MSK: Part 5 Flashcards
What is Paget’s disease of Bone
It is a condition involving disorder of bone remodelling and deformities
What age does Paget’s effect
Under 40
Where is Paget’s most prevalent
Europe and North England
What gender is Paget’s most common
Females
Risk factors in Paget’s
- Aetiology known
- Measles
- Family history
results from latent viral infection (canine distemper virus, measles or respiratory syncytial virus) in osteoclasts in generally susceptible individuals
Pathophysiology of Paget’s
- Increased osteoclastic bone resorption followed by formation of WEAKER NEW BONE, increased local bone blood flow and fibrous tissue
- Formation exceeds resorption but new woven bone is WEAKER than normal bone - leads to deformity and increased fracture risk
- Diseases doesn’t spread but can become symptomatic at previously silent sites
Clinical presentation of Paget’s
- Pelvis, lumbar spine, femur, thoracic spine, skull and tibia
- Bone pain
- Most are asymptomatic
- Joint pain - when an involved bone is close to a joint (leading to cartilage damage and osteoarthritis)
- Deformities, in particular bowed tibia and skull changes
- High-output cardiac failure and myocardial hypertrophy due to increased bone blood flow (RARE)
- Osteosarcoma
- Deafness from 8th cranial nerve involvement
- Paraparesis - partial paralysis of lower limbs
- Hydrocephalus due to blockage of aqueduct of Sylvius
Diagnostics of Paget’s
- FBC
- X-ray
- Isotope bone scans
FBC result of Paget’s
- Increased serum alkaline phosphatase with normal calcium and phosphate (reflects increased bone turnover)
- Urinary hydroxyproline excretion is raised
What does X-ray show in Paget’s
- Localised boney enlargement and distortion
- Sclerotic changes (increased density)
- Osteolytic areas (loss of bone and reduced density)
What does isotope bone scans show in Paget’s
- Determines extent of skeletal involvement but unable to distinguish between page’s and sclerotic metastatic carcinoma
How is Paget’s treated
- BISPHOSPHONATES (ORAL ZOLENDRONATE or ALENDRONATE)
- NSAIDs (IBUPROFEN)
- Disease activity monitored by symptoms and measurement of serum alkaline phosphatase or urinary hydroxyproline
What is osteomalacia
- Normal amount of bone but its mineral content is LOW (there is excess uncalcified osteoid and cartilage) - DEFECTIVE MINERALISATION
How does osteomalacia compare to osteoporosis
- Mineralisation is unchanged, overall there is bone loss
What is rickets
Defective mineralisation during bone growth at epiphyseal growth plates
Osteomalacia vs rickets
Osteomalacia is defective mineralisation after fusion of epiphyses
What can cause osteomalacia and rickets
Vit D deficiency
Why is Vit D important
Required for calcium and phosphate absorption and incorporation into bone
Where is Vit D produced
Skin
How is Vit D produced
- UV B sunlight on 7-dehydrocholesterol
Where is Vit D found in food
Oily fish
Egg yolks
Margarine
What happens to Vit D produced in the skin
Converted to 25-hydroxy Vit D in the LIVER
What happens to 25-hydroxy Vit D
Converted to 1,25-hydroxy Vit D (calcitriol) in the KIDNEY
What is the most common cause os osteomalacia
- Hypophopsphataemia (excess release of parathyroid hormone causes decreased absorption of phosphate in the kidneys - HYPERPARATHYROIDISM)
- Vit D deficiency
- Renal failure
- Drug induced
- Liver disease
- Tumour-induced osteomalacia
How does Vit D cause osteomalacia
- Vit D is fat soluble so GI disease can result in malabsorption
- Poor diet
- Lack of sunlight
How does renal failure cause osteomalacia
- Inadequate conversion of 25-hydroxy vit D to 1,24-hydroxy vit D
How do drugs cause osteomalacia
Anticonvulsants induce liver enzymes leading to increased breakdown of 25-hydroxy Vit D
Rifampicin
How do liver diseases cause osteomalacia
Reduce hydroxylation of Vit D to 25-hydroxy Vit D (cirrhosis)
Clinical presentation of osteomalacia
- Muscle weakness - leading to waddling gait, difficulty climbing stairs and getting out of a chair
- Widespread bone pain - dull ache that is worse on weight-bearing and walking
- Bone pain and tenderness
- Fractures of the femoral neck
Clinical presentation of rickets
- Growth retardation
- Knock knees, bowed legs
