Korean J Ophthalmol.  2012 Dec;26(6):481-484. 10.3341/kjo.2012.26.6.481.

Congenital Glaucoma from Sturge-Weber Syndrome: A Modified Surgical Approach

• Yuen NS
• Wong IY

• KMID: 1499691
• DOI: http://doi.org/10.3341/kjo.2012.26.6.481

Abstract

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder that causes congenital glaucoma. Previous experiences have shown that drainage procedures are often required to control associated glaucoma. The conventional surgical approach in trabeculectomy carries a significant risk of intraoperative expulsive hemorrhage. Here, we describe a modified approach of the conventional trabeculectomy technique, which may lower the risk of expulsive hemorrhage. A viscoelastic device was employed to maintain a steady intraocular pressure throughout the procedure. Details of the surgical technique and material used are described. One patient with congenital glaucoma associated with SWS underwent a successful trabeculectomy using the modified technique. Postoperative intraocular pressure was successfully reduced and no intraoperative complications occurred. We describe a successful case of trabeculectomy in a SWS case where a modified technique was applied.

Keyword

Congenital glaucoma; Ocular viscoelastic device; Sturge-Weber syndrome; Trabeculectomy; Trabeculotomy

MeSH Terms

Glaucoma/*congenital
Humans
Infant, Newborn
*Intraocular Pressure
Male
Sturge-Weber Syndrome/*complications/diagnosis/surgery
Trabeculectomy/*methods
Visual Acuity

Figure

• 

  Fig. 1 (A) Clinical photo showing right facial haemangioma, characteristic of Sturge-Weber syndrome. (B) Right eye before primary operation showing features of bulphthalmos. (C) Normal left eye before the primary operation. (D) Operated eye one-year after operation, showing a well formed bleb superiorly.

• 

  Fig. 2 Intraoperative photos. (A) Cauterisation of prominent episcleral vessels. (B) Injection of viscoelastics prior to anterior chamber penetration. (C) Appearance immediately prior to creating the inner window with slit knife. (D) Appearance of the inner window immediately after penetration with slit knife. Picture shows only a trace amount of viscoelastic egress from the anterior chamber. (E) Enlargement of inner window with a Kelly punch. (F) Testing aqueous outflow with the releasable suture tied.

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  Fig. 3 Post-operative intraocular pressure trend in the right eye. The intraocular pressure (IOP) decreased significantly after the primary trabeculectomy. Although there was a surge in the second week post-operatively, the IOP decreased to a lower level after the removal of the releasable suture on post-operative day 11. Pre-op = pre-operative.

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