Legg-Calve-Perthes disease

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Legg-Calve-Perthes disease

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A 7-year-old boy is brought to the pediatrician’s office for evaluation of left hip pain. The patient’s parent noticed a left-sided limp several weeks ago and initially attributed it to a fall at the playground. The pain has persisted and is minimally relieved with rest or ibuprofen. The patient has no significant past medical history. He takes no daily medications, and immunizations are up to date. Temperature is 37°C (98.6°F), pulse is 82/min, and blood pressure is 116/72 mm Hg. The child is in the 50th percentile for height and 40th percentile for weight. Examination shows a well-appearing and cooperative child. With internal rotation and abduction of the left hip, range of motion is limited secondary to pain. The remainder of the examination is noncontributory. An X-ray of the patient's hip is demonstrated below. Which of the following is the most likely diagnosis?
  
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Legg–Calvé–Perthes disease is a childhood hip disorder that occurs when the blood supply to the head of the femur gets disrupted and leads to death or necrosis of the tissue.

The disease was named after three doctors; Arthur Legg, Jacques Calvé, and Georg Perthes.

The hip joint is a ball and socket type, because the ball-shaped head of the femur sits and rotates inside the cup-shaped socket called the acetabulum.

This gives the hip joint the ability to move nearly in all directions, as long as the ball can smoothly rotate inside the socket.

Now, the head of the femur is supplied by branches of 3 arteries, the medial and lateral femoral circumflex arteries, as well as the artery of ligamentum teres.

The arteries send branches that climb up the neck of the femur to supply the head of the femur, providing the tissue with the nutrition it needs to grow and maintain its spherical shape.

In Legg–Calvé–Perthes disease, for some children, the blood supply to the femoral head becomes interrupted for some reason - and it’s not known why exactly.

The result is that the tissue begins to die off - a process called avascular necrosis.

Over time, there’s new blood vessel formation into the necrotized bone and that allows the dead tissue to get removed by immune cells called macrophages.

That process causes the head of the femur to lose mass, leaving it weak and prone to fractures.

When this happens, the head of the femur becomes misshapen and can no longer smoothly rotate inside the concave acetabulum, which results in reduced range of motion.

Now, over time, Legg–Calvé–Perthes disease typically self-resolves and the bone is able to heal - once again it’s not known exactly why or how this happens.

When bone remodeling occurs, new bone replaces the necrosed bone, and the spherical shape of the head of the femur gets restored and properly fits in the acetabulum again.

Summary

Legg �Calv� �Perthes disease (LCPD) refers to a childhood hip disorder that occurs when the blood supply to the head of femur is disrupted for unknown reasons, resulting in avascular necrosis of the head of femur. The disease was named after three doctors; Arthur Legg, Jacques Calv�, and Georg Perthes. Children with LCPD develop hip limping and pain which worsens with activity, and it may be referred to the knee. The condition is often treated with surgery and physiotherapy to help restore movement and function.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Maternal Smoking During Pregnancy, Other Prenatal and Perinatal Factors, and the Risk of Legg-Calvé-Perthes Disease" Pediatrics (2008)
  6. "Legg-Calvé-Perthes Disease" American Academy of Orthopaedic Surgeon (2010)
  7. "Pathophysiology and New Strategies for the Treatment of Legg-Calvé-Perthes Disease" Journal of Bone and Joint Surgery (2012)