FIBRIN SEALANT IN THE TREATMENT OF RETINAL DETACHMENT IN MORNING GLORY SYNDROME Marc G. Saab, MD, Ghassan P. Cordahi, MD, Flavio A. Rezende, MD, PhD
Purpose To report the usage of fibrin sealant in the treatment of retinal detachment in morning glory syndrome. Methods: A case report of an 8-year-old boy with a recent, macula-off, inferior, bullous retinal detachment associated to his morning glory syndrome that was first treated by a pneumatic retinopexy. The following day, his examination revealed a total retinal detachment and subretinal gas. The patient underwent a 25-gauge pars plana vitrectomy, incomplete peeling of the glial tuft, 360° laser photocoagulation around the peripapillary staphyloma, 20% perfluoropropane endotamponade, and fibrin sealant injection to temporarily plug the slitlike retinal break visualized on the inferior border of the peripapillary staphyloma. Results: Complete reattachment of the retina persisted after 6 months of follow-up with slight improvement in visual acuity noted by the patient. Conclusion: The authors believe that the usage of fibrin sealant seems to be a useful alternative in cases of retinal detachment related to morning glory syndrome with deep posterior peripapillary staphyloma, especially when persistent residual traction is suspected. RETINAL CASES & BRIEF REPORTS 5:326–329, 2011
displacement to the temporal edge of the disk.3–6 Retinal detachments have been reported in one third of patients with morning glory syndrome. In most cases, a retinal tear is not found and the detachment is usually limited to the posterior pole, but some cases of extensive bullous retinal detachments have been described.7 We report a case of an 8-year-old boy with a recurrent bullous retinal detachment associated with morning glory syndrome that was successfully treated by a 25-gauge pars plana vitrectomy, gas endotamponade, and fibrin sealant (Tisseel; Baxter International, Inc).
From the Retina Service, Department of Ophthalmology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.
M
orning glory syndrome is a rare congenital ocular anomaly. It was first described in the German literature in 1929 by Handmann,1 but Kindler2 was the first to use the descriptive term of ‘‘Morning Glory’’ because of its resemblance to the morning glory flower. Usually unilateral, it is characterized by an enlarged optic disk, with a central whitish gray glial tuft, peripapillary annulus of hyperpigmentation or hypopigmentation, and anomalous straight vessels radiating from the edge of the disk. Axial retrodisplacement of the optic nerve and peripapillary funnel-shaped scleral staphyloma are frequently present. In addition, there is a variable foveal
Case Report An 8-year-old boy was followed at another institution since December 6, 2006, for a left exotropia since birth and a morning glory syndrome of the same eye. The patient had a nephrotic syndrome secondary to minimal change disease for which he had no particular treatment. The results of the cerebral magnetic resonance imaging were normal, and a genetic analysis for PAX mutation was negative. His best-corrected visual acuity was 20/20 on the right eye and counting fingers on 6 feet on the left eye, with a refractive error of +0.50 diopters and 21.00 diopters, respectively.
Presented at the Canadian Surgical Meeting, Lake Louise, Alberta, Canada, February 18, 2010. The authors have no proprietary interest in this report. Reprint requests: Marc G. Saab, MD, Retina Service, Department of Ophthalmology, Maisonneuve-Rosemont Hospital, 5415 boul. de l’Assomption, Montreal, Quebec, Canada H1T 2M4; e-mail: [email protected]
326
327
RETINAL DETACHMENT IN MORNING GLORY SYNDROME Pertinent findings were confined to the posterior segment with a normal fundus examination on the right eye and a characteristic morning glory syndrome on the left eye (Figure 1). His condition was stable until his last annual follow-up on August 13, 2009, when he had a decreased visual acuity to light perception and a ‘‘macula-off,’’ inferior bullous retinal detachment. The patient was taken to the operation room, and an examination under general anesthesia revealed no visible retinal tears. The decision was made to do a pneumatic retinopexy with 0.3 mL of C3F8 100%. Patient was asked to keep face down positioning. The following day, his examination revealed a total retinal detachment with subretinal gas bubbles. The patient was then referred to us, and he underwent a 25gauge pars plana vitrectomy. After a core vitrectomy, triamcinolone was used to better visualize the posterior attachments of the vitreoretinal interface over the peripapillary staphyloma. A complete posterior vitreous detachment was achieved despite strong adhesions. A tiny slitlike break was found within the staphyloma under a grayish white fibroglial tuft covering the central part of the optic disk and producing traction on the peripapillary retina. A 25-gauge end-gripping forceps was used to peel the fibroglial tuft that covered the slitlike retinal break at the inferior border of the peripapillary staphyloma. Because of the very posterior location of the tuft, we had a lot of difficulty in peeling all the traction because the length of the