Here I answer the most frequently asked questions that I always hear from parents of children who have just been diagnosed with Perthes disease.
Based on my educational knowledge as a diagnostic radiographer, but also my in-depth reading in medical scientific publications.
Happy reading!😃
Have any questions, remarks, or experience to share? Feel free to use the comments section at the end of the article!🙏
Last updated: Oct 2024. Written by Juliet Semakula, diagnostic radiographer.
Disclaimer: no affiliate links
Summary: Most children with Perthes’ disease eventually recover, but it can take anywhere from two to five years for the femoral head to regrow and return to normal, or close to normal.
▶️What do we know about Perthes disease in children?
Perthes’ disease also known as (Legg-Calve-Perthes disease or coxa plana).
It’s a rare condition that affects children between the ages of 3 and 12 years old. With the highest rate of occurrence at 5 to 7 years
Perthes disease occurs when too little blood reaches the ball portion of the hip joint (proximal femoral epiphysis) for a short time. which is called ischaemia.
Without enough blood, this bone becomes weak and collapses which sometimes can be the irreversible death of body tissue or cells which is called necrosis.
When the blood supply to the head of the thigh bone is disrupted, the bone will start to deteriorate.
The cause of the reduced blood flow is unknown. Many hypotheses have been put forward, but none has been proven.
▶️What really causes Perthes disease.
The causes of Perthes’ disease as reported in different literature varies widely between and within countries. The origin of the disease is still unknown.
Perthes has been attributed to:
🔵Gender: It is 3 to 4 times more common in boys than girls.
🔵Social economic environmental factors are often implicated. They may include socio-economic deprivation and passive smoking.
Johansson 2017:
🔵Genetic factors in either causing the disease or increasing the vulnerability of an individual.
One article had a possible hypothesis that Perthes may be related to a polymorphism in the gene encoding endothelial nitric oxide synthase (eNOS), which is associated with lower plasma levels of nitric oxide.
Zhao et al .2016.
▶️What signs and symptoms you may notice with your child.
🟠Pain: Your child will start feeling pain which intensifies during and after physical activity. The pain can radiate into the thigh and knee.
🟠Limping or lameness when they walk which is usually the main sign they go to the doctor for consultation.
🟠Limited movement of the hip joint.
🟠Severe morbidity
▶️Diagnosis of Perthes disease
♦️Physical examination:
When you go to the doctor, a thorough physical examination will be done examining the range of movement of the hip and any symptoms such as pain.
♦️X-ray
Then a follow up X-rays will be requested to look at your child’s bones. X-rays can show evidence of Perthes syndrome, as well as the stage and severity of the disease.
X-ray images showing an AP and flag leg projections of a 6-year-old pelvis with limited range of movement in the right hip.
♦️MRI: In some cases, your doctor will order an MRI (magnetic resonance imaging) to see certain features in more detail if needed.
♦️Blood tests will be done to rule out infection or other conditions.
▶️Why is it important for Perthes femoral head to regrow?
The goal of all forms of treatment is to prevent deformity of the femoral head and incongruence of the affected hip.
Here are 4 reasons why it is beneficial for your femoral head to regrow when it is affected with Perthes.
1️⃣To prevent deformity of the femoral head and incongruence of the affected hip.
2️⃣Reduce pain and discomfort.
3️⃣Maintain your body structure and blood flow to the hip joint
4️⃣Regain your full mobility and the ability to do things.
Good femoral head regrow is what allows you to regain 100% of your abilities and to avoid future complications.
▶️How long does it take for Legg calves Perthes femoral head to regrow in children?
Delving through different research, people have been trying to better understand the stages of Legg calve Perthes regrows, and how long it takes.
Here are the main 4 stages of research that help estimate this.
Each stage of Perthes disease has key indicators, which signifies the progression of the disease through the various stages
Phase1️⃣:Onset stage,
Here the blood supply is absent to the femoral head which causes the hip joint to become inflamed and stiff.
You may start feeling mild pain and sometimes x-ray may appear normal. This stage lasts a few weeks to months.
Phase2️⃣: Fragmentation stage.
Here the body starts absorbing the dead bone attempting to repair itself and form new bone.
On x-ray the bone will appear Swiss cheese with patchy holes. This stage lasts 6 months to a year.
Phase 3️⃣: Reconstitution stage:
Here the bone begins to fill in the holes of the ball. On x-ray it will appear white, and the shape of the ball may improve at this stage.
This stage becomes present at 18 months, and it can take 2 to 3 years for the entire ball to be filled with new bone.
