Summary
Omphalocele is due to the defective closure of the abdominal wall in the embryo before 9 weeks of gestation, i.e. 11 weeks of amenorrhea. Associated malformations are frequent (in particular craniofacial and cardiac malformations), affecting almost 1/2 of the patients. Omphalocele is involved in many polymalformative syndromes, in particular in Wiedemann and Beckwith's syndrome. It is a large hernia of the abdominal wall centered on the umbilical cord in which the protruding viscera are protected by a sac. It is classified in the group of abdominal coelosomies. The size of the abdominal opening ranges from a simple hernia of the cord containing a few intestinal loops to giant omphaloceles in which a large part of the liver protrudes. Therapeutic problems vary according to the form. Prevalence of omphalocele is estimated at 1/2,000 births. In Europe, the average recorded prevalence of Omphalocele is 2.71/10,000 births (including livebirths, stillbirths, and terminations of pregnancy following prenatal diagnosis). The prognosis of an isolated omphalocele - when it is not giant - is good if surgery can be early performed in good conditions. Diagnosis is usually made before birth at the time of ultrasonography. Associated malformations can then be sought for and management of the infant can be timely prepared in specialized units. * Author: Prof. F. Bargy (Apr. 2002) *.
