Dripping Wax Bone Disease – Melorheostosis
Description
Patient Data
Age, Gender, and Occupation: Homemaker, 28 years old.
Location: Chennai, India's Sri Ramachandra Medical Centre.
The main complaint is three months of left knee pain.
Current Illness History
Onset: Slowly developing and sneaky.
Features of pain:
exacerbated by walking and bearing weight.
NSAIDs provide some relief.
There was no history of fever, trauma, or systemic symptoms.
Clinical Assessment
General: No skin lesions; systemic examination is normal.
Local analysis:
Examination:
little left limb shortening (1 cm).
There is wasting of the calf muscles.
Feeling:
No soft tissue swelling or localized warmth.
soreness and bony irregularity along the proximal tibia and medial femoral condyle.
Motion Range:
Full range of motion; little discomfort during terminal flexion.
Research Laboratory Examinations:
increased levels of alkaline phosphatase in the serum.
other blood metrics that are within normal ranges
Picture:
X-ray results:
dense, asymmetrical endosteal and periosteal surface hyperostosis.
Characteristic “candle dripping wax” look on medial femoral condyle & proximal tibia.
comparable alterations in the ipsilateral femur's subtrochanteric area.
MRI Results:
Leg & Thigh:
reduced signal strength across all sequences.
On the affected side, there is modest muscular atrophy and fatty infiltration.
No intraosseous fluid collection or marrow edema.
Subcutaneous tissues and neurovascular structures are normal.
Identification:
Final Diagnosis: Femur and tibia-related Melorheostosis (monomelic variety).
Clinical presentation, biochemical support, and distinctive radiographic findings form the basis of the diagnosis.
Protocol
Medical Management:
NSAIDs: To alleviate pain.
Pamidronate, a bisphosphonate, 30 mg daily for 6 days:
less pain in the bones.
Alkaline phosphatase levels returned to normal after six weeks.
Physiotherapy:
Exercises for improving muscle strength and mobility.
Follow-up:
every six months for a year.
At every appointment, the patient had no symptoms.
Pain and Functional Limitation Issue: The patient's mobility and day-to-day activities were hindered by persistent pain in the left knee and thigh.
Management:
Nonsteroidal anti-inflammatory medications (NSAIDs) were used to treat pain at first in order to provide instant relief. Additionally, the patient was given the bisphosphonate pamidronate for six days. By preventing bone resorption and regulating aberrant bone formation, this medical treatment assisted in lowering pain and inflammation.
Nursing Interventions:
Nurses kept an eye on patients' pain levels, gave prescription drugs as directed, made sure patients followed their treatment plans, and gave information on how to manage pain, including how to use assistive technology and take breaks.
Limb Shortening and Muscle Wasting Issue: The patient's gait and lower limb strength were impacted by modest calf muscle wasting and left lower limb shortening.
Management:
Range-of-motion exercises and muscle strengthening were the main goals of the physiotherapy that was recommended. The goals of this intervention were to reduce contractures, increase joint mobility and muscle function, and promote greater functional independence.
Nursing interventions included monitoring muscle tone and strength, assessing limb measurements, encouraging regular involvement in physiotherapy, supporting rehabilitative exercises, and motivating the patient to continue activities as tolerated.
Problems with Bone Deformity Diagnosis Problem:
The condition manifested as distinctive X-ray bone alterations (like a candle dripping wax appearance), which required monitoring and confirmation.
Management:
Blood tests were utilized to track disease activity (elevated alkaline phosphatase), and imaging was utilized to establish the diagnosis. Only in cases of severe deformity or functional impairment was surgical intervention taken into consideration.
Nursing Interventions:
By getting the patient ready, helping with imaging, explaining procedures, and making sure laboratory results were followed up on, nurses helped to facilitate diagnostic procedures.
Psychological Impact and Patient Education Issue:
Because melorheostosis is a chronic condition, its enduring symptoms and rarity might cause anxiety, frustration, or a decreased quality of life.
Management:
Emphasis was placed on patient education and supportive counseling about the condition, its course, and the expected course of therapy. When it came to linking patients with options like counseling or support groups and offering emotional support, nurses were essential.
Nursing Interventions:
Nurses gave patients regular comfort, listened to their worries, helped them communicate with the medical staff, and gave them thorough instruction on how to take care of themselves, take their medications as prescribed, and manage their illnesses over the long term.
Notes
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