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</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</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</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</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</p> Signup and view all the answers

    What is the incidence of esophageal atresia with proximal fistula?

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

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

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

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

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

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

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

    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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    Description

    This quiz covers congenital abnormalities including Omphalocele, Gastroschisis, and Umbilical cord hernia, which are defects in the abdominal wall. Learn about the characteristics and differences between these conditions.

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