Pediatric GI Pathology

Question 1

What is the clinical presentation of esophageal atresia or TEF?

Answer 1

Aspiration, regurgitation and respiratory distress noted with initial feeds

Question 2

What are things that are seen with esophageal atresia?

Answer 2

50% have other congenital abnormalities (i.e cardiac)

Observed with VATER, trisomy syndromes

Question 3

Where are most cases of duodenal stenosis observed?

Answer 3

In the proximal portion, close to the ampulla of Vater

Question 4

What are two causes of duodenal stenosis?

Answer 4

Web or annular pancreas

Question 5

How does duodenal stenosis present? Both clinical signs and imaging

Answer 5

Vomiting at birth; if billous, stenosis is distal to ampulla

On imaging see a “double-bubble”

Question 6

How many jejunoileal atresias are single? How many are multiple?

Answer 6

85% single

15% multiple

Question 7

What is the cause of most jejunoileal atresias?

Answer 7

Most cases are due to intrauterine vascular accidents or vascular insults such as volvulus, hernias or necrotizing enterocolitis

Question 8

What is the clinical presentation of jejuno-ileal atresias? Describe for proximal and distal

Answer 8

Proximal atresia result in vomiting

Distal atresias manifest with abdominal dissension and dilated loops on radiographs

Question 9

What is the difference between low and high anorectal atresias?

Answer 9

Low atresia associated with perineal fistula
High atresia associated with fistula of GU tract
Both result in imperforate anus

Question 10

What is an omphalocele? What is its etiology?

Answer 10

Extruded intestine covered by an amniotic sac through the umbilicus due to a failure of retraction through the abdominal wall. It is corrected by surgery

Question 11

What is gastroschisis?

Answer 11

Intestinal extrusion through umbilicus. There is no amniotic sac covering so prognosis is worse

Question 12

What are complications of omphalocele and gastroschisis? (4)

Answer 12

Infection
Atresia
Necrosis
Short gut

Question 13

What is short bowel syndrome ? How is it treated?

Answer 13

Massive loss of bowel resulting in decreased fluid/electrolyte reabsorption, chronic diarrhea, nutrient deficiency.
It is treated with total parental nutrition or bowel transplantation

Question 14

GI Duplications: which side does it usually appear?

Answer 14

On the mesenteric side of the bowel; it shares common wall with intestine but does not communicate with it.

Question 15

What are two types of GI duplications?

Answer 15

Tubular duplication– often asymptomatic

Cystic duplication: secretes fluid and causes obstruction of primary organ

Question 16

On which side are most Gi diverticula observed? What is the most common form?

Answer 16

Gi diverticula are the result of a failure in vitelline obliteration. They are usually on anti mesenteric side of bowel

Meckel’s diverticulum is the most common form

Question 17

What is the presentation of Meckel’s diverticulum?

Answer 17

Meckel’s diverticula can have gastric mucosa– results in gastric acid secretion that causes abdominal pain, rectal bleeding and sometimes perforation

Question 18

Neurenteric remnants/cysts:

Answer 18

Persistence of neural tissue from GI tract in cervical/lumbar area: can cause GI obstruction, respiratory distress, paralysis, infectious/chemical meningitis

Question 19

What are clinical consequences of malrotation of bowel?

Answer 19

Bad looping can lead to volvulus, vascular insults due to internal strangulations

Question 20

What is pseudobstruction? Name some primary and secondary causes

Answer 20

Symptoms of obstruction without mechanical obstruction

Primary: Hirschsprung disease, visceral myopathies
Secondary: Infections, CT disorders, muscular distrophies

Question 21

What are three plexuses of GI Tract?

Answer 21

Meissner’s plexus: superficial submucosa
Henley’s plexus: Deep submucosa
Auerbach’s plexus: Between smooth muscle layers

Question 22

What is clinical finding of hirschsprung disease?

Answer 22

Failure to pass meconium

Question 23

How far is the extent of angangliosis in most Hirschsprung patients?

Answer 23

75% is rectosigmoid

17% is long segment

Question 24

What are genetics of Hirschsprung?

Answer 24

Many cases involve RET mutation

10% incidence in Down’s syndrome

Question 25

What are important contributing factors to necrotizing enterocolitis?

Answer 25

90% of infants are premature and have very low birthweight

Contributing factors: intestinal ischemia, intestinal immaturity, bacterial colonization of gut, enteral feeding

Question 26

What is clinical presentation of necrotizing enterocolitis?

Answer 26

Appears during first two weeks of life with abdominal distension, blood in stools, apnea
Radiology: shows gas in bowel wall

Question 27

What areas are most commonly affected in necrotizing enterocolitis?

Answer 27

Can affect any segment but terminal ileum, cecum, ascending colon most frequently involved (watershed areas)

Question 28

What are consequences of necrotizing enterocolitis?

Answer 28

Healing leads to fibrous strictures, atresia/stenosis

Loss of intestine due to necrosis or resection results in short bowel syndrome

Question 29

What is the pathogenesis of intussusception?

Answer 29

Invagination of one intestinal segment into another

Question 30

What are some of the “lead points” of an intussusception? (3)

Answer 30

Masses, meckel’s diverticulum, lymphoid hyperplasia

Question 31

What are GI abrnormalities associated with CF?

Answer 31

Intestinal: Meconium ileus, distal intestinal obstruction, rectal prolapse
Pancreatic: insufficiency, pancreatitis
Hepatic: chronic hepatic disease, cirrhosis
Nutritional: failure to thrive, hypoproteinemia

Question 32

What is CF effect on pancreas?

Answer 32

Acinar destruction and fibrosis with diffuse fatty replacement leads to loss of exocrine function

Question 33

What are common results of CF on the liver?

Answer 33

Raised LFTs
Hepatomegaly
Hepatic steatosis