Omphalocele

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Omphalocele

ETP Gastrointestinal System - HV

ETP Gastrointestinal System - HV

Anatomy of the gastrointestinal organs of the pelvis and perineum
Anatomy of the oral cavity (dentistry)
Anatomy of the pharynx and esophagus
Anatomy of the anterolateral abdominal wall
Anatomy of the abdominal viscera: Blood supply of the foregut, midgut and hindgut
Anatomy of the abdominal viscera: Esophagus and stomach
Anatomy of the abdominal viscera: Small intestine
Anatomy of the abdominal viscera: Large intestine
Anatomy clinical correlates: Anterior and posterior abdominal wall
Abdominal quadrants, regions and planes
Development of the digestive system and body cavities
Development of the gastrointestinal system
Development of the teeth
Development of the tongue
Gallbladder histology
Esophagus histology
Stomach histology
Small intestine histology
Colon histology
Liver histology
Pancreas histology
Gastrointestinal system anatomy and physiology
Anatomy and physiology of the teeth
Liver anatomy and physiology
Escherichia coli
Salmonella (non-typhoidal)
Yersinia enterocolitica
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Enterobacter
Salmonella typhi (typhoid fever)
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Vibrio cholerae (Cholera)
Shigella
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Bacillus cereus (Food poisoning)
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Enteric nervous system
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Gastric motility
Gastrointestinal hormones
Chewing and swallowing
Carbohydrates and sugars
Fats and lipids
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Intestinal fluid balance
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Bile secretion and enterohepatic circulation
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Cleft lip and palate
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Warthin tumor
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Dental caries disease
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Gingivitis and periodontitis
Temporomandibular joint dysfunction
Nasal, oral and pharyngeal diseases: Pathology review
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Esophageal web
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Barrett esophagus
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Esophageal cancer
Esophageal disorders: Clinical
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GERD, peptic ulcers, gastritis, and stomach cancer: Pathology review
Gastroesophageal reflux disease (GERD)
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Helicobacter pylori
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Pyloric stenosis
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Zinc deficiency and protein-energy malnutrition: Pathology review
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Congenital gastrointestinal disorders: Pathology review
Congenital diaphragmatic hernia
Imperforate anus
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Omphalocele
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Disorders of carbohydrate metabolism: Pathology review
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Flashcards

Omphalocele

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Questions

USMLE® Step 1 style questions USMLE

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A 26-year-old woman, G2P1, comes to the emergency department in active labor. She has not received prenatal care during this pregnancy. A 3500g boy is delivered by an uncomplicated vaginal delivery. His temperature is 36.7°C (98°F), pulse is 120/min, respirations are 40/min, and blood pressure is 85/60 mmHg. Physical examination shows low-set ears, micrognathia and clenched fingers. The umbilical cord inserts at the edge of a midline abdominal wall defect through which bowel loops herniate, covered by a thin, translucent membrane. Which of the following is the most likely cause of this patient's abdominal findings?  

Transcript

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Content Reviewers

With an omphalocele, omphalo- refers to the naval, also known as the bellybutton—or more formally the umbilicus—which is the attachment site of the umbilical cord, and -cele relates to hernia or swelling.

Omphalocele, therefore, is when some of the bowels herniate out into the umbilical cord.

During the fourth week of fetal development, the embryo starts to change shape from a flat, three-layer disc to something more shaped like a cylinder, a process called embryonic folding.

In the horizontal plane, the two lateral folds eventually come together and close off at the midline, except for at the umbilicus, where the umbilical cord connects the fetus to the placenta.

This folding allows for the formation of the gut within the abdominal cavity.

During around the sixth week of development, the liver and intestines grow really quickly, and because the abdominal cavity’s still pretty small, there’s limited space, which causes the midgut to herniate through the umbilical ring into the umbilical cord, and this happens normally.

At about week 10, though, the abdominal cavity typically has grown enough to allow the midgut to come back from the umbilical cord.

With omphalocele, the midgut—along with potentially other organs from the abdominal cavity—fail to return back to the abdominal cavity, and therefore stay in the umbilical cord all the way through fetal development and even after birth.

Now, since the intestines and potentially other organs aren’t meant to be in the umbilical cord, there can be complications like the abdominal cavity not growing to its normal size, as well as pinched blood vessels and loss of blood flow to an organ.

So with an omphalocele, after birth the abdominal organs protrude out of the body, but are contained within the umbilical cord, meaning the organs are sealed by a peritoneal layer.

Key Takeaways

An omphalocele is an abdominal wall birth defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen in a sac, as a result of a defect in the development of the muscles of the abdominal wall. The sac, which is formed from an outpouching of the peritoneum, protrudes in the midline through the umbilicus.

Omphalocele shouldn't be confused with another condition called gastroschisis, which also involves the herniation of abdominal contents out of the abdominal cavity. Unlike in omphalocele, herniated bowels in gastroschisis lack a peritoneal layer covering.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Prenatal and postnatal management of omphalocele" Prenatal Diagnosis (2008)
  6. "Early development of the abdominal wall, stomach and heart from 7 to 12 weeks of gestation: a longitudinal ultrasound study" Ultrasound in Obstetrics and Gynecology (1995)