What are the potential complications of a port wine stain on an infant?

Port Wine Stain Complications in Infants

Port wine stains in infants are capillary malformations that can cause Sturge-Weber syndrome (neurological complications including seizures), glaucoma and other ocular abnormalities, and progressive soft tissue/bone hypertrophy, with the highest risk occurring when the lesion involves the V1 (ophthalmic) distribution of the trigeminal nerve. 1, 2

Neurological Complications

Sturge-Weber syndrome is the most critical complication to identify, occurring when leptomeningeal vascular malformations accompany facial port wine stains. 3

• Risk stratification by location: Port wine stains affecting the entire V1 distribution carry a 78% risk of neurological and/or ocular involvement 3
• Overall V1 involvement risk: Any partial or complete V1 distribution involvement carries a 26% risk of neurological and/or ocular complications 3
• Seizures are the most common neurological manifestation, with epilepsy being a primary concern requiring ongoing surveillance 3
• Developmental delays may occur and should be systematically screened for in any infant with V1 involvement 2

Critical Pitfall in Neurological Screening

Negative screening brain MRI cannot exclude Sturge-Weber syndrome - false-negative rates reach 23.1% in high-risk infants, and these false-negatives may delay seizure recognition by an average of 14 months compared to those who develop seizures earlier. 4 This means that regardless of MRI findings, seizure education and monitoring remain essential. 4

Ocular Complications

Glaucoma is the most common ocular complication in infants with port wine stains involving the periocular region. 3

• Ipsilateral choroidal involvement can occur, leading to various ocular manifestations 3
• Ongoing ophthalmological surveillance is mandatory for any V1 distribution involvement, even with negative initial screening 3, 4

Anatomical and Structural Complications

Port wine stains cause progressive changes beyond the skin surface:

• Soft tissue hypertrophy: Involvement of underlying subcutaneous tissue is common 3
• Bone hypertrophy: Underlying bone can be affected, leading to facial asymmetry 3
• Mucosal extension: Adjacent mucous membranes and conjunctiva may be involved 3
• Progressive darkening and thickening: The lesion darkens from pink/red to port-wine color over time and may develop nodular changes 5, 6

Dermatological Complications

• Port-wine stain-associated dermatitis can develop either spontaneously or during laser treatment 7
• This dermatitis occurs exclusively or most severely within the port wine stain itself 7
• Laser therapy must be deferred to dermatitis-affected areas until inflammation resolves to minimize adverse effects 7

Risk Stratification Algorithm

High-risk features requiring immediate specialist referral and screening: 2, 3

1. V1 distribution involvement (forehead, upper eyelid, periocular area)
2. Bilateral facial involvement
3. Complete hemifacial involvement
4. Midline or juxta-midline lumbosacral location (risk of spinal dysraphism) 2

Associated symptoms requiring urgent evaluation: 2

• Urologic dysfunction (incontinence, frequent urination, recurrent UTIs)
• Bowel dysfunction (infrequent movements, hard stools, fecal incontinence)
• Motor abnormalities (leg weakness, abnormal gait, foot deformities)
• Orthopedic concerns (progressive scoliosis, limb length discrepancy)

Essential Distinction from Infantile Hemangiomas

Port wine stains are fundamentally different from infantile hemangiomas - this distinction is critical for appropriate management: 1, 5

• Port wine stains: Flat, present at birth, do not proliferate, persist throughout life 1
• Infantile hemangiomas: Raised, highly vascular, appear before 4 weeks of age, proliferate rapidly, then involute 1
• Kasabach-Merritt phenomenon does NOT occur with port wine stains 1