disposable 25-gauge end-gripping forceps did not allow us to reach the base of the deep peripapillary staphyloma. We then proceeded to a complete peripheral vitrectomy with trimming of the vitreous base. No peripheral retinal tears were visualized. An air–fluid exchange was performed, and subretinal fluid was drained through the slitlike posterior retinal break using a 25-gauge silicone soft tip cannula. After the retina was completely reattached, we applied 360° endolaser photocoagulation around the staphyloma and then injected enough Tisseel to cover the entire staphyloma to temporarily plug the retinal break and prevent subretinal gas migration in case of residual traction. C3F8 20% endotamponade was used. Postoperatively, the patient was kept on supine positioning for 1 day to give enough time for the Tisseel to adhere properly followed by prone position for 6 days. Six months after his vitrectomy, the retina was still reattached (Figures 2 and 3) and the patient improved his visual acuity to hand motions. We performed a time-domain optical coherence tomography (Stratus OCT; Carl Zeiss Meditec, Inc) that showed only the
Fig. 1. Morning glory syndrome with a large round disk, a central whitish glial tuft, abnormally radiating vessels, annulus of hyper- and hypopigmentation, and nasal dragging of the xanthophyll pigment.
Fig. 2. Completely reattached retina after 6 months of follow-up showing the 360° laser scars around the peripapillary staphyloma.
persistence of a fluid-filled cavity within the peripapillary staphyloma with the slitlike break still open (Figure 4).
Discussion The exact pathophysiology of retinal detachments in morning glory syndrome is controversial. Communications of the subretinal space with the vitreous cavity, subarachnoid space, and orbital tissues have all been proposed.4 Chang et al8 demonstrated a communication between the subarachnoid space and the subretinal space in a case of total retinal detachment associated to a morning glory syndrome, and retinal reattachment was achieved after an optic nerve fenestration. Harris et al9 demonstrated a combined tractional–rhegmatogenous mechanism in retinal detachment in morning glory syndrome, with the existence of a retinal break within the peripapillary staphyloma providing communication between the vitreous cavity and the subretinal space. And Irvine et al10 demonstrated an interconnection among the vitreous, subretinal, subarachnoid, and even orbital spaces because of the incomplete differentiation and
328
RETINAL CASES & BRIEF REPORTS´ 2011 VOLUME 5 NUMBER 4
Fig. 3. B-scan 6 months after the patient’s vitrectomy showing the completely attached retina and the axial posterior displacement of the optic disk.
porous nature of the herniated tissues in the morning glory syndrome. In our case, the passage of gas under the retina after a pneumatic retinopexy and face down positioning was a sign of communication between the vitreous cavity and the subretinal space and possibly the existence of a tractional component inside the staphyloma. During the surgery, we found a tiny slitlike break at the margin of the peripapillary staphyloma, and we insisted on
removal of the epi- and peripapillary tissues to release any existing traction. The fact that the retina was reattached and no gas bubble leaked under the retina after C3F8 tamponade confirmed our impression of a combined tractional and rhegmatogenous pathophysiology. We chose not to use either perfluorocarbon liquid or silicone oil intraoperatively to avoid subretinal migration.4–7 The persistence of fluid-filled cavity inside the staphyloma despite all our treatment could be explained by the persistence of residual traction or by the possibility of a communication between the cerebrospinal fluid and the subretinal space.8 Tisseel is a commercial fibrin sealant that reproduces the last stages of the natural clotting cascade. The sealant forms by mixing fibrinogen and thrombin at the time of delivery. Thrombin, a highly specific protease, converts the fibrinogen into fibrin monomers. These fibrin monomers are then polymerized in a linear fashion and stabilized by cross-linking (catalyzed by Factor XIII) to form an insoluble fibrin clot. Aprotinin, which is a plasmin inhibitor, is included in the formulation to slow the natural process of in vivo fibrinolysis.11 The glue is applied with two identical disposable syringes that are joined by a common plunger to assure that the two components are delivered in equal volumes. During delivery, the solutions travel through a common joining piece and meet through a blunt-end cannula to form a gellike material. To the best of our knowledge, there have been no cases in the literature of intravitreal injection of Tisseel accompanying gas endotamponade in the treatment of retinal detachment associated with morning glory syndrome. Although Tisseel seems to be a safe and useful adjuvant in cases of complicated retinal detachments associated with morning glory syndrome and peripapillary staphyloma, further larger series are needed to better understand its role in these cases. Key words: retinal detachment, morning glory syndrome, peripapillary staphyloma, pars plana vitrectomy, Tisseel.
