Vascular Malformations
- Hemangiomas and Other Benign Vascular Lesions of the Skin
- Pigmented Lesions of the Skin
- Port-Wine Stains
- Klippel-Trenaunay Syndrome
Hemangiomas and Other Benign Vascular Lesions of the Skin
Benign vascular malformations of the newborn have been difficult to classify because of their great variety and overlap in clinical and histopathologic appearance. One method of classification is based upon whether or no the lesion has proliferative and regressive phases: hemangiomas do have such phases, and are called cellularly dynamic; vascular ectasias, such as port-wine stain and salmon patches, are called cellularly adynamic because they lack proliferative/regressive phases.
Hemangiomas and lymphangionas are the most common benign tumors of the skin and deeper tissues in the newborn. They represent sequestration of fetal tissue, and may be present at birth or appear in the first months after birth. clinically classified, capillary hemangiomas include port-wine stain, strawberry hemangioma and salmon patch. Other clinical classifications of hemangiomas are capillary/cavernous, cavernous, and verrucous, keratotic or venous.
Vascular malformations also include lymphangiomas, lymphangiohemangiomas, arteriovenous malformations and vascular gigantism.
These benign tumors of the skin range in size and appearance from very small and negligibly disfiguring to very large and overwhelmingly disfiguring. Some cellularly dynamic hemangiomas such as the so-called strawberry lesion my regress and disappear during the first weeks or months of the infant's life. On the other hand, large, critically located hemangiomas and lymphangiomas can be life-threatening as well as substantially disfiguring. Multiple hemangiomas of the newborn with diffuse involvement of the skin, lever, and intestinal tract pose a significant mortality risk.
Pigmented Lesions of the Skin
Pigmented lesions in the newborn often pose a dilemma for the pediatrician, obstetrician or family physician--e.g., determine which are benign and which are malignant or potentially malignant. Large pigmented lesions are disfiguring, whether on the face or body. Family history and histopathology may confirm heritability. Early consultation with a plastic surgeon is recommended to begin planning for surgical removal or other appropriate treatment, and providing objective information and treatment options to the child's parents and family.
Port-Wine Stains
Port-Wine Stains (PWS) are intradermal capillary malformations. They are present at birth and remain persistent throughout life without regression. Some PWS are seen in combination with other vascular anomalies. The incidence of PWS in newborns is 0.3%.
PWS may occur anywhere on the body, but are more frequently found on the face. They often follow the distribution of the fifth cranial nerve around the eye or cheek.
Each PWS is different in terms of size, color, and texture. PWS may darken and thicken as the child grows older. They may develop nodules which eventually need to be excised. Due to these changes, as well as for psychological and cosmetic concerns, it is often medically necessary to treat PWS with a laser. If not treated, 60-70% of lesions undergo these changes.
New Health Information: If your child is older than the age of 12 years and has a resistant port wine stain or has been treated in the past without complete fading, new treatment is available. Please contact our office to schedule an appointment and discuss treatment options
Klippel-Trenaunay Syndrome
This syndrome is characterized superficially by a patchy port-wine stain on an extremity that overlies a combined venous and lymphatic malformation with associated bony enlargement. This bony enlargement usually is not present at birth, but may appear within the first few months or years of life. It may become a problem particularly during puberty. The anomaly may be involving a single extremity or more than one as well as the face or trunk. There may be periods of rapid enlargement and then cessation of growth. There is no evidence of this syndrome being an inheritable disorder.
Symptoms vary with the severity of the dominant vascular component. Examples, if lymphatic anomalies are paramount, there will be soft tissue swelling and enlargement. If venous malformations abound, intermittent episodes of painful thrombosis (clotting) can be expected. Rectal and Urinary tract bleeding may be seen in rare instances if gastrointestinal lesions are involved.
Also through Pastoral Care on a 24-hour basis there is spiritual support available to patients and families.
If you have any questions, please contact our office at (469) 375-3838.
