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Hip Surgery May Not Be Advised for Children with Steel Syndrome

Posted on: 04/21/2010
It doesn't happen very often that someone with hip dislocations is told it is best to leave well enough alone -- in other words, don't operate, just leave the hips be. But that is the case for a group of Puerto Rican people with Steel Syndrome.

Steel Syndrome, named for the physician who first reported on this condition 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, 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.

At the time that Dr. Steel first studied this syndrome, there were 23 people identified with this syndrome. Since that time, another 14 people have been added to the group. A total of 32 of those two groups were followed long-term by the authors of this article who bring us the results of treatment.

When Dr. Steel did the original study, he found that efforts to surgically reduce the dislocated hips (put them back in the socket) were unsuccessful. That was back in 1993. This report brings us up-to-date on those early patients as well as takes a look at the new patients found with Steel Syndrome.

But first, let's take a look at what is known about Steel Syndrome. As mentioned, it is found among a particular ethnic group (Puerto Ricans). 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.

The main focus of this new study was the results of treatment for the hip dislocations. What makes the type of hip dislocations in people with Steel syndrome different from other syndromes is the fact that the joints are not hyperlax (super loose or flexible). This unique clinical feature makes this syndrome of interest to orthopedic surgeons.

Although surgical treatment for the scoliosis was done for one-third of the group, the results of those surgeries will be evaluated and reported separately. For those with elbow dislocation, limited motion and a funny looking bump because of the dislocation were the main problems. Treatment improved the cosmetics (how the elbow looks) but didn't improve the elbow function. The wrist and foot problems didn't bother any of the patients and therefore didn't require any treatment.

So that brings us back to the issue of the dislocated hips. Comparing patients who had surgery to try and correct the dislocation with those who had no surgery, the group with untreated dislocations had better results. That was true no matter what type of surgery was done (e.g., closed reduction with spica cast, open reduction, osteotomy, skeletal traction, Pavlik harness).

Failure was reported for all patients who had surgery to reduce the dislocated hips. The hips remained either subluxed (partially dislocated) or fully dislocated. Patients in the untreated group had less pain, more function, and less disability. There were also fewer emotional and behavioral problems among the children who had untreated hip dislocations. School performance was better for the untreated group with fewer limitations all the way around.

The authors conclude that Dr. Steel was right when he recommended against treatment for hip dislocation in Puerto Rican children with Steel Syndrome. It may seem like unusual advice from a surgeon, but the evidence available so far does not support efforts to reconstruct the hips in this particular group of children.

John M. Flynn, MD, et al. Steel Syndrome: Dislocated Hips and Radial Heads, Carpal Coalition, Scoliosis, Short Stature, and Characteristic Features. In Journal of Pediatric Orthopaedics. April/May 2010. Vol. 30. No. 3. Pp. 282-288.

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