory disc anomaly. Case report and literature review. J. Pediatr. Ophthalmol. Strabismus 17:81, 1980. 4. Beyer, W. B., Quencer, R. M., and Osher, R. H.: Morning glory syndrome. A functional analysis including fluorescein angiography, ultrasonography, and computerized tomography. Ophthalmology 89:1362, 1982. 5. Brown, G., and Tasman, W.: Congenital Anomalies of the Optic Disc. New York, Grune· and Stratton, 1983, p. 157. 6. Koening, S. B., Naidich, T. P., and Lissner, G.: The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology 89:1368, 1982. 7. Caprioli, J., and Lesser, R. L.: Basal encephalocele and morning glory syndrome. Br. J. Ophthalmol. 67:349, 1983. 8. Yamana, T., Nishimura, M., Ueda, K., and Chijiiwa, T.: Macular involvement in morning glory syndrome. Jpn. J. OphthalmoL 27:201, 1983. 9. Hamada, S., and Ellsworth, R. M.: Congenital retinal detachment and the optic disk anomaly. Am. J. Ophthalmol. 71:460, 1971. 10. Haik, B. G., Greenstein, S. G., Smith, M. E., Abramson, D. H., and Ellsworth, R. M.: Retinal detachment in the morning glory syndrome. Ophthalmology 9O(suppL):76, 1983. 11. Galbraith, J. E. K., and Sullivan, J. H.: Decompression of the perioptic meninges for the relief of papilledema. Am. J. Ophthalmol. 76:687, 1973. 12. Chignall, A. H., Carruthers, M., and Rahi, A. H. S.: Clinical, biochemical and immunoelectrophoretic study of subretinal fluid. Br. J. Ophthalmol. 555:525, 1971. 13. Sweeney, D. B., Balazs, E. A., and Karlin, D. B.: Biochemistry of retinal fluid. In Schepens, C. L., and Regan, C. Di (eds.): Controversial Aspects of the Management of Retinal Detachment. Boston, Little Brown, 1962, p. 315. 14. Pedler, C.: Unusual coloboma of the optic nerve entrance. Br. J. Ophthalmol. 45:803, 1961. 15. Rack, J. H., Wright, G. F.: Coloboma of the optic nerve entrance. Br. J. Ophthalmol. 50:705, 1966. 16. Cogan, D. G.: Coloboma of optic nerve with overlay of peripapillary retina. Br. J. Ophthalmol. 62:347, 1978. .
AND JOSE A. BERROCAL, M.D. San Juan, Puerto Rico
A I4-year-old girl had headaches and sudden loss of vision as a result of total retinal detachment in association with the morning glory optic nerve anomaly. Metrizamide eisternography with contrast dye introduced into the subarachnoid space disclosed migration of metrizamide with radiographic enhancement in the subretinal space. The demonstration of an abnormal communication between the subarachnoid and subretinal spaces suggested that the subretinal fluid is of cranial origin. The patient underwent surgical removal of a window of dura from the optic nerve sheath. This resulted in retinal reattachment and improvement of visual acuity to 20/200. The retina was still attached after a I5-month follow-up period. The morning glory syndrome is an unusual congenital anomaly of the optic nerve characterized by an enlarged optic disk with deep excavation of the optic cup resembling the flower after which the syndrome is named.l" The optic disk tissue frequently appears elevated. The retinal vessels appear to originate from deep within the optic disk excavations, travel along the peripheral optic disk tissue, and exit radially. Glial tissue may obscure or fill the anomalous optic cup. The optic disk may also appear to be elevated and there may be associated periretinal trac-
Accepted for publication Feb. 22, 1984. From the Departments of Ophthalmology (Drs. Chang, Haik, and Ellsworth) and Radiology (Dr. St. Louis), Cornell University Medical College/New YorkHospital, New York, New York, and the Department of Ophthalmology, University of Puerto Rico, San Juan, Puerto Rico (Dr. Berrocal). Reprint requests to Stanley Chang, M.D., Department of Ophthalmology, New York Hospital, 525 E. 68th St., New York, NY 10021. 596
