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CASE REPORT
Year : 2018  |  Volume : 4  |  Issue : 2  |  Page : 100-104

Bilateral Sturge–Weber Syndrome With Glaucoma: A Rare Presentation


1 Department of Ophthalmology, Lady Hardinge Medical College & Associated Hospitals, India
2 ESI Model Hospital, Basaidarapur, New Delhi, India

Date of Web Publication28-Aug-2018

Correspondence Address:
Dr. Geetika K Taneja
F 1/18, Budh Vihar Phase 1, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mamcjms.mamcjms_85_17

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  Abstract 


Sturge–Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a rare congenital and dermatological disorder. It is one of the phakomatoses and is associated with unilateral port-wine stain of the face, ipsilateral glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. However, different variants have been described. We describe the case of a 13-year-old female patient with bilateral SWS associated with advanced glaucoma in both the eyes and absent neurological involvement. Surgical treatment with Ahmed glaucoma valve was performed in both the eyes at an interval of 2 weeks for achieving a lower level of intraocular pressure.

Keywords: Ahmed glaucoma valve, bilateral Sturge–Weber syndrome, glaucoma


How to cite this article:
Taneja GK, Taneja A, Garg R. Bilateral Sturge–Weber Syndrome With Glaucoma: A Rare Presentation. MAMC J Med Sci 2018;4:100-4

How to cite this URL:
Taneja GK, Taneja A, Garg R. Bilateral Sturge–Weber Syndrome With Glaucoma: A Rare Presentation. MAMC J Med Sci [serial online] 2018 [cited 2018 Dec 18];4:100-4. Available from: http://www.mamcjms.in/text.asp?2018/4/2/100/240000




  Introduction Top


Sturge– Weber syndrome More Details (SWS) is one of the hamartomatoses or phakomatoses. It is a rare neuro-oculocutaneous disorder with an estimated incidence of one in 50,000 births.[1] Clinically, the neurological manifestations include epilepsy, mental retardation, and hemiplegia; the ocular manifestations include glaucoma and vascular malformations of the conjunctiva, episclera, choroid, and retina; and the dermal manifestations include nevus flammeus or port-wine stain.[2]

Glaucoma is a frequent association with SWS. It is seen in as many as 50% of the cases when the port-wine stain involves the maxillary and ophthalmic branches of the trigeminal nerve.[3] However, the prognosis of glaucoma associated with SWS, despite treatment, is often poor.[4]

In this case report, we describe the case of a 13-year-old female patient with bilateral SWS associated with advanced glaucoma in both the eyes and absent neurological involvement.

Case Report

A 13-year-old female patient was referred from the Paediatric Department for the evaluation of the ocular manifestations of SWS and low visual acuity in the right eye. She had a bilateral facial angioma sparing the right side of the forehead since birth [Figure 1]. Best-corrected visual acuity was 1/60 in the right eye [−4.50 diopter cylinder (DC) × 90°] and 6/9 in the left eye (−3.0 DC × 90°). Grade 3 relative afferent papillary defect was present in the right eye. Dilated episcleral vessels were noted in both the eyes [Figure 2]. Intraocular pressure measured by Goldmann applanation tonometer on presentation was 50 mmHg in the right eye and 42 mmHg in the left eye at 10.00 AM. Fundoscopy revealed total glaucomatous optic atrophy in the right eye and cup-to-disc ratio of 0.7 in the left eye [[Figure 3] and [Figure 4]]. Central corneal thickness was 508 and 525 μm in oculus dexter (OD) and oculus sinister (OS), respectively. Horizontal and vertical corneal diameter was 13 mm in both the eyes. On gonioscopy, anterior chamber angle was open in both the eyes with blood in the Schlemm’s canal. A visual field assessment of the left eye showed severe constriction with a sparing of central vision [[Figure 5] and [Figure 6]]. A visual field assessment of the right eye was not possible due to low visual acuity. No evidence of choroidal vascular malformations was seen in either eye. Axial length was 22.76 mm in the right eye and 22.32 mm in the left eye. Ultrasound B scan revealed increased optic nerve head cupping in both the eyes. The magnetic resonance imaging (MRI) of the brain revealed normal findings.
Figure 1: Clinical photograph of the patient showing bilateral facial angioma

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Figure 2: Clinical photograph of the patient showing the dilated episcleral vessels

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Figure 3: Fundus photograph of the right eye

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Figure 4: Fundus photograph of the left eye

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Figure 5: 30-2 Humphrey’s field analysis of the left eye

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Figure 6: 10-2 Humphrey’s field analysis of the left eye

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The patient was started on oral and topical medical therapy for the control of intra ocular pressure (IOP) and planned for glaucoma surgery in the left eye with a drainage device.

