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1.2 Lectures > Week 6 (Congenital Disorders of the GI Tract) > Flashcards

Week 6 (Congenital Disorders of the GI Tract) Flashcards

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1
Q

What are the clinical presentations of Esophageal Atresia?

A

Drooling/choking with first feeds, respiratory distress, inability to pass NGT, abdominal distension

Associated anomalies include VACTERL

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2
Q

What are the symptoms of an isolated Tracheoesophageal Fistula?

A

Coughing and choking with feeding, recurrent pneumonia, failure to thrive

Symptoms typically appear several months of age

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3
Q

What is the first step in the correction of EA/TEF?

A

Division of TEF and closure of trachea

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4
Q

What is the second step in the correction of EA/TEF?

A

Mobilization of esophagus and anastomosis of esophagus

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5
Q

What is the third step in the correction of EA/TEF?

A

Correction of EA/TEF

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6
Q

What abnormality is indicated by a newborn infant unable to tolerate feedings?

A

Esophageal Atresia

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7
Q

What is the significance of the Double Bubble Sign?

A

Indicates Duodenal Atresia

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8
Q

What is the incidence of Duodenal Atresia?

A

1/7000 live births

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9
Q

What mechanism causes Duodenal Atresia?

A

Failure of recanalization of duodenum after epithelial proliferation

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10
Q

What is the common association with Trisomy 21 in relation to Duodenal Atresia?

A

1/3 of infants with DA have Trisomy 21

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11
Q

What embryologic structure develops into the gut tube?

A

Primitive foregut

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12
Q

At what week does the gut tube develop along the length of the embryo?

A

Week 3

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13
Q

What is the result of normal embryologic intestinal rotation?

A

270 degree counterclockwise rotation around the axis of the SMA

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14
Q

What condition can arise from abnormalities of rotation and fixation of the intestine?

A

Midgut volvulus with ischemia

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15
Q

What are Ladd’s Bands?

A

Adhesive bands between proximal jejunum and cecum

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16
Q

Who is known as the Father of American Pediatric Surgery?

A

William E. Ladd, M.D.

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17
Q

What is the embryological timeline for the separation of the trachea and esophagus?

A

Separation begins at 4 weeks and is completed by 6 weeks

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18
Q

What is the milestone for swallowing development in fetal anatomy?

A

Swallowing by 16 weeks

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19
Q

Fill in the blank: The gut returns to the abdomen at week _____

A

10

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20
Q

What is the abnormality that involves a failure of umbilical coelom formation?

Study These Flashcards
A

Gastroschisis

Gastroschisis occurs when the developing gut lacks space to expand and ruptures out of the abdominal wall.

21
Q

Where is gastroschisis typically located?

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A

Always to the right of the umbilicus

This is due to weakness from the involution of the right umbilical vein.

22
Q

What are the consequences of gastroschisis?

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A
  • Bowel exposed to amniotic fluid results in inflammatory rind
  • Loss of abdominal domain
  • Vascular compromise
  • Infarction
  • Atresia
  • Short bowel syndrome

The exposure to amniotic fluid can cause significant complications.

23
Q

What are the treatment options for gastroschisis?

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A
  • Primary reduction and closure immediately at birth
  • Staged reduction with silo followed by delayed closure

Treatment aims to manage the exposed bowel effectively.

24
Q

What is the abnormality characterized by a large abdominal wall defect covered by a membrane?

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A

Omphalocele

Omphalocele can contain organs such as the liver, spleen, and intestines.

