Paediatric Orthopaedics - Complex Needs Flashcards
What is torticollis?
“Crick in the neck”
Abnormal, asymmetrical head or neck position, which may be due to a variety of causes - underlying anatomical distortion causing torticollis is a shortened sternocleidomastoid muscle
What is Scoliosis?
Lateral spinal curvature with secondary vertebral rotation - Thoracic or lumbar curvature can is greater than 10o
What is lordosis?
The normal inward lordotic curvature of the lumbar and cervical regions of the spine
What is kyphosis?
Convex kyphotic curvature of the spine as it occurs in the thoracic and sacral regions
What is Klippel-Fiel Syndrome?
Bone disorder, present from birth, characterized by the abnormal fusion of two or more spinal bones in cervical vertebrae
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What are the features of Klippel-Fiel Syndrome?
- Short neck
- Low hairline - back of the head
- Limited ROM in the neck
What are Muscular Dystrophies?
Progressive, generalised diseases of muscle, most often caused by defective / absent glycoproteins (e.g. dystrophin) in muscle wall membrane ⇒ Tissue most affected by its absence is skeletal muscle
How do the genetic abnormalities in muscular dystrophies lead to progressive muscle weakness?
- Dystrophin provides structural stability to the dystroglycan complex in cell membranes
- Defect ⇒ ongoing cell membrane depolarisation due to Ca2+ entering cells ⇒ degeneration and regeneration of muscle fibres
- Degeneration is faster than regeneration ⇒ progressive necrosis of muscle tissue
- Muscle cell proteins are replaced by adipose and connective tissue ⇒ muscle weakening
What is Spina Bifida?
- Birth defect - incomplete closing of the backbone and membranes around the spinal cord. There are varying degrees of which are classified into the following categories:
- Myelomeningocele
- Myeloschisis
- Meningocele
- Spina bifida Occulta
What is spina bifida with meningocele?
This is spina bifida with herniation of the meninges and CSF without involvement of the spinal elements
What is spina bifida with meningomyelocele?
Spina bifida with herniation of both the meninges, nerve roots and/or spinal cord into the sac.
What is Myeloschisis?
Herniation of the meninges and spinal elements, but is distinctly characterised by flattened, plate-like mass of disorganised neural tissue in the sac. This is the most severe form, as the spinal cord area is left open due to failue of the neural folds to fuse
What is Spina Bifida Occulta?
Vertebral fusion defect only, without spine involvement and of no clinical significance. Skin covers the abnormality, so there are no external visible signs
What are the two main phases of locomotion?
- Stance
- Swing
What are the 3 main tasks of the gait cycle?
- Weight Acceptance
- Single limb Support
- Limb Advancement
What are the 8 subphases of the gait cycle?
- Initial contact
- Loading Response
- Mild Stance
- Terminal stance
- Pre-swing
- Initial swing
- Mid swing
- Terminal Swing
What are the prerequisites of a normal gait?
- Stability in stance
- Clearance in swing
- Preposition of foot
- Adequate step length
- Energy conservation
What is an Apraxic Gait?
Motor disorder in which the individual has difficulty with the motor planning to perform tasks or movements. In the lower limbs, this is characterised as loss of the ability to have normal function of the lower limbs such as walking
What is Antalgic Gait?
Gait altered to reduce experience of pain from affected limb
What is foot drop gait?
- High-stepping to allow clearance of the weak foot
- Occurs in common peroneal nerve palsy
What is an Ataxic Gait?
An unsteady, uncoordinated walk, with a wide base and the feet thrown out, coming down first on the heel and then on the toes with a double tap
What is Trendelenberg Gait?
Abductor function is poor when weight bearing on affected side, so the contralateral hemipelvis falls
What is cerebral palsy?
A permanent and non-progressive motor disorder due to brain damage before birth or during the first 2 years of life
The lesion is static but the clinical picture is not
What are the different classifications of cerebral palsy?
- Spastic - Monoplegia, Hemiplegia etc.
- Dyskinetic - Dystonia, Athetoid, Chorea
- Ataxic - Abnormal force and rhythm
- Rigid
- Hemiballistic
- Mixed (combination of spasticity and athetosis)
What areas of the brain can be involved in cerebral palsy?
- Primary Motor cortex +/- corticospinal/vestibulospinal tracts
- Basal ganglia
- Cerebrellum
What are the prenatal causes of Cerebral palsy?
