Orthopaedic Issues in Individuals with Down Syndrome: the hips

       There are four phases of hip instability ranging from the initial phase to the fixed phase, where the hip will remain dislocated.  In between that is the dislocation phase where the hip will move in and out of dislocation.  This phase usually occurs between 3 to 6 years of age.  The laxity of the ligaments can cause the hip to continually dislocate.  This can be heard by a persistent clunking noise during diaper changes or other activities.  These initial dislocations are painless, but they can eventually lead to other issues, such as pain, arthritis, and loss of the ability to walk.



       The 3 courses of treatment for hip instability are 1.  leave it alone.  2.  wait and see and do a hip replacement when necessary.  3.  or more aggressive treatment.  Most doctors have been going the "wait and see" route, because surgeries have shown to have a 50% failure rate in patients with Trisomy 21.  There has also been a high rate of infections after the surgery.  Children's Hosptial of Philadelphia, however, has worked to find better answers and possibilities for treating their patients with Down Syndrome.  After researching the cause of the hip instability, they found that patients with Down Syndrome have hips that are tilted backward, and they dislocate to the back.  They perform a procedure called "complete redirectional acetabular osteotomy", which provides more support and stability in the back of the hip.  They actually reorient the socket to prevent the it from slipping back.  The success rate has gone from 50% to 91% with this new technique.  That alone makes me LOVE CHOP!

       The other hip disorder is called a slipped epiphysis or SCFE.  This is common in adolescents, and it is higher is boys and the African American population.

 Factors that affect the SCFE are endocrine or horomone factor, overweight, and Trisomy 21.  The symptoms are hip, KNEE, and thigh pain.  Knee pain is highlighted, because it is a common complaint, even though it is actually the hip causing the problem.  The child may also have a limp or worse gait than usual.  The SCFE is often misdiagnosed as a groin pull.  Since patients with Down Syndome can often have a limp, it can be difficult to diagnose this problem.  The patient may not express pain, but their limp or walk will change.  These are all signs to watch out for.  Doctors find that treating the SCFE an be difficult, because the patient may not comply afterward with staying off of their feet to heal.  There is a high rate of bone death from a SCFE.



       Thinking of all of these things can be overwhelming.  I often wonder about Kamdyn, because her hips are very loose, as most children with Down Syndrome are.  I try to push away the worry, and just file these things in the back of my mind, just in case I need them one day.  Writing it out like this also helps me remember it. 

       I am really excited about my next post.  It's going to be about making a Care Notebook.  I am just getting started, but I am very excited about it. 

  A little sneak peek. 

Orthopaedic Issues with the neck and spine in Individuals with Down syndrome

       The most contributing factor to ortho issues in kids with Down syndrome is ligamentous laxity, also know as loose joints and ligaments.  In other words, we have some flexible kids!  The first area of concern is in the neck.  Laxities in the spine occur at different levels.  There can be abnormal motion between the head and the first vertebrae, between the first and second vertebrae (the most common), or lower in the spine.  Doctors use x-ray, CT scan, and MRI along with precise measurements to determine if the range of motion is in the normal range or not.



        Most cases in the neck are asymptomatic, meaning the patient does not feel uncomfortable.  Actually, on 1-2% of cases present with symptoms of discomfort.  The risk for this type of abnormality is neurologic or spinal cord damage.  There are rare and infrequent, 13 ever recorded, of a patient suffering sudden paralysis or death.  The treatment for this type of problem is either restricted activities or surgery.  If the range of motion is slightly above normal, the doctor will recommend restricting high risk activities, such as diving, gymnastics, boxing, football, ice hockey, rugby, soccer, wresting, and trampolines.

 This particular doctor actually said that he discourages trampolines for any child, because they are dangerous, and he often sees patients as a result of trampoline accidents.  When the range of motion, however, is too far above normal, the only course of action is surgery. 

       Cervical spine screening should be done after 5 years of age, because it is too difficult to read these tests in young children.  Also the bones of young children are not completely formed yet, and they will not be accurate before this age.  The American Academy of Pediatrics does not recommend cervical spine screening on the basis that there is no treatment for severe cervical spine abnormalities other than surgery.  The Special Olympics, however, requires that children do one before they are permitted to play sports.  Cervical spine screenings are also important to do before a child receives any anesthesia, because the neck has to be tilted back during anesthesia.  If the original results come back normal there is a low risk of developing instability later, although it is possible.  If x-rays show an abnormality in the spine, the test should be redone every 2-3 years.

       Another spinal issue that children with Down syndrome are at a higher risk for is scoliosis.  Scoliosis is a condition where the spine forms in the shape of an S.  The S shape curve of the spine will continue as the child grows. 

Scoliosis can occur for unknown reasons or as a result of cardiac surgery.  After open heart surgery, the ribs can stick together causing the spine to grow abnormally.  Children are screened at school, as well as the pediatrician.  Mild cases of scoliosis will be observed.  Braces can be used, but they are not shown to be successful treatment in children with Down syndrome. 

In severe cases, where the spine is curved over 50 degrees, and can affect the heart and lungs at 80 degrees, surgery is recommended.  Individuals with Down syndrome, however, are shown to have a higher rate of complications following the surgery. 






Next time: the hip