Larsen's syndrome

(Loren Joseph Larsen, 20th century, American orthopaedic surgeon), inherited disorder in which the children have a distinctive facies with a flat face, depressed nasal bridge and prominent forehead, without clefting abnormality. The main and dominant lesion is that of multiple joint laxity with multiple dislocations, particularly affecting the elbows, hips and knees. Sensineuronal or hearing loss is common secondary to abnormal ear anatomy. Radiologically, the joint dislocations are demonstrated. There are often also abnormalities of the metacarpals and metatarsals with spatulated thumbs. There may be a degree of micrognathia. Imaging of the ear will show the underlying ear anomalies. With progression of time, secondary degenerative changes occur at the affected joints. Inheritance is autosomal dominant but most cases are sporadic.

HC