Paeds MSK/Rhem Flashcards
Define DDH
Structural abnormality in the hips leading to instability and potential for subluxation or dislocation
What are the 6 RF for DDH
- First degree Fx
- Female
- Breech presentation
- High birth weight
- Oligohydramnios
- Prematurity
When is DDH screened for ?
-Neonatal examination at birth and at 6-8 weeks
What findings on a NIPE may suggest DDH?
- Different leg lengths
- Restricted hip abduction on one side
- Significant bilateral restriction in abduction
- Difference in the knee level when hips are flexed
- Clunking of the hips on special tests
What are the 2 special tests to look for DDH?
- Ortolani test
- Barlow test
What is the ortolani test ?
- Looks for DDH
- With the hips and knees flexed, gentle pressure is used to abduct the hips and apply pressure behind the legs to see if the hips will dislocate anteriorly
What is the barlow test ?
- Looks for DDH
- Gentle downward pressure is placed on the knees through the femur to see if the femoral head with dislocate posteriorly.
What is the diagnostic test for DDH?
-USS
How is DDH managed if presents at <6 mnths ?
- Pavlik harness
- Keeps the baby’s hips flexed and abducted.
- Usually on for 6-8 weeks.
How is DDH managed if presents >6 mnths or if harness fails?
- Surgery
- After surgery an hip spica cast is used to immobilise the hips
Define osteogenesis imperfecta
-Genetic condition resulting in brittle bones that are prone to fracture
What causes osteogensis imperfecta ?
- Genetic mutations that affect the formation of collagen
- 8 different genetic abnormalities with varying severities
How does osteogenesis imperfecta present ?
- Recurrent and inappropriate fractures
- Hyper-mobility
- Blue/grey sclera
- Triangular face
- Short stature
- Deafness from early childhood
- Dental problems
- Bone deformities : esp bowed legs and scoliosis
- Joint and bone pain
How is osteogenesis imperfecta diagnosed ?
- Usually clinical
- X-ray used in fractures and for bone deformitis
How is osteogenesis imperfecta managed ?
- Bisphosphonates to increased bone density (e.g. alendronate)
- Vit D supplementation to prevent deficiency
- Physio and occupational therapy
Define rickets
-Defective bone mineralisation causing ‘soft’ and deformed bones
What 2 vitamin deficiencies cause rickets ?
- Vitamin D
- Calcium
What are the sources of vitamin D?
- Sunlight
- Eggs, oily fish or forified cereals
What are the sources of calcium ?
- Dairy productes
- Green vegetables
What is a rare form of rickets?
- Hereditary hypophosphataemic rickets
- Genetic defect causing low phosphate
- Most common : X-linked dominant
What are the potential symptoms of rickets ?
- Lethargy
- Bone pain
- Swollen wrists ! widening of joint space
- Bone deformity
- Poor growth
- Dental problems ! no teeth development
- Muscle weakness
- Pathological or abnormal fractures
What 5 bone deformities can occur in rickets?
- Bowing of the legs
- Knock knees
- Rachitic rosary : ribs expand at the costochondral junctions causing lumps along the chest
- Craniotabes : soft skull with delayed closure of sutures and frontal bossing
- Delayed teeth
What are the RF for rickets ?
Anything that increases the risk of vit d deficiency
- Darker skin
- Low exposure to sunlight
- Live in colder climates
- Spend majority of their time indoors
What are the 5 common blood results in rickets ?
- Serum 25-hydroxyvitamin D of less than 25 nmol/L
- Calcium : low
- Phosphate : low
- Alkaline phosphatase : high
- Parathyroid hormone : high
What investigation is required to diagnose rickets ?
- X ray
- May show joint widening and osteopenia (more radiolucent bones)
How is rickets prevented ?
- Breastfeeding women should taken vitamin D supplements (10 micrograms per day)
- Formula fed babies are at lower risk as it is fortified with vitamin D
How is rickets managed ?
