Parents, if your darling new baby has a red birthmark, you will want to know the difference between a port wine stain and a hemangioma, two common types of vascular birthmarks, and what some of the related complications might be.

I can tell you what I know – which, admittedly, isn’t much, and is, I’m afraid, heavily anecdotal. But, no matter; I’ll still share it.

Port Wine Stain vs. Hemangioma

A port wine stain, which is what Addy has, is usually flat at birth, but then it grows over time. That growth means that a port wine stain will often become raised & nodular, especially if left untreated.

A port wine stain is a proliferation of blood vessels associated with a particular nerve. That nerve, otherwise responsible for telling its partner blood vessels in utero to “Stop growing!”, missed giving that critical signal at some point during development – so the blood vessels kept growing and growing. (In Addy’s case, the error is associated with the middle branch of the trigeminal nerve, on the right side of the face.) As a result, the capillaries are abnormally wide in diameter (like, over 40x wider).

A hemangioma, on the other hand, is raised at birth – you can feel it if you run your finger over the skin – and it may grow a bit in the first year, but then it will recede or ‘implode’ on its own.

A hemangioma is a cluster of blood vessels by the surface of the skin, sometimes medically referred to as a benign ‘tumor’ of blood vessels. It’s often very bright red. These are the common “strawberry marks” we all know on babies. They usually fade on their own.

I can’t tell you how many birthmark misdiagnoses I’ve heard. Many of them were directed to us by well-wishers, including nurses who told us “Oh, that’s just a hemangioma, it’ll go away!”; others have been directed to some of my readers by aloof primary-care doctors who weren’t familiar with port wine stains. Get your child to a specialist who understands vascular malformations (a good dermatologist should know the difference), and get the right diagnosis.

Here’s why: if your child has a hemangioma, it will probably disappear on its own, and if it doesn’t, well, you can cross that bridge when you get there. But they usually go away without intervention.

If your child has a port wine stain, though, it will not go away on its own; in fact, it will grow with your child. And as your child develops, their blood vessels and the skin above them will only develop & toughen, making it harder to eliminate the stain later. The worst medical advice in that critical first year is “Ehhh, let’s just see if it goes away on its own.” It won’t. It will only toughen. We were lucky – Addy went in for her first surgery at 5 weeks old, and had 9 more before her first birthday.

Complications

Complications can arise depending on how ‘deep’ the birthmark is, and where it’s located, which are determined by when the error occurred in utero.

If the error of a port wine stain occurred earlier (i.e., the nerve missed giving that critical signal to “Stop Growing!” to its associated blood vessels), then the port wine stain will be deeper in the skin, and wider on the body.  For example: Addy’s stain covers much of the right side of her face (along the V2 branch of the trigeminal nerve), and is relatively difficult to treat, hence the 43 surgeries so far.  It’s deep.

Meanwhile, her friend has a small stain along the exact same V2 branch of the trigeminal nerve, but it’s the size of a half-dollar coin on her cheek, and it disappeared after a half-dozen laser treatments. That’s because the friend’s error occurred later in utero, while Addy’s occurred earlier.

Other kids have massive vascular malformations that erred even earlier than Addy’s; one young woman told me that her brother was born with a large one that totally covered one shoulder and down onto his back, and it was so large (and deep, and thick) at birth that the doctors weren’t sure if he would be okay. He avoided contact sports, but otherwise has been living a healthy life.

Depending on where the stain is, a port wine stain can cause other complications. For example, if the stain is on the V1 branch of the trigeminal nerve (the optical branch, along the forehead), the risk is significantly higher for Sturge-Weber syndrome, which is a constellation of issues stemming from having malformed blood vessels in the head, including calcifications in the brain (which can hinder brain development) and seizures. An MRI helps diagnose Sturge-Weber issues in the brain.

Since Addy’s port wine stain touches onto her forehead, she had an MRI at 5 months of age, to make sure that there were no vascular malformations in her brain; all was clear.

But glaucoma is definitely a concern, since the proliferation of blood vessels can also cause undue pressure on her eye. She is checked for glaucoma annually.

Of course, glaucoma isn’t the only eye issue; as you’ve read here, Addy’s tear drain was pinched off by the pressure of all the encroaching blood vessels around it. She had a tube put in a few years ago to try to prop it open. Even though it fell out shortly afterward, it did successfully open up the plumbing for a while, and it bought her some development time. Unfortunately, she’s been tearing up again in her right eye, so we may have to do it again.

The port wine stain is also in her mouth. This is fascinating! As the right and left sides of the head develop in utero, they ‘meet’ in the middle; thus, any error tends to only show up on one side of the face. Addy’s face is split down the middle, even down to her tongue and uvula! If you ask her to open up her mouth, you’ll see her tongue is half red, half pink – and if you look farther back, even her uvula is precisely half red, half pink.

The extra bloodflow to the right side of her mouth meant that her teeth developed earlier, and popped out of her gums earlier. (And, luckily, they popped out fast. Teething wasn’t so bad!) As a baby, she had an adorably lopsided smile for a short time until her left side came in.

But now, all that extra bloodflow and tooth development means a lot of crowding; the well-populated, well-nourished right side of her mouth is crowding out the left, and an extra tooth is popping in above the others (affectionately called ‘The Snaggletooth’), because it doesn’t have any place to go. So, we can add significant orthodontia to our 2019 budget.

When she brushes her teeth, bleeding from the right side isn’t uncommon. In the wintertime, she gets one-nostril nosebleeds on the right side. We try to minimize all possible injury to her face, gums, nose, and scalp on the right side, because she bleeds more than the average bear in those places. And, if you look closely, you’ll see that her bones are actually a bit bigger on the right side of her face, due to the extra bloodflow. It’s not actually a problem (or even noticeable) for Addy, but I know another little girl with a port wine stain who had to see a craniofacial surgeon to see if anything could be done about some too-fast bone growth along her stain.

So, there are complications to these port wine stains. Hemangiomas probably bring various complications, too, especially very large ones, but my experience is limited to port wine stains on the face. These are simply some of the things we’ve watched & worried over with Addy. Each birthmark is unique, so please don’t hesitate to contact me with questions and pictures if you want a mom’s advice. I’m not a doctor, and I will tell you to ‘get thee to a dermatologist’ if you haven’t yet, but I’m happy to navigate these waters with you.

For the sake of trivial interest in birthmarks, I can tell you that there’s another type of birthmark called a nevus, which is a proliferation of skin pigmentation, rather than blood vessels. (Have you ever seen someone with a really dark brown or black birthmark? Sometimes hairy? That’s a nevus.) We know a little girl with a nevus on her forehead, and the complication her parents have to worry about is skin cancer. Their pre-emptive treatment is skin grafting. (I’ll take lasers any day!)

Also, among blood-red marks, there’s even a third type after Port Wine Stain and Hemangioma: Hematoma.  In short, a hematoma is a red spot that looks like a hemangioma, but it usually comes about by injury or disease. Clarence ended up with a small hematoma on his face after an injury, and he got to have a laser session with Dr. Z., but I’ll tell you more about that in another post.

So, there: you’re ready to win obscure trivia questions about birthmarks! You’re welcome.

It’s not always easy to see, but check out the way her teeth are pushed over from the port wine stain on her right to the left — lots of activity on the right side! Lots of extra blood flow for growth.

Addy graciously let me photograph the inside of her mouth; the port wine stain invades her mouth, but only on the right side.