CONGENITAL DEFORMITIES Flashcards
1
Q
acquired before birth and are not due to known external environmental factors during the birth or postbirth period
either genetic or influenced by some extrinsic factor – e.g. alcohol or a drug
Acquired disabilities usually are the result of trauma, infection, or other causes.
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CONGENITAL DEFORMITY
2
Q
HISTORY
o Pre-natal – start of the pre-natal check up, ultrasound, drugs and medications taken
o Natal – birth, labor – whether easy or difficult, APGAR, birth weight
o Post-Natal – vaccinations, diseases, diet, milestones
PHYSICAL EXAMINATION o Height, weight, head circumference o Review of systems o Reflexes o Standardized tests
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EVALUATION OF pATIENTS WITH CONGENITAL DEFORMITIES
3
Q
- establishment of medical and functional prognoses for a child with a disability.
- the specific objectives and plans of management can be formulated that will guide the daily activities of the child and family
- In general, it is important to emphasize the importance or cognition and communication over ambulation
- Comfort and a stimulating environment are key
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REHABILITATION PLAN
4
Q
- disorder of movement control and posture
from a non-progressive lesion to an immature brain, occurring in utero, near the time of delivery or within the first 3 years of life. - More than 50% of cases has no etiology
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CEREBRAL PALSY
5
Q
o Pre-Natal Period: - Congenital Infections – TORCH 0 Toxoplasma – from cats, 0 Rubella – German measles, 0 Cytomegalovirus, 0 Herpes – STDS - severe microcephaly, 0 seizures 0 spastic quadriplegia, to mild diplegia. - Iodine – diplegia - Mercury - quadriplegia - Intrauterine subdural hemorrhage - diplegia
o Perinatal period
- complications of prematurity:
include birth weight under 800 g
- Grade III and IV intraventricular hemorrhage
- prolonged seizures
- APGAR score of less than 3 at 20 minutes
Appearance Pulse Grimace Activity Respiration
o FULL TERM GESTATIONS
o NEONATAL ASPHYSXIA - abruptio placenta, placenta previa, nuchal cord, or meconium aspiration
o HYPERBILIRUBINEMIA - Rh disease, G6PD, or ABO incompatibility resulting in Kernicterus
o bacterial or viral sepsis or meningitis - especially within the first 6 months can cause residual motor impairments.
o Traumatic brain injury - can be due to child abuse, trauma and accidents
o Traumatic delivery
o Stroke syndromes and clotting disorders
o Congenital heart diseases
o Heavy metal and organophosphate ingestion can cause quadriplegia.
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CEREBRAL PALSY – RISK FACTORS
6
Q
- gross motor delay is seen in all such patients
- lack of sitting after 6 months being the most common initially recognized deficit
- Poor head control may be recognized earlier in the more involved child
- Delayed ambulation – after 16 – 18 months in a child that is mildly involved
- Motor delay – poor prognostic indicator
- Abnormal motor characteristics (quality of movement) – often mistaken as “early milestones”
- “rolling” at 2 months by opisthotonic posturing,
- “handedness” at less than 1 year in hemiplegics,
- “walking” at 4 months by reflex steppage.
- W-sitting and sacral sitting with posterior pelvic tilt due to hamstring spasticity
- bunny hop crawling
- coming to stand through symmetric extension of the lower limbs due to poor pelvic dissociation in children with diplegia
- buttock hitching in hemiplegics
- abnormal gait patterns (crouch gait, “jump” stance)
A
CEREBRAL PALSY - SYMPTOMS
7
Q
- may result from focal perinatal injury, upper limb is - invariably more involved than the lower limb.
- has the highest incidence of CT/MRI abnormalities - in - the distribution of the middle cerebral artery.
failure to use the involved hand, although hitching on the buttocks rather than crawling on hands and knees is also seen. - Growth retardation of the affected side, associated parietal lobe syndrome are seen in 50%.
- Long-term disability is usually more cosmetic than functional.
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HEMIPLEGIA
8
Q
- the most common type of CP seen in premature babies.
- There is disproportionate involvement of the lower limbs, although upper limb abnormalities, manifested as motor perceptual dysfunction, are very common.
- both lower limb orthoses and upper limb devices may be required and distances covered varies for assistance
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DIPEGLIA
9
Q
- total body involved have the highest incidence of significant cognitive disability with 25% severely involved, 50% moderately involved, and 25% mildly involved.
- Lower limbs are usually more involved than upper limbs with asymmetries not unusual.
