History Questions for Infantile Port Wine Stain
When evaluating an infant with a port wine stain, your primary goal is to determine the anatomical location and extent of the lesion to stratify risk for associated neurological and ocular complications, particularly Sturge-Weber syndrome.
Essential Location and Distribution Questions
Ask specifically about the exact anatomical distribution of the port wine stain, with particular attention to trigeminal nerve involvement:
- Does the lesion involve the forehead, upper eyelid, or area around the eye (V1 distribution)? This is the single most critical question, as V1 involvement carries a 26% risk of neurological and/or ocular complications 1
- Does the lesion cross the midline or involve both sides of the face? 1
- Does it extend to involve the cheek (V2) or lower face/jaw (V3) regions? 1
- Is the lesion located on the scalp, neck, trunk, or limbs in addition to the face? 2
- Is the lesion midline or juxta-midline over the lumbosacral spine? This location is classified as intermediate-risk and may be associated with spinal dysraphism 2
Lesion Characteristics
Confirm the physical characteristics to distinguish port wine stain from other vascular lesions:
- Was the lesion present at birth? 3, 4, 5
- Is the lesion flat (not raised)? 2
- What color is it—pink, red, or darker red-purple? 2
- Are the borders well-defined or poorly defined? (Well-defined borders suggest true port wine stain versus nevus flammeus simplex) 2
- Has the lesion changed in color, thickness, or developed any nodules since birth? 3, 4
Associated Neurological Symptoms
For any V1 involvement, systematically screen for Sturge-Weber syndrome manifestations:
- Has the infant had any seizures or unusual jerking movements? 1
- Have you noticed any developmental delays or concerns? 2
- Any episodes of decreased responsiveness or staring spells? 1
Ocular Involvement Screening
V1 port wine stains require specific questioning about eye involvement:
- Does the lesion involve the eyelid or conjunctiva? 1
- Have you noticed any cloudiness or enlargement of either eye? (screening for glaucoma) 1
- Has the infant been evaluated by an ophthalmologist? 1
Associated Spinal Dysraphism Questions (for lumbosacral lesions)
If the port wine stain is located over the spine, ask about tethered cord symptoms:
- Urologic function: Any urinary incontinence, frequent urination, or recurrent urinary tract infections? 2
- Bowel function: Bowel movements every second day or less often? Hard stool consistency? Any fecal incontinence? 2
- Motor function: Any weakness in the legs, abnormal gait, or foot deformities? 2
- Orthopedic concerns: Any progressive scoliosis or limb length discrepancy? 2
Other Cutaneous Markers
Screen for additional high-risk cutaneous markers that may coexist:
- Is there a tuft of hair (hypertrichosis/"fawn's tail") over the spine? 2
- Any dimples, skin tags, or lumps along the spine? 2
- Any other birthmarks or skin lesions elsewhere on the body? 2
Birth and Perinatal History
- Was the infant born at term or preterm? 6
- What was the birth weight? 6
- Were there any complications during pregnancy or delivery? 6
- Has the infant had any hospitalizations or emergency visits since birth? 6
Family History
- Does anyone in the family have similar birthmarks or vascular lesions? 4
- Any family history of seizures, developmental delays, or eye problems? 1
Functional Impact and Parental Concerns
- Does the lesion involve the mouth, making feeding difficult? 4
- Have you noticed any bleeding from the lesion? 4
- What are your primary concerns about this birthmark? 6
Critical Pitfall to Avoid
Do not confuse port wine stains with infantile hemangiomas—the latter are raised, highly vascular lesions that develop after birth and proliferate, whereas port wine stains are flat, present at birth, and do not proliferate. 2 This distinction is crucial because infantile hemangiomas have completely different risk stratifications and management approaches.