Pediatrics

Question 1

Electrolytes in pyloric stenosis?

Answer 1

Hypokalemia hypochloremic metabolic alkalosis with paradoxical aciduria

Question 2

Most common solid abdominal malignancy in children

Answer 2

neuroblastoma

Question 3

favorable/unfavorable factors for neuroblastoma?

Answer 3

children less than 1 have more advanced disease, N-myc oncogene have unfavorable prognosis

Question 4

congenital lung lesion, presents with hemodynamic instability, hyperinflated lobe – dx/tx?

Answer 4

congenital lobar overinflation (emphysema) - cartilage failed to develop in bronchus, leads to air trapping with expiration – leads to HD instability (similar to tension PNX) – tx w/ lobectomy

Question 5

lung tissue w/ abnormal arterial supply, presents w/ infection - dx/tx?

Answer 5

pulmonary sequestration (Extra vs intralobar – extra = azygos drainage, intra = pulmonary vein drainage), does not communicate with tracheobronchial tree, present w/ infection, tx w/ lobectomy (kids) segmentectomy (adults)

Question 6

cystic lesion that communicates with airway, and poorly developed alveolar structure, recurrent infection - dx/tx?

Answer 6

congenital pulmonary adenoid malformation - tx w/ lobectomy

Question 7

Which CPAM types are associated with malignancy?

Answer 7

CPAM 1 (most common type) and 4

Question 8

what is a type 1 choledochal cyst? what is the treatment?

Answer 8

type 1 (most common ~85%)- fusiform dilation of the entire common bile duct – generally treat with resection and roux-en-Y hepaticojejunostomy

Question 9

what is a type 2 choledochal cyst? what is the treatment?

Answer 9

type 2 - true diverticulum that hangs off the common bile duct – generally able to resect off of the common bile duct to avoid a hepaticojejunostomy

Question 10

what is a type 3 choledochal cyst? what is the treatment?

Answer 10

type 3 - dilation of the intramural common bile duct, involving the sphincter of Oddi - generally treat if symptomatic or young – low malignant risk – possible endoscopic sphincterotomy, vs surgical transduodenal cyst excision

Question 11

what is a type 4 choledochal cyst? what is the treatment?

Answer 11

type 4a is both intra and extahepatic cysts, type 4b with extrahepatic cysts only – if 4b usually resect CBD and treat with hepaticojejunostomy, if there are intrahepatic cysts like in 4a, would also need partial hepatectomy, if extensive intrahepatic disease / symptom would need txp

Question 12

what is a type 5 choledochal cyst? what is the treatment?

Answer 12

type 5 is ‘caroli’s disease’, all intrahepatic cysts, get hepatic fibrosis, often need liver txp

Question 13

tender neck mass just lateral to the SCM: dx / tx

Answer 13

cystic hygroma, resection

Question 14

most common congenital diaphragm location?

Answer 14

posteriolateral most common (Bochladek), anteromedial is less common (Morgagni)

Question 15

most common liver tumor in children

Answer 15

hepatoblastoma

Question 16

most common lung tumor in children

Answer 16

carcinoid

Question 17

most common cause of painless bleeding in children? preferred treatment?

Answer 17

bleeding Meckel’s diverticulum. segmental resection is preferred to diverticulectomy to ensure removal of ulcer

Question 18

embryologic origin of Meckel’s diverticulum

Answer 18

persistent vitelline duct

Question 19

what is the most common type of tracheo-esophageal fistula? second most common?

Answer 19

type C (85%)– 1) blind esophagus 2) distal TE fistula – cannot pass NG to stomach; type A (5%) – similar to type C – blind esophagus (esophageal atresia) but no fistula, no gas is observed in abdomen

Question 20

explain VACTERL

Answer 20

vertebral anomalies, imperforate anus, cardiac abnormalities, TE fistula, radial/renal abnormalities, limb anomalies

Question 21

explain the steps of a Ladd’s procedure

Answer 21

1) eviscerate and detorse counterclockwise if any volvulus is present 2) release Ladd’s cecal bands 3) broaden small bowel mesentery 4) appendectomy 5) place small bowel on right and colon on the left

Question 22

what is cantrell’s pentalogy?

Answer 22

cardiac defects, pericardium defects, sternal cleft, diaphragmatic septum absence, omphalocele

Question 23

gastroschisis vs omphalocele - how to differentiate

Answer 23

gastroschisis is usually off midline (to the right), has NO peritoneal sac covering – due to intrauterine rupture of umbilical vein; omphalocele is sac through cord

Question 24

most significant physiologic abnormality in patients with CDH?

Answer 24

pulmonary hypertension

Question 25

newborn with drooling, coughing, and cyanosis after first feeding – resolves quickly and spontaneously. What is the next step?

Answer 25

pass OG tube – if unable to pass, obtain CXR to confirm DX of esophageal atresia w/ or w/o TE fistula

Question 26

explain paradoxical aciduria in pyloric stenosis

Answer 26

as vomiting continues, serum potassium drops, in an effort to conserve K+, kidney absorbs more Na+ and releases H+ into urine (which in turn leads to more K+ reabsorbed)

Question 27

key factors in determining prognosis of neuroblastoma

Answer 27

age <1 year best prognosis, >3 copies of N-myc amplification have poor prognosis – these tumors are most commonly found in adrenal medulla, can produce catecholamines

Question 28

fetal mesenteric vascular accidents lead to….

Answer 28

jejunal/ileal atresias

Question 29

failure of recanalization of the bowel leads to…

Answer 29

esophageal/duodenal atresias

Question 30

lack fo proper rotation of the bowel leads to…

Answer 30

midgut volvulus / malrotation

Question 31

most common indication for ECMO in neonates

Answer 31

meconium aspiration

Question 32

2 year old, abdominal mass – raccoon eyes, blueberry muffin skin lesions: dx

Answer 32

neuroblastoma (periortbital metastases - proptosis and ecchymosis), met to skin give blueberry muffin lesion)

Question 33

2 year old, abdominal mass, aniridia, GU abnormalities, and mental retardation

Answer 33

WAGR - wilms, aniridia, GU abnormalities, mental retardation

Question 34

most common cause of pathologic lead point for intussusception in children

Answer 34

Meckel’s diverticulum

Question 35

during surgery for type 1 choledochal cyst, cyst found to be adherent to portal vein – best management?

Answer 35

excise anterior portion of cyst with mucosectomy of the posterior cyst to prevent malignant degeneration