15- Paediatric Orthopaedics and Rheumatology Flashcards
Reactive arthritis background
Inflammation and joint pain triggered by an infection in another part of the body
- GI
- Genitals
- Urinary tract
Reactive arthritis pathophysiology
Sterile synovitis developing after a distant infection
Post diarrheal/ dysentery
o Salmonella
o Shigella
o Campylobacter
Post urethritis/cervicitis (STI)
o Chlamydia trachomatis
presentation of reactive arthritis
- few days – 2 weeks post infection
- acute asymmetrical lower limb arthritis develops.
- Red eye
- Other features: skin (circinate balanitis, keratoderma blennorrhagica), eye (conjunctivitis, uveitis), enthesitis.
- Affected joints
o Knee
o Ankles
o feet
investigations for reactive arthritis
serology/microbiology, inflammatory markers raised, may need joint aspirate to rule out septic/crystal arthritis
management of reactive arthritis
- Treat infection (this may not improve arthritis).
- NSAIDs and joint injections.
- Most will resolve within 2 years.
- Those that do not (esp if HLA-B27+) may need DMARDs.
prognosis of reactive arthritis
Usually goes within 12 months
septic arthritis background
- Septic arthritis refers to infection inside a joint.
- This can occur at any age, but is most common in children under 4 years.
- Infection in a joint is an emergency, as the infection can quickly begin to destroy the joint and cause serious systemic illness.
- Septic arthritis has a mortality around 10%..
- Septic arthritis is a common and important complication of joint replacement.
o It occurs in around 1% of straight forward hip or knee replacements. This percentage is higher in revision surgery.
pathogenic cause of septic arthritis
- Staphylococcus aureus most common
Other: - Neisseria gonorrhoea (gonococcus) in sexually active teenagers
- Group A streptococcus (Streptococcus pyogenes)
- Haemophilus influenza
- Escherichia coli (E. coli)
presentation of septic arthritis
Septic arthritis usually only affects a single joint.
This is often a knee or hip. It presents with a rapid onset of:
- Hot, red, swollen and painful joint
- Refusing to weight bear
- Stiffness and reduced range of motion
- Systemic symptoms such as fever, lethargy and sepsis
Septic arthritis can be subtle in young children, so always consider it as a differential when a child is presenting with joint problems.
investigations of septic arthritis
- X-ray of joint
- Joint aspiration
o Gram staining, crystal microscopy, culture and antibiotics sensitivities
management of septic arthritis
- Empirical IV antibiotics (given until sensitivity are known)
- Long term abx for 3-6 weeks
- May require surgical drainage and washout of the joint to clear the infection
management of septic arthritis
- Empirical IV antibiotics (given until sensitivity are known)
- Long term abx for 3-6 weeks
- May require surgical drainage and washout of the joint to clear the infection
what sort of disease is Henoch-schonlein purpura (HSP)
IgA vasculitis (IgAV)
background Henoch-Schonlein Purpura
- IgA vasculitis (IgAV)
- Palpable purpura without thrombocytopenia
- Most common form of systemic vasculitis in children,
- Unknown cause: 50% develop after URTI
- Peak age 3- 15 years
- Clinical manifestations develop over days and weeks- rash and arthralgia first
pathophysiology of HSP
- Inflammation occurs in affected organs due to IgA deposits in blood vessels
- Rash is caused by inflammation and leaking of blood from small blood vessels under the skin-> purpura
presentation of HSP
- skin
- arthritis/anthralgia
- GI
- kidney
HSP skin
THINK LOWER LIMBS- GRAVITY
Palpable purpura in the lower limbs and buttocks with neither thrombocytopenia or coagulopathy.
- Rash often begins with erythematous, macular wheals – may be itchy; rarely painful.
- Evolves into typical ecchymoses, petechiae and palpable purpura; typically in crops.
- Symmetrically distributed, located in gravity/pressure dependent areas e.g. lower extremities.
HSP: arthritis
- Common (affecting up to 80%) but rarely presents as sole symptom
- Usually transient, oligoarticular (1-4 joints), non-deforming in large lower extremity joints.
