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
What is the cause of acquired hypertropic pyloric stenosis
Natural gastritis or peptic ulcers
26
Which conditions are associated with Hirshprung
Down syndrome
27
What is the condition of genital aganlionic megacolon
Hurshprungs
28
Which component of the GI is missing in Hirschsprung disease
-Meissner submucoasal and Auerbach myenteric plexus
29
Loss or mutation in which gene results in Hirschsprung disease
Receptor tyrosine kinase RET
30
What is the stain that can be used to look for aganlionic conditions in Hirschsprung
H and E stain looking for ascetylcholinesterase
31
What is an identifiying feature of Hirschsprung in newborns
Unable to pass meconium
32
What are the major life threading conditions associated with Hirschsprung disease
Enterocolitis Fluid and electrolyte imbalance Perforation Peritonitis