Citation Information :
Junttila TL, Alberto N, Winkels M, Greenwood MD. Successful Reduction of Intraocular Pressure in a Patient with Glaucoma Secondary to Sturge–Weber Syndrome Using a Suprachoroidal Shunt. J Curr Glaucoma Pract 2020; 14 (1):43-46.
Aim: We present a case of successful reduction of intraocular pressure (IOP) in a patient with Sturge–Weber syndrome (SWS) and moderate open angle glaucoma using a suprachoroidal shunt.
Background: Patients with SWS glaucoma often have elevated episcleral venous pressure resulting in elevated IOP. This makes the conventional pathway for aqueous humor outflow a poor target for IOP reduction, yielding it difficult to treat. Medication and procedures that facilitate uveoscleral outflow have been more successful.
Case description: We present a case where a suprachoroidal shunt device (CyPass®) was used to reduce IOP in a patient with SWS. The IOP reduction has persisted for 12 months without complication or the requirement for topical prostaglandin analog use.
Conclusion: The uveoscleral pathway is a preferred target for IOP reduction in patients with elevated episcleral venous pressure. The CyPass device offers a promising ab interno minimally invasive glaucoma surgery (MIGS) approach to reduce IOP in cases where a pathologic pressure gradient exists in the trabecular meshwork.
Clinical significance: Glaucoma in patients with SWS has been historically difficult to treat. Previous surgical procedures that have been successful are more invasive and have required the creation of alternative drainage routes of aqueous humor. The CyPass device offers a promising less invasive option to reduce IOP in these patients.
Kanada KN, Merin MR, Munden A, et al. A prospective study of cutaneous findings in newborns in the United States: correlation with race, ethnicity, and gestational status using updated classification and nomenclature. J Pediatr 2012;161(2):240–245. DOI: 10.1016/j.jpeds.2012.02.052.
Hidano A, Purwoko R, Jitsukawa K. Statistical survey of skin changes in Japanese neonates. Pediatr Dermatol 1986;3(2):140–144. DOI: 10.1111/j.1525-1470.1986.tb00505.x.
Ch'ng S, Tan ST. Facial port-wine stains - clinical stratification and risks of neuro-ocular involvement. J Plast Reconstr Aesthet Surg 2008;61(8):889–893. DOI: 10.1016/j.bjps.2007.05.011.
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-weber syndrome: a review. Pediatr Neurol 2004;30(5):303–310. DOI: 10.1016/j.pediatrneurol.2003.12.015.
Mantelli F, Bruscolini A, La Cava M, et al. Ocular manifestations of Sturge-Weber syndrome: pathogenesis, diagnosis, and management. Clin Ophthalmol 2016;10:871–878. DOI: 10.2147/OPTH.S101963.
Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004;23(2):87–98. DOI: 10.1016/j.sder.2004.01.002.
Paller AS. The Sturge-Weber syndrome. Pediatr Dermatol 1987;4(4):300–304. DOI: 10.1111/j.1525-1470.1987.tb00797.x.
Javaid U, Ali MH, Jamal S, et al. Pathophysiology, diagnosis, and management of glaucoma associated with Sturge-Weber syndrome. Int Ophthalmol 2018;38(1):409–416. DOI: 10.1007/s10792-016-0412-3.
Shiau T, Armogan N, Yan DB, et al. The role of episcleral venous pressure in glaucoma associated with Sturge-Weber syndrome. J AAPOS 2012;16(1):61–64. DOI: 10.1016/j.jaapos.2011.09.014.
Digiuni M, Fogagnolo P, Rossetti L. A review of the use of latanoprost for glaucoma since its launch. Expert Opin Pharmacother 2012;13(5):723–745. DOI: 10.1517/14656566.2012.662219.
Abdolrahimzadeh S, Scavella V, Felli L, et al. Ophthalmic alterations in the Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and the phakomatosis pigmentovascularis: an independent group of conditions? Biomed Res Int 2015;2015:786519. DOI: 10.1155/2015/786519.
Amini H, Razeghinejad MR, Esfandiarpour B. Primary single-plate molteno tube implantation for management of glaucoma in children with Sturge-Weber syndrome. Int Ophthalmol 2007;27(6):345–350. DOI: 10.1007/s10792-007-9091-4.
Overby DR, Stamer WD, Johnson M. The changing paradigm of outflow resistance generation: towards synergistic models of the JCT and inner wall endothelium. Exp Eye Res 2009;88(4):656–670. DOI: 10.1016/j.exer.2008.11.033.
Maepea O, Bill A. Pressures in the juxtacanalicular tissue and Schlemm's canal in monkeys. Exp Eye Res 1992;54(6):879–883. DOI: 10.1016/0014-4835(92)90151-h.
Bill A, Svedbergh B. Scanning electron microscopic studies of the trabecular meshwork and the canal of Schlemm–an attempt to localize the main resistance to outflow of aqueous humor in man. Acta Ophthalmol (Copenh) 1972;50(3):295–320. DOI: 10.1111/j.1755-3768.1972.tb05954.x.
Poley BJ, Lindstrom RL, Samuelson TW. Long-term effects of phacoemulsification with intraocular lens implantation in normotensive and ocular hypertensive eyes. J Catar Refract Surg 2008;34(5):735–742. DOI: 10.1016/j.jcrs.2007.12.045.
Vold S, Ahmed II, Craven ER, et al. Two-year COMPASS trial results: supraciliary microstenting with phacoemulsification in patients with open-angle glaucoma and cataracts. Ophthalmology 2016;123(10):2103–2112. DOI: 10.1016/j.ophtha.2016.06.032.
Friedman DS, Jampel HD, Lubomski LH, et al. Surgical strategies for coexisting glaucoma and cataract: an evidence-based update. Ophthalmology 2002;109(10):1902–1913. DOI: 10.1016/s0161-6420(02)01267-8.