CTC and Case reviews

Question 1

most common soft tissue mass in trachea of a kid

Answer 1

hemangioma

Question 2

what percent of meconium aspiration leads to pnx

Answer 2

20-40%

Question 3

how to differentiate btw beta hemolytic step pna and RDS

Answer 3

beta hemolytic strep pna has pleural effusions

Question 4

what type of ca can CCAM transform into

Answer 4

pleuropulmo blastoma, rhabdomyosarcoma

Question 5

where is CLE most common? what is the rx?

Answer 5

LUL, lobectomy

Question 6

what infection is associated with CDH on the right?

Answer 6

GBS pna

Question 7

what is pappiloomatosis, what is the imaging appearnace

Answer 7

assoc with perinatal HPV infection, 2 percent risk of sq cell ca, see soft tissue masses/nodules in the airways and lungs

Question 8

what nuc med tracer has affinity for thymic rebound

Answer 8

FDG

Question 9

what type of neuroblastoma in a kid has better prognosis

Answer 9

thoracic

Question 10

what are the imaging features of pleuropulmo blastoma? what extra pulmo association does it have?

Answer 10

no calcs, no rib invasion, can be cystic or solid (looks like CCAM). associated with multilocular cystic nephroma in 10%

Question 11

most common type of TE fistula? what do u see in the stomach?

Answer 11

N type - blind ending E with distal connection of E and T, see too much air in the stomach

Question 12

what percent of duodenal atresias have downs? what percent have polyhydramnios and premature?

Answer 12

30 percent have downs, 40 percent have ploy/PT

Question 13

what are the signs of duod atreasia vs duod obstruction (ex due to malrotation/volvulus)

Answer 13

duod atresia: double bubble with no distal gas

Question 14

what are the signs of duod obstruction?

Answer 14

double bubble with a small amount of distal gas, causes are web, stenosis, annular panc, ladd bands

Question 15

what are the measurements for Hypertrophic pyloric stenosis? age range?

Answer 15

4 mm single wall, 14 mm length, age 2-12 weeks (NOT after 3 mo of age)

Question 16

what are the signs of distal ileal atresia

Answer 16

long microcolon, contrast does NOT reach ileal loops

Question 17

risk of perforation with intusseception? why is air reduction better than barium?

Answer 17

0.5 percent. air causes less peritonitis (spillage of fecal material)

Question 18

what are enteric duplication cysts assoc with?

Answer 18

vertebral anomalies, 30 percent of the time

Question 19

what is the only parameter associated with decreased incidence of NEC?

Answer 19

breast feeding

Question 20

what part of colon is NEC most common in

Answer 20

right colon

Question 21

is maternal AFP higher in omphalocele or gastroschisis

Answer 21

gastroschisis

Question 22

what time frame is physiologic gut herniation seen

Answer 22

6-8 weeks, up to 11 weeks

Question 23

what liver tumors in kids have high AFP

Answer 23

hepatoblastoma (solid, calcs in 50%) and HCC

Question 24

what is most common type of choledochal cyst? what is carolis?

Answer 24

type 1 (focal dilation of CBD), carolis is type 5 and intrahepatic bil-dil only see central dot sign

Question 25

what is carolis associated with

Answer 25

ARPKD and medullary sponge kidney

Question 26

what do u see in the liver in osler weber?

Answer 26

liver AVMs, massively dilated hepatic a, cirrhosis

Question 27

what type of peds liver tumor is gallium avid

Answer 27

fibrolamellar HCC

Question 28

what is the most common extrahepatic sign of biliary atresia

Answer 28

absent GB

Question 29

what is seen in biliary atresia, what are the associations

Answer 29

no extrahepatic bile ducts, triangle cord sign on US (echogenic structure at porta hep), assoc with trisomy 18 and polysplenia

Question 30

what is fibrosing colonopathy

Answer 30

thickening of the proximal colon due to enzyme replacement threapy in CF patients

Question 31

what GU problems are seen with unilateral renal agensis

Answer 31

absent ipsi ureter, absent ipsi hemitrigone, absent ipsi vas def, seminal vesicle cyst on ipsi side

Question 32

what is the risk of wilms in horseshoe kidney

Answer 32

8 times higher risk of wilms

Question 33

what cancer can develop in a horseshoe kidney

Answer 33

renal carcinoid

Question 34

what percent of congenital UPJ obstruction is bilateral

Answer 34

20%

Question 35

what kind of bladder cancer is assoc with patent urachus

Answer 35

adenoca

Question 36

what is the mgmt for nephroblastomatosis

Answer 36

US q3 mo until the age of 7 to make sure it doesnt go to wilms

Question 37

what does mesoblastic nephroma look like

Answer 37

solid mass on infancy, usually involves the renal pelvis

Question 38

what is unique about clear cell variant of wilms

Answer 38

likes to met to the bones

Question 39

age presentation diff btw NB and wilms?

