pediatrics Flashcards
symbrachydactyly
sporadic
- associated with Polands
- insult to vascular ingrowth
- ectoderm present but mesoderm not
radial longitudinal deficiency work-up
CBC renal US cardiac echo spine xray chromosomal breakage test
TAR
no radius but thumb present check platelets severe bleeding resolved by 1 year 50% cows milk intolerance 50% LE abnormal 50% abnormal facies cardiac and renal anomalies
VACTERL
vertebral anal atresia cardiac tracheo-espphageal fistula renal limb (radial aplasia/tibial aplasia(club foot))
fanconi anemia
radial aplasia cafe au lait spots short stature high rates of leukemia chromosomal breakage test to diagnose treat with bone marrow transplant
holt-oram
bilateral radial dysplasia thumb hypoplastic thumb index syndactyly radioulnar synostosis cardiac (ASD, VSD, conduction abnormalities)
thumb hypoplasia Blauth classification
- small thumb
- abnormal intrinsices
- abnormal extrinsics
a. stable CMC
b. unstable CMC - floating
- absent
neonatal compartment syndrome
- birth trauma or low blood pressure
- presents with blister or eschar (sentinal skin lesion)
- good outcomes with early fasciotomy
treatment hypoplastic thumb
II-IIIA reconstruct. - first webspace deepinging - MCP joint stabilization - opposition transfer (FDS ring or ADM (Huber)) IIIB-V polliciziation
radial polydactyly
most common is Wassel 4 (duplicated proximal phalanx)
- treat at 12 months
- keep ulnar thumb with UCL
triphalangeal thumb
AD bilateral or can be sporadic
- can be associated with blackfan diamond anemia, holt-oram, fanconi’s anemia
syndactyly surgery timing
6-9 months border digit
12-24 months central digit
cleft hand
functional ok but social disaster
close cleft and widen first webspace
madelung
often bilateral
volar subluxation of carpus
short stature
vickers ligament tethers volar ulnar corner of radius to lunate - treament is to excise
- dome osteotomy to correct radius
- ulnar shortening
- associated with Leri-Weill dyschondrosteosis (SHOX-stature homeobox-gene)
arthrogryposis
soft tissue contracture of joints
pediatric trigger finger
18-24 months appears
30-40% resolve spontaneously
may wait until 3 years
pediatric anesthesia
try to avoid >3 hours in kids <3 years old
clinodactyly
associated with Downs syndrome
aperts syndrome
FGFR2
complex complete syndactyly
forearm proximal radioulnar synostosis -> rotational osteotomy in 10-20 degrees of pronation
cerebral palsy
perinatal brain injury
- irreversible and static
- deformity = shoulder internal rotation, adduction, elbow flexion, forearm pronation, wrist flexion/ulnar deviation, finger flexion, thumb in palm
botox
blocks release of acetylcholine at myotendinous junction
treatment elbow contracture in CP
anterior elbow release
biceps tendon lengthening
treatment forearm pronation posture in CP
pronator teres release vs re-routing
ossification carpal bones
capitiate and hamate 6-8 months triquetrum 2-3 years lunate 4 years scaphoid 4-5 years trapezium and trapezoid 5 years pisiform 6-8 years
macrodactyly syndromes
- klippel-trenaunay-weber - localized tissue hypertrophy, vascular malformations
- proteus syndrome - dysproportionate, asymmetric overgrowth of body regions, onset first year of life, overlying skin hypertrophic, hyperpigmented or nevi
lipofibromatous hamartoma
- can be associated with macrodactyly
- ultrasound “coaxial cable-like appearance” of nerve on axial and fusiform mass with longitudinal nerve bundles alternating hypo and hyperechoic bands on sagittal
- no cure. Excision and grafting leads to nerve defects.
treatment for cerebral palsy
- elbow flexion contracture - anterior elbow release, biceps tendon lengthening
- forearm - pronation posture - pronator teres release versus re-routing
- wrist - wrist flexion and ulnar deviation - FCU->ECRB, wrist arthrodesis with PRC, flexor pronator slide
- thumb in palm (metacarpal adduction) adductor contracture - thenar release, EPL rerouting
- flexion contracture of the digits
- -impaired active extension then fractional lengthening of the FDS/FDP
- -static deformity then superficialis to profundus transfer, MP or IP fusion, intrinsic release
- swan neck due to intrinsic/extrinsic imbalance then lateral band rerouting
pediatric fractures
- forearm fractures in proximal 1/3 most likely to have loss or reduction
- dislocation of PRUJ = Montessia
- dislocation of DRUJ = Galeazzi
sprengels deformity
dysplasia and failure of descent of scapula
restricted shoulder movement
thumb in palm
thenar slide - release flexor pollicis brevis, adductor pollicis, first dorsal interosseous
