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.
Beals RK: Auriculo-osteodysplasia: A syndrome of multiple osseous
dysplasia, ear anomaly, and short stature. J Bone Joint Surg Am 49A:1541, 1967.