HSP: GI
abdominal pain
- affects about 50%
Severity varies:
- from mild: nausea, vomiting, abdominal pain, paralytic ileus.
- to severe: GI haemorrhage, ischaemia, necrosis, intussusception , perforation
HSP: Kidney
o IgA nephritis affects about 20-50%
o Haematuria (most commonly), proteinuria, elevated creatinine ± hypertension.
Investigations for HSP
Exclude serious pathology
- Meningococcal septicaemia
- Leukaemia
Bloods
- FBC
- Blood film for thrombocytopenia
- CRP
- Renal profile (U and E)
- Serum albumin (nephrotic syndrome)
- Blood culture for sepsis
Urine dipstick for proteinuria
- Urine protein: creatinine ratio
BP for hypertension
diagnosis of HSP
Diagnosis: EULAR/PRINTO/PRES criteria from 2010. This requires the patient to have palpable purpura (not petichiae) + at least one of:
* Diffuse abdominal pain
* Arthritis or arthralgia
* IgA deposits on histology (biopsy)
* Proteinuria or haematuria
management of HSP
- Supportive management: analgesia, rest and proper hydration
- +- steroids (debatable
- Monitor closely
o Urine dipstick for renal involvement
o Blood pressure to monitor HTN
prognosis for HSP
Prognosis
- Abdominal pain resolves within days
- Kidneys recover within 4 to 6 weeks
- 1/3 have recurrence iwithin 6 months
- Some will develop end stage renal failure
osteosarcoma background
- Bone cancer
- Usually presents in adolescents and younger adults
->10-20yo - Femur most commonly affected
-> Tibia
-> Humerus - Types- depending on how well-differentiated the cells are when oncogenic events occur
-> Osteoblastic- most highly differentiated
->Chrondroblastic
-> Fibroblastic- least highly differentiated
Risk factors for osteosarcoma
- Usually tall for age
- Males
- Li-Fraumeni syndrome
- RB1 mutation
- Prior radiation therapy
- Pagets/ osteochondromas
Risk factors for osteosarcoma
- Usually tall for age
- Males
- Li-Fraumeni syndrome
- RB1 mutation
- Prior radiation therapy
- Pagets/ osteochondromas
pathophysiology of osteosarcoma
Mutation occurs in rapidly dividing osteoblasts e.g. pubertal growth spurt
presentation of osteosarcoma
- Persistent bone pain
- Worse at night
- May wake them up
- Others:
-> Bone swelling
-> Palpable mass
-> Restricted joint movement - Weight loss, fatigue, headache
investigations for osteosarcoma
- VERY URGENT XRAY within 48 hours
- If Xray suggest sarcoma -> VERY URGENT SPECIALIST ASSESSMENT within 48 hours
- X-ray findings
-> Poorly defined lesion in bone, with destruction of normal bone and ‘fluffy’ appearance
-> Periosteal reaction (irritation of bone lining)- “sun-burst” appearance
->Soft tissue mass
- BLOOD TESTS
Raised alkaline phosphatase (ALP)
Other investigations
- CT\MRI
- Bone scan
- PET
- Bone biopsy
management of osteosarcoma
- Aggressive surgical resection of lesion = LIMB AMPUTATION
- Adjuvant chemotherapy improves outcomes
- MDT support
o Paediatric oncologists and surgeons
o Specialist nurses
o Physiotherapy
o OT
o Psychology
o Dietician
o Prosthetics and orthotics
o SS
Complications of osteosarcoma
- Prognosis poor
- Leading cause of death in the young
- Pathological fractures
- Metastatic disease
Ewings sarcoma background
- Second most common primary bone cancer
- Most commonly occurs in bone, rare cases in soft tissue (extraosseous ewings)
- Requires aggressive treatment
- Flat bones
o Tibia
o Fibula
o Knee joint
o Femur
o Pelvis
o Ribs
risk factors for Ewing sarcoma
- Males
- 10-20yo
- Rare in those older than 30yo
pathophysiology of Ewings sarcoma
- Ewing sarcoma is a small, round blue cell tumour
- Genetic mutation in 95% e.g. translocation between chromosome 11 and 22
- Due to uncontrolled cell turnover and tumour formation
presentation of Ewings sarcoma
- Bone pain: can be misinterpreted as growing pain or sports injuries
- Restricted movement of joint
- Fatigue, weight loss, fever
- Fracture susceptibility
- Red flags
o Unexplained bony lump
investigations of Ewings sarcoma
URGENT X-RAY WITHIN 48 HOURS
- If x-ray suggest cancer- specialist assessment within 48 hours
- Bloods: FBC, UandE, LFTS, ESR< CRP, ALP and bone profile
-> Anaemia, leucotosis, elevated WBC, elevated ESR, elevated LDH, elevated ALP and elevated CRP - Imagine
-> Initial XRAY :destructive, diaphyseal lesion with layer periosteal calcification
-> MRI, CT, PET- staging - Bone biopsy- definitive
manaagment of ewings sarcoma
