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Robbins Pathology GI (Chptr 16,17,18,19) > Chapter 17-Congenital Abnormalities > Flashcards

Chapter 17-Congenital Abnormalities Flashcards

1
Q

What is the esophageal atresia

A

Incomplete development of the esophagus, leading to a thin, noncanalized cord that causes obstruction

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2
Q

Where is esophageal atresia most commonly occurring

A

At the tracheal bifurcation

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3
Q

What is esophageal atresia normally associated with

A

-fistula connecting the upper and lower esophageal pouches to the bronchus or the trachea

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4
Q

What can the presence of the fistula lead to

A

Aspiration, suffocation,pneumonia, fluid and electrolyte imbalance

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5
Q

What conditions are developmental abnormalities of the esophagus associated with

A
  • Congenital heart defects
  • genitalurinary malformations
  • Neurological disease
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6
Q

What is the most common form of congenital intestinal atresia and what is the cause

A

Imperforate anus, which is due to the failure of the cloacal diaphragm to involat

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7
Q

What is the process of stenosis

A

Incomplete atresia due to the reduction in lumen size as a result of fibrosis

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8
Q

What is a diaphragmatic hernia

A

Incomplete formation of the diaphragm allows the abdominal viscera to herniate into the thoracic cavity

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9
Q

What complications is associated with diaphragmatic herniation

A

Pulmonary hypoplasia incompatible with life

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10
Q

When does an omphaloceole occur

A

Closure of the abdominal musculature is incomplete and the abdominal viscera herniate into a ventral membranous sac

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11
Q

When does a gastroschisis occur

A

Similar to an omphaceole, except every layer is involved, including the skins and peritoneum

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12
Q

What is normally associated with omphalocele formation

A

Other birth defects (40%)

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13
Q

What is the most commone site of ectopic gastric mucosa and what is it referred to as

A

Upper third of the esophagus, where it is known as an inlet patch

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14
Q

How is gastric heterotrophs present as

A

Small patches of gastric mucosa in the small bowl or colon will present with occult blood loss due to peptic ulceration

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15
Q

What is the most common diverticulum and where does it commonly occur

A

Meckels diveriticulum, and occurs in the ileum

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16
Q

Which embryo structure leads to formation of Meckel’s diverticulum

A

Viteline duct (connects the gut to the yolk sac)

17
Q

What are the rule of 2s, with Meckels diverticulum

A
  • 2% of population
  • within 2 feet of illeocecal valve
  • 2 inches long
  • 2x more likely in male
  • symptomatic by age 2
18
Q

What is the most common location of acquired diverticula

A

Sigmoid colon

19
Q

What is the location of the blind pouch commonly seen in Meckels

A

Antimesenteric side of the small bowel

20
Q

What is the gender that is more common for Congentical hypertrophic pyloric stenosis

A

3-5x more likely in males

21
Q

Which conditions increases the risk of Congentical hypertrophic pyloric stenosis

A
  • Turner syndrome
  • Trisomy 18
  • erythromycin or azithromycin in first two weeks of life
22
Q

When does congenital hypertrophic pyloric stenosis commonly present

A

Between third and sixth week of life

23
Q

What are the symptoms that will normally present for congenital hypertrophic pyloric stenosis

A

New onset of regurgitation
Projectile, nonbilious vomiting after feeding
Demands for refeeding

24
Q

What are the physical exam findings in a patient with congenital hypertropic pyloric stenosis

A

Firm, ovoid mass in the abdominal wall

-Left to right hyperperistalsis during feeding and before vomiting

25
Q

What is the cause of acquired hypertropic pyloric stenosis

A

Natural gastritis or peptic ulcers

26
Q

Which conditions are associated with Hirshprung

A

Down syndrome

27
Q

What is the condition of genital aganlionic megacolon

A

Hurshprungs

28
Q

Which component of the GI is missing in Hirschsprung disease

A

-Meissner submucoasal and Auerbach myenteric plexus

29
Q

Loss or mutation in which gene results in Hirschsprung disease

A

Receptor tyrosine kinase RET

30
Q

What is the stain that can be used to look for aganlionic conditions in Hirschsprung

A

H and E stain looking for ascetylcholinesterase

31
Q

What is an identifiying feature of Hirschsprung in newborns

A

Unable to pass meconium

32
Q

What are the major life threading conditions associated with Hirschsprung disease

A

Enterocolitis
Fluid and electrolyte imbalance
Perforation
Peritonitis

Robbins Pathology GI (Chptr 16,17,18,19) (32 decks)

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