Ortho & Neuro

Question 1

Kid w/ limp–MCC

Answer 1

trauma

Question 2

Kid w/ limp–18mo F w/ asymmetric gluteal folds on exam

Answer 2

Developmental Hip Dysplasia

Question 3

Developmental Hip Dysplasia–RF?

Answer 3

first born female, +FH, breech position

Question 4

Developmental Hip Dysplasia–Dx; Tx

Answer 4

clunk on Barlow, US hip if unsure (pelvis ossifies at 4-6 mo so xray no good); Pavilk harness or sx

Question 5

Kid w/ limp–5 y/o M initially w/ painless limp now has pain in his thigh

Answer 5

Legg-Calve’-Perthes Disease. (avascular necrosis).

Question 6

Kid w/ limp–5 y/o M initially w/ a cold 1wk ago now presents w/ a limp & effusion in the hip. X-rays are normal and ESR is 35 (↑), T = 99.8, WBCs = 10K

Answer 6

Transient Synovitis

Question 7

Transient Synovitis–tx

Answer 7

bed rest for 1 wk + NSAIDs

Question 8

Kid w/ limp–14 y/o lanky M w/ nagging knee pain and

decreased ROM of the hip on exam

Answer 8

SCFE. Remember they’re not always fat!

Question 9

SCFE–tx

Answer 9

Surgically close and pin the epiphysis to avoid osteonecrosis

Question 10

Kid w/ limp–14 y/o basketball player has knee pain and swelling of tibial tubercle

Answer 10

Osgood-Schlatter. Overuse injury from jumping

Question 11

Dx–12 y/o F w/ 2 wk hx daily fevers to 102 and a salmon colored evanescent rash on her trunk, thighs and shoulders. Left knee and right knee are swollen.

Answer 11

JRA

Question 12

JRA–good prognostic factor

Answer 12

+ANA

Question 13

JRA–bad prognostic factor

Answer 13

+RF, also polyarticular and older age @ onset

Question 14

JRA–Tx

Answer 14

1st line=NSAIDs, 2nd line=MXT, 3rd line=steroids

Question 15

Dx–2y/o F w/ a 2 wk hx daily fevers to 102 and desquamating rash on perineum. Swollen hands and feet, conjunctivitis and unilateral swollen cervical lymph node.

Answer 15

Kawasaki

Question 16

Kawasaki–lab findings

Answer 16

↑plts (wk2-3), ↑ urine WBC, ↑LFTs, ↑CSF protein

Question 17

Kawasaki–best first test

Answer 17

2D echo and EKG. Repeat the Echo after 2-3wks of tx

Question 18

Kawasaki–tx

Answer 18

Acute = IVIG + high dose aspirin; then aspirin + warfarin

Question 19

Kawasaki–most serious sequellae

Answer 19

coronary artery aneurysm or MI

Question 20

Dx–If F. More common if hx of retinoblastoma or previous radiation. “Onion skinning” on xray. (layers of periosteal development)

Answer 20

Ewing Sarcoma

Question 21

Ewing Sarcoma–tx

Answer 21

rads +/- sx

Question 22

Dx–If >10, more likely. M>F. See “sunburst” and “Codman’s triangle” on xray

Answer 22

Osteogenic sarcoma

Question 23

Osteogenic sarcoma–tx

Answer 23

chemo +/- sx

Question 24

Dx–More diffuse bone pain in a patient w/ petechiae, pallor and increased infections

Answer 24

Bone cancer; Don’t forget bone pain can be presenting sx for leukemia

Question 25

Hydrocephalus--Anytime you see a meningocele or myelomeningocele, do a ____

Answer 25

CT (looking for hydrocephalus, Arnold Chiari II)

Question 26

Anytime you see an infant with a head circumference >95th %, consider _____; sxs

Answer 26

hydrocephalus; bulging fontanelle, ↑DTRs, HA, vomiting.

Question 27

Hydrocephalus--Noncommunicating

Answer 27

Stenosis of CA, tumor/malformation near 4th ventricle

Question 28

Hydrocephalus--Communicating

Answer 28

SAH, pneumoncoccal/TB meningitis, leukemia

Question 29

Dx--Infant with increasing head size, prominent occiput, cerebellar ataxia and delayed motor development.

Answer 29

Dandy-Walker malformation

Question 30

Dandy-Walker--what do you see on CT/MRI?

Answer 30

Cystic expansion of 4th ventricle. Can see Agenesis of cerebellar vermis.

Question 31

Dx--1 y/o develops a fever to 102.4. Four hours later, the parents bring her in after she has a 3-4 minute tonic-clonic seizure

Answer 31

febrile seizure (6mo-6yo)

Question 32

Febrile seizure--next step

Answer 32

Give acetamenophen. NO ↑risk for epilepsy

Question 33

Dx--8 yo M gets in trouble in school because he is always “staring into space”. Episodes last seconds, have lip smacking, and he goes right about his business after they are done

Answer 33

Absence seizure

Question 34

Absence seizure--EEG finding

Answer 34

Hz spike and wave discharge

Question 35

Absence seizure--tx

Answer 35

ethosuxamide or valproic acid

Question 36

Dx--6mo w/ multiple symmetric contraction episodes of neck, trunk and extremities that occur in spells

Answer 36

Infantile spasms (can be associated w/ tuberous sclerosis)

Question 37

Infantile spasms--EEG finding

Answer 37

Hypsarrhythmia = asynchronous, chaotic, bilateral

Question 38

Infantile spasms--tx

Answer 38

1st line = ACTH; 2nd line = prednisone

Question 39

Dx--8y/o w/ difficulty w/ balance while walking, no DTRs, bilateral Babinski and “explosive, dysarthric speech”

Answer 39

Friedrich Ataxia (AR, trinucleotide repeat)

Question 40

Friedrich Ataxia--MCC death

Answer 40

HOCM --> CHF

Question 41

Dx--2y/o w/ gait disturbance, loss of intellectual fxn, nystagmus and optic atrophy. Cresyl violet w/ metachromatic staining

Answer 41

Metachromic leukodystrophy (AR)

Question 42

Metachromic leukodystrophy --deficiency of _____ and accumulation of _____

Answer 42

arylsulfatase A; cerebroside sulfate

Question 43

Dx--12y/o w/ decreased school performance, behavior changes, ataxia, spasticity, hyperpigmentation, ↑K, ↓Na, acidosis

Answer 43

Adrenoleukodystrophy (XLR)

Question 44

Adrenoleukodystrophy--prognosis

Answer 44

death in 10 years

Question 45

Dx--9mo who had previously been reaching milestones starts to lag. Seizures, hypotonia, cherry red macula

Answer 45

Tay Sachs (XLR)

Question 46

Tay Sachs--deficiency of ____; accumulation of _____

Answer 46

hexosaminidase A; GM2

Question 47

Dx--3mo infant lays in the “frog-leg” position,

Answer 47

SMA 1 = Werdnig Hoffman

Question 48

Werdnig Hoffman--prognosis

Answer 48

most die before 2yo

Question 49

Dx--6y/o is brought in 2/2 “clumsiness” and freq falls. Lower leg w/ dec muscle bulk and appears “stork- like”. Multiple sm injuries on hands/feet. Pes cavus and claw hand

Answer 49

Marie-Charcot-Tooth Disease

Question 50

Marie-Charcot-Tooth Disease--dx

Answer 50

Decreased motor/sensory nerve vel, sural nerve bx. *CPK is normal

Question 51

Marie-Charcot-Tooth Disease--Tx

Answer 51

Stablize ankles w/ surgical fusion. Usually normal lifespan and most remain ambulatory.