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Perthes Disease - Causes, Stages, Symptoms, and Treatment

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Perthes disease is a childhood hip disorder initiated by a disruption of blood supply to the head of the femur. Read this article to know more about Perthes disease.

Written by

Dr. Deepiha. D

Medically reviewed by

Dr. Anuj Gupta

Published At November 9, 2022
Reviewed AtNovember 9, 2022

Introduction:

The long bone of the thigh is called the femur. The upper portion of the femur bone contains a round, dome-shaped structure known as the head of the femur. The head of the femur articulates with a concave cavity (the acetabulum) on either side of the hip bone and forms the hip joint. It is a ball and socket type of joint surrounded by muscles, ligaments, and tendons. The hip joint's main function is to support the weight of the body. In addition, it helps with the wide range of movements of the lower limb.

What Is Perthes Disease?

Perthes disease is a hip disorder affecting the hip joint. The disease was initially thought to be associated with tuberculosis (TB). It was in 1910 that the disease was recognized as a separate entity by three physicians working independently. Their names were Arthur Legg, Jacques Calve, and Georg Perthes. Hence, this disease is also called Legg-Calve-Perthes disease, named after these three physicians.

Perthes disease is most common in children aged five to ten years. Boys are more prone to develop this condition, but the severity of the disease is high in girls. In a few cases, both the hip joints are affected. The other names of Perthes disease are coxa plana, pseudocoxalgia, osteochondritis deformans coxa juvenilis, and osteochondrosis capital femoral epiphysis.

Which Bones Are Affected by Perthes Disease?

The femur (thigh bone) is the only bone affected by Perthes disease. In children with Perthes disease, the round spherical head of the femur, which articulates with the hip socket, stops growing. This is due to the disruption of blood supply to the femur head, and as a consequence, the bone cells die (avascular necrosis) by the lack of oxygen supply.

Over the course, the healing of the bone occurs by new penetrating blood vessels which remove the dead bone cells. As a result, there is a loss of bone mass and weakening of the femur head. Ultimately the bone loss leads to the collapse of the femur head with permanent deformity in the shape of the bone.

What Are the Causes of Perthes Disease?

The exact cause of the disease is idiopathic (unknown reason). Both the developmental and environmental factors contribute to disease development. Some proposed hypotheses are,

  • Inherited protein C or protein S deficiency (factors that help in blood clotting).

  • Blood clotting in the veins.

  • Block or abnormalities in the arteries.

  • High pressure inside the bone tissue.

  • Infection in the joint space.

  • General bone disorders.

Another most accepted theory says that the femoral head blood supply is compromised after the age of 5 years. Normally, the femoral head is supplied by three arteries and in natural course of development, one of the arteries get subdued after the age of 5 years. The blood supply is restored after the age of 10 years. Hence, the child is most prone to develop Perthes between 5 to 10 years of age.

What Are the Stages of Perthes Disease?

The condition can be categorized into four stages depending on the evolution of the disease. They are;

  • Stage of Avascular Necrosis:

This is the initial stage of the disease. There is a lack of blood supply to the femur head, and the upper-end part of the bone is necrosed (dead). The space between the femur head and the hip bone socket is increased. This stage may last for several months.

  • Stage of Revascularization or Fragmentation:

In this stage, there is ingrowth of new blood vessels and tissues. The dead bone cells are removed and replaced by immature bone tissues. Thus, the bones are very weak in this phase. It is at this stage that there is collapse and loss of structural integrity of the femoral head leading to deformation. The femoral head may extrude from the hip socket.

  • Stage of Ossification or Healing:

New bone starts forming in this stage. The re-ossification stage is the longest stage that may exist for a few years.

  • Remodeling or Residual or Healed Stage:

During this stage, complete healing is done, and depending on the severity of the disease, the residual shape of the femur head may be spherical or distorted.

What Are the Symptoms of Perthes Disease?

Characteristic presentations of Perthes disease are:

  • Limping but without pain.

  • Pain may be present along with limp in some cases.

  • Difficulty in spreading the legs away.

  • Altered walking style.

How to Diagnose Perthes Disease?

  1. Clinical Examination: Physicians may check the movements of the hip joint.

  2. Imaging Studies: An X-ray of the pelvis which includes both hips is the most appropriate view. The opposite hip is required for comparison. A lateral view should also be obtained to look for the position of the femur head in the acetabulum. With the easy availability of magnetic resonance imaging (MRI), early diagnosis of Perthes disease is possible even when X-rays are normal.

How to Treat Perthes Disease?

The goal of Perthes disease treatment is to prevent deformation of the femoral head, prevention of stiffness and maintenance of a good range of movements, and prevent or correct the growth disturbances.

Containment of the femoral head within the hip socket helps to prevent deformation as the socket acts as a mold to keep the head spherical. This can be done in two methods,

Conservative Method:

  • Medication: Painkillers are recommended to reduce the pain.

  • Rest and Weight Relief: This method was primarily directed at avoiding weight by bed rest for a prolonged period (up to two years).

  • Supporting Devices: Casting and bracing are used to temporarily contain the femur head within the hip socket until definitive treatment is undertaken. During the developmental stage, the casting helps to mold the femur head to a normal position. Petri cast is a type of casting that has two long leg cats that hold the legs apart and are supported by a bar in the middle. The application of the cast can be performed under the guidance of an arthrogram (a small amount of dye is injected into the hip joint, and a series of special X-rays are made).