25
What distinguishes a giant omphalocele from a regular omphalocele?
Size and contents ## Footnote Giant omphaloceles contain larger amounts of abdominal contents and have higher risks of associated anomalies.
26
What are the associated anomalies with omphalocele?
* Chromosomal anomalies * Cardiac anomalies * Pulmonary hypoplasia ## Footnote The risk of complications depends on the size of the omphalocele and associated anomalies.
27
What is the abnormality indicated by greenish drainage from the umbilicus in a healthy full-term infant?
Patent omphalomesenteric duct remnant ## Footnote This condition requires operative exploration to manage.
28
What does the omphalomesenteric (vitelline) duct connect?
Extracoelemic yolk sac to primitive gut ## Footnote This duct typically obliterates prior to birth.
29
What is the most common vitelline duct remnant?
Meckel’s diverticulum ## Footnote It occurs in 1-2% of the population and may contain heterotopic tissue.
30
What are the potential presentations of Meckel’s diverticulum?
* Bleeding * Intussusception * Inflammation * Bowel obstruction ## Footnote These presentations can lead to significant clinical issues.
31
What is the incidence of jejunoileal atresia?
1:4000 - 1:5000 ## Footnote Approximately 10-12% of jejunoileal atresia cases are associated with cystic fibrosis.
32
What are some potential causes of jejunoileal atresia?
* Vascular accident * Complicated perforation ## Footnote The exact pathophysiology is unknown and may involve multiple factors.
33
What is the classification of jejunoileal atresia based on?
Variability in presentation ## Footnote It is emphasized that the classification should not be memorized but understood for its variability.
34
What is Type 3B?
Jejunal ## Footnote Refers to a type of intestinal atresia affecting the jejunum.
35
What are the challenges associated with jejunal atresia?
Loss of bowel length, size mismatch, loss of contractility in dilated proximal segment, instability of distal mesentery ## Footnote These challenges complicate surgical intervention and management.
36
What is Hirschsprung’s disease?
Aganglionosis of the colon ## Footnote Characterized by the absence of ganglion cells in the enteric nervous system.
37
What part of the nervous system controls bowel motility and secretion?
Enteric Nervous System ## Footnote It is a branch of the autonomic nervous system.
38
What are the two major plexuses of the enteric nervous system?
* Myenteric plexus * Submucosal plexus
39
What causes the failure of plexus development in Hirschsprung’s disease?
Aganglionosis of both plexuses ## Footnote This results in a lack of ganglion cells necessary for bowel function.
40
What are the three basic hypotheses explaining the lack of ganglion cells in Hirschsprung’s disease?
* Migration failure * Microenvironmental support failure * Immunologic destruction
41
What is the genetic aspect of Hirschsprung’s disease?
Familial clusters and increased risk in siblings but not in Mendelian pattern ## Footnote Multiple genes have been identified, including RET and its ligands.
42
What are some clinical presentations of Hirschsprung’s disease?
* Delayed passage of meconium * Neonatal constipation * Abdominal distension * Enterocolitis * Explosive diarrhea * Sepsis * Perforation from enterocolitis
43
What is the diagnostic method for Hirschsprung's disease?
Suction Rectal Biopsy ## Footnote This method allows for the identification of aganglionic segments.
44
What is a key morphological feature of Hirschsprung's disease?
Variable length of aganglionic segment ## Footnote The proximal segment may be dilated while the distal segment is contracted.
45
What is the significance of the cloaca in anorectal malformations embryology?
Cloaca formed at 21 days gestation ## Footnote It is the precursor to both the urogenital and anorectal cavities.
46
What separates the urogenital from the anorectal cavity?
Septum grows to separate them over 6 weeks ## Footnote This process is critical for normal development.
47
What are VACTERL Associated Anomalies?
* Vertebral defects * Anorectal malformations * Cardiac defects * Esophageal atresia/TEF * Renal and genitourinary anomalies * Radial limb deformities
48
What is the conclusion regarding congenital anomalies of the gastrointestinal tract?
Broad spectrum of congenital anomalies ## Footnote Most previously lethal lesions can be surgically corrected.
49
What can structural defects in the gastrointestinal tract result in?
Metabolic abnormalities such as fluid and electrolyte problems and short bowel syndrome ## Footnote These complications can significantly impact patient care.

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