Pediatric Sx

Question 1

_______ presents with excessive salivation noted shortly after birth or choking spells when first feeding is attempted.

Answer 1

Esophageal atresia

Question 2

MC form of EA

Answer 2

If there is normal gas pattern in the bowel, the baby has
the most common form of the 4 types, in which there is a blind pouch in the upper esophagus
and a fistula between the lower esophagus and the tracheobronchial tree

Question 3

What should be ruled out if with EA

Answer 3

rule out associated anomalies (the vertebral, anal, cardiac, tracheal, esophageal, renal, and radial [VACTER] constellation

Question 4

Workup for possible VACTER

Answer 4

• Look at the anus for imperforation
• Check the x-ray for vertebral and radial anomalies
• Do echocardiogram looking for cardiac anomalies
• Do U/S for renal anomalies

Question 5

In EA, Primary surgical repair is preferred, but if it has to be delayed, do a _____ to protect the lungs from acid reflux

Answer 5

gastrostomy

Question 6

_______may be the clinical presentation (noted on physical exam) for the VACTER collection of anomalies

Answer 6

Imperforated anus

Question 7

For the imperforated anus itself, look for ____

Answer 7

a fistula nearby (to vagina or perineum).

Question 8

Mx of Imperforate anus with FIA Mx

Answer 8

If present FIA, repair can be delayed until further growth (but before toilet training time).

Question 9

Mx of Imperforate anus with out FIA Mx

Answer 9

If not present, do a colostomy for high rectal pouches (and definitive repair at a later date)

Question 10

In Imperforate anus,

A primary repair can be done right away if_____

Answer 10

the blind pouch is almost at the anus

Question 11

How to do xrays for Imperforate anus

Answer 11

The level of the pouch is determined with x-rays taken upside down (so that the gas in the pouch goes up), with a metal marker taped to the anus.

Question 12

___________ is always on the left and results in bowel residing in the chest

Answer 12

Congenital diaphragmatic hernia

Question 13

In CDH, The real problem is not the mechanical one, but the hypoplastic lung that still has_________

Answer 13

fetal-type circulation

Question 14

When must CDH replay happen?

Answer 14

Repair must be delayed 3–4 days to allow maturation

Question 15

CDH Mx

Answer 15

Babies are in
respiratory distress, and need endotracheal intubation, low-pressure ventilation (careful not
to hyperinflate the contralateral lung), sedation, and NG suction.

Question 16

In _________, the cord is normal (it reaches the baby), the defect is to the right of the cord (lateral), there is no protective membrane, and the bowel looks angry and
matted

Answer 16

gastroschisis

Question 17

In _________ the cord goes to the defect (central), which has a thin membrane
under which one can see normal-looking bowel and a little slice of liver

Answer 17

omphalocele,

Question 18

Small defects can be closed primarily, but large ones require construction of a ________to
house and protect the bowel

Answer 18

Silastic “silo”

Question 19

Babies with gastroschisis also need vascular access for parenteral nutrition, because _______

Answer 19

the angry-looking bowel will not work for about 1 month.

Question 20

__________ is also an abdominal wall defect, but over the pubis (which is not fused), with a medallion of red bladder mucosa, wet and shining with urine

Answer 20

Exstrophy of the urinary bladder

Question 21

Bladder exstrophy, til when should you repair?

Answer 21

repair can be done within the first 1–2 days of life. Delayed repairs do not work

Question 22

Green vomiting and a “double-bubble” picture in x-rays (a large air-fluid level in the stomach and a smaller
one to its right in the first portion of the duodenum) are found in

Answer 22

duodenal atresia, annular pancreas, or malrotation.

Question 23

________ is diagnosed with contrast enema (safe, but not always diagnostic) or upper GI study (more reliable, but more risky).

Answer 23

Malrotation

Question 24

_______ also shows up with green vomiting, but instead of a double bubble there are multiple air-fluid levels throughout the abdomen

Answer 24

Intestinal atresia

Question 25

________ is seen in premature infants when they are first fed. There is feeding intolerance, abdominal distention, and a rapidly dropping platelet count (in babies, a sign of sepsis)

Answer 25

Necrotizing enterocolitis

Question 26

________ is seen in babies who have cystic fibrosis (often hinted at by the mother having it). They develop feeding intolerance and bilious vomiting

Answer 26

Meconium ileus

Question 27

Meconium ileus Xrays

Answer 27

X-rays show multiple dilated | loops of small bowel and a ground-glass appearance in the lower abdomen

Question 28

Meconium ileus Gastrografin enema is both ___ and _____

Answer 28

diagnostic (microcolon and inspissated pellets of meconium in the terminal ileum) and therapeutic (Gastrografin draws fluid in, dissolves the pellets).

Question 29

_______ shows up age ~3 weeks, more commonly in first-born boys, with non-bilious projectile vomiting after each feeding

Answer 29

Hypertrophic pyloric stenosis

Question 30

PE of Hypertrophic pyloric stenosis

Answer 30

they are dehydrated, with visible gastric peristaltic | waves and a palpable “olive-size” mass in the right upper quadrant

Question 31

TX of Hypertrophic pyloric stenosis

Answer 31

correction of the hypochloremic, hypokalemic metabolic alkalosis, followed by pyloromyotomy

Question 32

``` _______ should be suspected in babies age 6- to 8 weeks who have persistent, progressively increasing jaundice (which includes a substantial conjugated fraction). ```

Answer 32

Biliary atresia

Question 33

When to do HIDA scan in suspected biliary atresia

Answer 33

do HIDA scan after 1 week of phenobarbital (which | is a powerful choleretic)

Question 34

HIDA scan of Biliary atresia If no bile reaches the duodenum even with phenobarbital stimulation, do _______

Answer 34

surgical exploration is needed

Question 35

What is the 1/3 rule in Biliary atresia

Answer 35

* 1/3 of cases can get a long-lasting surgical derivation * 1/3 of cases need liver transplant after surviving for a while with a surgical derivation * 1/3 of cases need transplant right away

Question 36

X-rays show distended proximal colon (the | normal one) and “normal-looking” distal colon,

Answer 36

Hirschsprung’s disease (aganglionic megacolon

Question 37

_______ is seen in chubby, healthy looking babies ages 6- to 12 months, who have episodes of colicky abdominal pain which makes them double up and squat.

Answer 37

Intussusception

Question 38

Signs of child abuse

Answer 38

• Subdural hematoma plus retinal hemorrhages (shaken baby syndrome) • Multiple fractures in different bones at different stages of healing • All scalding burns, particularly burns of both buttocks (child was held by arms and legs and dipped into boiling water)