tion folds. Alterations in the retinal pigment epithelium surrounding the optic disk are common. Visual acuity is generally poor and the condition may be bilateral. In unilateral cases, congenital anomalies such as optic disk coloboma, microphthalmos, and the anterior cleavage syndrome may be present in the contralateral eye."! Systemic abnormalities associated with this anomaly include encephalocele, cleft lip and palate, agenesis of the corpus callosum, and renal anomalies. 6,7 Commonly associated fundus findings are retinal vascular abnormalities and macular ectopia with displacement of the macula toward the optic disk. 4,8 Retinal detachment is less common and has been described as nonrhegmatogenous with subretinal fluid not extending to the ora serrata. 1,5,9,10 In a group of ten patients with this pattern of retinal detachment Haik and associates'? found that four had spontaneous resolution of the subretinal
©AMERICAN JOURNAL OF OPHTHALMOLOGY 97:596-600, 1984
VOL. 97, NO.5
MORNING GLORY SYNDROME
fluid and reattachment of the retina. In one patient with known morning glory syndrome without retinal detachment a totally organized fixed retinal detachment developed several years later. The cause of the retinal detachment in this syndrome is uncertain. We recently treated one patient with retinal detachment and bilateral morning glory syndrome. These findings may provide insight into the mechanisms for and treatment of retinal detachment in this syndrome. CASE REPORT The patient, a 14-year-old girl, was examined because of a retinal detachment in her left eye. She had had poor vision and nystagmus since birth. There was no history of prematurity. The right eye had been smaller since birth and vision was better in her left eye. Seven months earlier, the patient had developed headaches above both eyes and sudden loss of vision in her left eye. Four months later she was found to have a total retinal detachment with telangiectasis in the left eye. Her distance visual acuity was R. E.: 5/200 and L. E.: hand movements. Sensory nystagmus and a left exotropia of 30 prism diopters (Hirschberg) were present. The right eye and cornea appeared to be somewhat smaller than the left. Each pupil measured 3 mm and was reactive with the left pupil exhibiting an afferent pupillary defect. Slit-lamp findings were
597
unremarkable. Ophthalmoscopy of the right eye disclosed a morning glory appearance to the optic disk. The retina appeared to be atrophic and there were large radial folds extending from the optic disk temporally. These were associated with some peripapillary intraretinal or subretinal hard yellow exudates. The pigment epithelium was diffusely atrophic with widespread areas of pigment clumping suggestive of previous retinal detachment. Ophthalmoscopy of the left eye disclosed a total bullous retinal detachment with early funnel formation obscuring good visualization of the optic disk .(Fig. 1). Fixed folds were present temporally; these were associated with retinal telangiectasis in the temporal periphery. The retina appeared to be atrophic with mild preretinal fibrosis and subretinal strand formation. No retinal breaks were found and the subretinal fluid did not shift. Ultrasonography showed the axial length of the right eye to be approximately 18 mm. There was a localized elevation of the retina extending temporally from the optic disk. The choroid in this area was also acoustically dense, suggesting calcification. The optic nerve pattern demonstrated sonolucent areas in the region of the optic disk and parabulbar region, suggesting an orbital cyst or coloboma formation. The axial length of the left eye was 22 mm. The retina was totally detached. The optic nerve pattern disclosed deep excavation in the region of the optic nerve with irregularity of the optic nerve pattern in the parabulbar area. Computed tomography demonstrated a colobomatous area in the region of the optic nerve head in both eyes (Fig. 2). There was increased radiodensity in the region of the right optic disk, suggesting optic nerve drusen or calcification (Fig. 2). Computed tomography after metrizamide
Fig. 1 (Chang and associates). Left, Drawing of the left eye shows total retinal detachment with telangiectasis temporally. Right. The fundus photograph shows an abnormal optic disk that is almost obscured by the retinal detachment.
