GI anomolies (2) Flashcards
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?
did Meckels scan
How is a Meckels Diverticula a True diverticula
true diverticulum of small bowel with all 4 layers: mucosa, submucosa, muscle propr., serosa
Pathogenesis of Meckels:
failure of involution of ______; cnx btw lumen and developing intestine in the yolk sac
vitelline duct
Explain the rule of 2’s in Meckels Diverticulum
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
Syptoms of Meckels diverticulum
bleeding into intestine, blockage of intestine, inflammation = diverticulitis
Describe histology of Meckels Diverticulum
See gastric mucosa present amidts small bowel: this can cause ulcers bc produces acid
Treatment for Meckels
surgical resection and bowel resection with anastomosis
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
Hischsprungs
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
suction rectal biopsy
Congenital defect in intestinal innervtion more common in males, linked with Downs and trisomy 21
Hirschsprungs
What genetic mutations are seen in Hirschsprungs
RET mutaiton common (50% familial and 15% sporadic)
= tyr kinase to transduce growth and diff signals in developing tissues including neural crest cells
Pathogenesis of Hirschsprungs disease
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
What happens to part of colon distal to innervation in Hirschsprungs?
Get segment of distal colon results w/out nerve cells; BOTH Meissner submucosal AND Auerbach Myetneric
lacking = Agangliosos
Transition point btwn aganglionated and ganglionate intestine: peristalsis is absent =
Functional obstruction
MOst common area affected by Hischsprungs
rectosigmoid but can be any part of colon
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?
necrotizing enterocolitis or Sepsis with Ileus
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
Necrotizing Enterocolitiz
Pathology of necrotizing colitis
Prematurity + Dysbiosis + Formula feeding; see impaired mucosal defense, circulatory instability of intestinal tract and Meds that cause intestinal mucosal injury or enhance microbial overgrowth
What are some possible tx options to look into for necrotizing colitis in the future?
get babies exposed to better biome; probiotics or fecal transplant
What are some diagnostic indicators of Necrotizing Enterocolits?
Abdominal distension, feeding intolerance/emesis, rectal bleeding, abdominal wall erythema, temperature instability/ bradycardia/ pnemotosis on xray =air in intestinal wall
INitial treatment of babies with necrotizing enterocolitis
stop enteral feeds! Broad spectrum IV antiBiotics. Gastric decompression. Serial Exams
Medical support: mechanical ventilation, IV resuscitation, tranfution, inotropes
When do we consider surgical treatment for baby with necrotizing enterocolitis?
- pneumoperitoenium or free air
- Clical deterioration or lack of improvement
3?? discolored abdomen, ‘fixed loop’or obstruction
How to babies with Necrotizing enterocolitis do?
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
