pediatrics Flashcards

1
Q

symbrachydactyly

A

sporadic

  • associated with Polands
  • insult to vascular ingrowth
  • ectoderm present but mesoderm not
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2
Q

radial longitudinal deficiency work-up

A
CBC
renal US
cardiac echo
spine xray
chromosomal breakage test
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3
Q

TAR

A
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
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4
Q

VACTERL

A
vertebral
anal atresia
cardiac
tracheo-espphageal fistula
renal
limb (radial aplasia/tibial aplasia(club foot))
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5
Q

fanconi anemia

A
radial aplasia
cafe au lait spots
short stature
high rates of leukemia
chromosomal breakage test to diagnose
treat with bone marrow transplant
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6
Q

holt-oram

A
bilateral radial dysplasia
thumb hypoplastic
thumb index syndactyly
radioulnar synostosis
cardiac (ASD, VSD, conduction abnormalities)
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7
Q

thumb hypoplasia Blauth classification

A
  1. small thumb
  2. abnormal intrinsices
  3. abnormal extrinsics
    a. stable CMC
    b. unstable CMC
  4. floating
  5. absent
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8
Q

neonatal compartment syndrome

A
  • birth trauma or low blood pressure
  • presents with blister or eschar (sentinal skin lesion)
  • good outcomes with early fasciotomy
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9
Q

treatment hypoplastic thumb

A
II-IIIA reconstruct.  
- first webspace deepinging
- MCP joint stabilization
- opposition transfer (FDS ring or ADM (Huber))
IIIB-V polliciziation
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10
Q

radial polydactyly

A

most common is Wassel 4 (duplicated proximal phalanx)

  • treat at 12 months
  • keep ulnar thumb with UCL
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11
Q

triphalangeal thumb

A

AD bilateral or can be sporadic

- can be associated with blackfan diamond anemia, holt-oram, fanconi’s anemia

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12
Q

syndactyly surgery timing

A

6-9 months border digit

12-24 months central digit

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13
Q

cleft hand

A

functional ok but social disaster

close cleft and widen first webspace

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14
Q

madelung

A

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)

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15
Q

arthrogryposis

A

soft tissue contracture of joints

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16
Q

pediatric trigger finger

A

18-24 months appears
30-40% resolve spontaneously
may wait until 3 years

17
Q

pediatric anesthesia

A

try to avoid >3 hours in kids <3 years old

18
Q

clinodactyly

A

associated with Downs syndrome

19
Q

aperts syndrome

A

FGFR2
complex complete syndactyly
forearm proximal radioulnar synostosis -> rotational osteotomy in 10-20 degrees of pronation

20
Q

cerebral palsy

A

perinatal brain injury

  • irreversible and static
  • deformity = shoulder internal rotation, adduction, elbow flexion, forearm pronation, wrist flexion/ulnar deviation, finger flexion, thumb in palm
21
Q

botox

A

blocks release of acetylcholine at myotendinous junction

22
Q

treatment elbow contracture in CP

A

anterior elbow release

biceps tendon lengthening

23
Q

treatment forearm pronation posture in CP

A

pronator teres release vs re-routing

24
Q

ossification carpal bones

A
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
25
Q

macrodactyly syndromes

A
  • 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
26
Q

lipofibromatous hamartoma

A
  • 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.
27
Q

treatment for cerebral palsy

A
  • 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
28
Q

pediatric fractures

A
  • forearm fractures in proximal 1/3 most likely to have loss or reduction
  • dislocation of PRUJ = Montessia
  • dislocation of DRUJ = Galeazzi
29
Q

sprengels deformity

A

dysplasia and failure of descent of scapula

restricted shoulder movement

30
Q

thumb in palm

A

thenar slide - release flexor pollicis brevis, adductor pollicis, first dorsal interosseous