A special need defined: Giant Congenital Nevus

*I wrote this post back in March 2009 shortly after we were matched with our new daughter Ava. Ava's special need is Giant Congenital Nevus. I have added some additional detail now that she is home.**

I have had several people email me requesting that I post some information about Ava’s special need. A commenter suggested that I should provide some information in the hopes that it may assist another family in accepting a child with a special need like Ava’s.

Ava’s special need is called giant congenital nevus. The plural of nevus is nevi. Essentially, Ava has a large birthmark on her face and scalp as well as some large birthmarks on the trunk of her body, her arms and her legs. A birthmark is skin that is highly pigmented with melanin. Melanin is human pigment. Normally melanin is distributed evenly throughout the skin. In instances of congenital nevi, the pigmented cells are not evenly distributed. When many of these types of cells are clumped together, they can result in moles on the skin because of the higher concentration pigment.

The term congenital is used if the birthmark is present at birth. One in every 50-100 people is born with a small mole. Moles can be very small or they can cover a very large part of the body and be so large that they are diagnosed medically as "giant nevi."

A nevus is considered large if it covers more than 2% of the patient’s total body surface area, or is larger than the palm of the patient’s hand. The term giant is also used when describing nevi. By definition, a giant nevus covers a very large part of the body, usually involves the torso, and is usually accompanied by a number of satellite nevi. Satellite nevi are smaller nevi. When a child is born with a giant nevus, it is common for satellite nevi to be present at birth. It is also common for satellite nevi to appear after birth.

Approximately 1 in 20,000 newborns is found to have a large congenital nevus. Large congenital nevi form in the womb very early in development, within the first twelve weeks of pregnancy. They are caused by a defect during embryologic development. There is no known method of prevention. They appear in either sex, in all races, and on any areas of the skin.There are several characteristics of nevus skin. Normally, nevus skin has fewer functioning sweat glands and too little fat where the nevus is located. Besides producing increased pigmentation, the area covered by a nevus often produces increased amounts of hair. Large nevi can also be delicate and tear easily. People with large nevi also have a higher risk of skin cancer however the medical community is unsure of how high the risk actually is. The risk of cancer is the main concern with congenital nevus.

In a small percentage of cases, pigment cells can be found in the brain and spinal cord which is a complication of large nevi called neurocutaneous melanocytosis (NCM). I would have to say that this is the complication that most parents of children with giant nevi are worried about.NCM can cause neurological issues. Studies have shown that most individuals with NCM do not develop neurological issues however those with neurological issues can have complications that are very serious. An MRI of the brain and spine can rule NCM out. Most professionals consider NCM to be a rare occurrence.

Currently the best method for removing large nevi is surgical excision by a plastic surgeon. Adjacent skin is stretched using a technique called "tissue expansion. During tissue expansion a series of expanders which look like deflated balloons are surgically placed under the skin. At regular intervals the expanders are slowly inflated with saline. The inflation of the expanders can take as long as 4 months. As the expanders are inflated the skin will slowly begin to stretch. When the skin has stretched as necessary the nevus will be excised and the expanded skin is used to cover the area where the nevus was removed.

This procedure has a very high success rate and after looking at before and after pictures I was stunned! This is something I am struggling with… we have every intention of working towards having Ava’s nevus removed primarily because we are concerned about the risk of skin cancer. On the other hand… her nevus is such a part of her… of who she is. It will be an emotional time on many levels.

I found the most fabulous support group called Nevus Outreach. The group consists of family, friends and medical professionals who are dealing with congenital nevus as well as individuals who have congenital nevus. We have been able to locate 2 doctors who are very highly skilled at treating facial nevus. I have learned so much and I have actually found several families who have adopted from China on that group!

This special need is consider moderate to severe primarily because of the treatments necessary to remove the nevus. A high percentage of children with this special need have no additional medical issues. I have had people tell me that her special need looks overwhelming. It may look like that on the outside but once your heart is invested… that special need just becomes a part of who they are.

Now that she is home I can tell you with my whole heart that we truly don’t SEE her nevi. We see Ava’s beautiful eyes, her full cheeks, her vibrant smile and her silky hair. That nevus just fades into the background… which is exactly how it should be.