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  • Shaken Baby Syndrome

    Tuesday, 21 July 2015 16:28
  • Amniotic fluid problems

    Thursday, 14 May 2015 12:54
  • Choosing a pre-school

    Friday, 10 April 2015 17:50
  • Newborn reflexes

    Tuesday, 03 March 2015 15:49
  • Mastitis

    Tuesday, 03 March 2015 15:41
  • Pelvic floor exercises

    Wednesday, 11 February 2015 17:20
  • Colic

    Wednesday, 11 February 2015 17:11
  • Antenatal Classes

    Monday, 03 June 2013 09:34
  • Strap-in-the-Future

    Thursday, 30 June 2011 13:52

Perthes’ Disease

perthesPerthes’ disease is a rare disorder of the hip joint in children. The condition is characterized by the temporary loss of blood flow to the hip area, resulting in irritation and inflammation. The disease particularly affects the femoral head, the rounded top of the thigh bone (femur), which sits inside the hip socket (acetabulum). Due to the lack of blood supply, the bone cells in the femoral head die, becoming soft, and the bone can fracture or become distorted. This process is called necrosis.

As the child grows, the blood vessels regrow and the blood supply returns to the affected area. This process is similar to the healing that occurs after any normal break or fracture to the bone. However, in the case of Perthes’ disease, the regrowth and remodeling of the bone can take several years. The primary goal of treatment is to ensure that the femoral head forms a good rounded or spherical shape. It is essential for the femoral head to fit into the hip joint, to ensure mobility and prevent wear and tear.

Perthes’ disease is also known as Calvé-Legg-Perthes disease, after the three separate doctors who initially described it in 1910. Although the term “disease” is used, Perthes’ is a complex process consisting of several phases. The disease generally affects children between the ages of 3 and 11. Perthes’ disease is more common in boys than girls, with a ratio of 4:1. In most cases, the disease only affects one hip but can occur bilaterally (in both hips).  If both hips are affected, it is usually at separate times.

Cause

The exact cause of the blood vessel problem in the femoral head is unknown. Perthes’ disease is not due to injury or a general blood vessel problem. Despite pain and limping, children with the condition are otherwise healthy. 

Phases

  1. Initially there is a loss of blood flow to the ball-shaped end of the upper thigh (femoral head). This causes the ball to soften and collapse (Initial phase: 6-12 months).
  2. As bone death occurs in the ball of the hip, the ball develops a fracture of the supporting bone. This fracture signals the beginning of bone reabsorption by the body (Reabsorption phase: 1 year or more)
  3. As bone is slowly absorbed, it is replaced by new tissue and bone (Reossification phase)
  4. Healing occurs when the new bone reshapes (Healing phase)

Stages 3 and 4 may go on for many years.

Symptoms

Symptoms tend to occur gradually and may include:

  • Limping that becomes worse and more frequent over a period of weeks or months
  • Pain in the hip or groin area
  • Referring pain may also be experienced in other parts of the leg, such as the knee or thigh
  • The pain tends to be worse when weight is placed on the leg or if the hip is moved
  • Stiffness and reduced range of movement in the affected area
  • Irritation around the hip area can result in muscle spasms
  • In time, the affected leg may become slightly thinner due to the wasting of muscles in the upper thigh
  • The affected leg may appear shorter than the unaffected one

Diagnosis

In the early stages, Perthes’ disease may not be detected on an x-ray. As a result, your doctor may request a bone scan, MRI or ultrasound scan to make a definitive diagnosis.

X-rays can show if the femoral head is broken or damaged. A healthy femoral head looks rounded in the hip socket. In the case of Perthes’ disease, it has a flattened or mushroom-like appearance. It no longer fits perfectly in the socket. X-rays are taken of both hips to make a comparison and are done every few months, to monitor the progression of the breakdown and healing, as new bone is made and reformed.

Other scans provide a more detailed picture and assess the extent of the damage to the femoral head.

Blood tests, or a sample of fluid from the hip joint, may be required to rule out other problems such as infection.

Treatment

Treatment depends on the age of the child and the severity of the condition.  Treatment of Perthes’ may require periods of immobilization or limitations on usual activities. The main concern is that the hip joint heals and reforms correctly, to reduce hip irritability, to prevent the ball from extruding or collapsing, and restoring and maintaining hip mobility.

If children are under the age of 5 and if the hip joint is only mildly affected, a “wait and see” approach may be adopted. Any advice will be tailored to meet the child’s needs. Bed rest or crutches may be recommended to relieve the hip joint from weight-bearing movements. High impact activities such as running and jumping should be avoided until the hip joint has healed. Observation, together with physiotherapy and home exercises, monitor healing and ensure that the hip joint is mobile and in the correct position in the socket. In some cases, bed rest in traction may be needed.

If the condition is more severe or the child is older, a plaster cast or special brace may be used to restrict some movements but still allow for weight bearing and walking. These devices aim to keep the leg slightly abducted and help return range of motion and mobility. Casts or braces may need to be worn for many months.

In severe cases, surgery may be required to realign the bones of the hip. The alignment is kept in place with screws and plates that need to be removed at a later stage. In some cases, the socket needs to be made deeper because the femoral head grew outside the socket during the healing process. This surgical intervention, allows the femoral head to fit snuggly in the socket and optimizes hip function. If the muscle has shortened due to limping, a procedure known as a tenotomy may be performed, to release the atrophied muscle. After any surgical procedure, the child is usually placed in a cast from head to toe for 6-8 weeks. This is followed by physical therapy to increase the range of motion and support the hip during the final stages of healing.

Anti-inflammatory medications may be prescribed to relieve pain and reduce inflammation of the hip joint or synovium (sleeve of tissue surrounding the hip joint).

Risk factors

Possible risk factors include:

  • Children who are small for their age
  • Children who are extremely active
  • The disease is found more frequently in Asians, Eskimos and Whites than those of other descent
  • There is a link between exposure to second hand smoke and Perthes’ disease

Prognosis

In most cases, the long-term prognosis is good. However, it may take from two to five years to repair the damaged bone. If the femoral head is not seriously deformed, normal hip function will return. The outcome is dependent on several factors, including the child’s age, gender and the severity of the condition. Younger children have a better prognosis because there is sufficient time for the hip to remodel before they finish growing. Children, who develop the disease after the age of 8 or 9, have less time to grow and therefore have a greater risk of permanent hip joint problems, such as premature arthritis or persistent stiffness. Boys tend to fare better than girls.

Emotional support

Recovery from Perthes’ disease is a slow process. Children may feel frustrated and anxious because they want to lead the same active lifestyles as their peers. Be understanding and supportive of your child’s feelings. Help them find non-weight bearing activities that they enjoy. Swimming and gentle cycling are possible options. Reassure your child that despite the lengthy process, they will eventually be able to resume their normal activities. After 18 months to 2 years of treatment, most children return to normal activities without major limitations.

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