Glaucoma Management in Sturge-Weber Syndrome Using the Delphi Process

We sought to identify areas of consensus and nonconsensus in the ophthalmic screening, diagnosis, and management of Sturge-Weber syndrome (SWS).

Modified Delphi methodology.

North American glaucoma experts with prior experience managing glaucoma in patients with SWS.

A modified Delphi process was used as a systematic and structured communication technique, consisting of 2 rounds of electronic questionnaires to a wider group, followed by an in-person meeting of selected experts. Questions that did not reach agreement were reformulated in each round, with the aim of reaching consensus.

Consensus was defined as agreement among at least 85% of participants for the electronic questionnaires or a minimum of 70% during the in-person meeting.

Among other recommendations, the panel concluded that all patients with a facial port wine birthmark should be assessed for glaucoma within a month of birth, with shorter screening intervals thereafter in patients with bilateral eyelid involvement, choroidal hemangioma, and signs of elevated episcleral venous pressure. There was no consensus on timing of follow-up visits. In children aged ≥4 years with signs of glaucoma, medical intervention is the initial consideration. In children aged <4 years, the primary intervention is likely to be surgical, with some form of goniosurgery as the first-choice procedure.

Consensus for screening, diagnosis, and management in patients with SWS is designed to hopefully improve clinical practice and patient outcomes. Questions where consensus was not reached may highlight variations in practice, conflicting evidence, or areas that might benefit from further research and investigation.

Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.