VACTERL

The following report from the MHC-database is based on the latest version of the Mun-H-Center Observation Chart that has been in use since 2008. Please note, that this report exclude children younger than 3 years.

Reports based on the original forms (1996-2008) are presented at the bottom of this page - below the description of the diagnosis


Synonyms
VATER

ICD-10
Q87.2

Estimated occurrence
1-2: 1 000 live births.

Etiology
Unknown. The malformations are thought to be caused by an interruption of the cell formation during early foetal life.

General symptoms
Low birth weight and premature birth are common. The term VACTERL is an acronym with each letter representing the first letter of the organ in which the anomaly may occur.

V Vertebral (vertebrae, bones of the spinal column)
A Anorectal (anus /rectum)
C Cardiovascular (heart)
T Tracheal (windpipe)
E Esophageal (tube connecting the throat to the stomach))
R Renal (kidney)
L Limb (arms/ legs)

The diagnosis is made if anomalies occur in at least three of the above groups. Other anomalies may also occur, for example hydrocephalus and choanal atresia

Orofacial/odontological symptoms
Feeding difficulties are common in children with breathing difficulties, heart defects and esophageal atresia. Some children therefore need to be tube fed during infancy or later. Gastroesophageal reflux (leakage of stomach fluids into the esophagus) and vomiting are relatively common and contribute to eating difficulties and increase the risk of caries.

Orofacial/odontological treatment
  • Children with eating difficulties are often in need of supplementary dental care e.g. help with oral hygiene and fluoride treatments.
  • Feeding and swallowing difficulties are investigated and treated by a specialist team at the hospital or multidisciplinary treatment centre.
  • When treating medically compromised patients always contact their doctors for medical advice.

Sources
The rare disease database of the Swedish National Board of Health and Welfare.
The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
The Newsletter of the Ågrenska Center.

Swedish association
Svenska Vacterlföreningen


Reports based on the original Observation Chart and Questionnaire (1996-2008):

Report from Questionnaires
Report from Observation Chart

Print version Print version

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