Pediatric GI Pathology Flashcards
What is the clinical presentation of esophageal atresia or TEF?
Aspiration, regurgitation and respiratory distress noted with initial feeds
What are things that are seen with esophageal atresia?
50% have other congenital abnormalities (i.e cardiac)
Observed with VATER, trisomy syndromes
Where are most cases of duodenal stenosis observed?
In the proximal portion, close to the ampulla of Vater
What are two causes of duodenal stenosis?
Web or annular pancreas
How does duodenal stenosis present? Both clinical signs and imaging
Vomiting at birth; if billous, stenosis is distal to ampulla
On imaging see a “double-bubble”
How many jejunoileal atresias are single? How many are multiple?
85% single
15% multiple
What is the cause of most jejunoileal atresias?
Most cases are due to intrauterine vascular accidents or vascular insults such as volvulus, hernias or necrotizing enterocolitis
What is the clinical presentation of jejuno-ileal atresias? Describe for proximal and distal
Proximal atresia result in vomiting
Distal atresias manifest with abdominal dissension and dilated loops on radiographs
What is the difference between low and high anorectal atresias?
Low atresia associated with perineal fistula
High atresia associated with fistula of GU tract
Both result in imperforate anus
What is an omphalocele? What is its etiology?
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
What is gastroschisis?
Intestinal extrusion through umbilicus. There is no amniotic sac covering so prognosis is worse
What are complications of omphalocele and gastroschisis? (4)
Infection
Atresia
Necrosis
Short gut
What is short bowel syndrome ? How is it treated?
Massive loss of bowel resulting in decreased fluid/electrolyte reabsorption, chronic diarrhea, nutrient deficiency.
It is treated with total parental nutrition or bowel transplantation
GI Duplications: which side does it usually appear?
On the mesenteric side of the bowel; it shares common wall with intestine but does not communicate with it.
What are two types of GI duplications?
Tubular duplication– often asymptomatic
Cystic duplication: secretes fluid and causes obstruction of primary organ
On which side are most Gi diverticula observed? What is the most common form?
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
What is the presentation of Meckel’s diverticulum?
Meckel’s diverticula can have gastric mucosa– results in gastric acid secretion that causes abdominal pain, rectal bleeding and sometimes perforation
Neurenteric remnants/cysts:
Persistence of neural tissue from GI tract in cervical/lumbar area: can cause GI obstruction, respiratory distress, paralysis, infectious/chemical meningitis
What are clinical consequences of malrotation of bowel?
Bad looping can lead to volvulus, vascular insults due to internal strangulations
What is pseudobstruction? Name some primary and secondary causes
Symptoms of obstruction without mechanical obstruction
Primary: Hirschsprung disease, visceral myopathies
Secondary: Infections, CT disorders, muscular distrophies
What are three plexuses of GI Tract?
Meissner’s plexus: superficial submucosa
Henley’s plexus: Deep submucosa
Auerbach’s plexus: Between smooth muscle layers
What is clinical finding of hirschsprung disease?
Failure to pass meconium
How far is the extent of angangliosis in most Hirschsprung patients?
75% is rectosigmoid
17% is long segment
What are genetics of Hirschsprung?
Many cases involve RET mutation
10% incidence in Down’s syndrome
What are important contributing factors to necrotizing enterocolitis?
90% of infants are premature and have very low birthweight
Contributing factors: intestinal ischemia, intestinal immaturity, bacterial colonization of gut, enteral feeding
What is clinical presentation of necrotizing enterocolitis?
Appears during first two weeks of life with abdominal distension, blood in stools, apnea
Radiology: shows gas in bowel wall
What areas are most commonly affected in necrotizing enterocolitis?
Can affect any segment but terminal ileum, cecum, ascending colon most frequently involved (watershed areas)
What are consequences of necrotizing enterocolitis?
Healing leads to fibrous strictures, atresia/stenosis
Loss of intestine due to necrosis or resection results in short bowel syndrome
What is the pathogenesis of intussusception?
Invagination of one intestinal segment into another
What are some of the “lead points” of an intussusception? (3)
Masses, meckel’s diverticulum, lymphoid hyperplasia
What are GI abrnormalities associated with CF?
Intestinal: Meconium ileus, distal intestinal obstruction, rectal prolapse
Pancreatic: insufficiency, pancreatitis
Hepatic: chronic hepatic disease, cirrhosis
Nutritional: failure to thrive, hypoproteinemia
What is CF effect on pancreas?
Acinar destruction and fibrosis with diffuse fatty replacement leads to loss of exocrine function
What are common results of CF on the liver?
Raised LFTs
Hepatomegaly
Hepatic steatosis
