VACTERL

Reports from the MHC data base

Report from the Observation Chart (PDF)

Synonym

VATER

Codes

ICD-10: Q87.2W
ORPHA: 887

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 Documentation from the Ågrenska national competence centre for rare diseases. 
Updated: 2017-09-13 08:38