Phase 4️⃣: Residual or healing stage:
Here the ball has been replaced with bone. When the ball is round, and the shape of the upper thigh bone is normal, long-term outcome is very good and no arthritis is expected in most patients.
It is important to identify the symptoms of perthes’ and get treatment when the child is still young because it helps to prevent the ball from becoming very flat.
▶️Do you grow out of Perthes disease?
Most children with Perthes’ disease recover completely. It may take 2 to 5 years for the body to repair the damaged bone.
If the femoral head is not seriously deformed, normal hip function will return.
About 60 percent of children with Perthes’ disease recover without any treatment.
It is important, however, for all children to be carefully followed up by their doctor during the disease. They usually must attend a clinic every 3-4 months for examinations and X-rays.
Caution!⚠️However Excessive deformity may lead to continued stiffness and early development of arthritis in the hip.
▶️What is the best treatment for Perthes disease?
There is no such specific treatment for Perthes disease. The best treatment will be evaluated on an individual case based on the disease progression, age, health, and severity.
The therapeutic spectrum ranges from observation and follow-up all the way to extensive surgical reconstructions of the hip.
Usually, treatment options consist of:
Conservative treatment
♦️Constant monitoring may at first consist of no more than mechanical stress reduction and further observation. This is usually possible only for children under age 6 who have a good range of motion of the hip.
♦️Anti-inflammatory medication to help with pain.
Pain treatment in the initial stages of the disease when inflammatory is present, the joint should be moved as little as possible.
A non-steroidal anti-inflammatory drug such as ibuprofen is usually given (Nelitx 2009.)
♦️ Physical therapy to keep the hip muscles strong and promote hip movement.
️♦️ Bracing: using a hip abduction bracing or casting to hold the head of the thigh bone in the hip socket and approve limited joint movement.
♦️Bed rest is part of the treatment process to help with the healing process.
Surgery options
If the disease takes an unfavourable course, or if conservative therapy fails, a few operative methods to help improve containment may be indicated.
♦️Acetabuloplasty for reducible hinge abduction.
♦️Arthrogram
♦️ Proximal femoral valgus osteotomy
A case with a satisfactory outcome after shelf acetabuloplasty for reducible hinge abduction
(A) A radiograph of an 8-year-4-month-old boy shows a subluxated femoral head in the fragmentation stage.
(B) Arthrograms suggest reducible hinge abduction; the labral position is assessed as uncomfortable
(C) but as comfortable (>35° in abduction).
(D) An abduction cast with tenotomy of adductor and psoas
(E) Was followed by shelf acetabuloplasty.
(F) A radiograph taken at 6 years and 10 months after surgery shows a round femoral head with Stulberg type 2 deformity,
Result shows a suggestive of satisfactory hip remodelling with increased acetabular depth growth and prevention of hip subluxation.
A case with a satisfactory outcome of a restoration of containment by varus osteotomy and salter’s pelvic osteotomy after “hinge abduction” has been ruled out intraoperatively. using screws and plates to try to reline the bone at the fracture site.
Restoration of containment by means of varus osteotomy (left) or Salter’s pelvic osteotomy (right), after “hinge abduction” has been ruled out intraoperatively.
During the entire course of treatment, the main treatment goal is the attention of free mobility of the hip in a free rotation and abduction, with maintenance of the central position of the femoral head in the acetabulum.
▶️Is Perthes considered a disability?
If your combination of limitations would make you unemployable, then you can get Social Security Disability due to your Perthes disease.
Social Security Disability Insurance (SSDI) or “Disability” provides monthly payments to people who have a disability that stops or limits their ability to work.
You will be classified as disabled if your combined symptoms keep you from being able to do any job 8 hours a day, 5 days a week, week in and week out.
If you live and work in the United Kingdom, you can apply through the benefit and financial support.
▶️Can Perthes disease cause problems later in life?
Adults who experienced Perthes as children may present with a range of deformities around the hip joint, including:
🟣femoral-acetabular impingement (FAI).
🟣 Labral, cartilage and ligament tears.
🟣Some patients will end up with painful arthritis at some stage in adult life and may require a hip replacement.
🟣In a small number of severely affected children, the symptoms of pain and stiffness persist for years even though the disease is no longer active.
These may contribute to instability and associated loss of movement and function. Always being a constant distraction to your daily activities.
▶️Recommendations for physiotherapy and physical activity for children with Legg Calve Perthes disease.
Restrictions of physical activity, and weightbearing are part of the treatment of children with Legg Calve Perthes disease.
