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Revista chilena de pediatría

versión impresa ISSN 0370-4106


DA SILVA, GLORIA et al. Amniotic band syndrome: 3 case-reports. Rev. chil. pediatr. [online]. 2008, vol.79, n.2, pp.172-180. ISSN 0370-4106.

Amniotic Band Syndrome is a sporadic condition with a spectrum of clinical presentations that include constriction rings, pseudosyndactily, amputations, multiple craniofacial - visceral - body wall defects and spontaneous abortion. The incidence ranges from 1: 1 200 to 1: 15 000 newborns, creating controversy regarding its pathogenesis. We report 3 cases with different clinical manifestations of this entity and review the different etiological hypotheses for this syndrome. Two main pathogenic mechanisms are proposed: the exogenous theory with early amnion rupture leading to fibrous bands that entrap the fetal body and the endogenous theory that establishes a germ plasm defect with vascular disruption and disturbance of morphogenesis during early gastrulation. However, the exact etiology of Amniotic Band Syndrome remains unknown and its natural evolution is unpredictable. The observed geographic difference in birth prevalence is useful in studying specific genetic and environmental factors involved. The management of this disease must be multidisciplinary and the outcome depends on malformations severity

Palabras clave : Amniotic Band Syndrome; constriction rings; amputations; etiopathological; exogenous theory; endogenous theory.

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