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

• Hypertelorism, Short Midface, Arachnodactyly, Coloboma of Iris and Delayed Bone Age Syndrome

What is Vagneur Triolle Ripert Syndrome? (Definition/Background Information)

• Vagneur Triolle Ripert Syndrome is a group of disorders characterized by eye abnormalities, lymphedema of the legs and frequent problems with the respiratory system
• The exact cause of Vagneur Triolle Ripert Syndrome is not known

Who gets Vagneur Triolle Ripert Syndrome? (Age and Sex Distribution)

• Vagneur Triolle Ripert Syndrome is a rare congenital disorder. The presentation of symptoms may occur at birth
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Vagneur Triolle Ripert Syndrome? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Vagneur Triolle Ripert Syndrome

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 Vagneur Triolle Ripert Syndrome? (Etiology)

The exact cause of development of Vagneur Triolle Ripert Syndrome is unknown.

What are the Signs and Symptoms of Vagneur Triolle Ripert Syndrome?

The signs and symptoms of Vagneur Triolle Ripert Syndrome may include the following:

• Widely-spaced eyes
• Hole(s) in the colored part of eyes (coloboma of iris)
• Disproportionality in face, with a short midface
• Long, slender fingers
• Bone abnormalities and delayed bone age
• Breathing difficulties
• Under-development of reproductive organs
• Intellectual deficiency
• Seizures

How is Vagneur Triolle Ripert Syndrome Diagnosed?

Vagneur Triolle Ripert Syndrome may be diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

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 Vagneur Triolle Ripert Syndrome?

The complications of Vagneur Triolle Ripert Syndrome may include:

• Failure to thrive after birth
• Vision impairment
• Risk of falling and injury due to seizures
• Intellectual deficiency leading to learning disabilities

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Vagneur Triolle Ripert Syndrome Treated?

There is no cure for Vagneur Triolle Ripert Syndrome, since it is a congenital condition. The treatment is usually given to manage the signs and symptoms and any complication that develops.

How can Vagneur Triolle Ripert Syndrome be Prevented?

• Vagneur Triolle Ripert Syndrome may not be preventable, since it is a congenital disorder
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Vagneur Triolle Ripert Syndrome ? (Outcomes/Resolutions)

• The prognosis of Vagneur Triolle Ripert Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Vagneur Triolle Ripert Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/