Perthes disease, also known as Legg-Calvé-Perthes disease, is a rare childhood condition affecting the hip joint. It involves a temporary disruption of blood supply to the femoral head, the ball at the top of the thigh bone. While it primarily manifests in children, its effects can persist into adulthood, influencing mobility and overall well-being.
Understanding Perthes Disease in Childhood
Perthes disease affects the hip in children, typically between ages 4 and 10, though it can occur from 2 to 15. It is more common in boys, affecting them four to five times more frequently than girls. The condition begins when blood supply to the femoral head is temporarily interrupted, leading to avascular necrosis. This causes the weakened bone to break down and lose its spherical shape.
The disease progresses through stages: initial necrosis, fragmentation, reossification, and healing. During fragmentation, the weakened femoral head can collapse and flatten. Over time, blood supply returns, and the bone regrows and remodels. The final shape of the femoral head, influenced by age at onset and damage extent, determines the long-term outcome.
Adult Health Implications
Adults who had Perthes disease as children often face ongoing health challenges due to the altered shape of the femoral head and hip socket. A common consequence is premature osteoarthritis, where protective cartilage on bone ends wears down. This occurs because the hip joint may not fit optimally after healing, leading to abnormal wear. Osteoarthritis can cause chronic pain, stiffness, and reduced range of motion in the affected hip.
Chronic pain is common, often interfering with daily activities and sleep. The pain can be localized to the hip or groin, or sometimes felt in the thigh or knee. Limited range of motion is also common, making movements like internal rotation and abduction difficult. Some adults may also experience a limb length discrepancy, where the affected leg is shorter by typically 1 to 1.5 cm, impacting gait and posture.
As osteoarthritis progresses, individuals may require hip replacement surgery (total hip arthroplasty). While effective in reducing pain and improving mobility, this procedure can be technically challenging for surgeons due to unique hip joint deformities from Perthes disease, such as a misshapen femoral head or shallow socket. About 22% of adults who had Perthes disease reported having at least one total hip arthroplasty, and 30% had some type of hip surgery in adulthood.
Navigating Life with Adult Perthes
Managing the adult implications of Perthes disease involves a multi-faceted approach to reduce pain, preserve joint function, and improve quality of life. Pain management often includes over-the-counter medications like acetaminophen or ibuprofen, and sometimes stronger prescriptions. Non-pharmacological approaches, such as heat therapy, can also provide relief.
Physical therapy maintains hip mobility and strength. Tailored exercise programs focus on gentle stretching, strengthening hip muscles, and promoting proper gait. Low-impact activities like swimming and cycling are encouraged to preserve joint function without excessive hip stress.
Lifestyle adjustments are often necessary. This includes avoiding high-impact activities like running and jumping, which can exacerbate joint wear and pain. Maintaining a healthy weight reduces pressure on the hip joint. Assistive devices like canes or crutches may be used to reduce weight-bearing stress and improve stability.
Surgical interventions, such as hip preservation surgery, may reshape the femoral head or improve its coverage by the acetabulum, aiming to delay or prevent osteoarthritis progression. When degenerative changes are extensive, total hip replacement becomes an option, significantly improving pain and mobility despite surgical complexities. Post-surgery, physical therapy is crucial for recovery and regaining function.
Long-Term Trajectory and Quality of Life
The long-term outlook for adults who had Perthes disease is variable, depending on the extent of femoral head deformity from childhood healing. While many individuals can lead active lives, some experience ongoing symptoms and limitations. A study indicated that by age 30, approximately one-third of affected individuals have no symptoms, one-third experience intermittent hip pain, and one-third develop arthritis requiring treatment.
Ongoing medical care is often needed to monitor joint health and manage symptoms as they evolve. Patient-reported outcomes suggest that adults with a history of Perthes disease may experience worse pain, physical, mental, and social health compared to individuals without the condition. Despite these challenges, appropriate management strategies can help individuals maintain functionality and a reasonable quality of life, allowing them to participate in many activities.