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Clinical bottom line
Children with bilateral port-wine stain (PWS), PWS involving more than one trigeminal dermatome, PWS of the upper or lower eyelids or unilateral PWS involving the V1 dermatome have the highest risk of developing glaucoma (≥5.8%). (Grade B)
Glaucomatous changes in patients with PWS involving unilateral V2 or unilateral V3 dermatomes alone are rare. (Grade C)
A newborn baby was observed to have a unilateral port-wine stain (PWS) in the distribution of the maxillary branch of the trigeminal nerve on the right side of the face, including the right lower eyelid. Further examinations, including neurological and ophthalmological assessments, were unremarkable and the patient was not routinely followed up for ophthalmic assessment, in keeping with the hospital guidelines. Twelve years later, the patient presented to their optometrist complaining of worsening vision in their right eye, secondary to glaucoma. As early detection of glaucoma improves long-term visual outcomes, you wonder whether all children with a PWS on their face, not just those with a forehead mark, should be offered routine ophthalmic surveillance.
Structured clinical question
In a neonate or child presenting with a facial port-wine stain (population), what is the chance of developing glaucoma (outcome) depending on facial distributions of the port-wine stain mark (risk factor)?
Cochrane Library was searched in January 2020 using “port wine OR nevus flammeus OR naevus flammeus OR facial …
AM, MG and AV contributed equally.
Contributors All authors contributed equally to this manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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