- Needs to be aggressive: chemo, surgery radiotherapy
-
Chemotherapy
-> Shrinks tumour prior to surgery and destroy remaining tumour cells which may have spread from primary site -
Surgery
->Limb sparing surgery
-> Resection of tumour and either a metal implant or autologous bone graft
-> If tumour spread to widely- amputation performed -
Radiotherapy
o Not used in every patient
o Can be used to shrink tumour prior to surgery
complications of Ewings
Complications
- Metastatic disease
o Lungs
o Bones
o Bone marrow
- Recurrence
Osgood-schlatters disease background
- Pain in the tibial tuberosity where the patella ligament inserts
- Common cause of anterior knee pain in adolescents
- Unilateral
pathophysiology of Osgood-Schlatters
The patella tendon inserts into the tibial tuberosity. The tibial tuberosity is at the epiphyseal plate. Stress from running, jumping and other movements at the same time as growth in the epiphyseal plate result in inflammation on the tibial epiphyseal plate. There are multiple small avulsion fractures, where the patella ligament pulls away tiny pieces of the bone. This leads to growth of the tibial tuberosity, causing a visible lump below the knee. Initially this bump is tender due to the inflammation, but has the bone heals and the inflammation settles it becomes hard and non-tender.
Risk factors for Osgood-Schlatters
- 10-15 yo
- Male
presentation of Oschgood-Schlatters
Gradual onset of symptoms
- Visible or palpable hard and tender lump at the tibial tuberosity
- Pain in the anterior aspect of the knee
- The pain is exacerbated by physical activity, kneeling and on extension of the knee
management of Osgood-schlatters
- Reduce inflammation
o Reduction in physical activity
o ICE and NSAIDS - Once symptoms settle: stretching and PT
prognosis of Osgood-Schlatters
- Patient left with hard boney lump on knee
complication of Osgood-Schlatters
avulsion fracture- rare
presentation of hip pain
- Limp
- Refusal to use the affected leg
- Refusal to weight bear
- Inability to walk
- Pain
- Swollen or tender joint
- acute or chronic
causes of hip pain in 0-4 year olds
- Septic arthritis
- Developmental dysplasia of the hip (DDH)
- Reactive arthritis/ Transient sinovitis
hip pain 5 – 10 years:
- Septic arthritis
- Reactive arthritis/ Transient sinovitis
- Perthes disease
hip pain 10 – 16 years:
- Septic arthritis
- Slipped upper femoral epiphysis (SUFE)
- Juvenile idiopathic arthritis
red flags hip pain
- Child <3
- Fever
- Waking at night with pain
- Weight loss
- Anorexia
- Night sweats
- Fatigue
- Persistent pain
- Stiffness in morning
- Swelling or red joint
general management of hip pain
Referral for assessment in a limping child if:
- Child under 3 years
- Child older than 9 with a restricted or painful hip
- Not able to weight bear
- Evidence of neurovascular compromise
- Severe pain or agitation
- Red flags for serious pathology
- Suspicion of abuse
management of hip pain
- Blood tests including inflammatory markers (CRP and ESR) for JIA and septic arthritis
- X-rays are used to diagnose fractures, SUFE and other boney pathology
- Ultrasound can establish an effusion (fluid) in the joint
- Joint aspiration is used to diagnose or exclude septic arthritis
- MRI is used to diagnose osteomyelitis
Developmental dysplasia of the hip background
Background
- a condition where there is a structural abnormality in the hips caused by abnormal development of the fetal bones during pregnancy
- causes instability in hips and tendency for subluxation or dislocation
screening for DDH
- Screening: Normally pick up on during new born examinations (NIPE)
o 6-8 weeks
o Looking for symmetry in hips, leg length, skin folds and hip moveemnts
o 2 specific tests: Ortolanis and Barlows
DDH Rf
female
- First born
- First degree family history
- Breech presentation
- Multiple pregnancy
NIPE examination
- Different leg lengths
- Restricted hip abduction on one side
- Significant bilateral restriction in abduction
- Difference in the knee level when the hips are flexed
- Ortolanis and Barlows
Ortolanis and Barlows
involves dislocating hip and popping it back in
barlows test
Barlows is done with the baby on their back with the hips adducted and flexed at 90 degrees and knees bent at 90 degrees. Gentle downward pressure is placed on knees through femur to see if the femoral head will dislocate posteriorly.
