Conditions We Treat
Anorectal malformations: Any abnormality of the perineum or bottom of a baby boy or girl is called an anorectal malformation or ARM. Conditions range from an absent anus or anal atresia, complex connections of the rectum to the bladder, and exposure of the organs on the lower half of the abdomen, known as exstrophy. We have teams of providers who work together no matter what the baby needs.
Imperforate anus: Babies can be born without an anus and many times this condition is not picked up on ultrasound before the baby is born. The first step for some of these babies is to create an ostomy on the abdomen so they can eat and grow. There are multiple procedures to create an anus and at Phoenix Children’s Hospital, our physicians have expertise in all types of operations that a baby might need.
Cloaca: One complex form of anorectal malformation is known as a cloaca. In these babies, the intestines, bladder and reproductive organs are all connected. Many times there is only one opening on the bottom and newborns will need an ostomy created on the abdominal wall to let stool out so they can be fed. After that, it is time to sort out the connections and start to reconstruct the openings on the bottom. At Phoenix Children’s we use a minimally invasive approach whenever possible and we are national leaders with this technique.
Cloaca Exstrophy: When babies have an opening in the lower half of their abdominal wall, the bladder and intestines are exposed. This is a rare problem and is usually seen on the prenatal ultrasound. Our team of physicians plan a closure of the open area and create an ostomy on the abdomen to allow stool to pass. At a later date, babies can have reconstructions to be able to pass stool and urine on the bottom but this kind of problem has many challenges as the child grows. Our team follows patients with exstrophy their whole lives.
Bladder Exstrophy: When the lower part of the abdomen is open and the bladder is exposed, the baby will need a closure and reconstruction of the urethra, the area where pee comes out. In some cases, children will need a channel created on the abdomen to drain the bladder and this is done in later years. We follow children with bladder exstrophy throughout their lives to make sure they stay healthy and so that we can optimize reproductive health later in life.
Hirschsprung Disease: When babies are born without the proper nerves in the lining of the rectum, they are not able to pass stool very well. If we are concerned, we perform a small biopsy of the lining looking for normal nerves, called ganglion cells. When no ganglion cells are present, we remove the portion of the rectum that is diseased and connect the intestines that have normal nerves to the bottom. At Phoenix Children’s Hospital, we use minimally invasive surgery whenever possible to allow the babies to heal quickly.
Ostomy: A baby without an anus that can pass stool will have a connection made from the intestines to the abdominal wall through a small operation in the first few days of life. In most cases, the colon is used and connected in two locations, one for stool to pass and one that goes downstream. The baby can be fed very soon after the operation and stool passes into a bag called an ostomy appliance. Our nurses help with training and follow up while the ostomy is needed.
Anoplasty: In some cases, an ostomy to relieve obstruction is not necessary because stool can come out of an anus that is too small and not in the proper position within the anal sphincter muscles. We evaluate each case individually and many times we recommend moving the anus to a spot within the muscles. This is done all on the bottom with no incisions on the abdomen. Babies can sometimes go home the same day or have a short hospital stay depending on their age.
Anorectal pull through procedure: The first step for a baby who is born without an anus is an operation to make an ostomy to allow stool to pass so the baby can be fed. After the baby gets bigger, a study called a colostogram is done so we can see if and how the intestines are connected to the bladder. Once we know that, we can plan to disconnect the rectum and make a new connection on the bottom. This is done with an open or laparoscopic procedure depending on the connections. If the ostomy does not have to be redone, the baby can usually be fed within a day or two of the surgery and go home when feeding well. Dilators will be needed to keep the new connection open as it heals.
Laparoscopic-assisted cloaca repair: We have found that using a camera and small instruments to take apart the abnormal connections and to help connect the rectum to the bottom can be a great technique for a baby with a cloaca. We help families think about their child in terms of different organs systems and we “fix” things as soon as the baby is ready. Dividing the goals of stooling and peeing into separate categories allows us do the reconstructions when most appropriate and avoid complications. If we can establish a way to pee on the bottom and open up the vaginal opening at the same time that we make an anus, we will. This would be a laparoscopic anorectoplasty with a urogenital sinus repair. Urology, surgery and gynecology all work together during these operations. Babies stay in the hospital for a few days.
Revision surgery: Older children and teenagers may develop a narrowing called a stricture at one of their new connections. Surgeries to open up those strictures can be simple or complex depending on how much needs to be done. We involve the patient, at any age, in the decision making and answer all their questions.
Ostomy takedown or reversal: When the new connections on the bottom are healed, we can reverse the ostomy that was created in the first few days of life. Now stool will be able to pass from the bottom for the first time. We pay special attention to the skin to help protect it from a rash. This operation requires a few days in the hospital to get back on food and to watch for any problems.
What to expect at your appointment
We already know you: Patients known to us before birth or patients we care for in the newborn period will automatically be scheduled for our colorectal clinic. You will have an appointment for your baby and a number to call in case something comes up that needs more immediate attention. You will have the contact information for our colorectal nursing team who know all about ostomies and other issues facing families with a baby with an anorectal malformation.
We are just meeting you: If you are calling our clinic for the first time we will ask you some basic questions and then one of our colorectal nursing staff will call to get some details and start the process of obtaining records and x-ray images from other facilities. We will arrange to see you soon and the provider can review your case ahead of time. You may get a call from a Phoenix Children’s patient representative to discuss any out of pocket costs you may have associated with your insurance provider.
What we do first: We will ask a lot of questions about the basics…Is your child eating ok? Gaining weight? How is the stool? If there is an ostomy, how is it working? If your child has had a anorectal surgery, are they constipated? Do they have any pain? Are they able to play and go to school? Sometimes we need to get new x-rays or schedule procedures and that will be discussed in your first visit.
Ongoing care: We will continue to care for your family as your child grows up and we have many tips and tricks for potty-training, entering school, participating in sports and dealing with the challenges that come with the teen years.