References
Fig. 4. Optical coherence tomography 6 months after the patient’s vitrectomy showing the persistence of a fluid-filled cavity with the slitlike open break (arrow).
1. Handmann M. Hereditary, probably innate, central gliotic degeneration of the optic nerve with special participation of the central vessels. Klin Monatsbl Augenheilkd 1929;83:145. 2. Kindler P. Morning glory syndrome: unusual congenital optic disc anomaly. Am J Ophthalmol 1970;69:376–384. 3. Harasymowycz P, Chevrette L, De´carie JC, et al. Morning glory syndrome: clinical, computerized tomographic, and ultrasonographic findings. J Pediatr Ophthalmol Strabismus 2005;42:290–295.
RETINAL DETACHMENT IN MORNING GLORY SYNDROME 4. Bartz-Scmidt K, Heimann K. Pathogenesis of retinal detachment associated with morning glory disc. Int Ophthalmol 1995;19:35–38. 5. Ho CL, Wei LC. Rhegmatogenous retinal detachment in morning glory syndrome pathogenesis and treatment. Int Ophthalmol 2001;24:21–24. 6. Manschot WA. Morning glory syndrome: a histopathological study. Br J Ophthalmol 1990;74:56–58. 7. Coll GE, Chang S, Flynn TE, Brown GC. Communication between the subretinal space and the vitreous cavity in the morning glory syndrome. Graefes Arch Clin Exp Ophthalmol 1995;233:441–443.
329 8. Chang S, Haik BG, Ellsworth RM, St Louis L, Berrocal JA. Treatment of total retinal detachment in morning glory syndrome. Am J Ophthalmol 1984;97:596–600. 9. Harris MJ, de Bustros S, Michels RG, Joondeph HC. Treatment of combined traction-rhegmatogenous retinal detachment in the morning glory syndrome. Retina 1984;4:249–252. 10. Irvine AR, Crawford JB, Sullivan JH. The pathogenesis of retinal detachment with morning glory disc and optic pit. Trans Am Ophthalmol Soc 1986;84:280–292. 11. Margalit E, Fujii GY, Lai JC, et al. Bioadhesives for intraocular use. Retina 2000;20:469–477.
Purpose To report the usage of fibrin sealant in the treatment of retinal detachment in morning glory syndrome. Methods: A case report of an 8-year-old boy with a recent, macula-off, inferior, bullous retinal detachment associated to his morning glory syndrome that was first treated by a pneumatic retinopexy. The following day, his examination revealed a total retinal detachment and subretinal gas. The patient underwent a 25-gauge pars plana vitrectomy, incomplete peeling of the glial tuft, 360° laser photocoagulation around the peripapillary staphyloma, 20% perfluoropropane endotamponade, and fibrin sealant injection to temporarily plug the slitlike retinal break visualized on the inferior border of the peripapillary staphyloma. Results: Complete reattachment of the retina persisted after 6 months of follow-up with slight improvement in visual acuity noted by the patient. Conclusion: The authors believe that the usage of fibrin sealant seems to be a useful alternative in cases of retinal detachment related to morning glory syndrome with deep posterior peripapillary staphyloma, especially when persistent residual traction is suspected. RETINAL CASES & BRIEF REPORTS 5:326–329, 2011
displacement to the temporal edge of the disk.3–6 Retinal detachments have been reported in one third of patients with morning glory syndrome. In most cases, a retinal tear is not found and the detachment is usually limited to the posterior pole, but some cases of extensive bullous retinal detachments have been described.7 We report a case of an 8-year-old boy with a recurrent bullous retinal detachment associated with morning glory syndrome that was successfully treated by a 25-gauge pars plana vitrectomy, gas endotamponade, and fibrin sealant (Tisseel; Baxter International, Inc).
From the Retina Service, Department of Ophthalmology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.