- Widened epiphyses at the wrists
- Hypocalcaemic tetany may occur (intermittent muscular spasms)
Diagnosis of rickets and osteomalacia
- FBC
- Biopsy
- X-ray
FBC test in osteomalacia
- Low ca and phosphate
- Raised serum alkaline phosphatase
- Elevated parathyroid hormone
- Low 25=hydroxy vit D
Biopsy in osteomalacia
Shows incomplete mineralisation
X-ray test in osteomalacia
- Shows defective mineralisation
2. Looser’s pseudo fractures (low density bands extending from cortex inwards in the shafts of the long bones)
Treatment of osteomalacia
- Vit D replacement:
Calcium D3 FORTE (dietary cure) - Oral ERGOCALCIFEROL or IM CALCITRIOL (hepatic disease and malabsorption)
- ALFACIDOL or CALCITRIOL (in renal disease)
What diseases are associated with Vit D insufficiency
Typ 2 diabetes, several cancers, CVD
What is Acute disc disease
Prolapse of intervertebral disc results in ACUTE BACK PAIN (LUMBARGO)
What aged people are effected by acute disc disease
Younger people as disc degenerates with age and in the elderly it is no LONGER ABLE TO PROLAPSE
What causes SCIATICA (Acute disc disease in elderly)
Compression (as opposed to prolapse) of the nerve root by osteophytes in the lateral recess of the spinal canal
Clinical presentation of acute disc disease
- Sudden onset of severe back pain
- Pain is often clearly related to position and aggravated by movement
- Muscle spasm leads to sideways tilt when standing
- Radiation of pain and clinical findings depend on the disc affected - LOWER THREE DISCS being most commonly affected
Where is root lesson in S1 felt
Buttock down back of thigh to ankle
What reflex is lost in S1 root lesion
Ankle Jerk
What other sign is found in S1 root lesions
Diminished straight leg raising
Pain felt in L5 root lesion
- Buttock to lateral aspect of leg and top of foot
What reflex is lost in L5 root lesion
None
Other signs of L5 root lesions
DIMINISHED STRAIGHT LEG RAISING
Pain in the L4 root lesions
- LATERAL ASPECT OF THIGH TO MEDIAL SIDE OF CALF
What reflex is lost in L4 root lesions
Knee Jerk
Other signs of L4 root lesions
- Positive femoral stretch test
Diagnosis of Acute Disc Disease
- X-rays NORMAL
2. MRI
How is Acute Disc Disease treated
- Acute Stage - bed rest on a firm mattress, analgesia and epidural corticosteroid injections
- Surgery for severe near impairment (foot drop or bladder symptoms)
- Physio in recovery phase (helping correct posture and restore movement)
What is Chronic Disc Disease
Associated with degenerative changes in the lower lumbar discs and facet joints
What causes pain in chronic disc damage
Aggravated by movement
Describe distribution of pain in CDD
- Sciatic radiation in the buttocks radiating into posterior thigh
Is there a cure to chronic disc disease
Long-standing with no cure
Surgical intervention for CDD
Fusion at single identifiable level if possible and decompression of affect nerve root
Treatment for CCD
- NSAIDs
- Physiotherapy
- Weight reduction
Describe pain in Primary and secondary bone tumours
- Unremitting and worst at night
When are primary tumours of the bone seen
- YOUNG
Osteosarcomas
Fibrosarcomas
Chrondromas
Ewing’s tumour
Where can bone tumours metastasise to
- Lungs
- Breast
- Prostate (often osteosclerotic)
- Thyroid
- Kidney
What do bone tumour symptoms rely on
Anatomical position of the tumour with local bone pain
What are systemic symptoms
Malaise, pyrexia as well as aches and pains occur and occasionally related to hypercalcaemia
Diagnostics of Primary and Secondary tumours
- Skeletal isotope scan
- X-rays
- MRI
- FBC
What would skeletal isotope scan show
Bony metastases as ‘hot’ areas BEFORE radiological changes occur
What would X-rays show in tumours
- Metastasis as osteolytic areas with bony destruction
2. Osteosclerotic (increased bone density) metastases
What defines osteolytic areas
Lost greater than 60% bone density
What do MRIs show in tumours
- Vertebral lesions
FBC results in primary and secondary tumours
- SEURM ALKALINE PHOSPHATASE raised
- HYPERCALACAEMIA
- PSA raised in prostatic metastases
How are bone tumours treated
- Analgesics and Anti-inflammatory drugs
- Local radiotherapy to relive pain and reduce fractures
- Chemotherapy for SOME
