Anorectal Malformation

How is an Anorectal Malformation Diagnosed?

Anorectal malformations are usually diagnosed shortly after birth. One of the first signs is that your baby does not have a normal anal opening or is unable to pass stool in the first day or two of life.

Your care team will examine your baby gently and may order imaging tests to understand better how the rectum is formed and where it connects. These tests may include:

• X-rays to check how far the rectum reaches toward the skin
• Ultrasound to check nearby organs
• Contrast studies to trace how stool or gas moves through the intestines

These tests help doctors decide the safest next steps and plan the right treatment for your child's specific anatomy.

First Steps after an Anorectal Malformation Diagnosis

If your baby is born with an anorectal malformation, care begins right away. Your medical team will gently examine your baby to understand how the rectum has formed and whether there's an opening for stool to pass. If no opening is present or in the wrong place, a plan will be made quickly to keep your baby safe and healthy.

In most cases, your baby will need a temporary colostomy within the first few days of life. This surgery creates a small opening in the belly so stool can leave the body and into a bag. This gives the lower digestive tract time to rest and heal while doctors prepare for the next stage of care.

Imaging tests may be done during this time to learn more about your baby's anatomy. Once the care team has a full picture, they'll work with you to plan the reconstruction surgery, usually done a few months later when your baby is bigger and stronger.

What is the Long-term Outlook for Children with Anorectal Malformations?

Every child's health journey is unique. Long-term outcomes depend on the type of anorectal malformation, its complexity, and the success of the surgical repair.

With the right care, many children can develop good bowel control and lead happy, active lives. Some may need ongoing support, like bowel management support or follow-up procedures, especially as they grow. Early treatment, regular follow-ups, and a supportive care team all play a key role in helping your child thrive.

Here are some things your child may experience as they grow:

Bowel control: Many children with anorectal malformation, or ARMs, can achieve good bowel control, but some may require ongoing bowel management programs, including dietary modifications, laxatives, or enemas to maintain regular bowel movements.

Continence: Achieving continence, or the ability to control bowel movements, may vary. Children with simpler forms of ARM, such as those with perineal fistulas, often have better outcomes than those with more complex forms, who may need additional support or interventions.

Urinary function: If a fistula was present or the malformation affected the urinary tract, some children may experience urinary issues, requiring monitoring and possibly additional treatments.

Quality of life: With proper management, most children can lead healthy, active lives. However, ongoing follow-up with a multidisciplinary team, including surgeons, gastroenterologists, and urologists, is often necessary.

Psychological and social support: Some children may require psychological support to help them cope with their condition, especially as they grow older and become more aware of their body image and social interactions.

Long-term follow-up is crucial to address any functional issues and to provide support as your child grows and develops.

Treatment for Anorectal Malformation

The primary surgery to correct an anorectal malformation is called a Posterior Sagittal Anorectoplasty, or PSARP, also known as the Peña Procedure. The goal of the surgery is to create a normal anal opening and properly position the rectum.

Steps of Posterior Sagittal Anorectoplasty:

• Pre-surgical preparation: In many cases, a colostomy (diversion of stool through an opening in the abdomen) may be created shortly after birth to allow the baby to pass stool while awaiting the definitive surgery.
• PSARP surgery: During the PSARP, the surgeon makes an incision in the midline between the buttocks to expose the pelvic muscles. The rectum is then carefully positioned within the muscle complex, and a new anal opening is created in the correct location.
• Reconstruction: The rectum is brought down and sutured to the skin at the newly created anal opening. If a colostomy is placed in the abdomen, it may remain temporarily until the new opening heals. Once healed, a second surgery would be performed to close the colostomy.

What is the Aftercare Following a PSARP?

After the PSARP surgery, careful post-operative care is crucial to ensure proper healing and function:

Hospital stay: The child will stay in the hospital for several days to a week, where they will be closely monitored for pain, infection, and any complications.

Pain management: Pain control is managed with medications. Parents will receive instructions on how to administer pain relief at home.

Wound care: It's important to keep the surgical site clean and dry is essential to prevent infection. Parents will be guided on how to perform daily wound care.

Dilatations: After surgery, some children may need regular anal dilatations to help keep the new opening from becoming too narrow. This involves gently inserting a small dilator into the anus to maintain its size. Parents are taught how to do this at home with guidance from the care team. For older babies—or when dilatations aren't the best option—a small procedure called a local stricturoplasty may be done during stoma closure to safely widen the opening.

Diet and bowel management: At first, your baby may be on a liquid or soft diet. Long-term bowel management may include stool softeners to ensure smooth passage of stool without straining.

Follow-up visits: Regular follow-up appointments are important to monitor healing and bowel function. The health care team will evaluate the need for any additional interventions.

Colostomy care: If a colostomy was performed, parents will be instructed on how to care for it until it is surgically reversed.

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At CHRISTUS Health, your child's care is led by a team who understands the complexities of anorectal malformations and the emotions that come with them. Your care team may include pediatric colorectal surgeons, urologists, general surgeons, and gastroenterologists, all working closely together to support your child's health and development.

From the first diagnosis to follow-up care years later, you're not alone. We're here to guide you, listen to you, and walk this journey with you, every step of the way.