• Users Online: 152
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 526-528

Massive serous macular detachment after an uneventful combined trabeculotomy with trabeculectomy in two cases of Sturge-Weber syndrome resolved with oral propranolol

VST Glaucoma Center, L V Prasad Eye Institute, Banjara Hills, Hyderabad, Telangana, India

Date of Submission20-Sep-2020
Date of Acceptance29-Jan-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Sirisha Senthil
L.V Prasad Eye Institute, Kallam Anji Reddy Campus, L V Prasad Marg, Road No: 2, Banjara Hills, Hyderabad, Telangana - 500 034
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3022_20

Rights and Permissions

Managing glaucoma in Sturge-Weber syndrome (SWS) is refractory needing surgery, which is often associated with serious complications. We report massive serous macular detachment in two patients with SWS following uneventful combined trabeculotomy with trabeculectomy (CTT). Two children with SWS and choroidal haemangioma underwent CTT with all pre-, peri- and postoperative precautions. Few days postoperatively, a large serous macular detachment was noted with a significant drop in vision. They were treated with oral propranolol (2 mg/Kg bodyweight with pediatricians' monitoring for 6-weeks) which helped in complete resolution of subretinal fluid in 1-month with good improvement in visual acuity and well-controlled IOP.

Keywords: Choroidal haemangioma, combined trabeculotomy and trabeculectomy, propranolol, serous macular detachment, Sturge-Weber syndrome (SWS)

How to cite this article:
Gajbhiye MP, Senthil S. Massive serous macular detachment after an uneventful combined trabeculotomy with trabeculectomy in two cases of Sturge-Weber syndrome resolved with oral propranolol. Indian J Ophthalmol Case Rep 2021;1:526-8

How to cite this URL:
Gajbhiye MP, Senthil S. Massive serous macular detachment after an uneventful combined trabeculotomy with trabeculectomy in two cases of Sturge-Weber syndrome resolved with oral propranolol. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:526-8. Available from: https://www.ijoreports.in/text.asp?2021/1/3/526/320097

 Sturge- Weber syndrome More Details More Details (SWS) is associated with facial and ocular haemangiomas. The ocular complications like glaucoma arise due to developmental angle anomaly in early onset congenital presentation and, due to raised episcleral venous pressure in late onset glaucoma in older children.[1],[2],[3] Diffuse choroidal haemangioma is one of the commonest ocular manifestations of Sturge-Weber syndrome (SWS), typically involving more than half of the fundus with ill-defined borders.[1] The associated reported complications are foveal distortion, amblyopia, and exudative retinal detachment.[2] Glaucoma in SWS is often refractory needing surgery for intraocular pressure (IOP) control. Postoperative choroidal effusion in eyes with SWS is a serious complication,[3] and spontaneous serous macular detachment can occur,[4] however, massive serous macular detachment post-trabeculectomy is not reported. Unlike choroidal effusion, macular detachment would need prompt treatment to prevent permanent photoreceptor damage that may occur with delayed resolution. Various treatment options for management of choroidal haemangiomas with complications are external beam radiotherapy, Ruthenium -106 plaque brachytherapy, photodynamic therapy, and a less invasive use of oral propranolol.[5],[6],[7] We present two cases that presented with massive macular retinal detachment following glaucoma filtration surgery in eyes with SWS and diffuse choroidal haemangioma. Both the children were treated with oral propranolol that resulted in complete resolution of the condition with improved vision.

