follow @PCDSUKCreated: 25th April 2012 | Last Updated: 19th January 2013
Introduction
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A port-wine stain is a vascular malformation of developmental origin with ectasia of superficial dermal capillaries. It is occasionally associated with congenital glaucoma, the Sturge-Weber syndrome (intracranial angiomas) and the Klippel-Trenaunay-Weber syndrome (limb hypertrophy). |
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History
- A port-wine stain is almost always present at birth
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The Sturge-Weber syndrome:
- The usual cutaneous finding is a unilateral port-wine stain, involving roughly the areas served by the ophthalmic and maxillary divisions of the trigeminal nerve
- Epilepsy occurs in up to 90% of cases - once seizures start neurological deterioration with mental retardation can be rapid
- Patients without seizures fare much better
- Glaucoma and other eye complications arise in 50-60% of all cases
- Early referral is indicated
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The Klippel-Trenaunay syndrome:
- A rare disorder with a port-wine stain extending down the limb, increased limb size, varicose veins and other soft tissue swellings
- The condition is associated with many other developmental defects and high rates of deep vein thromboses and pulmonary emboli
Clinical findings
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Distribution
- The face is the most commonly affected site
- The nape of the neck and upper trunk are other common sites, although any part of the body can be affected
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Morphology
- Pink to deep red / purple patches
- Often unilateral with a distinct cut off
- Lesions tend to persist, darken and thicken with age
Images
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Figure: 1 Port-wine stain and Sturge-Weber syndome |
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Figure: 2
Port-wine stain and Sturge-Weber syndome |
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Figure: 3 Port-wine stain |
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Figure: 4 Port-wine stain |
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Figure: 5
Klippel-Trenaunay syndrome and unaffected twin |
Management
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Facial lesions need early referral for the following reasons:
- To consider the possibility of the Sturge-Weber syndrome
- The cosmetic impact of such lesions can be substantial - referral to a dermatology department with laser facilities is appropriate
- Lesions on or near the eyelid can be associated with glaucoma - where the face is affected both above and below the eye the risk of glaucoma is particularly high. 40% of the cases of glaucoma arise in infancy, but glaucoma can also arise in adults and those at risk need early assessment and long-term follow up with an ophthalmologist