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Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS)

A diagnosis of megacystis microcolon intestinal hypoperistalsis (MMIHS) can leave you unsure of how to best care for your child and concerned for the future. We can help. The experienced, compassionate team at Children’s Health focuses on education and support paired with advanced diagnosis and treatment options. We work with you to develop a care plan that produces the best possible outcome.

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Fax: 469-488-7001
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What is Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS)?

MMIHS is a very rare disorder in which the muscles that line your child’s bladder and intestines do not move the way they should. In some cases, they do not move at all. Children with MMIHS have a smaller than normal colon and an enlarged bladder. This affects your child’s digestive system and their ability to receive nutrition by eating and drinking. It also affects your child’s ability to urinate. This can cause several health issues, including:

Inability to digest food - makes it difficult for your child to get adequate nutrition. Most children with MMIHS need some form of assistance, like a feeding tube, to get the nutrients essential for growth.
Intestinal malrotation - occurs when your child’s intestines don’t fold as they should and instead twist abnormally, causing blockages and affecting digestion.
Intestinal pseudo-obstruction - mimics a blockage of the intestines when an obstruction does not actually exist and reduces your child’s ability to digest nutrients from food. It causes partially digested food to build up in the intestines, which can cause vomiting, abdominal swelling and pain.
Reduced ability to pass urine - makes it difficult for your child to completely empty their bladder. This causes your child’s bladder to grow larger and their abdomen to swell. In many cases, a thin tube, or catheter, is needed to help remove urine when your child’s bladder is not working properly.

MMIHS is a serious, life-threatening condition that requires ongoing medical care and monitoring to help your child achieve a normal, active life.

What are the signs and symptoms of Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS)?

The signs and symptoms of MMIHS are usually similar for the children it affects, but the severity of the disorder and the impact it has on their daily life may be different.

Symptoms include:

Bile-stained vomit
Enlarged bladder, called megacystis
Increased amniotic fluid in late pregnancy
Sluggish or non-existent intestinal movements, called hypoperistalsis
Swollen abdomen
Undescended testes (UDT) in boys
Very small colon, called a microcolon

How is Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS) diagnosed?

MMIHS can sometimes be determined using ultrasound before your baby is born, but it is most often diagnosed shortly after birth.

The team of experts at Children’s Health starts with a careful examination of your child and review of their medical history and symptoms. Lab work and a colon biopsy can help confirm whether MMIHS is present.

What are the causes of Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS)?

MMIHS is a genetic disorder. This means that it is caused by a mutation in one or more of the genes that help control the movements in your child’s urinary and intestinal tracts and allows them to empty urine and move food through their body.

Some children inherit MMIHS from one of their parents. In other instances, the condition happens with no previous family history. This is called a de novo mutation.

How is Pediatric Megacystis Microcolon Intestinal Hypoperistalsis (MMIHS) treated?

There is currently no cure for MMIHS. Treatment is ongoing and centers on your child’s bladder, kidney and digestive functions.

Treatment includes:

Catheterization - uses a tube (catheter) that’s inserted into your child’s bladder to drain urine. The process is done several times a day to reduce the strain on your child’s bladder and kidneys.
Feeding tubes - help provide nutrients by delivering food directly into your child’s stomach or intestinal tract. There are several different types, which can be tailored to your child’s specific needs.
Ileostomy – a surgical procedure in which an opening is made in the wall of your child’s belly, or abdominal wall. It provides a way for your child’s body to eliminate stool, which is then collected in a pouch worn on the outside.
Intestinal resection - a surgical procedure in which part or all of your child’s small intestine is removed to eliminate damaged or diseased portions that are interfering with your child’s ability to digest food.
Total Parenteral Nutrition (TPN) - a way for your child to receive nourishment without involving their digestive tract. A mixture of electrolytes, protein, vitamins, fats and other essential nutrients is pumped into your child’s body through a central line or port directly into their bloodstream.