Malrotation in Children

What is malrotation in children?

Malrotation is a congenital (present at birth) condition that affects the positioning of a baby’s small and large intestines within their abdomen (belly). The intestines form early in fetal development. As part of that process, the intestines coil and rotate until they reach the proper position and attach to the abdominal wall. But in some cases, they don’t coil and rotate properly. This is called intestinal malrotation.

Most children will need surgery to fix the positioning of their intestines, prevent complications and help the digestive system work as it should.

What is volvulus?

Volvulus is abnormal twisting of the intestines that compromises the blood supply to your child’s bowel. It’s the main complication of malrotation. You might also hear it called a twisted bowel or twisted colon.

Volvulus happens because your baby’s intestines aren’t attached to the abdominal wall in the correct spots. Without proper anchoring, the intestines may start to twist in ways they shouldn’t. This twisting can prevent digested food from passing through the intestines and even restrict blood flow. Picture a garden hose that twists in a loop and develops a kink. Water can’t pass through until you reposition the hose. With volvulus, the consequences can be much more serious.

Signs and symptoms usually appear in children before age 5 — often as early as the first month after they’re born — and include sudden vomiting (usually greenish in color) and bloating.

Volvulus is a surgical emergency. Untreated volvulus can lead to life-threatening complications, including:

• Bowel obstruction.
• Bowel perforation.
• Peritonitis.
• Sepsis.
• Shock.

What are the signs and symptoms of malrotation?

Signs and symptoms can vary but generally include frequent vomiting and abdominal pain. Malrotation itself may not cause symptoms until it leads to volvulus.

Call 911 or your local emergency number if your child has green or yellow-green vomit or blood in their poop. These can be signs of volvulus or other emergencies.

Signs of malrotation in infants

Signs and symptoms of malrotation in infants include:

• Frequent vomiting, which may be green or yellow-green due to the presence of bile.
• Abdomen (belly) that’s swollen, painful or tender to the touch.
• Severe abdominal colic pain.
• Rectal bleeding or bloody poop.

Babies with cramps and pain from malrotation frequently follow a typical pattern. They’ll:

• Draw their legs up and cry.
• Settle for about 10 to 15 minutes.
• Begin to cry again.

Signs of malrotation in children over age 1

Signs and symptoms in children over age 1 include:

• Abdominal pain or tenderness.
• Nausea and vomiting.
• Diarrhea.
• Bloated belly.
• Constipation.
• Rectal bleeding or poop that contains blood.
• Not growing as expected (failure to thrive).

Some research shows that kids over age 2 are less likely to produce green vomit than infants. Older children typically have recurrent vomiting (normal in color) and abdominal pain.

What causes intestinal malrotation?

Researchers don’t know exactly what causes malrotation. They know it’s a congenital condition, which means a person is born with it. The intestines form in a fetus around week 10 of pregnancy. Experts are still studying what makes intestines form incorrectly.

How is intestinal malrotation diagnosed?

Healthcare providers use physical exam findings and imaging tests to diagnose malrotation. A provider will give your child a thorough exam to evaluate their overall health and check for signs of malrotation. They’ll also use imaging tests that take pictures inside your child’s body. These include:

• Abdominal ultrasound and X-rays with contrast.
• Gastrointestinal (GI) exams.

Your child’s provider will tell you more about the specific tests your child needs and what you can expect.

What is the treatment for intestinal malrotation in children?

Babies and children with malrotation typically need surgery to realign their intestines. A surgeon, through a minimally invasive surgery technique (MIS surgery), will untwist your child’s intestines and reposition them within their belly.

There are two main surgeries that surgeons use to manage malrotation:

• Ladd’s procedure. Ladd’s procedure involves releasing abnormal bands of tissue that prevent the intestines from working as they should. A surgeon will also adjust how your child’s intestines are rotated and positioned. Surgeons can perform Ladd’s procedure via open surgery or laparoscopic methods. Although Ladd’s procedure helps ease symptoms, it’s possible for a child’s intestines to abnormally twist again in the future (recurrent volvulus).
• Kareem’s procedure. This involves rotating the intestines all the way to their proper position and connecting them to the abdominal wall. This technique may lower the risk of recurrent volvulus.

Your child’s care team will discuss the best approach to use in your child’s situation. along with the benefits and risks. Depending on the anatomy and health of their intestines, your child may also need:

• A temporary or permanent colostomy.
• A portion of their intestines removed.
• Intestinal reconstruction.
• Their appendix removed (if their anatomy would make diagnosing appendicitis difficult in the future).

Some babies and children need emergency surgery. This may happen if malrotation leads to volvulus that may cause serious damage to your child’s intestines. Emergency surgery can feel scary and overwhelming. Don’t hesitate to ask your child’s care team questions. They’ll help you understand more about what’s happening and what your child’s recovery will look like.

Can twisted intestines be prevented?

There’s no way to prevent malrotation or its main complication, volvulus, because experts aren’t sure what causes it. However, seeking care right away can lower your child’s risk of serious complications.

If something doesn’t seem right with your child — like they’re repeatedly vomiting and you can’t figure out why — reach out to a healthcare provider. Don’t assume it’ll go away on its own or that you’re over-worrying. Malrotation can become more serious the longer it goes on without treatment.

What can I expect if my child has intestinal malrotation?

Your child’s outlook depends on many factors, including:

• How soon they receive treatment.
• How damaged their intestines are.
• Any other health conditions they may have.

Surgery helps many children feel better and have a lower risk of future complications. Some children experience twisted intestines again, even after surgery. Newer surgical techniques are lowering this risk. Your child’s care team will tell you more about what to expect.

When should I seek medical care for my child?

Call a pediatrician if your child has signs or symptoms of intestinal malrotation. This condition can quickly lead to a medical emergency. Noticing early signs can help your child get treatment before serious complications occur.

Noticing recurrent vomiting in your child can be distressing. It’s important to seek medical care, but keep in mind there are other possible causes besides malrotation. Many other conditions (like reflux) have similar symptoms. Your pediatrician will find the cause and make sure your child gets the treatments they’ll need.

When should I go to the emergency room?

Seek emergency care right away if your child has:

• Green or yellow-green vomit.
• Unusually fast breathing.
• Fast heart rate.
• Confusion.
• Unconsciousness (passing out).
• Skin that’s cool, clammy and paler than usual.
• Abdominal distension with colicky pain.

Which questions should I ask my child’s healthcare provider?

Asking questions allows you to learn more about your child’s condition and available treatment options. You might consider asking:

• How does malrotation affect my child?
• Are there signs of complications?
• Does my child need surgery? If so, can you tell me more about what’s involved?
• What are the benefits and risks of surgery?
• What will my child’s recovery look like and how can I support them?
• What follow-ups will my child need?