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GI 3 > GI anomolies (1) > Flashcards

GI anomolies (1) Flashcards

1
Q

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

A

TEF

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2
Q

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

A

1

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3
Q

>50% association of TEF with VACTERL =

A

Vertebral, Anorectal, Cardiac, TEF, Renal and Limb

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4
Q

How to DX baby with TEF

prenatally:

postbirth:

A

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

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

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5
Q

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

A

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

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6
Q

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

A

Suspects H type atresia

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7
Q

Why is jaundice normal in a newborn

A

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

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8
Q

Peak jaundice for breast fed vs formula fed newborns

A

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

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9
Q

Irregular Jaundice of newborn

A

Beyond 2-3 weeks age; need to eval.

If Direct >2mg/dL

OR Total bill >12 mg (term)

or Total bili >14mg (premie)

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10
Q

Why do we worry about newborn abnromal jaundice?

A
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11
Q

What imaging is recommended for neonatal jaundice?

A

Metabolic Eval: TORCH, A1TA, CF

Ultrasound

Scintigraphy with PHenobarbital to see fluid conc in liver

Cholangiogram

Liver biopsy

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12
Q

Two neonatal causes of bile duct obstruction

A

Biliary atresia and Choledochoal cyst more common

: tumor, bile plug, trama more rare

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13
Q

How does Biliary atresia present?

A

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

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14
Q

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

A

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

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

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15
Q

Natural progression of infant with biliary atresia

A

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

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16
Q

Describe the pathogenesis of biliary atresia

A

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

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17
Q

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

A

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

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18
Q

This picture of biliary atresia represents what?

A

Intrahepatic ductal proliferation with CK19 stain

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19
Q

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

A

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

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20
Q

Tx for biliary atresia

A

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

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21
Q

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

A

KADE supplements and monitor serum vitamin levels

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

Ampicillin for prophylaxsis of cholantitis

22
Q

survival rates for biliary atresia

A

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

23
Q

most common reason for pediatric liver transplant

A

Biliary atresia

24
Q

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:

A

Cholelithiasis/Choledochalithiasis, Bile Plug Syndrome, Choledochal cyst

25
Q

What are Choledochal Cysts adn which are common?

A

abnormal devo of bile ducts

Type I (77-90%) and Type IV (15%) most common

26
Q

Pathogenesis of Choledochal cysts

A

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

27
Q

Embryologic reaosoning for choledochal cysts

A

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

28
Q

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

A

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

29
Q

More common presentation of choledochal cyts

A

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
30
Q

Dx for choledochal cysts

A

Ultrasound is usually enough

31
Q

Tx for type I and IV choledocal cyst

A

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

32
Q

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

A

malrotation of gut

33
Q

Embryology of malrotation of gut

A

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

34
Q

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

A

ligament of Treitz

35
Q

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

A

Midgut volvus

Ladd’s bands

36
Q

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

A

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

37
Q

Treatement for Malrotation of bowel

A

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

38
Q

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

A

Duodenal Atresia

39
Q

Pathogenesis of Duodenal Atresia

A

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

40
Q

Dx duodenal atresia prenatally

After birth

A

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

41
Q

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

A

80%

42
Q

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

A

Trisomy 21 or Downs

as well as: cardiac, GI and anoretal abnormalities

43
Q

Tx for duodenal atresia

A

Operative repair: duodenoduodenstomy with anastomosis of proximal to distal duodenum

44
Q

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

A

Jejunoilileal atresia

45
Q
A
46
Q

Pathogenesis of Jejuoileal atresia

A

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

47
Q

What are some causes of jejunoilieal atresia?

A

Inherited thrombophilia leading to spontaneous thrombosis can play a role; risk for clotting abrnomalities in children w/ intestinal atresia

Caused by maternal use of vasoconstrictive meds: pseudoephendrine, cocaine, nicotine

48
Q

Is Jejunoileal atreasia a familial inherited diseaes?

Are they associated with other extrainsteinal anomalies?

A

rare familial inheritance (only few cases reported); see low birth wt and prematurity d/t polyhydramnios

not associated with extra intestinal anomalies

49
Q

Treatement for Jejunoilial atresia

A

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

50
Q
A

GI 3 (15 decks)

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