Osteitis fibrosa cystica

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Osteitis fibrosa cystica
Synonyms	Von Recklinghausen's disease of bone
Pronounce	N/A
Specialty	N/A
Symptoms	Bone pain, fractures, skeletal deformities
Complications	Hypercalcemia, kidney stones, osteoporosis
Onset	Typically in adults
Duration	Chronic
Types	N/A
Causes	Hyperparathyroidism
Risks	Parathyroid adenoma, parathyroid hyperplasia
Diagnosis	Blood test, X-ray, bone scan
Differential diagnosis	Osteoporosis, Paget's disease of bone
Prevention	N/A
Treatment	Parathyroidectomy, bisphosphonates
Medication	Calcimimetics, vitamin D
Prognosis	Good with treatment
Frequency	Rare
Deaths	N/A

Other names

Osteitis fibrosa cystica; Hyperparathyroidism - osteitis fibrosa; Brown tumor of bone

Histology of bone showing osteitis fibrosa cystica

Definition

Osteitis fibrosa is a condition of bones when they become abnormally weak and deformed due to a complication of hyperparathyroidism.

Parathyroid glands

The parathyroid glands are 4 tiny glands in the neck. These glands produce parathyroid hormone (PTH). PTH helps control calcium, phosphorus, and vitamin D levels in the blood and is important for healthy bones.

Causes

Excessive parathyroid hormone (hyperparathyroidism) can lead to increased bone breakdown, which can cause bones to become weaker and more fragile.

Osteoporosis

Many people with hyperparathyroidism eventually develop osteoporosis.

Pathophysiology

• Not all bones respond to PTH in the same way.
• Some develop abnormal areas where the bone is very soft and has almost no calcium in it and this is osteitis fibrosa.
• In rare cases, parathyroid cancer causes osteitis fibrosa.
• Osteitis fibrosa is now very rare in people who have hyperparathyroidism who have good access to medical care.
• It is more common in people who develop hyperparathyroidism at a young age, or who have untreated hyperparathyroidism for a long time.

Symptoms

Osteitis fibrosa may cause bone pain or tenderness. There may be fractures (breaks) in the arms, legs, or spine, or other bone problems. Hyperparathyroidism itself may cause any of the following:

• Nausea
• Constipation
• Fatigue
• Frequent urination
• Weakness

Management

• Investigations include blood tests show a high level of calcium, parathyroid hormone, and alkaline phosphatase (a bone chemical).
• Phosphorus level in the blood may be low.
• X-rays may show thin bones, fractures, bowing, and cysts.
• Teeth x-rays may also be abnormal.
• A bone x-ray may be done.
• People with hyperparathyroidism are more likely to have osteopenia (thin bones) or osteoporosis (very thin bones) than to have full-blown osteitis fibrosa.

Treatment

Most of the bone problems from osteitis fibrosa can be reversed with surgery to remove the abnormal parathyroid gland(s). Some people may choose not to have surgery, and instead be followed with blood tests and bone measurements. If surgery is not possible, medicines can sometimes be used to lower calcium level.

Complications

Complications of osteitis fibrosa include any of the following:

• Bone fractures
• Deformities of bone
• Pain
• Problems due to hyperparathyroidism, such as kidney stones and kidney failure

Prevention

Routine blood tests done during a medical checkup or for another health problem usually detect a high calcium level before severe damage is done.