Legg-Calvé-Perthes Disease is a condition that affects the hip in children between the ages of four and eight. The condition is also referred to as Perthes disease. It was named in honor of the three physicians who each separately described the disease. Boys are affected five times more often than girls. In 10 to 15 per cent of children with this disease, both hips are affected.
In this condition, the blood supply to the growth center of the hip (the capital femoral epiphysis) is disturbed, causing the bone in this area to die. Hip pain, limited hip motion, and a limp bring the child into the physician’s office for diagnosis. The blood supply eventually returns, and the bone heals. But how the bone heals determines how much problem the condition will cause in later life. This condition can lead to serious problems and even permanent deformity in the hip joint later in life.
Perthes disease results when the blood supply to the capital femoral epiphysis is blocked. There are many theories about what causes this problem with the blood supply, yet none have been proven.
There appears to be some relationship to nutrition. Children who are malnourished are more likely to develop this condition. Depending on the birth mother’s prenatal care and nutrition and conditions at the orphanage, there may be a link here for your child.
There is some new evidence that Perthes disease is genetic as a result of a mutation (abnormal change) in the type II collagen (fibers that make up soft tissue structures). Previously there was no known increase in risk for children whose parent had Perthes disease as a child. But this belief may no longer be accurate.
Studies among Asian families who have many members with this disease have been found with this mutation in the type II collagen gene. Scientists think that the mutation results in weakening of the hip joint cartilage that also affects the blood vessels within the cartilage.
These are all theories that remain to be fully investigated and proven. The important thing is to make sure your child is followed closely by your pediatrician and orthopedic surgeon.
Early treatment can often be accomplished with conservative (nonoperative) care. If there are signs of disease progression and hip deformity, then surgery may be needed. Your surgeon will be able to advise you on this. Decisions are made based on the age of the child, condition of the bone, presence of bone fragmentation, and loss of blood supply at the time of diagnosis. The younger the child (under age six), the more likely conservative care can be effective.