- A brief period of hypotonia giving way to early spasticity is usually a poor prognostic sign for independent mobility.
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QUADRIPLEGIA
10
Q
- presents with relatively symmetric involvement of the lower limbs and marked asymmetric involvement of the upper limbs.
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TRIPLEGIA
11
Q
- (athetoid/dystonic) CP accounts for 5% to 8% of cases.
most cases were associated with kernicterus due to Rh disease. - Athetosis, dysarthria, sensorineural deafness, and paralysis of upward gaze were the usual signs, while intelligence was often normal, since cerebral cortex was spared.
- more commonly seen as part of the picture of diffuse hypoxia and may be associated with spasticity, seizures, and retardation
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DYSKINETIC
12
Q
- atonic (hypotonic) and ataxic CP.
- The latter is usually associated with hypotonia.
- If spasticity is present, progressive CNS diseases such as Friedrich’s ataxia must be ruled out.
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RARER TYPES
13
Q
- The clinical type of CP is important
- All children with hemiplegia will walk as will most with true ataxia, while those with atonia usually will not.
- if independent sitting occurs by 2 years, prognosis for ambulation is good
- ability to crawl on hands and knees by 1.5 to 2.5 years was a good prognostic sign
- Persistence of three or more primitive reflexes at 18 to 24 months is a poor prognostic sign
- Combinations of independent ambulation and use of wheeled mobility for greater distances should be introduced.
- Marginal ambulators during childhood may lose functional ambulation during or after adolescence due to:
;progressive orthopedic deformity
;insufficient muscle power and
;Insufficient apacity to accommodate increased height
;weight and social/emotional problems
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PROGNOSIS- CP
14
Q
- Mental retardation of moderate to severe degree is seen in one third, of mild degree in another one third.
- Those with pure dyskinesia as a rule are the brightest, while those with atonia and quadriplegia are most involved.
- Retardation is usually mild in those with diplegia and hemiplegia and may be confused with learning disabilities. Seizures
- up to 35% to 40% of children with CP
- most common in postnatal CP, and about 60% to 70% of those with hemiplegia and quadriplegia
- CT SCAN, MRI and EEG
- Anticonvulsant management
- Abnormal vision is seen in 50% of children with CP.
- Muscle imbalances cause esotropia, exotropia, or hyperopia and are most frequent in diplegia and quadriplegia
- Homonymous hemianopsia can be seen in hemiplegia.
- Paralysis of upward gaze is seen in pure dyskinesia.
- Nystagmus is seen in ataxia.
- Defective tracking can be seen in all types of CP
- HEARING LOSS - may be conductive due to abnormal - Eustachian tube function
- DENTAL PROBLEMS – malocclusions, enamel dysplasias secondary to palatal distortions and abnormal oromotor reflexes, dental caries
- Increased risk of respiratory infections is due to both extrinsic (abnormally high tone and poor control over chest muscles leading to poor sigh and cough mechanisms) and intrinsic (bronchopulmonary dysplasia) reasons.
- Malnutrition
- Abnormal oral motor function – increase risk of aspiration
- Disordered GI motility with reflux proximally and poor transit time distally is seen
- A “neurogenic” bladder can occur in the face of normal sensation
- either a disinhibited bladder or a spastic dyssynergic bladder due to external sphincter spasticity coupled with uncontrolled spastic detrusor contraction
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ASSOCIATED DISABILITIES- CP
15
Q
- true emotional lability as part of an organic pseudobulbar palsy consisting of dysarthria, drooling, and poor chewing.
- Attention deficit disorder with hyperactivity may be seen.
- Poor peer acceptance leading to a negative self-image, school phobia, depression, and anger may be exacerbated during normal periods of transition
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BEHAVIORAL DISORDERS- CP
16
Q
- “Physical” therapy consists of a hands-on approach by physical, occupational, and speech therapists to improve gross motor, fine motor, and oromotor function.
- emphasizes hands-on facilitation of movement and positioning to “normalize” tone and reduce the influence of abnormal postures.
- This can be further assisted by the use of positioning aids, such as sidelyers, adaptive seat inserts, and prone standers.
- “Educators” or “conductors” encourage spontaneous achievement of motor activity without regard for abnormal quality of movement.
- craniosacral manipulation, hyperbaric oxygen, and various electrical stimulation systems, both functional electrical stimulation (FES) and threshold electrical stimulation (TES).
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THERAPY- CP