- Vitamin D (ergocalciferol) : 6mnths to 12 yrs is 6,000 IU per day for 8-12 wks
- Calcium supplementation
Explain the pathophysiology behind rickets
- Low vitamin D
- This leads to malabsorption of calcium and phosphate
- Low calcium and phosphate leads to defective bone mineralisation
- Low calcium leads to secondary hyperparathyroidism
- Increased parathyroid hormone leads to increased calcium reabsorption from bone and further issues with bone mineralisation
What is transient synovitis ?
-Temporary irritation and inflammation in the synovial membrane of the joint
What age group is transient synovitis most common in ?
3-10 years
How does transient synovitis present ?
- Usually occurs within a few weeks of a viral illness
- Limp
- Refusal to weight bear
- Groin or hip pain
- Mild low grade temperature
What is a serious differential in a child with joint pain and fever ?
-Septic arthritis
How is transient synovitis managed ?
- Simple analgesia
- Safety net to attend A&E if symptoms worsen or they develop a fever !
Define septic arthritis
-Infection inside a joint
when is septic arthritis most common ?
<4 yrs of age
How does septic arthritis usually present ?
- Single joint
- Rapid onset : hot red swollen painful joint
- Refusing to weight bear
- Stiffness and reduced range of motion
- Systemic Sx : fever, lethargy, sepsis
What is the most common bacterial cause of septic arthritis ?
-Staph aureus
What 4 other bacteria can cause septic arthritis ?
- Neisseria gonorrhoea (sexually active teenagers)
- Group A strep
- Haemophilus influenza
- E.coli
Give 4 other differential diagnosis for septic arthritis
- Transient synovitis
- Perthes
- Slipped upper femoral epiphysis
- JIA
How is septic arthritis managed ?
- Joint aspiration for gram staining, crystal microscopy, culture and Abx sensitivities.
- Empirical IV abx
- Sugical drainage and washout where necessary
What is perthes disease?
- Idiopathic, avascular necrosis of the femoral head due to disruption of blood flow
- Affecting the epiphysis of the femur
At what age does perthes most commonly occur?
- Boys aged 5-8
- Can occur between 4-12 yrs
How does perthes disease present?
-Slow onset of : pain in the hip or groin, limp, restricted hip movements and possible referred pain to knee
How is perthes diagnosed ?
- X ray
- Technetium bone scan or MRI if sx persist
How is perthes managed ?
- <6 = conservative
- Older : surgical management
What is the main complication of perthes?
- Soft and deformed femoral head leading to early hip osteoarthritis
- this is due to the revascularisation and neovascularisation that occurs over time
What is a slipped upper femoral epiphysis (SUFE) ?
-Where the head of the femur is displaced along the growth plate
Who is SUFE more likely to occur in ?
- Boys (8-15 with an average age of 12)
- Obese children
What is the typical exam q for SUFE?
- Usually in an adolescent obese male going through a growth spurt
- Hx of mild trauma causing symptom onset -> pain is usually disproportionate to the trauma
How does SUFE present ?
- Hip, groin, thigh or knee pain
- Restricted range of hip movement
- Painful limp
- Restricted movement in the hip : particularly restricted internal rotation
On examination, what movement will be restricted in SUFE?
- > Internal rotation
- > They will prefer external rotation
How is SUFE diagnosed ?
X-ray !
Plain X-ray of BOTH hips (AP and frog-leg views)
Both hips as its bilateral in 20% of cases
How is SUFE managed ?
-Surgery
What is osteomyelitis ?
-Infection in the bone and bone marrow, typically in the metaphysis of long bone
What bacteria is the most common cause of osteomyelitis ?
-Staph aureus
Give 5 risk factors for osteomyelitis
- Male
- <10 yrs
- Open bone fracture
- Orthopaedic surgery
-Sickle cell anaemia : salmonella
What is chronic osteomyelitis ?
