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