Omphalocele is a congenital Congenital Chorioretinitis anterior abdominal wall Anterior abdominal wall The anterior abdominal wall is anatomically delineated as a hexagonal area defined superiorly by the xiphoid process, laterally by the midaxillary lines, and inferiorly by the pubic symphysis. Anterior Abdominal Wall: Anatomy defect in which the intestines are covered by peritoneum Peritoneum The peritoneum is a serous membrane lining the abdominopelvic cavity. This lining is formed by connective tissue and originates from the mesoderm. The membrane lines both the abdominal walls (as parietal peritoneum) and all of the visceral organs (as visceral peritoneum). Peritoneum: Anatomy and amniotic membranes. The condition results from the failure of the midgut Midgut Development of the Abdominal Organs to return to the abdominal cavity by 10 weeks' gestation. Omphalocele is frequently associated with genetic syndromes and chromosomal abnormalities. Prenatal diagnosis can be made by prenatal ultrasound, maternal blood elevated ⍺-fetoprotein, and fetal chromosomal analysis. Treatment includes immediate hemodynamic stabilization followed by primary or staged surgical repair.
Last updated: 8 Jan, 2021
Large omphalocele containing omphalomesenteric duct Omphalomesenteric duct The narrow tube connecting the yolk sac with the midgut of the embryo; persistence of all or part of it in post-fetal life produces abnormalities, of which the commonest is meckel diverticulum. Meckel’s Diverticulum cyst
Image: “Large omphalocele containing OMD cyst” by Yousuf Aziz Khan, MBBS, FCPS (Paediatric Surgeon), Department of Paediatric Surgery, National Institute of Child Health, Rafiquee Shaheed Road, Karachi – 75510, Sind, Pakistan. License: CC BY 3.0Diagram showing the normal process of intestinal
rotation
Rotation
Motion of an object in which either one or more points on a line are fixed. It is also the motion of a particle about a fixed point.
X-rays and herniation during embryologic development
A: The
midgut
Midgut
Development of the Abdominal Organs (multi-colored loop) before herniation
B1–B3: As it grows rapidly, the
midgut
Midgut
Development of the Abdominal Organs herniates through the umbilical ring and begins
rotation
Rotation
Motion of an object in which either one or more points on a line are fixed. It is also the motion of a particle about a fixed point.
X-rays.
C: The
midgut
Midgut
Development of the Abdominal Organs returns to the abdominal cavity.
Pathophysiology is not definitively understood; 2 main theories:
Most cases of omphalocele (90%) are diagnosed prenatally:
Obstetric ultrasound at 37 weeks showing an omphalocele containing intestines and part of the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy
Image: “Obstetric ultrasound at 37 weeks containing intestines and part of the liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy” by Gynaecology and Obstetrics Department I, University Hospital Hassan II, Fez, Morocco. License: CC BY 2.0A large omphalocele
Image: “Aspect of the omphalocele at birth” by Gynaecology and Obstetrics Department I, University Hospital Hassan II, Fez, Morocco. License: CC BY 2.0Staged closure of a giant omphalocele using synthetic mesh: Daily sequential Sequential Computed Tomography (CT) reduction of synthetic mesh silo content
Image: “Daily sequential Sequential Computed Tomography (CT) reduction of synthetic mesh silo content” by Department of Pediatric Surgery, GMC Hospital, Ajman, United Arab Emirates. License: CC BY 3.0Staged closure of a giant omphalocele using synthetic mesh: complete closure of a giant omphalocele
Image: “Complete closure of giant omphalocele” by Department of Pediatric Surgery, GMC Hospital, Ajman, United Arab Emirates. License: CC BY 3.0