'Vater Syndrome' Is Often Misused
The word Vater is an acronym was created to refer to a group of congenital anomalies with that tend to occur nonrandomly at the same time. These letters of the acronym are:
V = vertebral
A = anorectal malformation
TE = tracheal-esophageal fistula
R = renal and or radial
We often receive letters from parents of children who suffer from anorectal malformations and are very concerned because the pediatrician, neonatologist, or pediatric surgeon told them that their child suffers from Vater's syndrome.
We personally believe that the term Vater's syndrome should not be used for several reasons:
- It is not a term that reflects the real prognosis of the patient
- It is useless from the therapeutic point of view
- It causes parents to become overly concerned and upset
- Most of the time, the term is misused
For instance, a patient may have a minimal vertebral anomaly that requires no treatment, a benign anal malformation such as perineal fistula (an abnormal anal opening, the simplest form of anorectal malformation), esophageal atresia (a birth defect in which the esophagus, the organ that connects the mouth to the stomach, lacks the opening to allow food to pass into the stomach), a gap between the upper and lower segments of the esophagus, and a minor kidney abnormality.
This would be an example of a patient that has an excellent prognosis, will enjoy 100% bowel control, and will have an excellent quality of life in spite of meeting the criteria for the term of Vater's syndrome. On the other hand, a patient may have no vertebral anomaly, no esophageal atresia, no kidney malformation, but an anorectal malformation consisting of a fistula or abnormal passage between the rectum and bladder. This would be an example of a patient that does not have Vater's syndrome but does have a very bad prognosis concerning bowel control.
In medicine and biology, things are never black and white. Some vertebral anomalies are really incapacitating, require several operations, and the patients will suffer from a significant number of consequences. Some anorectal malformations are very serious, provoke fecal incontinence, and will require multiple operations, while other anorectal malformations are minimal, and require no surgical treatment. Most of the time, the term Vater syndrome is misused to describe patients that don't fulfill the original meaning of the term or patients that have minimal anomalies of the vertebrae or kidneys.
For all of these reasons and considering the wide spectrum of anorectal malformations and other disorders, we recommend that medical professionals and others no longer use term Vater syndrome.
Contact the Colorectal Center
For more information about the Colorectal Center at Cincinnati Children's, please contact us.