Answer 39

NB: usually before 2 years, can be born with it. Wilms: usually around 4 yo, never born with it

Question 40

what is the next best step if u suspect wilms on imaging?

Answer 40

NEVER biopsy bc u can seed the tract, remove it surgically/chemo first

Question 41

what is the best test for NB mets?

Answer 41

MIBG scan

Question 42

what is the paraneoplastic syndrome assoc with NB?

Answer 42

opsomyoclonus (dancing eyes and feet)

Question 43

what percent of SC teratomas are b9?

Answer 43

eighty percent

Question 44

what is the most common extratesticular mass in a kid?

Answer 44

embryonal rhabdo from the spermatic cord or epeidydmis

Question 45

what are the most common testicular germ cell tumors in a kid

Answer 45

yolk sac (v high AFP) and teratoma

Question 46

how do sertoli cell testicular tumors present

Answer 46

bilateral, burned out tumors, assoc with Peutz Jehgers

Question 47

in what condition is sindig larsen johansen syndrome seen?

Answer 47

kids with cerebral palsy

Question 48

what is the most common acquired cause of hypothyroidism in kids

Answer 48

hashimotos

Question 49

what is amelia?

Answer 49

absent limb

Question 50

what is rhizomelia?

Answer 50

proximal limbs are short (femur, humerus)

Question 51

what is acromelia?

Answer 51

distal limbs are short

Question 52

what is mesomelia?

Answer 52

middle limbs are short (forearm)

Question 53

what is a risk factor for achondroplasia?

Answer 53

advanced paternal age

Question 54

what is jaffe campanacci syndrome

Answer 54

multiple NOFs, MR, hypogonadism, cyrptochidism, multiple cafe au lait spots, cardiovasc probs

Question 55

what is the inheritance pattern for multiple hereditary exostosis? what is the risk of malignant degeneration in HME?

Answer 55

AD, risk of malignant deg: 1-20%

Question 56

what is the most common mechanism of elbow injury in kids?

Answer 56

hyperextension - usually results in posterior displacement of the fracture fragment

Question 57

what is the difference in managment for extension vs flexion type injuries in elbow fractures in kids?

Answer 57

flexion requires open reduction, extension usually closed reduction

Question 58

what are the most common types of elbow injuries in kids (most to least common)

Answer 58

supracondylar fx, lateral condylar fx, medical epicondyle

Question 59

what type of CCAM is associated with other anomalies?

Answer 59

type 2 (many cysts, uniform size) assoc with skeletal, cardiac, intestinal)

Question 60

what are the CNS manifestations of LCH?

Answer 60

hypertrophied, Low T1 pituitary stalk, diabetes insipidus

Question 61

what ribs show notching in coarctation of the aorta? what vessels supply the distal aorta in coarctation? what is the gender predominance?

Answer 61

inferior surface of the 3rd to 5th ribs. mediastinal, intercostal, superior epigastric vessels. male predominance.

Question 62

what is the most frequently occurring symptomatic vascular ring? iare cardiac anomalies more prevalent in right or left aortic arch with aberrant subclavian artery?

Answer 62

right aortic arch with aberrant left subclavian, cardiac anomalies are more common with a right aortic arch (as opposed to left aortic arch with aberrant right sublavian)

Question 63

what is denys drash syndome?

Answer 63

meduallary glomerulosclerosis, wilms tumor, ambigious genetalia. almost all have nephroblastomatosis (increased risk of wilms)

Question 64

what is the abnl relationship btw the SMA and SMV seen in malrotation?

Answer 64

SMA to the right of the SMV = abnormal

Question 65

are bladder ears seen in a distended or non distended bladder?

Answer 65

non distended

Question 66

what are bladder ears associated with

Answer 66

it is due to patent process vaginalis - assoc with inguinal hernia

Question 67

what is the most common cause of a palpable abdominal mass in a neonate?

Answer 67

UPJ obstruction

Question 68

what is potter SYNDROME

Answer 68

oligohydramnios assoc with pulmonary hypoplasia