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Gastroschisis

Orpha number ORPHA2368
Prevalence of rare diseases 1-5 / 10 000
Inheritance
  • Sporadic
Age of onset Neonatal/infancy
ICD 10 code
  • Q79.3
MIM number
Synonym(s) Laparoschisis

Summary

Gastroschisis is marked by viscera protruding, without a covering sac, from the fetal abdomen on the right lateral base of the umbilicus. It is due to defective embryo growth and other malformations are only exceptionally associated. In Europe, the average recorded prevalence of gastroschisis is 1/5,000 births. Associated intestinal lesions are linked to the quality of the mesenteric vascularization and to the contact between intestinal loops and amniotic fluid. When the hernia ring is tight or decreasing in size, necrosis or progressive ischemia of the protruding intestines may be observed, resulting in some cases with bad prognosis. The etiology remains unknown. Diagnosis is usually made before birth during prenatal ultrasound, allowing the birth to be arranged in a specialized unit where surgery can be performed immediately. Differential diagnoses include omphalocele (see this term), which is most frequent and associated with other malformations. Treatment is surgical and consists of closure of the abdominal wall. This is not always possible immediately and different techniques of delayed closure may be resorted to. Recently, in cases with a deficit of amniotic fluid, the possibility of performing amnio-infusions in the prenatal period has limited the number of intestinal lesions. Prognosis is linked to the functional quality of the intestinal loops, but is excellent in more than 90% of cases. *Author: Prof. F. Bargy (January 2009)*.

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
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