M
orning glory syndrome is a rare congenital ocular anomaly. It was first described in the German literature in 1929 by Handmann,1 but Kindler2 was the first to use the descriptive term of ‘‘Morning Glory’’ because of its resemblance to the morning glory flower. Usually unilateral, it is characterized by an enlarged optic disk, with a central whitish gray glial tuft, peripapillary annulus of hyperpigmentation or hypopigmentation, and anomalous straight vessels radiating from the edge of the disk. Axial retrodisplacement of the optic nerve and peripapillary funnel-shaped scleral staphyloma are frequently present. In addition, there is a variable foveal
Case Report An 8-year-old boy was followed at another institution since December 6, 2006, for a left exotropia since birth and a morning glory syndrome of the same eye. The patient had a nephrotic syndrome secondary to minimal change disease for which he had no particular treatment. The results of the cerebral magnetic resonance imaging were normal, and a genetic analysis for PAX mutation was negative. His best-corrected visual acuity was 20/20 on the right eye and counting fingers on 6 feet on the left eye, with a refractive error of +0.50 diopters and 21.00 diopters, respectively.
Presented at the Canadian Surgical Meeting, Lake Louise, Alberta, Canada, February 18, 2010. The authors have no proprietary interest in this report. Reprint requests: Marc G. Saab, MD, Retina Service, Department of Ophthalmology, Maisonneuve-Rosemont Hospital, 5415 boul. de l’Assomption, Montreal, Quebec, Canada H1T 2M4; e-mail: [email protected]
326
327
RETINAL DETACHMENT IN MORNING GLORY SYNDROME Pertinent findings were confined to the posterior segment with a normal fundus examination on the right eye and a characteristic morning glory syndrome on the left eye (Figure 1). His condition was stable until his last annual follow-up on August 13, 2009, when he had a decreased visual acuity to light perception and a ‘‘macula-off,’’ inferior bullous retinal detachment. The patient was taken to the operation room, and an examination under general anesthesia revealed no visible retinal tears. The decision was made to do a pneumatic retinopexy with 0.3 mL of C3F8 100%. Patient was asked to keep face down positioning. The following day, his examination revealed a total retinal detachment with subretinal gas bubbles. The patient was then referred to us, and he underwent a 25gauge pars plana vitrectomy. After a core vitrectomy, triamcinolone was used to better visualize the posterior attachments of the vitreoretinal interface over the peripapillary staphyloma. A complete posterior vitreous detachment was achieved despite strong adhesions. A tiny slitlike break was found within the staphyloma under a grayish white fibroglial tuft covering the central part of the optic disk and producing traction on the peripapillary retina. A 25-gauge end-gripping forceps was used to peel the fibroglial tuft that covered the slitlike retinal break at the inferior border of the peripapillary staphyloma. Because of the very posterior location of the tuft, we had a lot of difficulty in peeling all the traction because the length of the disposable 25-gauge end-gripping forceps did not allow us to reach the base of the deep peripapillary staphyloma. We then proceeded to a complete peripheral vitrectomy with trimming of the vitreous base. No peripheral retinal tears were visualized. An air–fluid exchange was performed, and subretinal fluid was drained through the slitlike posterior retinal break using a 25-gauge silicone soft tip cannula. After the retina was completely reattached, we applied 360° endolaser photocoagulation around the staphyloma and then injected enough Tisseel to cover the entire staphyloma to temporarily plug the retinal break and prevent subretinal gas migration in case of residual traction. C3F8 20% endotamponade was used. Postoperatively, the patient was kept on supine positioning for 1 day to give enough time for the Tisseel to adhere properly followed by prone position for 6 days. Six months after his vitrectomy, the retina was still reattached (Figures 2 and 3) and the patient improved his visual acuity to hand motions. We performed a time-domain optical coherence tomography (Stratus OCT; Carl Zeiss Meditec, Inc) that showed only the
Fig. 1. Morning glory syndrome with a large round disk, a central whitish glial tuft, abnormally radiating vessels, annulus of hyper- and hypopigmentation, and nasal dragging of the xanthophyll pigment.
Fig. 2. Completely reattached retina after 6 months of follow-up showing the 360° laser scars around the peripapillary staphyloma.
persistence of a fluid-filled cavity within the peripapillary staphyloma with the slitlike break still open (Figure 4).
Discussion The exact pathophysiology of retinal detachments in morning glory syndrome is controversial. Communications of the subretinal space with the vitreous cavity, subarachnoid space, and orbital tissues have all been proposed.4 Chang et al8 demonstrated a communication between the subarachnoid space and the subretinal space in a case of total retinal detachment associated to a morning glory syndrome, and retinal reattachment was achieved after an optic nerve fenestration. Harris et al9 demonstrated a combined tractional–rhegmatogenous mechanism in retinal detachment in morning glory syndrome, with the existence of a retinal break within the peripapillary staphyloma providing communication between the vitreous cavity and the subretinal space. And Irvine et al10 demonstrated an interconnection among the vitreous, subretinal, subarachnoid, and even orbital spaces because of the incomplete differentiation and
328
RETINAL CASES & BRIEF REPORTS´ 2011 VOLUME 5 NUMBER 4
Fig. 3. B-scan 6 months after the patient’s vitrectomy showing the completely attached retina and the axial posterior displacement of the optic disk.
