GI anomolies (1)

Question 1

32wk newborn, can’t pass oral gastric tube. Healthy apperance, polyhyrdamnios, What do we expect?

Answer 1

TEF

Question 2

TEF type___ most common= stomach to trachea and see polyhydramnios if its EA only

Answer 2

1

Question 3

>50% association of TEF with VACTERL =

Answer 3

Vertebral, Anorectal, Cardiac, TEF, Renal and Limb

Question 4

How to DX baby with TEF

prenatally:

postbirth:

Answer 4

prenatal: dx see polyhydramnios, small stomach, prominent proximal pouch

Post: ‘mucousy’ baby w/ feeding intolerance or can’t pass NG tube.

Question 5

What type of tests/labs should we run on baby with TEF?

Answer 5

Should have pre-op screening,plain films/renal ultrasound; check for VACTERL associations

Question 6

You’re working with a baby that is older with recurrent cough/choking and your attending tells you to order a video esophagram, why?

Answer 6

Suspects H type atresia

Question 7

Why is jaundice normal in a newborn

Answer 7

newborns have decreased 1/2 life of RBC, liver has less ability to conjugate bilirubin, less enterohepatic circulation

Question 8

Peak jaundice for breast fed vs formula fed newborns

Answer 8

see peak jaundice in full term formula at day three w/ 6 mg/dL

-breast fed will peak at day 4 with 15-18 mg/dL of bilirubin

both should decrease around day 6-7

Question 9

Irregular Jaundice of newborn

Answer 9

Beyond 2-3 weeks age; need to eval.

If Direct >2mg/dL

OR Total bill >12 mg (term)

or Total bili >14mg (premie)

Question 10

Why do we worry about newborn abnromal jaundice?

Question 11

What imaging is recommended for neonatal jaundice?

Answer 11

Metabolic Eval: TORCH, A1TA, CF

Ultrasound

Scintigraphy with PHenobarbital to see fluid conc in liver

Cholangiogram

Liver biopsy

Question 12

Two neonatal causes of bile duct obstruction

Answer 12

Biliary atresia and Choledochoal cyst more common

: tumor, bile plug, trama more rare

Question 13

How does Biliary atresia present?

Answer 13

presents 4-6 weeks with DIRECT hyperbilirubinemia, acholic (clay colored) stools, dark pee

Question 14

What do we see on ultarsound in infant with biliary atresia?

Answer 14

dimunitive/absent GB; spleen/situs/PV then:

do IDA scan with 5 day phenobarbitol prep that gives 100% sensitivity and 94% specificity

Question 15

Natural progression of infant with biliary atresia

Answer 15

progressive, acute then chronic inflammatory cholangiography w/ complete obliteration of extra hepatic biliary structures

By 12 weeks of birth: cholestasis, neocholangiogenesis, hepatocellular injury —> cirrhosis, portal hypertension, end stage liver disease

Death by <2 w/out portoenterostomy and or transplantation

Question 16

Describe the pathogenesis of biliary atresia

Answer 16

Pathogenesis: in utero biliary epithelial injury—> leads to inflammation

Etiology = perhaps infection or toxogenic, most likely not genetic

Get fibrosising of hepatic duct, cystic duct, common bile duct and need to be replaced

Question 17

Whats going on in this picture of patient with biliary atresia?

Answer 17

bile plugs; see hepatocyts not in rows anymore, brown stuff is bile and there is proliferation as well = neocholangiogenesis

Question 18

This picture of biliary atresia represents what?

Answer 18

Intrahepatic ductal proliferation with CK19 stain

Question 19

What is the end result of biliary atresia that you see on histology

Answer 19

see accumulaiton of fibrous tissue with obliterated ducts and eventially cirrhosis which is blue collagen all over

Question 20

Tx for biliary atresia

Answer 20

Kasai procedure: remove fibrotic duct and hook small intestine right to liver and stomach enters downstream in a Roux-en-Y.: make sure to get rid of fibrotic shit before attachment

Question 21

Three keys for post-op care of biliary atresia pats that recieve Kasia procedure

Answer 21

KADE supplements and monitor serum vitamin levels

Ursodeoxycholic Acid given at 20mg/kg/day for 2 years

Ampicillin for prophylaxsis of cholantitis

Question 22

survival rates for biliary atresia

Answer 22

1/3 good long term w/ native liver (if dx before 90 days and bridging fibrosis)

1/3 progressive liver disease      

1/3 short term failure

Question 23

most common reason for pediatric liver transplant

Answer 23

Biliary atresia

Question 24

1.5 yo girl w/ adb pain and decrease PO, no meds, or PMHx, appears jaundice. T.bili = 9.6 and D. bill = 7.8 with elevated liver enZ, alkphos up, amylase elevated

DDx:

Answer 24

Cholelithiasis/Choledochalithiasis, Bile Plug Syndrome, Choledochal cyst

Question 25

What are Choledochal Cysts adn which are common?