A decision for surgical management in the form of Ahmed glaucoma valve implantation in both the eyes was taken because trabeculectomy in patients with SWS is associated with higher chances of failure due to trabecular dysgenesis and higher chances of postoperative complications such as choroidal effusion. The patient underwent Ahmed glaucoma valve implantation in the left eye [[Figure 7] and [Figure 8]] and was continued on topical medical therapy in the right eye. The valve was secured to the sclera using a 10-0 nylon suture and the conjunctiva was closed using 6-0 silk sutures, because both these steps warranted the use of nonabsorbable sutures. Postoperative outcome was satisfactory in the left eye. After 2 weeks, the patient underwent glaucoma valve implantation in the right eye as well. At 1-month follow-up, the IOP was 12 and 10 mmHg in OD and OS, respectively. Best-corrected visual acuity was maintained at 6/9 in the left eye. Visual acuity in the right eye was 1/60 with the projection of rays accurate in all quadrants due to advanced glaucomatous optic neuropathy.
Figure 7: Postoperative Day 1 clinical photograph of the patient

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Figure 8: Postoperative Day 1 slit lamp photograph of the patient

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  Discussion Top


Glaucoma associated with SWS is usually a unilateral condition.[3] The case described in this study is that of a bilateral presentation with asymmetric optic disc damage.

The pathophysiology of glaucoma in SWS has been under investigation for many years, and various mechanisms for the same are theorized. When glaucoma is present at birth, it is due to a malformation of the anterior chamber angle, and patients manifest the typical signs of congenital glaucoma, including buphthalmos, anisometropia, and amblyopia. In later-onset juvenile glaucoma, the anterior chamber angle mostly appears normal. It is suggested that elevated episcleral venous pressure plays an important role in glaucoma associated with SWS.[5] Another mechanism of glaucoma is thought to be caused by the premature ageing of the trabecular meshwork and Schlemm’s canal complex leading to early-onset chronic open angle glaucoma.[6]

Medical therapy for glaucoma in SWS is often inadequate, and most patients require surgical intervention for the control of intraocular pressure. Trabeculectomy is associated with a number of postoperative complications including choroidal effusion.[7] Goniotomy and trabeculotomy have been described as treatment modalities in early-onset glaucoma.[3] Less frequent complications have been found to be associated with glaucoma drainage devices.[8],[9] This was the reason for the choice of this procedure as primary treatment modality in our case.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatr Neurol 2004;30:303-10.  Back to cited text no. 1
    
2.
Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus 1992;29:349-56.  Back to cited text no. 2
    
3.
Allingham RR, Damji KF, Freedman S, Moroi SE, Rhee DJ. Shield’s Textbook of Glaucoma. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2005.  Back to cited text no. 3
    
4.
Oziebo-Kupczyk M, Urban B, Mrugacz M, Bakunowicz-Lazarczyk A. The evaluation of the efficacy of treatment in glaucoma associated with Sturge-Weber syndrome. Klin Oczna 2007;109:46-8.  Back to cited text no. 4
    
5.
Shiau T, Armogan N, Yan DB, Thomson HG, Levin AV. The role of episcleral venous pressure in glaucoma associated with Sturge-Weber syndrome. J AAPOS 2012;16:61-4.  Back to cited text no. 5
    
6.
Cibis GW, Tripathi RC, Tripathi BJ. Glaucoma in Sturge-Weber syndrome. Ophthalmology 1984;91:1061-71.  Back to cited text no. 6
    
7.
Bellows AR, Chylack LT Jr, Epstein DL, Hutchinson BT. Choroidal effusion during glaucoma surgery in patients with prominent episcleral vessels. Arch Ophthalmol 1979;97:493-7.  Back to cited text no. 7
    
8.
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.  Back to cited text no. 8
    
9.
Figueiredo LR, Silva Filho FJ, Rehder JR. Sturge–Weber syndrome and its ocular effects: A review. Rev Bras Oftalmol 2011;70:194-9.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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