Congenital Abdominal Wall Defects

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Questions and Answers

What is the characteristic of an Omphalocele?

  • A small defect in the abdominal wall
  • A defect that is not covered by a membrane or sac
  • A central abdominal wall defect larger than 4 cm at the umbilicus (correct)
  • A defect that only contains the midgut

What is the difference between Omphalocele and Gastroschisis?

  • The presence of a membrane or sac, with Omphalocele having none
  • The size of the defect, with Omphalocele being larger than 4 cm (correct)
  • The size of the defect, with Omphalocele being smaller
  • The location of the defect, with Omphalocele being on the side

What organs are usually found in an Omphalocele?

  • The liver and midgut
  • The liver, midgut, spleen, and one or both gonads (correct)
  • The liver, midgut, and spleen
  • Only the midgut

What is the location of an Omphalocele?

<p>At the umbilicus (B)</p> Signup and view all the answers

What is the characteristic of the membrane or sac in an Omphalocele?

<p>It is thin and transparent (D)</p> Signup and view all the answers

What is the location of the proximal end of the esophagus in relation to the azygos vein?

<p>At the level of the azygos vein (C)</p> Signup and view all the answers

What is the typical length of the distal esophagus?

<p>Short and often suspended by a fibrotic band (C)</p> Signup and view all the answers

What is the typical location of an H-type fistula?

<p>Thoracic aperture or higher in the neck (B)</p> Signup and view all the answers

What is the incidence of esophageal atresia with proximal fistula?

<p>2% (B)</p> Signup and view all the answers

What is the characteristic of the diameter of the fistula in esophageal atresia with proximal fistula?

<p>Variable, from tiny to large (C)</p> Signup and view all the answers

How many proximal fistulas are usually found in esophageal atresia with proximal fistula?

<p>Two or three (C)</p> Signup and view all the answers

What is the approximate incidence of esophageal atresia (EA) with tracheoesophageal fistula (TEF)?

<p>1 in 2500–3000 live births (B)</p> Signup and view all the answers

What is the main reason for the abnormal connection between the trachea and esophagus?

<p>Incomplete fusion of the lateral tracheoesophageal folds (C)</p> Signup and view all the answers

What is the name of the association that includes vertebral, anorectal, cardiac, tracheo-esophageal, renal, and limb abnormalities?

<p>VACTERL association (B)</p> Signup and view all the answers

What percentage of patients with EA/TEF have chromosomal anomalies?

<p>6-10% (B)</p> Signup and view all the answers

What is the name of the classification of EA where the proximal and distal esophagus end blindly?

<p>Pure esophageal atresia without TEF (B)</p> Signup and view all the answers

What is the approximate incidence of pure esophageal atresia without TEF?

<p>7% (B)</p> Signup and view all the answers

What is the risk of a second child with EA/TEF among parents of one affected child?

<p>0.5-2% (D)</p> Signup and view all the answers

At what level does the distal esophagus enter the trachea in esophageal atresia with distal fistula?

<p>At the level of the carina or 1-2 cm higher (B)</p> Signup and view all the answers

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Study Notes

Congenital Abdominal Wall Defects

  • Omphalocele is a central abdominal wall defect, larger than 4 cm, located at the umbilicus.
  • Omphalocele is always covered by a thin membrane or sac.
  • Omphalocele usually contains the liver, midgut, and may contain other organs such as the spleen, and one or both gonads.

Gastroschisis

  • Gastroschisis is a small defect.

Embryology of Esophageal Atresia (EA)

  • During the 4th week of gestation, the foregut differentiates into a ventral respiratory part and a dorsal esophageal part.
  • Incomplete fusion of lateral tracheoesophageal folds results in a defective tracheoesophageal septum and abnormal connection between the trachea and esophagus.

Incidence of EA and Tracheo-Esophageal Fistula (TEF)

  • Incidence of EA/TEF: 1 in 2500-3000 live births
  • Male preponderance: 1.26:1
  • No evidence linking EA/TEF to maternal age
  • Risk of a second child with EA/TEF: 0.5-2% (increases to 20% if more than one child is affected)
  • Chromosomal anomalies in 6-10% of patients (e.g., trisomy 18 and 21)

Associated Anomalies

  • EA can be divided into isolated EA and syndromic EA (roughly equal incidence)
  • Most frequent associated malformations in syndromic EA:
    • Vertebral anomalies (mainly thoracic region)
    • VACTERL association (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, and Limb abnormalities)
    • CHARGE association (Coloboma, Heart defects, Atresia of the choanae, developmental Retardation, Genital hypoplasia, and Ear deformities)

Classification of EA/TEF

  • Esophageal Atresia with Distal Fistula: 85%
    • Dilated proximal esophagus descends to 3rd or 4th thoracic vertebrae
    • Distal esophagus is slender and enters the trachea posteriorly
  • Pure Esophageal Atresia without TEF: 7%
    • Proximal and distal esophagus end blindly
    • Proximal esophagus is dilated and ends at the level of the azygos vein
  • H-Type Fistula without Esophageal Atresia: 4%
    • Fistula starts from the membranous trachea and enters the esophagus
    • Usually short, but diameter may vary
  • Esophageal Atresia with Proximal Fistula: 2%
    • Upper esophageal fistula is usually not found at the end of the pouch
    • Fistula starts proximally on the trachea and ends distally in the dilated proximal esophagus
  • Esophageal Atresia with Proximal and Distal Fistulas: incidence unknown

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