What is the difference in diagnosis and treatment between congenital hemangioma and port-wine stain?

From the Guidelines

Congenital hemangiomas and port-wine stains are distinct vascular anomalies with different diagnoses and treatment approaches, as emphasized by the International Society for the Study of Vascular Anomalies classification 1.

Key Differences

• Congenital hemangiomas are fully-formed vascular tumors present at birth, which may rapidly involute (RICH), partially involute (PICH), or remain stable (NICH), whereas port-wine stains are flat, pink to purple vascular malformations that grow proportionally with the child and never regress.
• Diagnosis of congenital hemangiomas often relies on clinical appearance, with imaging like ultrasound or MRI used in some cases, while port-wine stains are typically diagnosed clinically, with rare need for imaging 2.

Treatment Approaches

• Congenital hemangiomas often require only observation, as many involute spontaneously, with surgical excision reserved for those causing complications or for cosmetic concerns.
• Port-wine stains, however, require active intervention, with pulsed-dye laser therapy being the gold standard treatment, typically requiring multiple sessions starting in infancy for optimal results 1.

Important Considerations

• If left untreated, port-wine stains darken and may develop nodularity over time, while some congenital hemangiomas resolve completely without intervention.
• Port-wine stains associated with Sturge-Weber syndrome require additional neurological evaluation due to potential central nervous system involvement.

Classification and Nomenclature

• The classification of vascular anomalies, as adopted by the International Society for the Study of Vascular Anomalies, is crucial for accurate diagnosis and treatment, highlighting the distinction between vascular neoplasms (like congenital hemangiomas) and vascular malformations (like port-wine stains) 1, 2.

From the Research

Diagnosis of Congenital Hemangioma and Port-Wine Stain

• Congenital hemangiomas are rare solitary vascular tumors that do not proliferate after birth, characterized as either rapidly involuting congenital hemangiomas (RICHs) or noninvoluting congenital hemangiomas (NICHs) based on their clinical progression 3.
• Port-wine stains (PWS) are congenital vascular malformations characterized as ectatic capillaries and venules in the dermis, clinically appearing as a deep red to purple patch on the skin 4.
• Diagnosis of these vascular lesions can be done using optical coherence tomography (OCT) coupled with functional blood-flow Doppler modality, which can distinguish between normal skin and vascular lesions, and also between PWS and hemangiomas 5.

Treatment of Congenital Hemangioma and Port-Wine Stain

• Treatment with the pulsed dye laser (PDL) is the criterion standard for treating vascular lesions, and is well established as the most effective and safest treatment for port-wine stains 6, 4.
• PDL treatment can also be used for superficial hemangiomas, but its efficacy and adverse effects need to be compared with those of observation 6.
• Congenital hemangiomas may not require treatment, but indications for intervention include ulceration, prevention of disfigurement, and impairment of function or vital structures 7.
• Vascular-specific lasers are the gold standard in treating PWS, but some patients may require a combination of treatment methods or new innovations for treating recalcitrant PWS 4.

Differentiation between Congenital Hemangioma and Port-Wine Stain

• Congenital hemangiomas are fully formed at birth and do not proliferate after birth, whereas port-wine stains are present at birth but may progressively darken and become hypertrophic or nodular without treatment 3, 4.
• The vascular characteristics of congenital hemangiomas and port-wine stains differ, with PWS presenting blood vessels of mean diameter 114 μm and mean depth 304 μm, and hemangiomas presenting blood vessels of mean diameter 39 μm and mean depth 298 μm 5.
• The clinical characteristics and management of congenital hemangiomas and port-wine stains also differ, with congenital hemangiomas requiring different treatment approaches based on their clinical progression and associated complications 3, 7.