congenital anomalies of GI

Question 1

atresia and fistulas

Answer 1

often diagnoses shortly after birth d/t feed probs and regurgitaiton

Question 2

tracheoesophageal fistula

Answer 2

lesion occurs because of incomplete separation of the trachea and esophagus that begins during the 4th-5th week gestation

survival rate is >95% if no other issues

Question 3

risks of tracheoesophageal fistula and esophageal atresia

Answer 3

pneumonia, poor nutrition and gastric distention, impaired ventilation

Question 4

how is esophageal atresia usually diagnosed

Answer 4

immediately after birth when the newborn has excessive secretions, coughing, and choking after first feeding

additional indicators: recurrent pneumonias, OG cannot be passed

Question 5

surgical repair for TEF/EA

Answer 5

ligation of fistula and anastamosis of esophagus

Question 6

meckel diverticulum

Answer 6

can mimic appendicitis

occurs in ileum

a remnant of omphalomesenteric duct normally connects embryonic midgut to yolk sac providing nutrients to midgut during development

Question 7

rule of 2s for meckel diverticulum

Answer 7

occurs in 2% of population, 
2 inches in length 
within 2 feet of ileocecal valve
symptomatic by age 2
twice as common in males

Question 8

hirschsprung disease

Answer 8

congenital disorder that causes intestinal obstruction from birth d/t defective innervation of colon d/t failure of migration of neural crest cells

distal colon has no peristaltic contractions

always affects rectum

dx - ganglion cells absent in rectal biopsy

Question 9

treatment for hirschsprung disease

Answer 9

pull through surgery or resection to bypass and remove part of colon, ostomy, rectal irrigation

Question 10

malrotation and midgut volvulus

Answer 10

results from abnormal migration or incomplete rotation of intestines from yolk sac back into abdomen

intestines twist around superior mesenteric artery, can compress vasc supply
can cause intestinal ischemia

1/3 of cases present in first week of life with bilious vomiting, distended abdomen, and hemodynamic instability, bloody stools are ominous sign, rapidly compromised blood supply

this is s true surgical emergency

Question 11

surgery for midgut volvulus

Answer 11

reduce volvulus and relieve obstruction by dividing the fixation bands between teh cecum and the duodenum or jejunum and widening base of mesentery

Question 12

children with less than how much small bowel develop short bowel syndrome and require TPN?

Answer 12

30-40cm

Question 13

Ladd’s procedure

Answer 13

performed to alleviate malrotation of intestines

counterclockwise detorsion of the bowel, surgical division of ladd’s bands, widening of small intestine mesentery, appendectomy, reorientation of small bowel on right and cecum/colon on left