GI Infant Disorders

Question 1

When does cleft lip occur?

Answer 1

During embryonic development.

Question 2

What positive and negative teratogenic factors are associated with cleft lip?

Answer 2

Smoking and viral infections (positive), folic acid deficiency (negative).

Question 3

What happens to make a cleft lip occur? What weeks during embryonic development does this occur?

Answer 3

Maxillary and nasal structures don’t fuse. Week 5-8.

Question 4

What does CL often occur with?

Answer 4

Cleft palate

Question 5

What is cleft palate? What weeks during embryonic development does this occur? What is the treatment for CL and CP?

Answer 5

Incomplete fusion of palatine structures. Weeks 9-12. Surgery.

Question 6

CP has a strong link to what during pregnancy?

Answer 6

Smoking.

Question 7

What other structure is malformed with CP?

Answer 7

Nasal structures.

Question 8

Is pyloric stenosis (PS) a functional or congenital problem? What is it?

Answer 8

Functional. Hypertrophy and constriction at pylorus.

Question 9

When does pyloric stenosis occur in children? Who is it more common in?

Answer 9

2-8 weeks old. Females.

Question 10

What is the etiologic factor for PS? 3 possible links?

Answer 10

Idiopathic. Links: hypergastrinemia, PGE (prostaglandin e, local hormone) and erythromycin exposure.

Question 11

What is the patho for PS? Manifestation? Complications?

Answer 11

Hypertrophy - constriction - inflammation and exudate forms - obstruction - feed can’t pass into duodenum - stomach stretch. Projectile vomit. Dehydration and malnourishment.

Question 12

How is PS diagnosed? Treatment?

Answer 12

Palpable tiny mass in URQ and US. Surgery to open sphincter.

Question 13

Gastroesophageal reflux (GER): common in what age? What type of disorder is this?

Answer 13

0-3 months. Neuromuscular etiology, functional issue.

Question 14

What happens in GER? What is the patho of it?

Answer 14

Reflux occurs at the distal esophageal sphincter. Gastric contents move into esophagus - esophagitis.

Question 15

What are complications of GER related reflux?

Answer 15

Growth issues (baby not wanting to feed), esophageal erosion.

Question 16

What are treatment methods of GER? How long does this take to correct itself usually?

Answer 16

Antacid, PPI, H2RA, modify behaviour around feeding (positioning of infant) and modify feed (smaller feeds increased viscosity). Fundoplication to fortify cardiac sphincter?

Question 17

What is Hirschprung Disease? What causes this disease? Is it common?

Answer 17

Genetic problem. RET gene mutation on chr. 10. Not common.

Question 18

What is the RET gene? What do the proteins it codes for do?

Answer 18

RET gene codes for protein involved in cell signalling for formation of neural tissue in colon specifically.

Question 19

What is the patho of Hirschprung disease? Complications?

Answer 19

RET gene mutation - part(s) of colon lack parasympathetic ganglia (neural tissue) - no localized peristalsis - accumulation of contents - colon distention - abdominal distention. Rupture of colon, peritonitis.

Question 20

What is the treatment for Hirschprung disease?

Answer 20

Aganglionic segment of colon removed.

Question 21

What is intussusception? Where is the most common part in the GIT for this to occur? Why?

Answer 21

Invagination of intestine into adjoining part. Illeocecal valve because of a smaller diameter moving through to a larger diameter.

Question 22

What is the patho for intussusception? What are 3 complications? How is this treated?

Answer 22

Invagination - obstruction - inflammation - edema - ischemia due to increase intraluminal pressure. Necrosis perforation, peritonitis. Hydrostatic reduction (using water soluble contrast medium and air pressure). Surgery required sometimes.