Different recommendations are widely discussed and vary between paediatric orthopaedic surgeons and physiotherapists about what physical activity is good for Perthes disease.
Here are some consensus and elaborate guidelines which are widely recommended.
For strengthening exercises in the initial and fragmentation stages Children with Perthes disease should avoid these activities:
🟣No running.
🟣Avoid jumping on trampoline or take part in other high-impact activities that might speed up hip damage.
🟣Avoid gymnastics in the first 2 stages of the disease
🟣Crutches are Sometimes used to help protect the affected hip to avoid bearing weight on it. Using crutches can help protect the joint.
However, a survey done by Acta orthopaedical, where a web-based questionnaire was given to Perthes patients, they considered a good range of movement like stretching exercises, swimming good for their condition.
None of the participants recommended total non-weightbearing
Melin et al 2023.
Physiotherapy has been shown to improve articular range of motion and muscular strength, and reduce articular dysfunction
Exercises recommended for Perthes disease.
There was no clear agreement regarding the appropriate timeline for resuming full activities. Some authors suggest the re-ossification stage is the best time to resume activities.
Whether non-weightbearing and restriction of activities are effective as a treatment for Legg calve Perthes disease in children remains controversial
Here are some answers from the paediatric orthopaedic surgeons (POS)and physiotherapists (PTS) for activities in the initial stage, fragmentation stage, and re-ossification stage.
Data combined for do not allow/allow with restrictions (no) and allow/recommend (yes). Values are count
Timelines by stages: Recommendations for physiotherapy and physical activity for children with Legg–Calve–Perthes disease
Melin et al 2023
▶️How can I be sure that this is the right time to resume activities?
Your doctor or physiotherapist will be able to give you a go ahead based on the stage of the disease.
But your body should be able to give you a rough idea if it is best for you to resume any activity. Judgement could be done on how you feel pain wise, range of movement and treatment given.
You can begin with simple exercises that will not affect your hip, short walks and swimming. Stop when you feel pain or any kind of limitations.
Hope I have answered some of your frequently asked questions. I wish you a quick recovery!🙋
📚Source:
Rodríguez-Olivas AO, Hernández-Zamora E, Reyes-Maldonado E. Legg-Calvé-Perthes disease overview. Orphanet J Rare Dis. 2022 Mar 15;17(1):125. doi: 10.1186/s13023-022-02275-z. PMID: 35292045; PMCID: PMC8922924.
Hailer YD, Hailer NP. Is Legg-Calvé-Perthes Disease a Local Manifestation of a Systemic Condition? Clin Orthop Relat Res. 2018 May;476(5):1055-1064. doi: 10.1007/s11999.0000000000000214. PMID: 29481348; PMCID: PMC5916600.
Melin L, Rendek Z, Hailer YD. Recommendations for physiotherapy and physical activity for children with Legg-Calvé-Perthes disease: a survey of pediatric orthopedic surgeons and physiotherapists in Sweden. Acta Orthop. 2023 Aug 16;94:432-437. doi: 10.2340/17453674.2023.18341. PMID: 37592869; PMCID: PMC10436286.
Johansson T, Lindblad M, Bladh M, Josefsson A, Sydsjö G. Incidence of Perthes’ disease in children born between 1973 and 1993. Acta Orthop. 2017 Feb;88(1):96-100. doi: 10.1080/17453674.2016.1227055. Epub 2016 Sep 2. PMID: 27587239; PMCID: PMC5251271.
Choi IH, Yoo WJ, Cho TJ, Moon HJ. Principles of treatment in late stages of Perthes disease. Orthop Clin North Am. 2011 Jul;42(3):341-8, vi. doi: 10.1016/j.ocl.2011.05.004. PMID: 21742145.
Tuktiyeva N, Dossanov B, Sakalouski A, Syzdykbayev M, Zhunussov Y. METHODS OF TREATMENT OF LEGG-СALVÉ-PERTHES DISEASE (REVIEW). Georgian Med News. 2021 Apr;(313):127-134. PMID: 34103444.
Nelitz M, Lippacher S, Krauspe R, Reichel H. Perthes disease: current principles of diagnosis and treatment. Dtsch Arztebl Int. 2009 Jul;106(31-32):517-23. doi: 10.3238/arztebl.2009.0517. Epub 2009 Aug 3. PMID: 19730720; PMCID: PMC2735835.
Y. Zhao, S. Liao, R. Lu, J. Zhao, X. Ding.Endothelial nitric oxide synthase gene polymorphism is associated with Legg-Calvé-Perthes disease.Exp Ther Med, 11 (2016), pp. 1913-1917