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Fig. 2 (Chang and associates). Left, Computed axial tomogram with contrast demonstrates a colobomatous area in the region of the optic nerve head in both eyes. Right, Increased radiodensity suggests optic nerve drusen or calcification in the right eye.
contrast dye was injected into the subarachnoid space via a lumbar puncture in the IA-L5 space showed that the metrizamide diffused around both optic nerves. After the metrizamide injection a radiographic enhancement of the subretinal space in the left eye appeared (Fig. 3) but there was no radiographic enhancement in the vitreous space, suggesting that the sub retinal fluid was in communication with the cerebrospinal fluid. Because of these findings, the optic nerve sheath was surgically opened by a medial approach described by Galbraith and Sullivan. 11 The medial rectus muscle was disinserted to expose the optic nerve sheath. Clear fluid gushed out after a window of dura was removed from the nerve sheath. On the first postoperative day, the retina had flattened dramatically with complete flattening superonasally and opening of the funnel. Postoperatively, visual acuity improved to 5/200. A shallow retinal detachment persisted in the temporal and inferonasal quadrants (Fig. 4). Several observers were unable to find a retinal break and persistent vitreous traction was considered. Seven weeks post-
Fig. 3 (Chang and associates). Computed axial tomogram after metrizamide injection shows contrast enhancement of the left optic nerve sheath and subretinal space.
operatively, subretinal fluid was drained at the equator in the 3:30 o'clock meridian. The subretinal fluid was yellow and viscous. The retina flattened completely and visual acuity improved to 20/200 (Fig. 5). The retina was still completely flat at the most recent follow-up examination 15 months later. DISCUSSION
Retinal detachment is an intriguing finding in association with the morning glory optic disk anomaly. 9,10 Of 32 patients described by Haik and associates," 11 had retinal detachments. Ten of these detachments were nonrhegmatogenous;
Fig. 4. (Chang and associates). Fundus drawing of retinal detachment before external drainage of subretinal fluid. No retinal breaks were found.
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Fig. 5 (Chang and associates). Two 45-degree fundus photographs taken two months after drainage of subretinal fluid. The optic cup is covered by glial tissue (left). The retina is entirely flat. There is no evidence of the macula (right).
they usually appeared as an accumulation of subretinal fluid originating around the optic disk. When the fluid extends to the ora serrata, a retinal break usually cannot be found. Spontaneous resolution of the retinal detachment has been observed in some cases. In other cases there have been pigmentary changes in the retinal pigment epithelium suggesting previous retinal detachment. These clinical observations suggest that the accumulation of subretinal fluid is related to the defect in the optic disk. Possible sources of the fluid include liquid vitreous passing through the optic cup and into the subretinal space, leakage of the peripapillary retinal vascular abnormalities into the subretinal space, or a communicating defect from the subarachnoid space allowing cerebrospinal fluid to enter the subretinal space. The findings in this case strongly suggested a communicating defect between the subarachnoid and subretinal spaces. Because the intraocular pressure normally exceeds the cerebrospinal pressure, we hypothesize that either progressive vitreoretinal traction or peripapillary traction from fibroglial proliferation over the
