Hereditary Fibrosis of the Extraocular Muscles

Hereditary fibrosis of the extraocular muscles is an unusual condition. Many neurologists have never heard of it. It may be a source of puzzlement to ophthalmologists as well. Typically, a patient with this condition has ptosis; restricted extraocular movements even with forced ductions; and a hyperextended posture of the neck to compensate for the eye problems. There may be associated hyperopia; ambiyopia, or cataracts. At surgery for correction of ptosis and eye deviation, the extraocular muscle tissue is found to be partially or totally replaced by fibrous bands. Several families with affected members in 5 successive generations have been reported -- an autosomal dominant pattern of inheritance.