Monday, October 7, 2013

Treatment group at Children’s targets vascular anomalies

By: Dr. Brian D. Kulbersh of Pediatric ENT Associates is director of the Vascular Anomalies Treatment Group at Children’s of Alabama.


About 200 patients are referred every year to Children’s of Alabama for diagnosis, treatment and management of vascular anomalies. These conditions arise when blood or lymphatic fluid vessels develop improperly. They can range in severity from benign birthmarks to painfully swollen malformations of vessels.


To provide the best possible care for these young patients, Children’s of Alabama has formed the Vascular Anomalies Treatment Group. This team brings together seven disciplines: ENT, hematology/oncology, dermatology, interventional radiology, general surgery, plastic surgery and orthopedic surgery.


I lead the team, and members include: Drs. Amy Theos, Joseph Pressey, Ahmed Kamel, Souheil Saddekni and Mac Harmon. We all have developed sub-specialization in the field of vascular anomalies. We meet monthly to review medical histories, photographs, radiological images and pathology slides from patients who have been referred to us.


In general, our team usually sees two major groups of anomalies, hemangiomas and vascular malformations. The more common of the two groups, hemangiomas, are not present at birth. They can be red when they are in the top skin layers and blue if they are deeper. Sometimes they occur in combination. They are usually noticed several days or weeks into life.


Hemangiomas are more common in girls and low birth-weight babies. Eighty percent of them are found near the head and neck. They can grow for up to a year, and then usually begin to slowly fade and disappear when the child is 3 to 9 years of age. Sometimes, families choose surgical intervention.


On the other hand, vascular malformations are abnormal clusters of blood vessels that form during fetal development, although they may not be visible at birth. Symptoms include pain, swelling or bleeding. They, too, grow during the first year of life, but without treatment, they will not diminish or disappear.  


For example, arteriovenous malformations are abnormal connections between arteries and veins. They are present at birth, developing in the absence of a normal network of tiny capillaries that is needed to properly connect arteries and veins. These malformations slowly progress, and become larger, darker, warmer and more painful.

We also care for patients with lymphatic malformations, conditions that are often present at birth and cause swelling in the neck and face. These malformations do not involve blood vessels, but instead involve defective vessels that carry lymph, a fluid that is critical to the body’s immune system.  Some lymphatic malformations can interfere with eating and breathing. Treatment includes surgical intervention, sclerotherapy via interventional radiology and some new medical treatments such as Rapamycin.


Vascular anomalies, and some of conditions that cause them, include:

--Common birthmarks are the most familiar vascular anomalies, with names like “stork bite” and “strawberry mark.” These areas of discolored and/or raised skin are apparent at birth, or shortly thereafter. About 1-in-10 babies have a vascular birthmark. Most are benign and require no treatment.


--Hereditary hemorrhagic telengiectasia. Children with this syndrome tend to form blood vessels that lack capillaries between arteries and veins. Without tiny capillaries, arterial blood under high pressure flows directly into a vein and can rupture vessels, causing bleeding. This occurs mostly on the nose, facial skin, hands, mouth, GI tract, lungs and brain.

--Klippel-Trénaunay syndrome. This rare, congenital condition is marked by a large number of abnormal blood vessels. It is a complicated condition, and affects different children in different ways. It requires the skills of several specialists using a variety of interventions, including surgery.

--Sturge-Weber syndrome. This syndrome involves a vascular birthmark and neurological abnormalities. Between 75 to 90 percent of afflicted children develop seizures, which start before age 1, and may worsen. About a third of afflicted children are born with glaucoma on the side of their facial birthmark.

--Port wine stains. These birthmarks begin as flat areas of skin that are pink to dark red. They usually follow nerves on the face, arms or legs. A port wine stain on an eyelid of the forehead is sometimes associated with Sturge-Weber Styndrome, and similar lesions may be found in the brain, causing neurological problems.

--Venous malformations. These are collections of dilated veins usual present at birth as a painless, purple mass. They grow slowly, and tend to get larger during adolescence, especially if they are located below heart level.
--PHACE syndrome. This rare syndrome is marked by large hemangiomas and birth defects of the brain, heart, eyes head or neck.

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