-Deep seated, slow growing infection with more subtle features
How with acute osteomyelitis present ?
- Fever
- Refusing to use the limb or weight bear
- Pain
- Swelling
- Tenderness
How is osteomyelitis investigated ?
-MRI
-Blood culture
- Bone marrow aspiration or bone biopsy if necessary
How is osteomyelitis managed ?
- Antibiotics
- May require drainage and debridement
What is an osteosarcoma and where does it most commonly affect ?
- Bone cancer
- Femur = most common
- Tibia and humerus also commonly affected
What age group are usually affected with osteosarcomas ?
10-20 yrs
How does an osteosarcoma present ?
- Persistent bone pain
- Bone pain worse at night and may wake from sleep
- Bone swelling
- Palpable mass
- Restricted joint movement
How is an osteosarcoma diagnosed ?
-Urgent direct access xray within 48 hrs
What would an xray show in an osteosarcoma ?
- Poorly defined lesion in the bone with destruction of the normal bone and a ‘fluffy’ appearance
- Periosteal reaction = ‘sun-burst’ appearance due to irritation of the lining of the bone.
What may be raised in blood tests in an osteosarcoma ?
-Raised alkaline phosphatase (ALP)
What is the main complication of an osteosarcoma ?
- Pathological bone fractures and metastasis
- Surgical resection also often leads to limb amputation
What is Talipes ?
- Clubfoot : fixed abnormal ankle position
- Fixed abnormal ankle position presenting at birth
What is talipes equinovarus ?
-Ankle in plantar flexion and supination
What is talipes calcaneovalgus ?
-Ankle in dorsifelxion and pronation
How is clubfoot managed ?
-Ponseti method
What is the ponseti method ?
- Used to treat club foot
- The foot is manipulated towards a normal position and a cast used to hold it in position. This is repeated until the foot is in the correct position
- A brace is then used to hold the feet in the correct position when not walking until around 4 yrs of age
What is positional talipes ?
- The ankle is in a plantar flexion and supination position but is not fixed
- The muscles around the ankle are tight but the boney structure is not affected
- Managed using physio
What is achondroplasia ?
- Most common common cause of disproportionate short stature (dwarfism)
- It is a type of skeletal dysplasia
What is the cause of achondroplasia?
-Genetic mutations in the FGFR3 gene (fibroblast growth factor receptor 3) on chromosome 4 leading to abnormal function of the epiphyseal plates.
What kind of genetic inheritance leads to achondroplasia ?
- Autosommal dominant -> heterozygous
- > Homozygous gene mutations are fatal in the neonatal period
What are the main features of achondroplasia ?
- Average height of 4 feet
- Short digits
- Bow legs
- Disproportionate skull
- Foramen magnum stenosis
- Frontal bossing
Give 6 associations with achondroplasia
- Recurrent otitis media
- Kyphoscoliosis
- Spinal stenosis
- Obstructive sleep apnoea
- Obesity
- Cervical cord compression and hydrocephalus as a result of foramen magnum stenosis
What is osgood-schlatters disease?
Inflammation at the tibial tuberosity where the patella ligament inserts leading to anterior knee pain in adolescents
When does osgood-schlatters typically occur?
- 10 to 15 yrs old
- More common in males
How does osgood-schlatters present ?
Gradual onset :
- Pain at the anterior aspect of the knee
- Pain exacerbated by physical activity, kneeling and extension of the knee
- Visible or palpable heard and tender lump at the tibial tuberosity
How is Osgood-Schlatters managed ?
- Reduction in physical activity
- Ice
- NSAIDs
- Once symptoms have settled : stretching and physio
What is a rare complication of Osgood-Schlatters?
-Full avulsion fracture where the tibial tuberosity is separated from the rest of the tibia
Explain the pathophysiology behind Osgood-Schlatters
- Stress from running, jumping etc + growth in the epiphyseal plate results in inflammation on the tibial epiphyseal plate
- Multiple small avulsion fractures occur, where the patella ligament pulls away tiny pieces of bone
- This leads to growth of the tibial tuberosity = visible lump
- This lump is tender due to inflammation but as the bone heals it becomes hard and non-tender
What is kawasaki disease ?