porous nature of the herniated tissues in the morning glory syndrome. In our case, the passage of gas under the retina after a pneumatic retinopexy and face down positioning was a sign of communication between the vitreous cavity and the subretinal space and possibly the existence of a tractional component inside the staphyloma. During the surgery, we found a tiny slitlike break at the margin of the peripapillary staphyloma, and we insisted on
removal of the epi- and peripapillary tissues to release any existing traction. The fact that the retina was reattached and no gas bubble leaked under the retina after C3F8 tamponade confirmed our impression of a combined tractional and rhegmatogenous pathophysiology. We chose not to use either perfluorocarbon liquid or silicone oil intraoperatively to avoid subretinal migration.4–7 The persistence of fluid-filled cavity inside the staphyloma despite all our treatment could be explained by the persistence of residual traction or by the possibility of a communication between the cerebrospinal fluid and the subretinal space.8 Tisseel is a commercial fibrin sealant that reproduces the last stages of the natural clotting cascade. The sealant forms by mixing fibrinogen and thrombin at the time of delivery. Thrombin, a highly specific protease, converts the fibrinogen into fibrin monomers. These fibrin monomers are then polymerized in a linear fashion and stabilized by cross-linking (catalyzed by Factor XIII) to form an insoluble fibrin clot. Aprotinin, which is a plasmin inhibitor, is included in the formulation to slow the natural process of in vivo fibrinolysis.11 The glue is applied with two identical disposable syringes that are joined by a common plunger to assure that the two components are delivered in equal volumes. During delivery, the solutions travel through a common joining piece and meet through a blunt-end cannula to form a gellike material. To the best of our knowledge, there have been no cases in the literature of intravitreal injection of Tisseel accompanying gas endotamponade in the treatment of retinal detachment associated with morning glory syndrome. Although Tisseel seems to be a safe and useful adjuvant in cases of complicated retinal detachments associated with morning glory syndrome and peripapillary staphyloma, further larger series are needed to better understand its role in these cases. Key words: retinal detachment, morning glory syndrome, peripapillary staphyloma, pars plana vitrectomy, Tisseel.
References
Fig. 4. Optical coherence tomography 6 months after the patient’s vitrectomy showing the persistence of a fluid-filled cavity with the slitlike open break (arrow).
1. Handmann M. Hereditary, probably innate, central gliotic degeneration of the optic nerve with special participation of the central vessels. Klin Monatsbl Augenheilkd 1929;83:145. 2. Kindler P. Morning glory syndrome: unusual congenital optic disc anomaly. Am J Ophthalmol 1970;69:376–384. 3. Harasymowycz P, Chevrette L, De´carie JC, et al. Morning glory syndrome: clinical, computerized tomographic, and ultrasonographic findings. J Pediatr Ophthalmol Strabismus 2005;42:290–295.
RETINAL DETACHMENT IN MORNING GLORY SYNDROME 4. Bartz-Scmidt K, Heimann K. Pathogenesis of retinal detachment associated with morning glory disc. Int Ophthalmol 1995;19:35–38. 5. Ho CL, Wei LC. Rhegmatogenous retinal detachment in morning glory syndrome pathogenesis and treatment. Int Ophthalmol 2001;24:21–24. 6. Manschot WA. Morning glory syndrome: a histopathological study. Br J Ophthalmol 1990;74:56–58. 7. Coll GE, Chang S, Flynn TE, Brown GC. Communication between the subretinal space and the vitreous cavity in the morning glory syndrome. Graefes Arch Clin Exp Ophthalmol 1995;233:441–443.
329 8. Chang S, Haik BG, Ellsworth RM, St Louis L, Berrocal JA. Treatment of total retinal detachment in morning glory syndrome. Am J Ophthalmol 1984;97:596–600. 9. Harris MJ, de Bustros S, Michels RG, Joondeph HC. Treatment of combined traction-rhegmatogenous retinal detachment in the morning glory syndrome. Retina 1984;4:249–252. 10. Irvine AR, Crawford JB, Sullivan JH. The pathogenesis of retinal detachment with morning glory disc and optic pit. Trans Am Ophthalmol Soc 1986;84:280–292. 11. Margalit E, Fujii GY, Lai JC, et al. Bioadhesives for intraocular use. Retina 2000;20:469–477.
