Legg-Calve-Perthe’s Disease
Overview: Perthe’s ‘disease’ is an uncommon childhood condition that affects the hips which are ‘ball and socket’ joints. It occurs when the blood supply to the round head of the femur (thigh bone) is disrupted leading to death of bone cells culminating in Avascular Necrosis.
It is a complex process progressive in stages that can last several years.
The weak bone of the head (ball) of the femur gradually begins to break and collapse. With time, the blood supply to the head of the femur returns (revascularization) and the bone
begins an attempt to regrow and heal.
It generally occurs in children between 4 and 10 years old, more common in boys (five times) than in girls and if affected it is more likely to cause more damage to the bone. It is bilateral in 10% to 15% of all cases.
CAUSE of Perthe’s disease is not known. Few recent studies suggest possibility of a genetic link to the development of Perthe’s, but more research is required.
SYMPTOMS
The earliest signs of Perthe’s is a change in the way a child walks and runs (altered gait and limp), more apparent during sports. They have limited motion in the hip joint.
Pain in the hip or groin, sometimes ‘referred’ to other parts of the thigh or knee.
Pain that worsens with activity and is relieved with rest.
Painful muscle spasms that may be caused by irritation around the hip.
Symptoms are intermittent over a period of weeks or even months before a doctor visit is planned.
Timely precision diagnosis and treatment, can turn a painful hip in a growing child into a healthy hip that will last his life.
Impending pain, stiffness and inactivity is often associated with arthritis.
EXAMINATION
Physical examination tests.
Assess child’s range of motion in the hip. Perthe’s reduces the ability to move the leg away from the body (abduction), and twist the leg toward the inside of the body (internal rotation).
X-rays will show the condition of the bone in the femoral head and help the doctor evaluate the stage of the disease.
In this x-ray, Perthe’s disease has progressed to a early medial collapse of the femoral head (highlight).
Perthe’s hip can expect to have several x-rays taken over the course of follow up, which may be 2 years or longer. As the condition progresses, x-rays often look worse before gradual improvement is seen.
ADVANCED INVESTIGATIONS
Ultrasound will show fluid (effusion) in the joint in acute stage of pain
MRI confirms the diagnosis and the stage of the disease in a 3-dimensional image.
Bone Scans evaluate the current state of vascularity of the diseased head and has the ability to detect the disorder in its initial stages, thus allowing earlier treatment, and to provide prognostic information that may affect treatment.
TREATMENTS
Depends on the severity and complexity of the condition, age, overall health and the expectations for the course of the condition as a child grows.
The goals of treatment include:
⦁ preserving the round shape of head of the femur (thigh bone)
⦁ keeping the head of the femur in the socket (containment)
⦁ preventing deformity during its course
⦁ regaining hip motion
⦁ eliminating pain that results from the inflammation inside the joint resulting in tight muscles surrounding the hip
Treatment options depend on the degree of child’s hip pain, stiffness and x-ray changes over time — as well as how much of the head of the thigh bone has collapsed.
Legg-Calve-Perthe’s non-surgical treatment
Non-surgical approaches to treatment may include:
⦁ resting the joint (not total bed rest)
⦁ controlled restricted activity to limit joint movement
⦁ pain medication
⦁ bed rest and traction if required
⦁ casting or bracing to keep it ‘contained’ in the socket
⦁ allow the head to remodel itself into a round shape
⦁ physical therapy, to keep the hip muscles strong and promote hip range of motion
⦁ wheelchair or crutches (in some cases)
Legg-Calve-Perthe’s Surgical Treatment
If non-surgical treatments prove inadequate, child may need surgery to contain the head of the thigh bone in the hip socket. Surgery involves reorienting the affected bones (osteotomy) and stabilizing the realignment with screws and plates as required in children with more than 50% of hip damage in a child older than 8 years.
Long-term ‘outlook of Legg-Calve-Perthe’s?
The majority of children treated for Legg-Calve-Perthe’s disease have corrections that enable them to walk, play, grow and live active lives. Diagnosing and treating child’s Perthe’s disease early in its development greatly increase the likelihood of a successful outcome.
The two most important factors that determine the outcome are the child’s age (usually, the younger the better) and how much of the head of the thigh bone is affected by the condition. The more severe the case is, the greater the chance for the child’s hip motion to become limited and for him to have further hip problems later, including premature arthritis.
Legg-Calve-Perthe’s follow-up care
Periodic follow up by orthopedist until skeletal growth is complete is advised. He will monitor the hip joint over his whole growth period in order to last a lifetime. Most surgical operations for Legg-Calve-Perthe’s Disease occur without major complications. Hip problems can recur as the child grows even after successful treatment in childhood. In severe cases limited hip motion, different leg lengths and arthritis have a higher incidence.
References:
https://orthoinfo.aaos.org/en/diseases–conditions/perthes-disease