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My brother's child (our niece) has been diagnosed with Steel Syndrome. We have another friend whose child has this condition, so we know a little bit about it. Our niece has both hips dislocated and may need surgery. They are asking us to help financially. We are willing to help contribute to the cause but wanted to find out more about the success of this operation. Does it work? Will she need more surgery later?
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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 (or very similar) features: bilateral hip dislocations, elbow dislocations, short height, scoliosis (curvature of the spine), fused wrists, and abnormally high arches of the feet.
At the time that Dr. Steel first studied this syndrome in 1993, there were 23 people identified with this syndrome. Since that time, another 14 people have been added to the group. A total of 32 patients from those two groups have been followed long-term by Dr. Steel's successors so we have a few results to offer.
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. Remember, that was back in 1993. According to an updated report this year (2010) there is new information that brings us up-to-date on those early patients as well as takes a look at the new patients found with Steel Syndrome.
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 of the newer report concluded 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.
With this information in mind, the family may want to seek a second opinion before committing to a surgical procedure. This may be one time when conservative (nonoperative) care is really best.
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