4 .Hormonal therapy for tumours hormone dependant - Bisphosphanates (ALDENRONATE)
Name some primary bone tumours
Osteosarcoma
Ewing’s sarcoma
Chrondrosarcoma
Peak onset of osteosarcoma
15-19 yrs
What disease is osteosarcoma associated with
Paget’s
Where does osteosarcoma occur
Metaphases of long bones
Common sites of osteosarcomas
Knees or humerus
Clinical presentation of osteosarcomas
Painless tumour
Metastases of osteosarcomas
Destroys bone and spreads into surrounding tissues and rapidly metastasises to the LUNG
Diagnostics of osteosarcomas
X-ray:
- Bone destruction and formation
- Soft tissue calcification produces sunburst appearance
What cells cause Eqing’s sarcoma
Mesenchymal stem cells
Onset of Ewing’s sarcoma
15 years
Clinical presentation of Ewing’s sarcoma
1. Mass/swelling in long bones of the: arms legs pelvis chest skull and flat bones of trunk
Painful swelling, redness in area surrounding tumour, malaise, anorexia, weight loss, fever and paralysis
What is chrondrosarcoma
Cancer of the cartilage
Clinical presentation of chrondrosarcoma
- Dull, deep pain
2. Affected area is swollen and tender
Common sites effected in chondrosarcoma
- Pelvis
- Femur
- Humerus
- Scapula
- Ribs
Define fibromyalgia
- Widespread musculoskeletal pain AFTER other diseases are excluded
- Symptoms present for at least 3 months and other causes have been excluded
- Pain at 11/18 tender point sites on digital palpation (with enough pressure so that thumb blanches)
Clinical presentation of Fibromyalgia
- CENTRAL (non-nociceptive)chronic pain
- Depression
- Chronic headache
- Irritable bowel syndrome
- Chronic fatigue syndrome
- Myofascial pain syndrome
What gender does fibromyalgia effect
Females
Risk factors for fibromyalgia
- Female
- Middle age
- Low household income
- Divorced
- Low educational status
Where is pain found in fibromyalgia
- Neck and back
- Lower neck in front
- Base of skull
- Upper edge of breast
- Neck and shoulder
- Below side bone at elbow
- Upper outer buttock
- Hip Bone
- Just above the knee on inside
How is pain in fibromyalgia aggravated
Stress
Cold
Activity
CNS effect in fibromyalgia
Paraesthesiae of hands and feet
Fibromyalgia clinical presentations
- fatigue is extreme after minimal exertion
- Non-restorative sleep:
- Frequent waking during the night
- Waking unrefreshed
- Poor concentration and forgetfulness
- Low mood, irritable and weepy
- Lack of non-REM sleep causes functional pain
- Patient is anxious and angry for no reason
Differential diagnosis in fibromyalgia
- Hypothyroidism
- SLE
- Polymyalgia rheumatic
- High calcium
- Low Vit D
- Inflammatory arthritis
Diagnostics in fibromyalgia
- Pain at 11/18 tender point sites on digital palpation
- Thyroid function test (excludes hypothyroidism)
- ANA and SaSNA
- ESR and CRP raised
- Ca and electrolytes (exclude high ca)
- VIt D (exclude low Vit D)
- Examine patient and CRP (exclude inflammatory arthritis)
Treatment in fibromyalgia
1, Educat patient and family: Explain that they have real symptoms but pain doesn't always mean damage (migraine of joints) 2. Avoid unnecessary investigations 3. Rest pain thermostat: - correct non-restorative sleep - Improve aerobic fitness (tires them) 4. ORAL AMITRYPTILINE - cure depression ORAL PREGABALIN (anticonvulsants)
How long does it take for ORAL AMIARYPTILINE and ORAL PREGABALIN to function
1 month
Do NSAIDs and steroids work in fibromyalgia
No
Red flag signs for cancer
- Rest pain
- Night Pain
- Lump present
- Loss of function
- Neuro symptoms
- Unwell and weight loss
Why is a CT done for cancer
- Bone quality and staging
MRI scan for cancer
Soft tissue reactive changes
Bone scan for cancer
Skeletal metastases
X-Ray for cancer
- Bone density changes
- Zone of transition
- Periosteal reaction
Osteoblastoma vs osteosarcoma
X-ray in what conditions show Codman’s triangle
Osteosarcoma Equine's GCT Osteomyelitis Metastases
X-Ray in what conditions show sunburst appearance
Osteosarcoma and Ewing’s
X-ray in what condition shows onion-skin appearance of the bone
Ewing’s
What type of MRI is used to look at bone pathology
T2
Three phases of a bone scan
- Arterial (1 minute)
- Blood pooling (capillary dilation)
- Delayed (uptake within the bone)
Uses Gamma Camera
