Angela's Syndrome


This is nothing official. You won't find "Angela's Syndrome" in any medical books, and doing a google search on it only brings up stories about other Angela's. But because my diagnosis throughout the years have never really fit exactly what is going on with my body my doctor in Boston calls it Angela's Syndrome. 

So what do I have? The easiest way to describe my condition is to say that I have extra blood vessels on the left side of my body. These extra blood vessels, or venous malformations, are different than the normal blood vessel. According to Boston Children's Hospital "the blood vessel walls in a venous malformation are unusually thin." The doctors in the past have described it to me by saying that the extra "bad" blood vessels in my body are flat, where as normal blood vessels are more round. The roundness of the normal blood vessels allows blood to flow freely, while the flatness of the extra blood vessels can lead to blood clots because of the restrictiveness of the blood flow. One of the possible risks involved with my condition is that the blood clots formed in the extra blood vessels could dislodge and travel to my heart or lungs. Because of this risk the doctors have me on blood thinners to prevent blood clots. 

Besides my most recent above the knee amputation surgery, I have had four other surgeries. Three to remove vascular malformations and one "Chiari malformation decompression". My first surgery was when I was five years old. The doctor removed two vascular malformations, one from my thyroid and another from my inner left thigh. When I was 16, I had a vascular malformation removed from my abdomen. At the time the doctors said it was the single largest vascular malformation ever removed from anyone. During that surgery Dr. Fishman; head of the Vascular Anomalies Clinic at Boston Children's Hospital, and world renowned doctor in the field of vascular malformation disorders; removed several other small lesions from my abdomen along with the larger one he described as the size of a cantaloupe. 

In 2009 after suffering from severe headaches for about 2 years Dr. Smith performed surgery to remove a section of my skull and my top vertebrae in order to fix my Chiari malformation. There is a chance that due to my genetic condition (Angela's Syndrome) the lining of my spine called the Dura, is weaker than normal. Because of this there is a higher risk for a spinal leak. In 2007 I popped a Dura in my spine which caused a spinal leak. When the fluid leaked out of my spine it cause my brain to droop which in turn caused increasingly painful headaches. Finally, the doctors figure out what was wrong and patched my spinal leak. My brain had been drooping for so long that it caused a Chiari malformation. "Chiari malformation is an abnormality in the back of the head where the brain and spinal cord meet. It causes some of the brain tissue at the base of the skull to be pushed into the spinal canal. This can cause pressure on the brain and block the normal flow of spinal fluid in and around the brain"1 So even after the spinal leak was fixed my brain was stuck in the base of the skull, causing severe headaches. So in 2009 I had a Chiari decompression. It "is a surgical procedure performed to remove the bone at the back of the skull and spine. The dura overlying the tonsils is opened and a patch is sewn to expand the space, similar to letting out the waistband on a pair of pants. The goals of surgery are to stop or control the progression of symptoms caused by tonsillar herniation, to relieve compression of the brainstem and spinal cord, and to restore the normal flow of cerebrospinal fluid (CSF)."2 It was a tricky surgery, with a few scary moments, but overall it went well and after months of recovery the headaches began to go away. I still have headaches from a vascular malformation behind my eye, but nothing like the headaches I suffered during this time. 

The last surgery that I had was my above the knee amputation. To read all about the surgery and follow along during my recovery check out my blog. Here are a few post to check out:

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