Beals Syndrome

Genetic syndrome characterized by multiple osseous dysplasia, ear anomalies, and short stature.

Auriculo-Osteodysplasia. Do not confuse with congenital contractural arachnodactyly, which sometimes is also referred to as Beals syndrome.

Two families with autosomal dominant inheritance have been reported. Ratio of females to males is approximately 1:1. The genetic defect has not been identified.

Dysplasia of the radiocapitellar joint with or without dislocation of the radial head, characteristically shaped ears with elongated and attached earlobes, and an associated small lobule beneath are the hallmarks of this syndrome. Hip dislocation is more frequent in these families. Females tend to have masculine torsos. All affected individuals have a slightly reduced body height. Intelligence is normal.

Based on the described features, no specific implications for anesthesia are expected. However, positioning the patient requires care to prevent luxation of the affected joints, especially with use of neuromuscular blocking agents.