Marc A. AIMS To review the history of surgery for anorectal malformation and current concepts in its management To describe the spectrum of disease and associated defects To describe neonatal management To discuss definitive management in all groups To discuss complications and outcome. Significant advances have been made in the management of this complex condition in recent years. The background to these advances and the current management of the condition are considered here.
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An anorectal malformation ARM is a congenital it is present at birth disorder in which the bottom does not develop normally. In ARMs the anus bottom hole does not open in the normal position. In addition the urinary tract urethra wee tube , bladder and kidneys is affected most of the time. There are different types of ARMs, which are of different severity and might require different treatment.
They are classied according to the place where the bowel ends and whether there is an abnormal connection between the bowel and the urinary tract. This is the case in most boys. The abnormal connection is called a stula. Normal development Normally, two separate tubes develop when a baby boy is growing in the womb. Both tubes should have an opening to the outside world.
The tubes, openings and functions are as follows: rectum lower bowel and anus, which allow the passage of stool poo out of the body urethra, which allows the passage of urine wee out of the body The region where these openings are placed is called the perineum. It extends from bone at the front to bone at the back. How is an Anorectal Malformation detected? It is not usually possible to detect ARMs on antenatal scans so you may rst be told that something is wrong at the postnatal check.
Most ARMs in boys are obvious: when you look at the babys bottom there is no anus or your baby passes meconium black poo from his urethra. However, if the anus is present, but slightly further forward than usual this subtle abnormality may be missed until a child has problems with constipation.
The point where the bowel joins the urinary tract stula is usually not obvious by looking at your baby and can only be determined with a special X-ray. This X-ray micturating cystourethrogram MCUG and loopogram is usually done when your baby is six weeks to three months old. The X-ray involves passing X-ray dye contrast into the bowel and urinary tract through catheters and this allows us to work out where the two meet.
Rectoperineal stula In this type of ARM the anus is slightly closer to the scrotum further forwards than it should be sometimes called an anterior anus or anterior ectopic anus. This may be obvious at birth or it may only be diagnosed when the baby is older and develops constipation difculty passing stool.
This is a mild type of ARM. Sometimes the anus is covered with a thin layer of skin. This diagram shows a normal urethra. The anus is closer to the scrotum and urethra than it should be. The opening is in front of the muscles that control continence.
Rectourethral stula This is the most common ARM in boys. The rectum ends in a narrow tube stula that joins the urethra, rather than ending at his anus on his bottom.
It is usually obvious that the anus is not present often called an imperforate anus. This diagram shows the rectum joining the urethra.
This join can be at different levels. The anus is not present. Rectovesical stula This is a more severe form of ARM. The rectum ends in a narrow tube stula that joins the bladder organ were urine is stored rather than ending at his anus on his bottom. Rare ARM in boys If the anus or rectum does not form normally, there is usually an abnormal connection stula between the bowel and urinary tract.
Rarely there is no such stula. This can only be determined by MCUG. This type of ARM is sometimes called anal agenesis or rectal atresia.
What causes Anorectal Malformations and how common are they? The cause of ARMs is not known. As far as we know, there is nothing that you could have done to cause or prevent your baby from having an ARM. About one in 4, - 5, babies has an anorectal malformation. This diagram shows the rectum joining the bladder. ARMs can be associated with other anomalies.
The most common are anomalies of the urinary tract, genital tract and heart. V vertebra spine A anus C cardiac heart T trachea windpipe E esophagus food pipe R renal kidneys L limb arm or leg Will any investigations be necessary? As ARMs can be associated with other anomalies your baby will need investigations to nd out if this is the case.
The most common investigations are an ultrasound scan of the kidneys, spine and heart called an ECHO echocardiogram. Later, a contrast study of the urinary tract MCUG and lower bowel loopogram will be performed. Other investigations may be required and your doctor will explain these to you. How are ARMs treated?
