Externally recognizable Abdominal Malformations Gastroschisis and Omphalocele |
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click the images for more details |
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Incidence | |
Relatively frequent malformation of the abdominal wall and content: one gastroschisis in 15,000 (increasing); one omphalocele in 5,000 newborns. | |
Clinical significance | |
Due
to an abdominal wall defect in the region of the umbilicus, there is an
eventration of abdominal content such as intestine, liver and other organs. | |
Etiology | |
Unknown. | |
Pathology, anatomical types | Illustrations |
Gastroschisis:
During pregnancy the non-protected intestine remains outside the abdominal
cavity and undergoes severe reactive changes due to the amniotic fluid;
the malformation is combined with a narrow midline cleft of different
length. | show details |
Pathophysiology | |
The pathophysiological events result from the reactive changes of the intestinal wall in gastroschisis, and in both malformations from the operative reduction of the abdominal content in its cavity. | |
Clinical presentation, Gastroschisis | Illustrations |
There is a midline cleft of the abdominal wall located to the right of the umbilicus. The walls and the mesentery of the intestine are swollen due to an edema, covered by adherent coats, and stuck firmly together. | show details |
Clinical presentation, Omphalocele | Illustrations |
The anomaly consists of the sac of varying size from small to giant, initially being mostly transparent; therefore, the organs of the abdominal content are initially recognizable. In contrast to the gastroschisis not only the intestine, but the liver and other organs, too may be eventrated. The insertion of the umbilical cord is eccentricly located on the omphalocele sac, and the connection between the sac and the abdominal may be either narrow or large. | show details |
Natural history | Illustration |
Already during
pregnancy, either a volvulus or a strangulation of the eventrated intestine
may occur in gastroschisis with abortion, or a short bowel syndrome due
to an ischemic damage to the intestine. | show details |
Differential diagnosis | Illustrations |
If the clinical
picture of both malformations is well-known, and a precise and careful
clinical evaluation is perfomed in the individual case, there is no differential
diagnosis. | show details |
Work-up examinations | Illustration |
Prenatal
ultrasound for diagnosis of both malformations and as a follow-up to recognize
an imminent complication (see natural history). | show details |
Therapy | Illustrations |
Primary
closure, which is possible in more than 75 % in gastroschisis, less in
omphalocele. | show details |
Prognosis | |
In gastroschisis, the mortality is less than 10 %; in omphalocele, 30-40 %. The mortality is due to the side-effect of parenteral nutrition in the former, and severe associated anomalies in the latter. The longterm prognosis for the gastrointestinal tract is good except for frequent occurrence of gastroesophageal reflux.
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