Surgical Method:

Osteotomy is the most common and preferred surgical procedure to treat after effects or deformity caused due to Perthes disease, where the bone is cut and realigned or repositioned such that the femur head fits within the acetabulum.

  • Femur Osteotomy: A piece of bone is removed from the femoral shaft, and the bone is pushed into the hip socket minimizing the shortening of the bone.

  • Pelvic Osteotomy: In this method, the hip bone is altered to reorient the socket or create a bony shelf for the femur head.

Conclusion:

Perthes disease can affect the child’s normal living. The treatment outcome depends on the age of the patient, the severity of the condition, and the extent of movement at the hip. In most cases, the treatment outcome is good, and children may have normal adulthood without further hip problems. In a few cases, there will be persistent hip pain and a high risk of developing arthritis during adulthood.

Frequently Asked Questions

1.

Does Perthes Disease Have a Cure?

However,  Perthes disease can benefit from therapy. Most youngsters may resume their usual activities without restrictions after two to five years of therapy or supervision. Young children with  Perthes disease under six have a very good prognosis with only observation, but it can take the femoral head two to five years to regenerate and go back to normal or very near to normal.

2.

Which Treatment Is Most Effective for Perthes Disease?

The initial line of treatment for kids with mild  Perthes disease symptoms is normally physical therapy, which usually starts as soon as a kid is identified. Physical therapy can assist in restoring the hip joint range of motion, minimizing pain and discomfort, and safeguarding the joint as it recovers.

3.

Is Perthes Disease a Chronic Condition?

Perthes is a complicated process with stages that can span years. The weakened head of the femur ("ball-and-socket" joint of the hip) progressively gives way as the situation worsens. Most kids with  Perthes disease eventually make a full recovery. However, it can take the femoral head two to five years to grow and go back to normal or very near to normal.

4.

What Age Group Is Perthes Disease Most Likely to Affect?

Although it can affect kids of almost any age,  Perthes disease often develops between the ages of four and ten. Boys are nearly four times more likely than females to have  Perthes disease. Due to this  Perthes disease, the blood flow to the thigh bone's head is cut off, which damages the bone. This may result in hip joint discomfort, limping, and restricted movement.

5.

How Is Perthes Disease Contracted in Children?

Perthes disease develops when the hip joint's ball section receives insufficient blood supply. This femoral bone (thigh bone) becomes brittle and prone to fracture without enough blood supply. Unknown factors are still causing the sudden reduction in blood flow to the femoral head.

6.

Is Perthes an Impairment?

Perthes disease is often a temporary disability that can be treated successfully when detected early in childhood. The majority of the time, this illness has a fair prognosis and does not cause any deformities in adulthood. Rarely, though, failing to heal the bone on time can force the use of walking assistance, turning the condition into a handicap.

7.

Does Perthes Require Surgery?

Core decompression surgery is only advised in the initial stages of  Perthes disease before the femoral head has compressed or flattened and no longer fits precisely into the hip socket, also known as the acetabulum. On average, the hip joint recovers from core decompression surgery in two to three months. However, if bone grafting was done, it could take longer.

8.

Is Perthes Disease Hereditary?

The condition is more likely to affect children whose parents already have it. Approximately two to ten percent of kids with  Perthes disease have at least one affected family member. These situations, known as family instances, occur when an afflicted person inherits the mutation from one compromised parent.

9.

Can a Person With Perthes Disease Participate in Sports?

Yes, a person with  Perthes disease can participate in sports. Children with  Perthes disease are advised to refrain from high-impact sports like running and leaping until the hip joint has healed. Most kids who have  Perthes disease recover, although it can take up to two years for the bone to grow back and become normal.

10.

Does Perthes Disease Have an Impact on Height?

The development of the top end of the femur  (thigh bone) will slow in all patients with  Perthes disease. This may lead to a clinically important or troubling disparity in leg length. Growing data suggest that children with  Perthes disease may have a more extensive skeletal condition characterized by moderate low stature, disproportional growth retardation, and delayed bone marrow maturation.

11.

Is Perthes a Serious Condition?

There is no urgent need for inpatient care for  Perthes disease. To start, thorough monitoring is necessary to ascertain the level of necrosis. When treating children under six, observation may be a part of the treatment. Exercises to extend the flexion are advised, as well as bed rest.

12.

Is Obesity a Factor in Perthes Disease?

Perthes disease is thought to have obesity as a clinical risk indicator. Leptin (a hormone that the body fat secretes that helps in long-term weight maintenance) controls body weight and adipose tissue mass. It is largely released by the body's primary energy storage locations, which play a crucial role in endocrine function (adipocytes). Furthermore, there is little doubt that obesity is linked to higher leptin levels.

13.

Which Hip Disorder Is Most Common in Perthes Disease?

Children with  Perthes disease are more likely to grow up with hip arthritis (swelling or inflammation of one or more joints), especially if the hip joint heals in an unusual form. Children with  Perthes disease, a hip condition, are often between the ages of four and eight. The greatest weight-bearing joint in the human body is the hip. The upper leg may move thanks to a ball and socket joint in this area. Both hips are often affected by  Perthes disease.

14.

What Are the Subtypes of Avascular Necrosis?

Two types of avascular necrosis primarily affect children. The first is known as  Perthes disease, idiopathic osteonecrosis (an inflammatory condition that results in femoral head bone degeneration) of the femoral head, and the second is osteonecrosis in youngsters linked to a slipping capital femoral epiphysis.
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Dr. Anuj Gupta
Dr. Anuj Gupta

Spine Surgery

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