Answer 25

abnormal devo of bile ducts Type I (77-90%) and Type IV (15%) most common

Question 26

Pathogenesis of Choledochal cysts

Answer 26

Disordered organogenesis and seen prenatal by 15 weeks, Pancreaticobiliary Malunion (75%) or Acquired weakness or distal obstruction

Question 27

Embryologic reaosoning for choledochal cysts

Answer 27

Abnormal pancreatico-biliary junciton; if too long **\>2 cm** get *reflux of pancreatic* enZ to bile duct

Question 28

How does an infant or in vitro present with choledochoal cyst?

Answer 28

25% time see in infancy on maternal ultrasound: see mass in abdomen—\> born jaundice, FTT

Question 29

More common presentation of choledochal cyts

Answer 29

75% age \>1 yr most often have intermittent pain/ some jaundice and some mass in abdomen Can present with: pancreatitis, cholelithiasis, Cholangitis and perforation but these are RARE

Question 30

Dx for choledochal cysts

Answer 30

Ultrasound is usually enough

Question 31

Tx for type I and IV choledocal cyst

Answer 31

Excision with Hepatico-jejunostomy or duodenostomy +/- liver resection

Question 32

1 day old female, born 39 weeks gestation w/ Apgars 6 and 8. Cyanotic episode that resolved but resuscitated. Bilious emesis after 1st feed \*\*\*BILIOUS EMESIS = SURGICAL EMERGENCY PE: tachycardic, tachypnic, grunting. Give contrast and see ligament of treitz located to left, no contrast getting through

Answer 32

malrotation of gut

Question 33

Embryology of malrotation of gut

Answer 33

4-8 wks GI devos, extends out of abdomen—\> normal rotation 90 deg counterclock and returns at 8-10 weeks with another 180 degrees rotation. Fixed at Duodenal-JJ ligament of Treitz and colon

Question 34

Gut is fixed at the duodenal-JJ and colon via:

Answer 34

ligament of Treitz

Question 35

Pathogenesis of malrotation: narrow mesenteric base allows abnormal mobility of small bowel—\> mesentery can twist = ______ (entire midgut can die) \_\_\_\_\_\_ that cross duodenum can lead to obstruction—\> no blood supply

Answer 35

Midgut volvus Ladd’s bands

Question 36

What to look for on Xray if you suspect malrotation of gut

Answer 36

upper GI adn look for location of ligament of Treitz, should be to patients LEFT of spine

Question 37

Treatement for Malrotation of bowel

Answer 37

Tx: take to OR and do Ladd’s procedure: untwist bowel, take down Ladds bands, widen mesentery place small bowel to the right and colon to the left; give appendectomy

Question 38

Prenatal consult of 29 yo female; see polyhydromnios and double bubble on US

Answer 38

Duodenal Atresia

Question 39

Pathogenesis of Duodenal Atresia

Answer 39

failure of recanalization: duodenum has solid phase early on—\> lumen should re-establish at wk 8: can have duodenal stenosis/ web/atresia

Question 40

Dx duodenal atresia prenatally After birth

Answer 40

Prenatally with polyhydromnios from high intestinal obstruction and no reabsorption of fluid. “double bubble” d/t dilate stomach and proximal duodenum from obstruction W/in hours of birth: vomiting clear or bilious and ‘Double Bubble” on X-ray while rest of abdomen is gasless

Question 41

Level of obstruction in duodenal atresia can be pre or post-ampullary with ____ being post-ampullary and get BILIOUS vomit

Answer 41

80%

Question 42

Duodenal atresia is associated with what up to 40% of the time?

Answer 42

Trisomy 21 or Downs as well as: cardiac, GI and anoretal abnormalities

Question 43

Tx for duodenal atresia

Answer 43

Operative repair: duodenoduodenstomy with anastomosis of proximal to distal duodenum

Question 44

baby born at 34 weeks w h/o of pregnancy complicated by 3rd trimester polyhydramnios. baby didn’t pass meconium, fed had lots of green bilious emesis—\> to NICU PE: baby grunting, tachypnic, HR =190 and RR= 74, no murmrus and lungs CTA. Abdomen distended

Answer 44

Jejunoilileal atresia

Question 46

Pathogenesis of Jejuoileal atresia

Answer 46

in utero vascular disruption leading to ischemic necrosis of intestine—\> nectrotic intestine reabsorbed leading to blind proximal and vital ends with gap

Question 47

What are some causes of jejunoilieal atresia?

Answer 47

**Inherited thrombophilia** leading to spontaneous thrombosis can play a role; risk for clotting abrnomalities in children w/ intestinal atresia ## Footnote Caused by maternal use of vasoconstrictive meds: **pseudoephendrine, cocaine, nicotine**

Question 48

Is Jejunoileal atreasia a familial inherited diseaes? Are they associated with other extrainsteinal anomalies?

Answer 48

rare familial inheritance (only few cases reported); see low birth wt and prematurity d/t polyhydramnios not associated with extra intestinal anomalies

Question 49

Treatement for Jejunoilial atresia

Answer 49

do exploratory laparotomy, primary anastomosis and can have long term issues depending on intestinal length; can cause short gut issues