Perthes Disease

Children with Perthes' disease most commonly present with an insidious onset of a limp without a history of previous trauma. If pain is present, it is usually localized in the groin, the thigh or the knee - particularly after physical activity.

Perthes' disease, also known as Legg-Calve-Perthes disease (LCPD), is a condition affecting the hip joint in children, typically one side in boys between the age of 4 and 8 years. It is rare (9,3 in 100000 children are affected) and the etiology of the disease is still unknown.

The blood supply to the head of the femur is temporarily disrupted either partially or totally leading to bone death and eventual misshaping of the femoral head during the healing process. This may result in long-term joint problems such as arthritis of the hip in later years if left untreated.

Children with Perthes' disease most commonly present with an insidious onset of a limp without a history of previous trauma. If pain is present, it is usually localized in the groin, the thigh or the knee - particularly after physical activity.

The symptoms can vary in severity and may be mild and self-limiting initially which is why many patients do not present for medical attention until months after the clinical onset of the disease.

The symptoms nevertheless progress slowly over time leading commonly to a restricted range of movement (stiffness) of the hip joint, wasting of the muscles in the affected leg and eventually shortening of the affected leg over time. These symptoms may persist on and off for months to years. The disease itself lasts for a few years.

Diagnosing Perthes disease typically involves a combination of thorough medical history, physical examination and imaging tests with the key to right early diagnosis being a caregiver with clinical experience and good knowledge of the various conditions affecting the hip during childhood.

Physical examination includes not only assessment of the hip joints range of motion, pain and other abnormalities but focuses on the whole child as a person to not miss other medical conditions and be able to provide the best treatment plan with the wellbeing of the child being the highest priority.

Imaging tests as X-Ray usually suffice for the diagnosis and may need to be repeated after 3-4 months if the initial examination is normal. In a few selected cases other modalities such as magnetic resonance imaging (MRI), or computed tomography (CT) scans may be ordered to confirm the diagnosis and determine the extent of the damage to the hip joint.

Follow-up care is an important part of diagnosing and managing Perthes disease. The child's progress will be closely monitored through regular appointments with the children orthopedic specialist. Additional imaging tests may be ordered to assess the progression of the disease and the effectiveness of the treatment plan.

The treatment of Perthes disease aims to reduce pain, preserve and improve hip joint function, and prevent long term complications. The treatment plan may vary based on the severity of the disease and the child’s age and overall health.

Most children with Perthes' disease recover without any operative treatment with the prognosis being the best for younger children. It is important, however, for all children to be carefully followed up by the orthopedic surgeon during the course of the disease. They usually have to attend clinic every 3-6 months for examination and X-rays. In that way, the orthopedic specialist can detect any potential issues early and identify the children that are at risk of doing less well and treat them accordingly.

Long-term follow-up care is also important as some children may develop long-term complications, such as arthritis or hip deformities, later in life. The follow up plan is always individualized but follows the general recommendations of the Swedish Pediatric Orthopaedic QualityRegistry (SPOQ) for Perthes disease with the parent consent.

Non-surgical treatment options may include activity modification when the disease is active (avoid high impact activities such as running and jumping and contact sports), rest and physical therapy to strengthen the muscles around the hip joint and improve the range of motion. Anti-inflammatory and/or pain medications may also be used to manage symptoms.

In some cases, surgical intervention, such as an operation to re-shape the bone around the hip joint, may be necessary to improve hip joint function and prevent long term complications. When considering treatment, a procedure called arthrogram may be necessary to determine the best type of operation.

In a small number of severely affected children where the shape of the hip joint is greatly altered by the disease, the symptoms of pain and stiffness persist for years even though the disease is no longer active. These children may require additional operations in childhood or early in adulthood.