Because of the abnormal connection between the bowel and the urethra or bladder there is a risk of urinary tract infection UTI. To minimise the risk of urinary tract infection your baby will be started on prophylactic antibiotics. Until the contrast study is done, it is not possible to tell the difference between a rectourethral and a rectovesical stula type of ARM.
Most babies with an ARM need three operations. The rst operation happens in the rst days of life and allows the baby to page 7 pass stool. This is a life saving operation. This operation brings the bowel to the surface of your babys tummy. The articial opening is called a stoma. Stool passes directly from the bowel onto the skin of the lower tummy. A bag is stuck to the skin to protect the skin and catch stool; this is called a stoma bag.
Most parents quickly learn how to look after their babys stoma. The second operation reconstructs the tubes in the correct positions. This operation is called an anorectoplasty, and usually happens at about three months of age. The anorectoplasty can be a long delicate operation as it involves placing the rectum lower bowel that stores stool within the muscles sphincters that will help to keep your child continent and create an anus in the normal position.
After the second operation it is important to make sure that the new anus does not narrow while it is healing. It will therefore need dilatations stretching. This starts seven - ten days after the operation and needs to be done every day. A metal rod is inserted into the anus for about 30 seconds.
Your doctor will determine which size dilator is right for your baby and show you how to do the dilatations yourself at home. Increasing sizes of dilators are used until your babys anus is a normal size. The third operation happens when the reconstruction has healed and the anus is a normal size. This is usually about three months after the anorectoplasty. The operation involves assessing the reconstructed anus.
If the new anus is big enough to allow stool to pass, the stoma will be closed so that the baby will be able to pass stool out of his bottom and not into a bag. Babies with a rectoperineal stula may only require one operation. The surgeon treating your baby will explain if this is the case. All operations have a small risk of bleeding and infection. The operation to form a stoma carries the risk that the stoma may become too tight stenotic to allow stool to pass. The stoma may also prolapse fall out of place so that a length of bowel that should be on the inside slides outside.
These problems are rarely severe enough to require another operation to treat them. The main risk of the anorectoplasty is narrowing of the new anus this problem is called anal stenosis.
Unusual, but signicant risks of anorectoplasty are that the original opening re-opens this is called re-stulation , so that there are two openings. Rarely the reconstruction can break down this is called a dehiscence. Re-stulation and dehiscence are generally treated with a second anorectoplasty. After the third operation, closing the stoma, there is a very small chance that the join may leak.
Leaks happen in the rst few days after the operation and will require another operation to treat them. The join can become too narrow stenotic and this may require another operation.
Both these complications are rare. What happens before the operation? Whichever operation is planned, you will receive information on how to prepare your child for the operation in your admission letter and our welcome booklet. Your childs surgeon will explain the operation in more detail and discuss with you any concerns you may have. The surgeon will ask you to sign the consent form giving your permission for the operation to go ahead.
An anaesthetist will also visit you to explain about your childs anaesthetic in more detail and discuss options for pain relief afterwards. If your child has any medical problems, like allergies, page 9 please tell the doctors. Please also bring in any medicines your child is currently taking.
If you are at home before the operation you and your child might need to come into hospital one day before the operation. This is so your childs bowel can be empty of stool ready for the operation. Your child may have laxatives or a bowel washout. In the anaesthetic room A nurse and parent can accompany your child to the anaesthetic room.
Pena- Management Malformasi Anorektal
An anorectal malformation ARM is a congenital it is present at birth disorder in which the bottom does not develop normally. In ARMs the anus bottom hole does not open in the normal position. In addition the urinary tract urethra wee tube , bladder and kidneys is affected most of the time. There are different types of ARMs, which are of different severity and might require different treatment. They are classied according to the place where the bowel ends and whether there is an abnormal connection between the bowel and the urinary tract. This is the case in most boys.
MALFORMASI ANOREKTAL (ANOREKTAL MALFORMATION)