  Case Reports Top

Case 1

A 6-year-old girl had left-sided port wine stain [Figure 1]a, the visual acuity (VA) was 20/20p and intraocular pressure (IOP) was 28 mmHg on maximum medications in the left eye. The left eye showed megalocornea, open angles on gonioscopy, glaucomatous disc damage and diffuse choroidal hemangioma and the right eye was normal. The child underwent combined trabeculotomy with trabeculectomy (CTT) under general anesthesia. Appropriate pre- and perioperative precautions included preoperative intravenous mannitol, slow decompression of anterior chamber, preplaced sutures and quick closure after trabeculectomy, anterior chamber (AC) reformation with air and use of cycloplegics. Intravenous bolus dose of dexamethasone was given at the conclusion of surgery. Postoperative regimen included intense topical steroids, cycloplegics and antibiotic. Oral steroid at dose of 1 mg/kg bodyweight was started. On the first postoperative day, VA was 20/80 with shallow anterior chamber with air bubble in place, IOP was 12 mm Hg and no choroidal detachment. On the second postoperative day, the VA dropped to 20/500, AC was very shallow and IOP was 15 mmHg. B-scan ultrasonography was performed to assess the posterior segment, minimal peripheral choroidal detachment was noted with subretinal fluid [Figure 1]b and [Figure 1]c. The optical coherence tomography (OCT) showed a large area of neurosensory detachment in the macular area with clear subretinal space [Figure 1]d.
Figure 1: Clinical picture showing port wine stain on left side of the face (a), ultrasound B scan showing peripheral choroidal detachment (white arrow) (b) and subretinal fluid (yellow arrow) (c), macular optical coherence tomography (OCT) showing subretinal fluid (yellow arrow) (d) and resolution with treatment (e and f), wide-field fundus photograph showing diffuse choroidal hemangioma, resolved macular detachment with advanced glaucomatous disc damage (g)

Click here to view

Despite oral steroids, the child had this complication, hence oral propranolol was started under pediatricians' guidance and monitoring. The dose was escalated to 2 mg/kg body weight in two divided doses over 2 days. Serial clinical examinations and OCT at 1 week [Figure 1]e and 1 month [Figure 1]f showed gradual resolution of the subretinal fluid. Oral propranolol was tapered and stopped at 6 weeks. The vision improved to 20/20p with well-controlled IOP of 16 mmHg at 1 month which was maintained until 2 years followup.

Case 2

An 11-year-old boy had SWS with secondary glaucoma in the left eye with left-sided port wine stain [Figue 2]a, his VA was 20/20 and IOP was 25 mmHg in left eye. Gonioscopy showed open angles, fundus showed glaucomatous disc damage, diffuses choroidal haemangioma and normal macula [Figue 2]b in the left eye and right eye was normal. With all appropriate precautions CTT was performed. On day 1 post-surgery, vision was 20/100, AC was shallow but formed and IOP was 11 mmHg. Postoperatively, intense topical steroids, cycloplegics, and antibiotic were started. This child was not started on oral steroids. On the fifth postoperative day, the VA dropped to 20/200, with further shallowing of the AC and IOP of 14 mmHg. Fundus evaluation showed a large serous macular retinal detachment [Figue 2]c. The OCT showed a large area of neurosensory detachment in the sub-foveal region with clear subretinal space [Figue 2]d. The child was started on Oral propranolol at a dose of 2 mg/kg bodyweight under pediatricians' guidance. Repeat OCT at 1 month showed complete resolution of the subretinal fluid [Fig. 2e]. Fundus widefield photography at postoperative 1-month showed no macular detachment, diffuse choroidal haemangioma, and advanced glaucomatous optic disc damage (2f).
Figure 2: Clinical picture showing left-sided port wine stain (a), preoperative macular OCT showing normal macula and thickened choroid (b) postoperative fundus photograph showing large macular detachment (yellow arrow) (c) macular OCT showing subretinal fluid (yellow arrow) (d) and resolution with treatment (e), wide-field fundus photograph showing diffuse choroidal hemangioma, resolved macular detachment with advanced glaucomatous disc damage (f)