Ways we can scan for cancer
- PET-CT
- Whole body MRI
- T2 MRI
- Ultrasound
- X-Ray
Define a histologically beingn gradient, Low grade malignant and high grade malignant tumour
- Well defined margins
- Progressive growth limited by natural barriers and well defined margin with thin cortical bone
- Growth not limited by natural barriers
Where do the following bone tumours present: Osteosarcoma Parosteal osteosarcoma Chondrosarcoma Giant cel tumours Chordoma Adamantinoma
- Knee
- Distal femur
- Plevis
- Knee
- Sacrum
- Tibia
Clinical presentation of osteoid osteoma
- Localised pain and self-limiting
Produces Prostaglandin E2
What is seen on an X-ray for osteoid osteoma
Active reactive bone and nidus
Diagnostics for osteoblastoma
PAIN
X-ray: Bone destruction surrounded by reactive new bone
Histology: Interlacing trabecular + loose fibrovascular stroma
How is osteoblastoma treated
Excision with at least a marginal line of excision
What is an osteochondroma
Metaphysical lesion covered by cartilage cap that grows away from growth plate and stops growing after puberty
Clinical presentation of osteochondroma
Painless lumps
How is osteochondroma treated
Excision
What are osteosarcomas
Spindle cell neoplasms that produce asteroids
Name four types of osteosarcomas
- Intramedullary osteosarcoma
- Parosteal osteosarcoma
- Periosteal sarcoma
- Telangiectatic osteosarcoma
What is osteosarcoma associated with
p53 mutation
Chondrosarcoma
What are intramedullary osteosarcomas
These penetrate the cortex forming soft tissue mass
How is intramedullary sarcoma diagnosed
- X-ray: bone formation and destruction seen
2. MRI
Where do intramedullary osteosarcomas metastases to
LUNGS
What causes secondary osteosarcoma
- Paget’s
- Irradiation (radiotherapy)
- Fibrous dysplasia
Management of osteosarcoma
- Limb Salvage surgery
- Adjuvant Chemotherapy
- Chemotherapy pre-op (8-12 weeks)
What osteosarcoma has the best prognosis
Parosteal
Where are endchondromas seen
Small bones of hand and feel
What are enchondromas
Benign lesions that DO NOT destroy cells
Diagnostics for echondromas
- X-ray: Metaphysical popcorn
- Bone scan: HOT
- Histology: Island of cartilage
What is chondroblastoma similar to
Giant cell tumour - both metastasise to lungs
GCT has NOT SCLEROTIC RIM on imaging while chondroblastoma does
What is fibrous dysplasia
Failure to produce lamellar bone
What cause fibrous dysplasia
- Mutation of the GS-alpha protein
Diagnostics and treatment for fibrous dysplasia
- X-ray: Ground glass lesion with sclerotic margin
2. Histology: Chinese alphabet
Treatment of fibrous dysplasia
- Bisphosphonates
2. Curettage and cortical autograft - surgery
What is non-ossifying fibroma
- Diaphysial variant of normal growth - benign
Radiographic features of non-ossifying fibroma
- TO one side of the bone (eccentric)
- Metaphysical
- Sclerotic rim
- Overlying cortex thinned
- Lesion based on cortex
- CLEAR ZONE OF TRANSITION
What are unicameral bone cysts
- Metaphysical
- Purely lytic
- Epand the bone symmetrically and border the growth plate
- Fallen leaf sign - trabeculations after they fracture
Characteristics of Aneurysmal Bone cyst
Metaphysical and eccentric
Diagnostics of ABC
- X-ray: Eccentric, lytic and expansile lesion
2. MRI - fluid levels
How is ABC treated
Simple curettage
What are GCTs
- Locally destructive neoplasms with poorly defined cells
Diagnostics for GCT
- Xrays: Destructive lesion + no sclerotic rim
2. Histology: Multinucleate cells
How is GCT treated
- Curettage, bisphosphonates
What are adamantinoma
- Rare tumour of long bones with pain
Diagnostics of adamantinoma
- Xray: Radiolucent zones interlaced with sclerosis and cortical destruction
- Histology: Epithelial cells in fibrous storm
How is Adamantinoma treated
Wide surgical excision plus reconstruction
What four features of a soft tissue could indicate sarcoma
- Size > 5cm
- Deep to the fascia
- Enlarging
- Painful
Define the following surgical margins: INtralegional Marginal Wide Radical
Intraleisonal: Plane of surgery goes through the tumour
Marginal: Plane of surgery goes through reactive zone of the lesion
Wide: Plane of dissection through normal tissue
Radical: Entire anatomic compartment of the lesion is removed