- Placental insufficiency
- Toxaemia
- Smoking
- Alcohol
- Drugs
- TORCH
What are the perinatal causes of Cerebral palsy?
- Prematurity (most common)
- Anoxic injuries
- Infections
- Kernicterus
- Erythroblastosis fetalis
What are the postnatal causes of Cerebral Palsy?
- Infection (CMV, rubella)
- Head Trauma
What are the clinical features of Cerebral Palsy?
- Weakness
- Spasticity
- Lack of voluntary control
- Poor co-ordination
- Paralysis
- Language/speech problems
- Dynamic/fixed muscle contractures
If someone had spastic cerebral palsy, what area of the brain would be affected?
Lesion in the pyramidal system and motor cortex
If a child has cerebral palsy associated involuntary movements, what area of the brain is usually affected?
This suggests involvement of the basal ganglia
If somone has ataxic cerebral palsy, what area of the brain is affected?
Cerebellum and brainstem
If someone has dyskinteic (athetoid) cerebral palsy, what area of the brain is most commonly affected?
Extrapyramidal system and basal ganglia
How would you assess for cerebral palsy?
Clinical Criteria
- Instrumented Gait analysis
Imaging
- MRI head - visualise lesions (80% abnormality present in CP)
- X-ray - hips (subluxation?), spine
Bloods
- Metabolic Screen?
- Coag studies?
What are the orthopaedic priorities in the MDT management of cerebral palsy?
- Spine - tendancy for scoliosis
- Hip - dislocation common (1/3) - can’t keep perineal area clean
- Feet - planovalgus feet
- Torsional problems - twisting of leg bones - due to spasticity and increased tone
What can be used to maanage spasticity problems in cerebral palsy?
General
- Baclofen
- Diazepam
Specific
- Botulinum toxin
- Surgery
What are the general principles to the management of cerebral palsy?
Keep patient as active as possible
Physiotherapy
- Sitting balance
- Standing Posture
- Gait
What age does scoliosis commonly develop?
10-18 yrs
How is scoliosis classified?
- By aetiology - Congenital, idiopathic, neuromuscular, traumatic, infectious, syndromic, other
- By Age - Infantile, juvenile, Adolescent
- By geography - thoracic, lumber, thoracolumbar
What are non-structural causes of scoliosis?
Extrinsic Causes, e.g:
- Leg length discrepancy
- Hip problem
Scoliosis resolves when causal factor is addressed.
How would you examine someone with suspected scoliosis?
- Inspect posterior torso - spinal contour
- Foraward bend test - Structural scoliosis will look worse when bent forward into flexion.
- Note abnormal neuro or pain
- Hip ROM
What investigations would you do if you suspected scoliosis?
- AP Erect Whole spine +/- Lateral
- MRI - Cerebellar tonsils, Syrinx, Tethering, Vertebral anomalies
- Bone scan - pain
How would you manage someone with Scoliosis?
Treatment based on the severity of the curve and amount of growth remaining
- Observational monitoring
- Bracing
- Surgical spinal arthrodesis (fusion)
What is talipes equinvovarus?
Club Foot
Congenital deformity with unknown aetiology
What is the classic deformity in Talipes Equinvarus?
- Cavus
- Adductus (midfoot)
- Varus (hindfoot)
- Equinus (hindfoot) - fixed
What is the prevalence of talipes equinovarus?
1/1000 live births
Which sex is talipes equinovarus more prevalent in?
Males - 3:1
What percentage of those with talipes equinovarus have bilateral deformity?
50%
What is important in the history for those with talipes equinovarus?
Family history of club foot
How would you investigate a child for suspected talipes equinovarus?
Prenatal Diagnosis
Clinical Diagnosis
Imaging
- X-ray - DDH? Spinal abnormalities? Foot deformity (AP + lat.)
- CT - pre-operative planning
In those with talipes equinovarus, what other abnormalities are important to investigate for?
- DDH
- Spina Bifida
- Other spinal deformities
How would you manage talipes equinovarus?
Specific
- PONSETI METHOD - thumb is placed on the talar head while the forefoot is gently supinated and abducted
- Strapping
- Serial casting - supination and abduction, increasing levels
- Dennis Browne Boots
Surgery - Percutaneous tenotomy of achilles tendon (90% will need this)
What are the complications that can occur in scoliosis?
- Nerve root damage
- Cord traction injury
- Vascular injury
-
Problems of growth
- Growing rods
- Changing rods
- Crankshaft phenomenon
