Familial occurrence of gastroschisis: a population-based overview on recurrence risk, sex-dependent influence, and geographical distribution

Pediatr Surg Int. 2018 Mar;34(3):277-282. doi: 10.1007/s00383-018-4224-6. Epub 2018 Jan 15.

Abstract

Purpose: There is uncertainty over whether familial recurrences in gastroschisis might be higher. Moreover, scant information is available regarding its sociodemographic features. We aim to explore the recurrence risk, sex-dependent influence, and geographical distribution of familial gastroschisis.

Methods: A systematic review of the literature and data extraction from population-based studies published 1970-2017 (PubMed/MEDLINE) was independently performed by two reviewers. Familial ocurrence of gastroschisis, whereas sociodemographic features from 11 studies were pooled including 862 probands as a base. A descriptive analysis and Chi-square test were performed.

Results: Twenty-four probands had a positive family history of gastroschisis including 49 affected family members, for a recurrence risk of 5.7 and 3% adjusted for proband. Siblings' recurrence was 4.3%. Sex-dependent influence analysis (n = 879, from three studies) evidenced an increased susceptibility to gastroschisis in males (2.5%) compared to females (1.3%) adjusted for proband. Heterogeneity was identified by Fisher's exact test (P = 0.023).

Conclusion: Our findings support a greater liability attributable to familial factors on gastroschisis along with significant information for family and prenatal counseling. We suggest that future studies should include for a more accurate account for both familial and environmental confounding factors to uncover relatives and environmental exposures that more limited family histories may have missed.

Keywords: Familial; Gastroschisis; Genetics; Inheritance; Population based.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Gastroschisis / epidemiology
  • Gastroschisis / genetics*
  • Genetic Predisposition to Disease*
  • Humans
  • Recurrence
  • Risk
  • Sex Factors
  • Siblings