Weak lower muscles, use upper extremities Lose functioning in lower legs & moves up to head Wheel chair bound at 12, most die in adolescence, cardiac & resp failure Parents take great care, when pass, lose sense of self because life was dedicated towards care of child Diagnostic Tests DNA analysis of blood or muscle biopsy o Fatty infiltration Elevated serum enzymes (CK, aldolase, AST) before clinical signs appear EMG – electricity into muscles o Decreased amplitude Treatments No effective treatment Goal is to maintain functioning in muscles for as long as possible o Do not work muscles out more, cant rebuild or workout more (higher wasting away) Respiratory and cardiac problems eventually are focus of treatment Nutritional support
o Like to eat, but can’t when NPO on vent/GI issues Metatarsus Adductus Inward turning of front foot Intrauterine constricting Most common congenital foot deformity Exercise, PT, splint Casting if severe Talipes Equinovarus Clubfoot treatment Bones same length, weakened atrophies lower leg muscles, shortened achilles tendon Soles of feet almost touch Congenital anomaly – intrauterine position or genetic, fam hx = increased risk Both feet effected 1/2 time, other half just 1 foot 1/700-1000 births; males commonly Treatment Serial casting o Changed every 1-2 weeks o After casting done Denis Browne splint Reverse corrective shoes (toes pointing outward) Car seat tilt, backwards Surgery necessary if casting is not corrective Teach parents cast care Bathing, clothing Car seat use Clubfoot Developmental Dysplasia of the Hip DDH - Diagnosis Hip instability, subluxation, acetabular dysplasia
Hip with folds – extra folds on skin indicates dysplasia -skin folds should be symmetrical (on belly with knees bent Allis’ sign – pt on back, lift knees up together, knees not symmetrical o One knee lower than the other with knee flexion Left hip more common than right Genetic/mild from intrauteral positioning Common with breech presentation Can be cultural – positions or swaddling certain ways Ortolani & Barlow maneuvers o Barlow – adduct hips, apply gentle pressure forward & down Femur will dislocate o Ortolani – abduct thighs & push forward gently, both legs Clicks = Dislocate Xray or Ultrasound dx Treatment Pavlik Harness – trust in parents (removeable), assert correctness & constant o Less skin breakdown & bathing easier Spica Cast o Cast that does all the way up half torso and legs o Swelling! Watch for pulses & compartment syndrome ! Depends on severity of dysplasia Spot open for diaper area Skin care important, surgical incisions under cast under diaper area Ensure dont get stool or urine near incisions Double or triple diaper – absorbent part Large diaper around whole cast Legg-Calve’-Perthes Disease Legg-Calve’-Perthes A vascular necross if femoral head 2-12 yrs, common 4-8 yo 1/4000 children Family hx or mild traumatic hx
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- Spring '17