CHRONIC IDIOPATHIC INTESTINAL PSEUDO OBSTRUCTION
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ALTERNATE NAMES
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Chronic Intestinal Pseudo Obstruction; Intestinal Pseudo Obstruction; Congenital Idiopathic
Intestinal Pseudo Obstruction; Primary Intestinal Pseudo Obstruction
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DESCRIPTION
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Chronic Idiopathic Intestinal Pseudo Obstruction (CIIPO) is a rare gastrointestinal motility (movement) disorder characterized by impairment
of the muscle contractions that move food, fluid, stool or air through the gastrointestinal
(digestive) tract in the absence of any mechanical obstructions or lesion(s). This
condition may be caused by abnormalities or injury to the smooth muscles of the gastrointestinal
tract (myogenic) or from problems with the nerves that control the muscle contractions
(neurogenic). CIIPO leads to a buildup of partially digested food in the intestines.
This buildup may cause abdominal swelling (distention) and pain, nausea, vomiting,
and constipation or diarrhea. Affected individuals may experience loss of appetite
and impaired ability to absorb nutrients, which may lead to malnutrition and weight
loss.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: The clinical diagnosis of CIIPO is confirmed by a combination of:
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Gastrointestinal manometric studies (anal manometry or esophageal manometry);
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Transit time measurements;
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Radiological findings (dilated bowel with air fluid levels);
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Gastric emptying radionuclide scan;
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Intestinal radionuclide scan;
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Barium swallow, barium enema;
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Blood test for nutritional or vitamin deficiencies;
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Histological examination of a full thickness biopsy of the affected intestine.
Physical findings: Individuals with CIIPO may present with severe chronic obstructive symptoms:
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Nausea/vomiting, diarrhea and/or intractable constipation; or
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Malabsorption of nutrients leading to weight loss and/or failure to thrive.
ICD-9: 560.89
ICD-10: K59.8
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PROGRESSION
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CIIPO can occur in people of any age, but it develops primarily in children and may
be present at birth. In severe cases, intestinal transplantation may be utilized in
children who are dependent on total parenteral nutrition (TPN- an intravenous administration
of a solution of essential nutrient needed by individuals who are unable to ingest
food) or TPN management failure. In adults, CIIPO is commonly a secondary complication
of other conditions such as scleroderma and other connective tissue conditions, diabetes,
neurologic disease, use of narcotics with anticholinergic properties, hypothyroidism
infection, paraneoplastic syndromes, amyloidosis, and radiation enteritis. CIIPO is
a severe disease which may lead to potentially life-threatening complications over
time. Long term outcome is generally poor despite surgical and medical therapies.
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TREATMENT
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There is no definitive cure for CIIPO. Treatment is symptom specific to reduce complications.
Medications such as prokinetics (Domperidone, Cisapride, or Erythromycin) to increase
the frequency of contractions; analgesics (relief from pain); or antibiotics to treat
bacterial overgrowth may be prescribed; nutritional support (PEG jejunostomy, total
parenteral nutrition (TPN); and surgical interventions (colonoscopy, bypass, limited
resection, decompression stoma) may be utilized. Management of CIIPO requires a multidisciplinary
approach including a dietician to address nutritional deficiencies; gastroenterologist;
pediatric gastroenterologist; pain management specialist or psychologist depending
on the cause of the disorder, the extent and location of intestine involved and the
severity of symptoms.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment,
and physical findings; and
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Imaging studies including x-rays, ultrasound, computerized axial tomography (CAT)
scans, magnetic resonance imaging (MRI) and radionuclide scans.
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Suggested
Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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5.08
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Listing level severity must be documented.
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105.08 A and B
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Listing level severity must be documented.
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105.10
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Listing level severity must be documented.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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