Pediatric Motility Disorders

Chronic Intestinal Pseudo Obstruction (CIPO)

Chronic Intestinal Pseudo Obstruction is a rare disorder in which intestinal nerve or muscle problems prevent food, fluid, and air from moving through the stomach and intestines due to abnormalities affecting the involuntary, coordinated muscular contraction (called peristalsis) of the GI tract. (1)

Common Signs and Symptoms:

Nausea and vomiting
Abdominal distention
Abdominal pain
Constipation

Diagnosis:

Manometry: Gold standard for diagnosis as it measures the contractions of the intestinal tract and can help to localize the functioning areas of the gastrointestinal tract. Manometry can help evaluate functional areas prior to any surgical intervention or creation of a stoma.
Gastric emptying studies: Direct, noninvasive measurements of liquid or solid gastric emptying to assess function of the stomach.
Full thickness biopsy: Full thickness biopsies are useful in diagnosis autoimmune or mitochondrial etiology for chronic intestinal pseudo obstruction and can get a better understanding of abnormalities of the intestinal (enteric) nervous system.

Treatment:

Nutritional Support: Majority of patients will require nutritional support, often requiring total parenteral nutrition (TPN).
Prokinetics, such as erythromycin, octreotide, or tegaserod may be recommended.
Antibiotics may be used to treat small intestinal bacterial overgrowth.
Surgical: A decompressing gastrostomy or jejunostomy is reported to be a beneficial intervention in patients with chronic intestinal pseudo obstruction.
Small Bowel Transplant: May be indicated in patients who have TPN dependency and have TPN failure.

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS)

A rare congenital disorder that results from a developmental defect of the smooth muscle in the bladder and the colon. This interferes with the functioning of the stomach, intestines, kidneys, and bladder, thus interfering with urination and digestion. It is considered a sub-category of pseudo-obstruction that is characterized by:

Distended, non-obstructed urinary bladder microcolon.
Incomplete intestinal rotation.
Hypoperistalsis in the entire gastrointestinal tract.
Failure to pass meconium.

Diagnosis: The enlarged bladder is usually the first detected defect on ultrasound. After birth, it will be detected when your infant doesn’t pass his or her first meconium or produce proper urine. Imaging tests of your infant may show signs of microcolon (very small appearing colon), enlargement of the small intestine or stomach, an enlarged bladder, hydronephrosis, enlarged ureters, or other malformations of the urinary tract.

Treatment is supportive until an intestinal transplant can be performed.

Reference:

Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral transplantation. Gastroenterology Report. 2017;5(1):20-28. doi:10.1093/gastro/gow045.