GI anomolies (2)

Question 1

2 yo female with large amt of blood in diaper, otherwise healthy, baby pale, but comfortable, afebrile, tachy, hypotensive with hemoglobin at 4.8 and hematocrit of 14.6%—> What would you perform next?

Answer 1

did Meckels scan

Question 2

How is a Meckels Diverticula a True diverticula

Answer 2

true diverticulum of small bowel with all 4 layers: mucosa, submucosa, muscle propr., serosa

Question 3

Pathogenesis of Meckels:

failure of involution of ______; cnx btw lumen and developing intestine in the yolk sac

Answer 3

vitelline duct

Question 4

Explain the rule of 2’s in Meckels Diverticulum

Answer 4

in 2% population

2 types of abnormal lining (stomach + pancreas)

w/in 2 feet of ileocecal valve

2 in long and symptomatic

by 2 years of age

Question 5

Syptoms of Meckels diverticulum

Answer 5

bleeding into intestine, blockage of intestine, inflammation = diverticulitis

Question 6

Describe histology of Meckels Diverticulum

Answer 6

See gastric mucosa present amidts small bowel: this can cause ulcers bc produces acid

Question 7

Treatment for Meckels

Answer 7

surgical resection and bowel resection with anastomosis

Question 8

baby boy at 4 days old to ED with distened abdomen, bilious emesis and only small smear of meconium at birth, only BM with suppositories

Xray see stool throughout; baby was stabilized given IV fluids via IO line

Answer 8

Hischsprungs

Question 9

You see aganglionated to ganglionated transition in the rectosigomoid of baby you suspect has Hirschspurngs but you Need to do ______ to see if theres ganglion

Answer 9

suction rectal biopsy

Question 10

Congenital defect in intestinal innervtion more common in males, linked with Downs and trisomy 21

Answer 10

Hirschsprungs

Question 11

What genetic mutations are seen in Hirschsprungs

Answer 11

RET mutaiton common (50% familial and 15% sporadic)

= tyr kinase to transduce growth and diff signals in developing tissues including neural crest cells

Question 12

Pathogenesis of Hirschsprungs disease

Answer 12

enteric nueral plexus devos from neural crest cellsand migrating to bowel wall during embryogenesis; normal migration of neural crest cells from sm.int to rectum is disrupted

Question 14

What happens to part of colon distal to innervation in Hirschsprungs?

Answer 14

Get segment of distal colon results w/out nerve cells; BOTH Meissner submucosal AND Auerbach Myetneric

lacking = Agangliosos

Question 15

Transition point btwn aganglionated and ganglionate intestine: peristalsis is absent =

Answer 15

Functional obstruction

Question 16

MOst common area affected by Hischsprungs

Answer 16

rectosigmoid but can be any part of colon

Question 17

26 week twin at 3 weeks post delivery, tolerating full feeds till now, spitting up formula, abdominal distension, increased breathing difficulty: xray see gas all over

DDx?

Answer 17

necrotizing enterocolitis or Sepsis with Ileus

Question 18

Seen in premies under 34 weeks; seen in 10% infants with weight <1500 grams, rare in term infants, occurs weeks after birth, not associated with congenital anomalies

Answer 18

Necrotizing Enterocolitiz

Question 19

Pathology of necrotizing colitis

Answer 19

Prematurity + Dysbiosis + Formula feeding; see impaired mucosal defense, circulatory instability of intestinal tract and Meds that cause intestinal mucosal injury or enhance microbial overgrowth

Question 20

What are some possible tx options to look into for necrotizing colitis in the future?

Answer 20

get babies exposed to better biome; probiotics or fecal transplant

Question 21

What are some diagnostic indicators of Necrotizing Enterocolits?

Answer 21

Abdominal distension, feeding intolerance/emesis, rectal bleeding, abdominal wall erythema, temperature instability/ bradycardia/ pnemotosis on xray =air in intestinal wall

Question 22

INitial treatment of babies with necrotizing enterocolitis

Answer 22

stop enteral feeds! Broad spectrum IV antiBiotics. Gastric decompression. Serial Exams

Medical support: mechanical ventilation, IV resuscitation, tranfution, inotropes

Question 23

When do we consider surgical treatment for baby with necrotizing enterocolitis?

Answer 23

1. pneumoperitoenium or free air
2. Clical deterioration or lack of improvement

3?? discolored abdomen, ‘fixed loop’or obstruction

Question 24

How to babies with Necrotizing enterocolitis do?

Answer 24

Stage I: have feeding intolerance, IG better with medical management

Stage II: some penomatosis with 15% mortality but medical tx possible surgery

STage III: Advanced with ascities and perforation; 30-50% mortality

Question 25

Surgical options for necroziting enterocolitis

Answer 25

Peritoneal Drainage or surgical exporation and resection; Have to resect if there is dead bowel, if just air, do drainage

Question 26

Gross apperance of necrotizing enterocolitis:

Answer 26

ery thin mucosa, necrotic and pussy and nasty looking

Question 27

Histologic apperance of nectorizing enterocolitis

Answer 27

see ischemic necrosis with dead villi tips; absent epithelial layer while deeper tissue is more viable

Question 28

Long term outcome for babies with necrotizing enterocolitis

Answer 28

Long term outcome: Mortality = 20-30% with increase risk of Short gut syndomre, neuro delay and bronchopulmonary dysplasia; very $$$$

Question 29

Day 2 of life male has abdominal distention, can’t pass stool. Full term, no complications, no polyhydramnios, breast fed DO1 and on DOL2 resistant but has normal wet diapers

Answer 29

anorectal malformation

Question 30

Association of anorectal malformations

Answer 30

Associated with Down Syndrome and VACTERL: Vertebral/Anorectal/Carciac/TEF/Renal/Limbs

Question 31

Pathogenesis of anorectal malformaitons

Answer 31

abnormality of caudal descent of urorectum; failure of descent of hindgut to anus or fusion of urorectal ridges to walls of cloaca

Question 32

is most common ‘high’ with rectovesicular (rectum to bladder) or rectourethral fistula

Answer 32

MALE

Question 33

Anorectal malformaiton: femals often with ‘____’ malfomatios to perineum or vestibule OR____ displaced anus

*more present after a few months to years with consitpation

Answer 33

Low

anterior

Question 34

see a pt with anorectal malformation, what else needs to be dones for this pt.

Answer 34

Eval: rule out VACTERL complex with ECHO, renal US and good physical exam

Question 35

Tx option for anorectal malformations

Answer 35

no opening: colostomy with delayed repair OR opening: primary repair +/- colostomy

Question 36

outcomes: depends on severity and associated anomalies

_____ have periodic constipation and ___ have severe constipation of incontinent of stools

Answer 36

100%

<10%