Ortolani’s
RELOCTATES HIP AFTER BARTONS
is done with the baby on their back with the hips and knees flexed. Palms are placed on the baby’s knees with thumbs on the inner thigh and four fingers on the outer thigh. Gentle pressure is used to abduct the hips and apply pressure behind the legs with the fingers to see if the hips will dislocate anteriorly.
DDH Investigations
US (investigation of choice) and X-ray
Management of DDH
Pavlik harness (children <6 months of age)
-> Fitted and kept on permanently, adjusting for growth of baby
-> Holds femoral head in correct position to allow the acetabulum to develop into a normal shape for around 6-8 weeks
Surgery required if harness fails or diagnosis after 6 months- hip scipa cast used to immobilise the hip for a prolonged period post surgery
Perthes vs Sufe
Perthes
- P for primary school aged kids
- avascular necrosis
SUFE
- S for secofnary school aged kids
- obesity
- fracture
Perthes Background
- Disruption of blood flow to the femoral head -> avascular necrosis
- Affects epiphysis of the femur -> bone distal to the growth plate (physis)
RF for perthes
- Occurs in children aged 4-12 (mostly 5-8)-> primary school
- Male
pathophysiology of perthes
Pathophysiology
- Idiopathic
- One theory suggest repeated mechanical stress to the epiphysis may interrupt blood supply
- Overtime there is revascularisation or neovascularisation and healing of the femoral head
o Remodelling of the bone as it heals
presentation of Perthes
Slow onset
- Pain in the hip or groin
- Limp
- Restricted hip movements
- There may be referred pain to the knee
There will be no history of trauma.
If the pain is triggered by minor trauma, think about slipped upper femoral epiphysis, particularly in older children
investigations for Perthes
- X-ray – may be normal
- Squaring of bone
- Blood tests are typically normal esp inflammatory markers
- Technetium bone scan
- MRI scan
management of perthes
Aim: maintain healthy position and alignment in joint and reduce risk of damage or deformity to the head
- Bed rest
- Traction
- Crutches
- Analgesia
- PT
- regular x-rays to assess healing
- Surgery- in older children or those not healing
-> aims to improve alignment and function of femoral head and hips
Complications of Perthes
- Soft deformed femoral head -> early hip OA
Leads to an artificial total hip replacement
slipped upper femoral epiphysis (SUFE) background
- Head of femur becomes slipps along the growth plate
RF for SUFE
- More common in boys
- 8-15 (12)
- In females occurs at average age of 11
- Obesity
presentation of SUFE
Typical: obese, adolescent undergoing a growth spurt. May be history of minor trauma (disproportionate to severity of trauma)
- Hip, groin, thigh, knee pain
- Restricted range of movement
- Painful limp
- Restricted movement in hip
- Prefer to keep leg in external rotation – limited internal rotation
Investigations SUFE
- X-ray
- Blood tests: esp inflammatory to exclude other causes of joint pain
- Technetium bone scan
- CT/ MRI