What is arachnodactyly a sign of?

Congenital contractural arachnodactyly (CCA) is an extremely rare genetic disorder characterized by a Marfan-like body habitus (tall, slender), the permanent fixation of certain joints (e.g., fingers, elbows, knees, and hips) in a flexed position (contractures); abnormally long, slender fingers and toes (arachnodactyly …

Can you have arachnodactyly without Marfan syndrome?

This feature can occur on its own with no underlying health problems, or it can be associated with certain medical conditions, including Marfan syndrome, Ehlers–Danlos syndromes, Loeys–Dietz syndrome, congenital contractural arachnodactyly, and homocystinuria.

What causes Beals Hecht syndrome?

Beals-Hecht syndrome, also known as congenital contractural arachnodactyly, is caused by a defect in fibrillin as in Marfan syndrome. This syndrome is characterized by a multitude of clinical findings including arachnodactyly, narrow body habitus, scoliosis, congenital contractures, and external ear deformities.

Is Beals Hecht Syndrome a genetic disorder?

Congenital contractural arachnodactyly (Beals syndrome) is an autosomal dominantly inherited connective tissue disorder characterized by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. It is caused by a mutation in FBN2 gene on chromosome 5q23.

How common is arachnodactyly?

The prevalence of congenital contractural arachnodactyly is estimated to be less than 1 in 10,000 worldwide.

Can arachnodactyly be normal?

Considerations. Long, slender fingers can be normal and not associated with any medical problems.

Can you have arachnodactyly?

Congenital contractural arachnodactyly is a disorder that affects many parts of the body. People with this condition typically are tall with long limbs (dolichostenomelia) and long, slender fingers and toes (arachnodactyly).

What is arachnodactyly?

Arachnodactyly is a condition in which the fingers are long, slender, and curved. They look like the legs of a spider (arachnid).

What is congenital contractural arachnodactyly (CCA)?

Congenital contractural arachnodactyly (CCA) appears to comprise a broad phenotypic spectrum.

Is there a cure for arachnodactyly?

The common genetic disorders that affect the connective tissues causing arachnodactyly include Marfan syndrome, homocystinuria, and Ehlers-Danlos syndrome. Since arachnodactyly often is the result of genetic disorders, there are not any treatments that can cure this condition.