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Síndrome de bridas amnióticas, a propósito de 3 casos clínicos
| Content Provider | Semantic Scholar |
|---|---|
| Author | Silva, G. Beatriz Da Cammarata-Scalisi, Francisco González-Coira, Mercedes Cruz, Maria Angelina La Rendon, Belitza |
| Copyright Year | 2008 |
| Abstract | 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. El sindrome de bridas amnioticas es una condicion esporadica variable que presenta un espectro que incluye anillos de constriccion, pseudosindactilia, amputaciones, multiples alteraciones craneofaciales, viscerales, de la pared corporal y abortos espontaneos. El rango de incidencia es de 1 en 1 200 a 1 en 15 000 recien nacidos vivos. La patogenia del sindrome de bridas amnioticas es controversial. En el siguiente informe se reportan tres casos donde se describen las diferentes manifestaciones clinicas de esta entidad, ademas se revisaron las diferentes hipotesis etiologicas. Entre los dos mecanismos propuestos destacan la ruptura prematura del amnios (teoria exogena), que conlleva a la formacion de bandas fibrosas que afectan el cuerpo fetal y la teoria endogena como defecto germinal, disrupcion vascular y alteraciones en la morfogenesis durante la gastrulacion temprana. Sin embargo, la etiologia exacta del sindrome de bridas amnioticas es incierta y su curso natural es impredecible. Las diferencias geograficas observadas en las prevalencias de nacimiento puede ser una indicacion util para estudios de factores geneticos y ambientales candidatos. El manejo debe ser multidisciplinario y el pronostico depende de la gravedad de las malformaciones. |
| Starting Page | 172 |
| Ending Page | 180 |
| Page Count | 9 |
| File Format | PDF HTM / HTML |
| DOI | 10.4067/S0370-41062008000200007 |
| Volume Number | 79 |
| Alternate Webpage(s) | https://scielo.conicyt.cl/pdf/rcp/v79n2/art07.pdf |
| Alternate Webpage(s) | https://doi.org/10.4067/S0370-41062008000200007 |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