Click here to view

  Discussion Top

Diffuse choroidal haemangiomas in SWS may be associated with exudative retinal detachment with involvement of the macula and are often treated with external beam radiotherapy, Ruthenium -106 plaque brachytherapy, or photodynamic therapy.[5],[6],[7],[8] Oral Prednisolone is a noninvasive alternative that was tried for this condition. It was proposed that the effect of propranolol on haemangioma involves induction of endothelial vasoconstriction, decreased expression of hypoxia-inducible factors 1 alpha (HIF-1α), beta-fibroblast growth factor, and vascular endothelial growth factor through RAF, which is a type of proto-oncogene.[8] Raba Thapa and Carol L. Shields,[9] showed complete resolution of exudative retinal detachment secondary to diffuse choroidal haemangioma with oral propranolol therapy in SWS in a 17-year-old boy. In 2011, Arevalo et al.[10] reported use of oral propranolol in resolution of diffuse choroidal haemangioma induced retinal detachment in a 58-year-old woman with SWS over 6 weeks. We administered oral propranolol at the recommended dose of 2 mg/kg of body weight in two divided doses with weekly heart rate and blood pressure monitoring. The resolution of subretinal fluid started within a week of starting the oral propranolol and completely resolved in less than 4 weeks. This not only helped with resolution of the postoperative complication but also helped with restoring the preoperative visual acuity without any surgical intervention. In the first child since we were not aware of use of oral propranolol we had started the child on oral steroids at a dose of 1 mg/Kg BW. However, this did not seem to help as the serous macular detachment occurred despite being on oral steroids. Hence oral steroids were quickly tapered and we explored other option of using oral propranolol for its management.

Recently, Kaushik et al.,[11] reported the effectiveness of perioperative oral propranolol in preventing postoperative sight-threatening choroidal effusion in SWS.

  Conclusion Top

While massive choroidal effusion is a postoperative complication that we fear in eyes with SWS with choroidal haemangioma, our cases describes a rare complication of serous macular retinal detachment. This complication seems to occur 2-5 days after surgery and needs to be identified and treated appropriately to prevent vision loss.

Patients and/or their care takers should be counselled about this rare complication. Oral propranolol is a non-invasive, safe and economical treatment option to treat this complication and can be initiated in the peri-operative period.

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 Top

Tallman B, Tan OT, Morelli JG, Piepenbrink J, Stafford TJ, Trainor S, et al. Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications. Paediatrics 1991;87:323-7.  Back to cited text no. 1
Shields JA, Shields CL. Systemic hamartomatoses ("phakomatoses"). In: Shields JA, Shields CL, editors. Intraocular Tumors: A Text and Atlas. Philadelphia, PA: W. B. Saunders; 1992. p. 528-33.  Back to cited text no. 2
Mantelli F, Bruscolini A, La Cava M, Abdolrahimzadeh S, Lambiase A. Ocular manifestations of Sturge-Weber syndrome: Pathogenesis, diagnosis, and management. Clin Ophthalmol (Auckland, NZ) 2016;10:871-8.  Back to cited text no. 3
Dave T, Dave VP, Shah G, Pappuru RR. Diffuse choroidal hemangioma masquerading as central serous chorioretinopathy treated with oral propranolol. Retin Cases Brief Rep 2016;10:11-4.  Back to cited text no. 4
Murthy R, Honavar SG, Naik M, Gopi S, Reddy VA. Ruthenium-106 plaque brachytherapy for the treatment of diffuse choroidal haemangioma in Sturge Weber syndrome. Indian J Ophthalmol 2005;53:274-5.  Back to cited text no. 5
[PUBMED]  [Full text]  
Anand R. Photodynamic therapy for diffuse choroidal hemangioma associated with Sturge Weber syndrome. Am J Ophthalmol 2003;136:758-60.  Back to cited text no. 6
Paulus YM, Jain A, Moshfeghi DM. Resolution of persistent exudative retinal detachment in a case of Sturge-Weber syndrome with anti-VEGF administration. Ocul Immunol Inflamm 2009;17:292-4.  Back to cited text no. 7
Giatromanolaki A, Arvanitidou V, Hatzimichael A, Simopoulos C, Sivridis E. The HIF-2alpha/VEGF pathway activation in cutaneous capillary haemangiomas. Pathology 2005;37:149-51.  Back to cited text no. 8
Thapa R, Shields CL. Oral propranolol therapy for management of exudative retinal detachment from diffuse choroidal hemangioma in Sturge-Weber syndrome. Eur J Ophthalmol 2013;23:922-4.  Back to cited text no. 9
Arevalo JF, Arias JD, Serrano MA. Oral propranolol for exudative retinal detachment in diffuse choroidal hemangioma. Arch Ophthalmol 2011;129:1373-5.  Back to cited text no. 10
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.  Back to cited text no. 11


  [Figure 1], [Figure 2]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Reports
Article Figures

 Article Access Statistics
    PDF Downloaded13    
    Comments [Add]    

Recommend this journal