Chronic intestinal pseudo-obstruction (CIPO) is a rare, severe and disabling disorder of gastrointestinal motility. CIPO may involve multiple areas of the gastrointestinal tract, including the esophagus, stomach, small intestine, large intestine, and rectum. The bladder may also be affected in some children.
In children with CIPO, the coordinated muscle contractions in the intestinal tract (peristalsis) become altered and inefficient. Effective peristalsis is very important for digestion and is dependent on the muscles, nerves and hormones in the digestive tract working in a coordinated fashion. Because of the inefficient peristalsis, the intestines in children with CIPO act as if there is a true mechanical obstruction or blockage. However, when testing is performed, no physical evidence of a blockage is found.
The symptoms of pseudo-obstruction are caused by difficulties in how the muscles and nerves in the intestine work. CIPO is generally broken into two groups, primary CIPO and secondary CIPO.
Primary CIPO can be congenital, present at birth. It may be caused by an injury to the smooth muscle (myopathic) or the nervous system (neuropathic) of the gastrointestinal tract. There are also familial and sporadic causes of primary CIPO. Several genetic diseases have been identified in familial cases.
Secondary CIPO is acquired, such as after a viral illness. Autoimmune and metabolic disorders are also known to be associated with secondary CIPO. Specific diseases and infections that may be associated with secondary CIPO include Chagas disease, collagen vascular disorders, endocrine disorders, and muscular dystrophy.
In many cases, the cause of CIPO is unknown.
Clinical signs and symptoms of CIPO vary depending on what part of the intestine is affected. Due to the disruption in normal intestinal peristalsis, children may experience symptoms such as abdominal pain, nausea, abdominal bloating, vomiting, stomach pain, diarrhea and constipation. Severity of the symptoms can vary. Sometimes the symptoms are intermittent and only occur during an acute episode. In severe cases, the symptoms are more chronic and can potentially cause significant life-threatening complications during infancy or early childhood. Sometimes, bacterial infections and poor absorption of nutrients may occur, resulting in malnutrition.
Diagnosing CIPO includes obtaining a comprehensive medical, surgical and symptom history to identify risk factors and symptoms that may be suggestive of CIPO. The physical exam will help your physician evaluate for underlying disorders that have been linked CIPO.
Diagnostic screening testing may include imaging of the abdomen and bowel to evaluate for dilated loops of bowel and transit time, screening laboratory studies and motility studies. Motility studies of the esophagus, stomach, small intestine, large intestine and rectum may be completed.
Acquired pseudo-obstruction is managed by treating the predisposing or underlying condition. There is no cure for congenital pseudo-obstruction.
Doctors who specialize in the movement of the muscles and nerves of the gastrointestinal tract treat pseudo-obstruction.
The goals of treatment for CIPO are to provide nutritional support, minimize abdominal pain and bloating, regulate bowel movements, decrease nausea and vomiting.
Dietary management is aimed at encouraging patients with CIPO to eat five to six small meals throughout the day instead of larger meals and to follow a low-fat, low-fiber diet. Some children may also need to take daily multivitamins and nutritional supplements, which are beneficial for those who are malnourished. Some children may need IV nutrition is they are unable to tolerate food.
A variety of medications may be used to treat children with CIPO. Medications may help to control symptoms and avoid complications. Such medications include antibiotics, medications that improve gastrointestinal motility, and medications that target pain. The specific medication regimen used is based on each individual child’s history.
Early diagnosis and management of underlying and predisposing condition can lead to improvement of symptoms and quality of life for children with CIPO.
Children with CIPO require ongoing management based on symptoms and nutritional needs.
We have extensive experience in the diagnosis and management of pseudo-obstruction secondary to a variety of underlying factors and conditions. CHOP excels in providing comprehensive care for every patient, including medical, pharmaceutical, and nutritional management of pseudo-obstruction and underlying conditions.
Families can come to any CHOP location for medical management or a consultation. Manometry testing is only performed at CHOP’s Main Campus.