|Year : 2021 | Volume
| Issue : 2 | Page : 198-199
One stage non-valved glaucoma drainage device in refractory glaucoma under cover of oral propranolol for presumed choroidal hemangioma in Sturge-Weber syndrome
Vanita Pathak-Ray1, Supriya Chinta2
1 Department of Glaucoma, Centre for Sight, Banjara Hills, Hyderabad, Telangana, India
2 Department of Glaucoma, Aravind Eye Hospital, Hyderabad, Telangana, India
|Date of Submission||04-Jun-2020|
|Date of Acceptance||07-Oct-2020|
|Date of Web Publication||01-Apr-2021|
Dr. Vanita Pathak-Ray
Centre for Sight, Road No 2, Banjara Hills, Hyderabad - 500 034, Telangana
Source of Support: None, Conflict of Interest: None
Keywords: Aurolab aqueous drainage implant, childhood glaucoma, choroidal detachment, choroidal hemangioma, non-valved glaucoma drainage device, Sturge-Weber syndrome
|How to cite this article:|
Pathak-Ray V, Chinta S. One stage non-valved glaucoma drainage device in refractory glaucoma under cover of oral propranolol for presumed choroidal hemangioma in Sturge-Weber syndrome. Indian J Ophthalmol Case Rep 2021;1:198-9
|How to cite this URL:|
Pathak-Ray V, Chinta S. One stage non-valved glaucoma drainage device in refractory glaucoma under cover of oral propranolol for presumed choroidal hemangioma in Sturge-Weber syndrome. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 May 12];1:198-9. Available from: https://www.ijoreports.in/text.asp?2021/1/2/198/312342
Bilateral Sturge- Weber syndrome More Details More Details (SWS) is rare but glaucoma associated with it is common. Diffuse choroidal hemangioma (CH) may be associated with SWS and remain asymptomatic throughout childhood. Its presence, however, poses several sight-threatening challenges in the surgical management of glaucoma.,
| Case Report|| |
Our patient was diagnosed with bilateral SWS [Figure 1]a and glaucoma at 5 years of age. On fundoscopy [Figure 1]b and [Figure 1]c, retinal nerve fiber layer (RNFL) changes were seen mostly inferiorly in OS with multiple drusenoid deposits [Figure 1]c; notably, there was a subtle difference in color [Figure 1]b and [Figure 1]c. CH was excluded by optical coherence tomography (OCT). She had a history of trabeculectomy + mitomycin C in OS at 7 years and massive sight-threatening choroidal detachment (CD) involving the posterior-pole in all quadrants necessitating oral steroids for 3 weeks, in the immediate postoperative period. One-year later, intraocular pressure (IOP) remained uncontrolled in OS. The non-valved glaucoma drainage device (GDD), Aurolab Aqueous Drainage Implant (AADI) was implanted under cover of perioperative propranolol (2 mg/kg) with three occlusive ligatures [[Figure 2] left] along with three pairs of fenestrations anterior to it [[Figure 2] left and right].
|Figure 1: (a). Bilateral Port-wine stain in Sturge-Weber Syndrome. (b and c). Fundus photo right eye and left eye, showing inferior retinal nerve fiber layer loss (left much more than right) and multiple drusenoid deposits with a subtle difference in color on the left side|
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|Figure 2: Intraoperative photograph of Aurolab Aqueous Drainage Implant with three temporary occlusive ligatures with polyglactin – fenestrating vents being made anterior to the three occlusive ligatures (left, white arrow) and drainage seen through these vents (right, white arrows)|
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Postoperatively there was no hypotony or shallow anterior chamber (AC). Stepwise ligature autolysis ensured that there was no precipitous drop in IOP at around 5–6 weeks. At 1-year follow-up, the vision was 20/30 in OS with controlled IOP (13 mmHg) without medications; fellow-eye is controlled with medications.
| Discussion|| |
When CH is present, a sudden decompression of the globe or prolonged hypotony may lead to rapid transudation of fluid into the extravascular space causing choroidal and retinal effusions. Oral propranolol is hypothesized to stabilize the vascular membrane and was, therefore, used under careful systemic watch, commencing 1-week prior, and continued for 6-weeks postoperatively. CH is best diagnosed with an OCT or B-scan; even though OCT was equivocal, diffuse CH in the LE was suspected clinically—the subtle difference in the fundus color, presence of drusen-like structures, and the history of CD post initial filtration—all heightened that suspicion.
Standard treatment guidelines are not available in SWS but the use of valved GDD is emerging as the preferred option; despite flow restriction, they are not immune to a CD. Budenz et al. did use the non-valved device Baerveldt Glaucoma Implant (BGI) but only as a two-stage procedure, along with prophylactic posterior sclerotomies. We used indigenously manufactured AADI with safety and efficacy comparable to BGI (not available in India), and at a fraction of the cost of the valved device available.
The preemptive measures, in this case, made non-valved surgery safe and the need for a second stage and/or prophylactic posterior sclerotomy was obviated. It also highlights that index for clinical suspicion of CH should be high.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Formisano M, Abdolrahimzadeh B, Mollo R, Bruni P, Malagola R, Abdolrahimzadeh S. Bilateral diffuse choroidal hemangioma in Sturge Weber syndrome: A case report highlighting the role of multimodal imaging and a brief review of the literature. J Curr Ophthalmol 2018;31:242-9.
Mantelli F, Bruscolini A, La Cava M, Abdolrahimzadeh S, Lambiase A. Ocular manifestations of Sturge-Weber syndrome: Pathogenesis, diagnosis, and management. Clin Ophthalmol 2016;10:871-8.
Kaushik S, Kataria P, Joshi G, Singh R, Handa S, Pandav SS, et al
. Perioperative propranolol a useful adjunct for glaucoma surgery in Sturge-Weber syndrome. Ophthalmol Glaucoma 2019;2:267-74.
Abdolrahimzadeh S, Parisi F, Mantelli F, Perdicchi A, Scuderi G. Retinal pigment epithelium-photoreceptor layer alterations in a patient with Sturge-Weber syndrome with diffuse choroidal hemangioma. Ophthalmic Genet 2017;38:567-9.
Budenz DL, Sakamoto D, Eliezer R, Varma R, Heuer DK. Two-staged Baerveldt glaucoma implant for childhood glaucoma associated with Sturge-Weber syndrome. Ophthalmology 2000;107:2105-10.
Pathak Ray V, Rao DP. Surgical outcomes of a new affordable non-valved glaucoma drainage device and Ahmed glaucoma valve: Comparison in the first year. Br J Ophthalmol 2019;103659-65.
[Figure 1], [Figure 2]