We are getting ready to adopt a child who was born in Puerto Rico but is currently living in an orphanage in Miami. The social worker told us he has something called Steel Syndrome. We still want to adopt this special needs child. But can you tell us a little more about this problem?

Steel Syndrome, named for the physician who first reported on this condition in 1993. It is a rare problem among Puerto Rican children. As the word syndrome suggests, each child with this diagnosis has the same features: bilateral hip dislocations, elbow dislocations, short height, scoliosis (curvature of the spine), fused wrists, and abnormally high arches of the feet.

The children have a similar look about them. Besides being short in stature, their faces are long and oval-shaped. The head is slightly larger than normal with a prominent forehead. The ears are small, low, and rotated backwards slightly. The bridge of the nose is broad. Mental (cognitive) abilities are reportedly normal.

Genetic studies have shown that it is probably caused by a genetic mutation. But the specific gene linked with the physical changes seen with this syndrome has not yet been identified.

It doesn’t appear that Steel syndrome develops more in one particular geographic location (place on the island) than another. Boys and girls were affected in equal numbers. And there was no intermarriage among the parents to account for this syndrome.

With the limited number of people affected by this syndrome, future efforts to unravel the genetic connection may be slow. But there is a group of physicians who have been following Dr. Steel’s patients. They have added a few more patients to the group who have been diagnosed with Steel Syndrome. Efforts are underway to follow and report on long-term treatment efforts for the combined groups.