optic disk initiated the development of subretinal fluid. Metrizamide cisternography demonstrated that contrast dye could enter the subretinal space from the subarachnoid spaces of the optic nerve sheath. Creating a window in the dural sheath of the optic nerve probably fused the dural sheath to the optic nerve, thereby interrupting the pathway by which the cerebrospinal fluid progressed anteriorly. The residual subretinal fluid that required a secondary drainage procedure can probably be explained by residual vitreous traction or by the presence of viscous subretinal fluid. The cerebrospinal fluid normally has a higher protein content than the subretinal fluid found in recent rhegmatogenous detachments. 12 The protein content of subretinal fluid increases as the duration of retinal detachment increases, 13 resulting in delayed retinal reattachment. Histopathologic observations in the few reported cases supported our hypothesis concerning this case. 14-16 Connective tissue in the region of the optic cup is associated with redundant peripapillary retinal folds that may contribute to traction retinal detachment. Also, the his to-
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pathologic finding of a large scleral canal with defective fusion of the sclera to the optic nerve and its sheath, reported in all cases, could result in a communicating pathway from the subarachnoid space to the subretinal space in some patients. The surgical intervention in our case was justified because the only eye with useful vision had been involved for at least four months. It is probably advisable to observe patients in whom the opposite eye is normal because in many cases the retinal detachment resolves spontaneously. Additionally, the visual potential is usually lower in, the involved eye than in the normal eye. In theory, an alternative therapy may be considered. External drainage of subretinal fluid and peripapillary krypton laser photocoagulation after the flattening of the retina might be possible in cases of extensive retinal detachment. However, optic nerve sheath surgery appears to be the best method of interrupting the flow of cerebrospinal fluid, particularly in cases in which the subretinal fluid is restricted to the posterior pole. This procedure also preserves the most retinal function in an already compromised retina. REFERENCES 1. Kindler, P.: Morning glory syndrome. Unusual congenital optic disk anomaly. Am. J. Ophthalmol. 69:376, 1970. 2. Krause, U.: Three cases of the morning glory syndrome. Acta OphthalmoL 50:188, 1972.
MAY, 1984
3. Steinkuller, P. G.: The morning glory disc anomaly. Case report and literature review. J. Pediatr. Ophthalmol. Strabismus 17:81, 1980. 4. Beyer, W. B., Quencer, R. M., and Osher, R. H.: Morning glory syndrome. A functional analysis including fluorescein angiography, ultrasonography, and computerized tomography. Ophthalmology 89:1362, 1982. 5. Brown, G., and Tasman, W.: Congenital Anomalies of the Optic Disc. New York, Grune· and Stratton, 1983, p. 157. 6. Koening, S. B., Naidich, T. P., and Lissner, G.: The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology 89:1368, 1982. 7. Caprioli, J., and Lesser, R. L.: Basal encephalocele and morning glory syndrome. Br. J. Ophthalmol. 67:349, 1983. 8. Yamana, T., Nishimura, M., Ueda, K., and Chijiiwa, T.: Macular involvement in morning glory syndrome. Jpn. J. OphthalmoL 27:201, 1983. 9. Hamada, S., and Ellsworth, R. M.: Congenital retinal detachment and the optic disk anomaly. Am. J. Ophthalmol. 71:460, 1971. 10. Haik, B. G., Greenstein, S. G., Smith, M. E., Abramson, D. H., and Ellsworth, R. M.: Retinal detachment in the morning glory syndrome. Ophthalmology 9O(suppL):76, 1983. 11. Galbraith, J. E. K., and Sullivan, J. H.: Decompression of the perioptic meninges for the relief of papilledema. Am. J. Ophthalmol. 76:687, 1973. 12. Chignall, A. H., Carruthers, M., and Rahi, A. H. S.: Clinical, biochemical and immunoelectrophoretic study of subretinal fluid. Br. J. Ophthalmol. 555:525, 1971. 13. Sweeney, D. B., Balazs, E. A., and Karlin, D. B.: Biochemistry of retinal fluid. In Schepens, C. L., and Regan, C. Di (eds.): Controversial Aspects of the Management of Retinal Detachment. Boston, Little Brown, 1962, p. 315. 14. Pedler, C.: Unusual coloboma of the optic nerve entrance. Br. J. Ophthalmol. 45:803, 1961. 15. Rack, J. H., Wright, G. F.: Coloboma of the optic nerve entrance. Br. J. Ophthalmol. 50:705, 1966. 16. Cogan, D. G.: Coloboma of optic nerve with overlay of peripapillary retina. Br. J. Ophthalmol. 62:347, 1978. .