Systemic medium-sized vessel vasculitis
Who does kawasaki disease typically affect?
- Children <5 yrs
- Asian children (japanese or korean)
- Boys
What is a key complication of kawasaki disease ?
-Coronary artery aneurysm
What are the key features of kawasaki disease ?
CRASH AND BURN
- C : conjunctivitis (bilateral)
- R : rash (widespread erythematous maculopapular)
- A : adenopathy (cervical & usually unilateral)
- S : strawberry tongue and cracked lips
- H : hands (desquamation)
-BURN : high fever >5 days
Give 3 investigation results usually seen in kawasaki disease
- FBC : anaemia, leukocytosis and thrombocytosis
- LFTS : hypoalbuminaemia and elevated liver enzymes
- Raised ESR
How is kawasaki disease managed ?
- High dose aspirin
- IV immunoglobulins
- Echo
What is a risk of using aspirin in children ?
-Reye’s syndrome
What is rheumatic fever ?
- Autoimmune condition caused by group A beta-haemolytic streptococcal (typically streptococcus pyogenes causing tonsilitis).
- The antibodies to target the bacteria also target antigens on the cells of the person’s body (e.g. myocardium of the heart)
- Leads to type 2 hypersensitivity reaction where the immune system begins attacking cells through the body.
What is the diagnostic criteria for rheumatic fever
- Evidence of strep infection + two major criteria or 1 major and 2 minor
- Major :
- J : Joint arthritis
- O : Organ inflammation (e.g. carditis)
- N : Nodules
- E : Erythema marginatum rash
- S : Sydenham chorea
- Minor :
- F : Fever
- E : ECG changes(prolonged PR interval) without carditis
- A : Arthralgia without arthritis
- R : Raised inflammatory markers
What should be given for tonsilitis caused by streptococcus to prevent rheumatic fever ?
-Phenoxymethylpenicillin (penicillin V) for 10 days
What is Ehlers-Danlos syndrome ?
- Autosommal dominant
- Defect in collagen, resulting in hyper-mobility causing joint pain and soft and stretchy skin
What score is used to assess hypermobility on EDS
-Beighton score : palms flat on floor, elbows, knees, thumb and little finger hyperextend
What is a common cardiac involvement of EDS?
- Aortic regurgitation
- Postural Orthostartic tachycardia Syndrome
What is Henoch-Schonlein Purpura ?
-IgA vasculitis affecting the skin, kidneys and GI tract
What can tigger Henoch-Schonlein purpura and what are the 4 common features ?
- URTI or gastroenteritis
- Common in <10 yrs
- Purpura
- Joint pain
- Abdo pain : can lead to GI haemorrhage, intussusception and bowel infarction
- IgA nephritis : haematuria and proteinuria !
What would be a common exam presentation in henoch-scholein purpura
-8 yr old, recent gastroenteritis. Purpura starting on legs and speading to buttock. Arthralgia in knees and ankles. Abdo pain. Haematuria and proteinuria on dipstick.
What would be a common exam presentation in henoch-scholein purpura
-8 yr old, recent gastroenteritis. Purpura starting on legs and speading to buttock. Arthralgia in knees and ankles. Abdo pain. Haematuria and proteinuria on dipstick.
Give 4 non infective causes of a fever lasting >5 days in a child
- Stills Disease
- Leukaemia
- Rheumatic fever
- Kawasaki
How does systemic JIA present ?
- Subtle salmon-pink rash
- Joint pain
- High swinging fevers
What medications can be used to treat JIA?
- NSAIDs
- Steorid injections
- DMARDS (e.g methotraxate, sulfasalazine)
- Biologics : adalimumab, infliximab
