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Gastroschisis

Orpha number ORPHA2368
Synonym(s) Laparoschisis
Prevalence 1-5 / 10 000
Inheritance Not applicable
Age of onset Infancy
Neonatal
ICD-10
  • Q79.3
ICD-O -
OMIM
UMLS
  • C0265706
MeSH
  • D020139
MedDRA
  • 10018046
SNOMED CT
  • 72951007

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 more 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.

Expert reviewer(s)

  • Pr Frédéric BARGY

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