15b Devo abnoramlities

Question 0

VACTERL

Answer 0

Vertebral, anorecctal, cephalic, cardiac, TEF, Renal, Limb

Question 1

Esophageal atresia

Answer 1

Associated with VACTERAL

Question 2

Neonatal jaundice

Answer 2

Formula fed peak at 6mg/dL by day 3
Breast fed peak at 15-18 mg/dL by day 4
* Decr. by day 6

Question 3

Abnormal jaundice

Answer 3

*>2 wks
Direct bili >2mg/dL
Toatal >12(term or >14(preterm)

Question 4

Biliary atresia

Answer 4

*presents at 4-6 wks with jaundice and conj hyperblirubinemia
*Light stool, dark urine
inflammatory Distruction of extra-hepatic biliary tree.
*Bile plugs on liver biopsy
*Bile ductal proliferration (CK19 pos)
*Later total bile ducct obliteration and fibrosis

Question 5

Biliary atreasia Tx

Answer 5

ADEK supplement
Ursodeoxycholic acid 20 mg/day- 2 years
Ampicillin for cholangitis prophylaxis

Question 6

Malrrotation

Answer 6

Ladds bands- Attachment of cecum to gut wall

*Contrast doesnt cross spine- should go to ligament of treitz

Question 7

Duodenal atresia

Answer 7

• Double bubble sign

* Association with trisomy 21

Question 8

JEjunoileal atresia

Answer 8

Ischemic

• thromosis
• vasoactive medications

Question 9

Meckels diverticulum

Answer 9

True diverticulum from failed regression of vitelline duct
Rule of 2- 2% population, 2 feet of ileocecal valve, 2 inches in length, presents by age 2
*Gastric mucosa -true diverticulum with all layers

Question 10

Hirsprungs

Answer 10

Associated with Trisomy 21

• RET mutations- TKreceptor
• aganglionic (big round cells), Huge nerves

Question 11

Necrotizing enterocolitis

Answer 11

• Sometimes treatment with antibiotics or peritoneal drainage is sufficient
• 5% stage II with occasional need for surgery and 15% mortality
• 5% stage III with Needed surgery 28-54% mortality

Overal 20-30% mortality
*Short gut, nerodevelopmental dela, bronchiopulmonary dsplasia