What are the other Names for this Condition? (Also known as/Synonyms)

• CTS (Capillary Telangiectasia)
• Telangiectasia, Capillary Type

What is Capillary Telangiectasia? (Definition/Background Information)

• Capillary Telangiectasia (CTS) is a type of vascular malformation of the brain, in which clusters of dilated capillaries are formed in some areas of the brain, interspersed with normal brain tissue.
• The condition has generally been found in 3 regions of the central nervous system:
  • Pons: It is involved in relaying signals for respiration, sleep, bladder control, emotions, posture, and many other functions
  • Cerebellum: It is the region that receives sensory inputs; it is responsible for movement, respiration, coordination, posture, and many other functions
  • Spinal cord, which is an integral part of the central nervous system (CNS)
• Although this type of brain vascular malformation can occur at any age, it is most often found in young and middle-aged adults.
• The risk factors for Capillary Telangiectasia are not clearly explained. However, if the condition develops because of hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, a positive family history of the disorder can be a risk factor
• In most cases, Capillary Telangiectasia is asymptomatic. However, a small percentage of affected individuals may have headaches, hemorrhage, double vision, dizziness, hearing loss, and uncoordinated movements
• The disorder is typically diagnosed incidentally, or while performing an autopsy. If symptoms are noted, then a thorough physical examination, symptom assessment, evaluation of family medical history, and imaging scans can help make the diagnosis
• Intracerebral hemorrhage can be complication of Capillary Telangiectasia, which may cause headaches, weaknesses, and paralysis. Additionally, CTS can be a part of combined vascular deformity of the brain, typically in association with cavernous or arteriovenous malformations. In such cases, significant complications may be observed
• Owing to the location of Capillary Telangiectasia, and the condition being asymptomatic in most cases, a treatment for CTS is not usually recommended. The prognosis is generally good for malformations that do not present any symptoms

Who gets Capillary Telangiectasia? (Age and Sex Distribution)

• Capillary Telangiectasia occurs in 0.4% of the global population, and accounts for approximately 20% of all vascular malformations of the brain
• The age of manifestation of CTS can vary, but is typically observed in the 30s and 40s (in young and middle-aged adults)
• This brain vascular malformation affects males and females equally and no preference is noted
• The condition equally affects individuals belonging to all races and ethnicities

What are the Risk Factors for Capillary Telangiectasia? (Predisposing Factors)

• The risk factors for the formation of Capillary Telangiectasia are not currently known
• If the condition is associated with Osler-Weber-Rendu syndrome, then a family history of the disorder is a risk factor for being diagnosed with this condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Capillary Telangiectasia? (Etiology)

Currently, the cause of Capillary Telangiectasia is not known.

• However, if the condition is associated with Osler-Weber-Rendu syndrome, mutation(s) in the ACVRL1, ENG, and SMAD4 genes are known to cause malformations in the brain. These genes code for proteins that interact with growth factors and play a role in the formation of blood vessels
• The pattern of inheritance is autosomal dominant, in which a single copy of a defective gene is sufficient to cause this type of vascular brain malformation. Affected individuals inherit the disorder from an affected parent

What are the Signs and Symptoms of Capillary Telangiectasia?

In most cases, Capillary Telangiectasia does not produce any significant signs and symptoms. In symptomatic CTS, a combination of the following may be observed:

• Headaches
• Weakness
• Hemorrhage
• Double vision
• Dizziness, vertigo
• Ringing in the ears (tinnitus)
• Hearing loss
• Speech impediment
• Uncoordinated movements
• Exaggerated reaction to external stimuli (hyperflexia)
• Numbness, tingling, burning, or other sensations in various parts of the body (paresthesia)

How is Capillary Telangiectasia Diagnosed?

The diagnosis of Capillary Telangiectasia in most cases is incidental, since the condition is predominantly asymptomatic. The condition is sometimes also diagnosed during an autopsy (post-mortem).

In individuals who present symptoms, the diagnosis is made by the following:

• A thorough physical examination and assessment of symptoms
• An evaluation of the affected individual’s personal and family medical history
• Cerebral arteriography:
  • This test provides information on the location and characteristics of the feeding arteries and draining veins, as well as blockages
  • A thin tube is threaded up towards the brain and inserted into an artery in the groin; a dye is injected through this thin tube. The dye provides a contrast for X-ray analysis of the affected organ/area
• Computerized tomography (CT) scans of the brain; it may include CT angiogram that may be performed with a dye injection
• Magnetic resonance imaging (MRI) of the brain; it may include MRI angiogram with an injected dye

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Capillary Telangiectasia?

By itself, Capillary Telangiectasia is a benign condition in most cases that does not lead to any significant complications. However, in some cases, or in association with other vascular malformations, the following complications may occur:

• Hemorrhage
• Stroke
• Difficulty in speaking
• Loss of vision
• Unsteadiness that may be very severe
• Hydrocephalus (fluid build-up in the skull, pushing the brain tissue towards the skull)

How is Capillary Telangiectasia Treated?

No treatment is usually necessary for Capillary Telangiectasia. The condition being symptomatic, in most cases, makes treatment unnecessary.

• Also, the location of the abnormality predominantly in the pons region of the brain, may make treatment impractical
• If the condition is associated with other vascular disorders of the brain, or if CTS presents significant symptoms, then treatment may be considered on a case-by-case basis

How can Capillary Telangiectasia be Prevented?

Presently, there are no known methods or guidance available for the prevention of Capillary Telangiectasia.

• If the condition runs in the family, genetic counseling may help prospective parents ascertain the risks and plan for a child accordingly
• Routine and regular monitoring of the condition is highly recommended

What is the Prognosis of Capillary Telangiectasia? (Outcomes/Resolutions)

• Capillary Telangiectasia is a benign and predominantly non-symptomatic condition. Therefore, the prognosis is good if CTS occurs by itself (i.e., it is not associated with other conditions)
• However, the prognosis can be guarded if it coexists with other vascular malformations as part of mixed brain malformations

Additional and Relevant Useful Information for Capillary Telangiectasia:

• Dandy-Walker malformation (DWM) is a rare, congenital malformation of the brain that includes the cerebellum and the fluid filled spaces around it

The following link may help you find useful additional information on Dandy-Walker malformation:

http://www.dovemed.com/diseases-conditions/dandy-walker-malformation/