Anus Closure or Congenital AnoRectal Malformation (CARM)
Anus atresia or congenital anorectal malformation (CARM) is a congenital condition in which the anus is missing or not properly constructed. Every year in the Netherlands about forty to fifty babies are born with anal atresia. What is it and what treatments are possible?
Children with an anus atresia are born without an anus or with one that is very abnormally shaped. This prevents them from defecating properly and causes serious blockages in the intestine. In some cases, stool comes out through the urethra or vagina.
Cause
Until now, little is known about the cause of anal atresia. No association was found with drug use, smoking or illness during the pregnancy. Children with anal atresia often also have other birth defects. Such as, for example, diseases of the esophagus, heart, bones, kidneys and urinary tract. Children with anal atresia are therefore examined shortly after birth for further abnormalities.
Anus atresia can come in different forms and in varying degrees of severity. Sometimes the anus is completely missing, sometimes it is constructed, but very narrow. The anus may also be in a different location.
The diagnosis is usually made soon after birth, because no or no complete anus is visible. Sometimes it is not noticed until later when the midwife or maternity nurse wants to temperature the baby. Immediate intervention is necessary because the baby has to get rid of his stools.
A baby who is born with anus atresia should be operated on as soon as possible, but first we look at which treatment is best for the child.
Different treatments
If the anus is formed, but very narrow, only stretching may be sufficient. There are also children who do have an anus, but where it is covered with a kind of skin flap, so that stools cannot get out properly. A minor operation is often sufficient.
If there is hardly any anus, the surgeon will make a new anus during an operation, to which the rectum is attached. This often requires multiple operations. When the new anus is formed, the child may still have difficulty passing stools. This is because the sphincter is usually not properly constructed. The surgeon can make a new exit, but a sphincter cannot be made or repaired. In such a case, children often depend on colonic irrigation to get rid of their stools.
stoma
Sometimes it is not possible for a surgeon to make an anus at the buttock seam. For example, when there is no connection at all between the intestine and the perineum. The surgeon will then usually decide to create a stoma, an artificial exit through the abdominal wall. The rectum is attached to this. The faeces can then come out through the stoma.
In some cases, a stoma is temporary, for example if several operations are necessary to create a new anus. At least the faeces can then leave the body.
Potty trained
If the operations are successful, there is no guarantee that the function of the rectum and anus will return to full normal and a child will be able to become toilet trained. This is very difficult because the rectum, anus and pelvic floor muscles are not attuned to each other in anus atresia. It will probably never go as automatically and well as with a normally constructed anus.
Various problems can manifest themselves sooner or later. Each case in itself requires its own treatment and approach. One child will laxatives have to use, the other child will need colonics. Special workouts can help children gain control over their stools. Often it is not possible to assess the final state until puberty.
Which hospital?
In the Netherlands there are only six hospitals where they are allowed to treat a child with anal atresia. These are the academic hospitals of Groningen, Nijmegen, Amsterdam, Utrecht, Rotterdam and Maastricht. Until they reach adulthood, children with anus atresia should regularly return to the pediatric surgeon for a check-up.
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