The Vascular Birthmarks Foundation
Spinal Cord Vascular Birthmarks or Lesions
The following information refers to Spinal Cord Vascular Birthmarks/Lesions.
For ease in understanding, I am presenting them in two types: superficial
only and compound. The Superficial ONLY lesions are a vascular birthmark
(hemangioma, port wine stain, angiokeratoma, avm, etc.) that presents
at the base of the spine on the skin. The Compound spinal cord vascular
lesions have a stain (most of the time) on the outside of the skin
and an underlying vascular lesion (these lesions are often called
hemangiomas, spinal cord angiomas, angiokeratomas, avms, lymphatic
malformations, lymphangiomas, cystic-hygromas, etc.) Some have no
stain on the outside of the skin but do present with a lump under
the skin, at the base of the spine. These lesions are very, very
rare to find during life. Some anecdotal medical reports that I
have read indicate that they are "common" in about 10%
of autopsies, but never present as a probelm during life (so these
people likely never knew they had a spinal vascular lesion). Superficial
stains or birthmarks at the base of the spine with no underlying
component are not the lesions that provoke concern. When there is
an underlying vascular lesion, this is when you need to have an
MRI or MRA to rule out any damage or impingement on the spinal cord.
Stains at the base of the spine that are raised up and appear to
have any bulk to them or appear to have an underlying component
should be imaged as well as if the stain is flat but the individual
is having problems with walking, falling, etc. The vascular lesion
can wrap around the cord (called tethered cord) and cause complications
such as pain, partial or complete paralysis, problems with walking,
falling, etc. The birthmark is NOT what sends the patient to the
doctor, it is usually the associated problems (which usually indicate
an underlying vascular lesion).
There is a very rare syndrome and it is called COBB SYNDROME. Spinal cord vascular birthmarks may be indicative of this syndrome. Basically, you need to know what COBB SYNDROME is in order to diagnose it and with so few cases in the world, it is very difficult to even get it diagnosed correctly. COBB SYNROME in plain terms usually means a vascular stain at the base of the spine with an "underlying" vascular lesion - so just having a birthmark at the base of the spine does not indicate COBB, there must be an underlying vascular component that COULD or DOES impinge on the spine and could or does cause problems.
Treatment is controversial. A good Pediatric Dermatologist or a vascular birthmarks expert can diagnose COBB SYNDROME. There are various schools of thought on treatment. Interventional Radiologists use alcohol injections and report it is very successful with eliminating symptoms and surgeons report operating on the vascular lesion to remove it and any associated problems is the best treatment. If someone has a diagnosis of COBB SYNDROME the best course of action is to find (although it will be difficult) another individual who has been treated successfully for this syndrome. The internet is a good place to start as well as finding a physician who can confirm the diagnosis and present options to the family affected by the spinal cord vascular lesion. The physician may even be able to refer the family to another family who has been affected by this syndrome. Experts in spinal cord tumors (neurologists) are the most familiar with this syndrome as well as vascular birthmark/tumor experts.