VACTERL-Assoziation
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VACTERL
association • VACTERL - Ganzer Fall bei Radiopaedia
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VACTERL
association • VACTERL - Ganzer Fall bei Radiopaedia
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VACTERL
association • VACTERL association - Ganzer Fall bei Radiopaedia
VACTERL is an acronym that describes a non-random constellation of congenital anomalies. It is not a true syndrome as such and is equivalent to the VATER anomaly.
Epidemiology
The estimated incidence is 1 in 10,000-40,000 births .
Pathology
The acronym VACTERL derives from:
- V: vertebral anomalies
- hemivertebrae
- congenital scoliosis
- caudal regression
- spina bifida
- A: anorectal anomalies
- C: cardiac anomalies; cleft lip
- TE: tracheo-esophageal fistula +/- esophageal atresia
- R: renal anomalies; radial ray anomalies
- L: limb anomalies
At least three of the above features (in each category) is considered necessary for the diagnosis of this condition .
The association does not include cranial / CNS anomalies. If there is accompanying hydrocephalus, the term VACTERL-H is used by some authors
Genetics
The vast majority of VACTERL associations are thought to have a sporadic occurrence with occasional autosomal recessive forms. There is no known underlying genetic defect .
Siehe auch:
- Spina bifida
- Hydrocephalus
- angeborene Wirbelanomalien
- Polydaktylie
- Ösophagusatresie
- Analatresie
- caudal regression syndrome
- Lippen-Kiefer-Gaumen-Spalte
- Halbwirbel
- VATER association
- cardiac anomalies
- renal anomalies
- Hunter-MacMurray-Syndrom
- David-O'Callaghan-Syndrom
- VACTERL-H
- Radialstrahldefekte
und weiter:
- Blockwirbel
- congenital tracheo-oesophageal fistula
- angeborene renale Anomalien
- Tracheomalazie
- Syndaktylie
- nuchal translucency
- einseitige Nierenagenesie
- VATER
- Radiusaplasie
- caudal dysplasia sequence
- VATER anomaly
- esophageal atresia with H-type tracheo-esophageal fistula
- VACTERL-H association
- H-type tracheo-oesophageal fistula with no atresia
- Holt-Oram-Syndrom
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