COMPASSIONATE ALLOWANCE INFORMATION

CHRONIC IDIOPATHIC INTESTINAL PSEUDO OBSTRUCTION

ALTERNATE NAMES

Chronic Intestinal Pseudo Obstruction; Intestinal Pseudo Obstruction; Congenital Idiopathic Intestinal Pseudo Obstruction; Primary Intestinal Pseudo Obstruction

DESCRIPTION

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.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: The clinical diagnosis of CIIPO is confirmed by a combination of:

• gastrointestinal manometric studies (anal manometry or esophageal manometry),

• transit time measurements,

• radiological findings (dilated bowel with air fluid levels),

• abdominal x-rays,

• gastric emptying radionuclide scan,

• intestinal radionuclide scan,

• barium swallow, barium enema,

• blood test for nutritional or vitamin deficiencies,

• colonoscopy, and

• histological examination of a full thickness biopsy of the affected intestine.

Physical findings: Individuals with CIIPO may present with severe chronic obstructive symptoms: abdominal pain, distensions/fullness, nausea/vomiting, diarrhea and/or intractable constipation, or malabsorption of nutrients leading to weight loss and/or failure to thrive.

ICD-9: 560.89

ONSET AND PROGRESSION

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.

TREATMENT

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.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the impairment, and physical findings.

• Imaging studies including x-rays, ultrasound, computerized axial tomography (CAT) scans, magnetic resonance imaging (MRI) and radionuclide scans.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

5.08

105.08 A and B

105.10

Listing level severity must be documented.

Equals Listing

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.