How can Chronic Intestinal Pseudo-Obstruction (CIPO) be managed?

Management of Chronic Intestinal Pseudo-Obstruction (CIPO)

CIPO management requires a multidisciplinary team approach prioritizing nutritional support, symptom control with prokinetics and antibiotics for bacterial overgrowth, and avoidance of unnecessary surgery, with parenteral nutrition reserved for patients who cannot maintain nutrition enterally. 1

Multidisciplinary Team Composition

The management team must include gastroenterologists, gastrointestinal physiologists, surgeons, pain specialists, psychiatrists/psychologists, dietitians, specialist nurses, clinical biochemists, histopathologists, and pharmacists, with regional networking encouraged for support and data collection. 1

Treatment Goals

The primary aims are to: 1

• Reduce symptoms (pain, vomiting, distension, constipation/diarrhea, bloating)
• Reduce morbidity and mortality
• Achieve a BMI within the normal range
• Improve quality of life

Nutritional Management Algorithm

Step 1: Oral Nutrition Optimization

• Begin with oral supplements and dietary adjustments if the patient is malnourished or at risk. 1
• Recommend frequent small meals (4-6 per day) with low-fat, low-fiber content and liquid nutritional supplements, as many CIPO patients tolerate liquids better than solids. 1, 2
• Separate liquids from solids by avoiding drinking 15 minutes before or 30 minutes after eating to minimize bacterial overgrowth. 2

Step 2: Gastric Feeding

• If oral feeding is unsuccessful and the patient is not vomiting, attempt gastric feeding. 1
• This option is only viable in patients without significant gastroparesis. 1

Step 3: Jejunal Feeding

• If gastric feeding fails, try jejunal feeding initially via nasojejunal tube. 1
• If successful, insert a permanent tube endoscopically (through a gastrostomy [PEGJ] or as a direct jejunostomy) or surgically. 1
• A jejunostomy is also useful for drug administration. 1
• Note: Jejunal feeding often fails due to abdominal distension or pain as the feed is infused. 1

Step 4: Parenteral Nutrition

• If jejunal feeding fails and the patient is malnourished, parenteral support (home parenteral nutrition, HPN) is necessary. 1
• Do not delay HPN in malnourished patients if oral nutrition/enteral nutrition is obviously inadequate. 1
• The nutritional regime should follow the same criteria adopted for HPN in patients with other causes of chronic intestinal failure. 1

Pharmacological Management

Prokinetic Agents

• Always attempt a trial with prokinetics, even though they help only a minority of patients with generalized motility disorders. 1
• Main drugs used include metoclopramide, domperidone, erythromycin, octreotide, and neostigmine. 1
• Octreotide (50-100 mcg/day subcutaneously) has shown benefit in adults with scleroderma-associated CIPO. 1
• Prucalopride, a highly specific serotonin receptor agonist with enterokinetic effects, is the latest assessed agent. 1

Antibiotic Therapy for Bacterial Overgrowth

• Sequential antibiotic therapy is very effective in treating intestinal bacterial overgrowth and reducing malabsorption, improving nutritional status and sometimes bloating. 1
• Use rifaximin (550mg twice daily for 1-2 weeks) as first-line treatment due to its non-systemic action and favorable side effect profile. 3
• Rotate antibiotics in repeated courses every 2-6 weeks (with 1-2 week antibiotic-free periods between courses) to prevent resistance. 3
• Poorly absorbable antibiotics such as aminoglycosides and rifaximin are preferred, but alternating cycles with metronidazole and tetracycline may be necessary to limit resistance. 1
• Alternative antibiotics include amoxicillin-clavulanate, doxycycline, norfloxacine, cephalosporins, ciprofloxacin, or cotrimoxazole. 1, 3
• Bacterial overgrowth may lead to life-threatening bacterial translocation. 1

Pain Management

• Avoid high doses of opioids, as they worsen intestinal dysmotility and can lead to narcotic bowel syndrome. 1
• If the patient has taken long-term opioids, consider gradual supervised opioid withdrawal with involvement of a pain specialist. 1
• Use as few drugs as possible and avoid cyclizine and anticholinergic drugs. 1

Nutritional Supplementation

Vitamin Monitoring and Replacement

• Monitor for deficiencies in fat-soluble vitamins (A, D, E, K), as SIBO causes malabsorption through bacterial deconjugation of bile salts. 2
• Supplement with water-miscible forms: Vitamin A (10,000 IU daily), Vitamin D (3000 IU daily), Vitamin E (100 IU daily), and Vitamin K (300 mcg daily). 3
• Check vitamin B12 and iron status, as these are commonly depleted. 2

Management of Steatorrhea

• Consider bile salt sequestrants (cholestyramine or colesevelam) if steatorrhea persists, but be aware these can worsen fat-soluble vitamin deficiencies. 3, 2

Surgical Considerations

Critical Caveat: Avoid Surgery When Possible

• Avoid surgery in CIPO patients whenever possible due to the risk of postoperative worsening of intestinal function and need for subsequent reoperation. 1
• Surgery should be indicated only in a highly selected, well-characterized subset of patients. 4

Limited Surgical Options

• A venting ostomy (either endoscopically or surgically) can diminish symptoms in selected patients. 1
• A venting gastrostomy may reduce vomiting but can have problems (leakage, not draining, or poor body image). 1
• Optimize nutritional status before any surgical procedure whenever possible. 1
• Delay percutaneous endoscopic gastrostomy (PEG) or stoma placement in severely malnourished or physiologically unfit patients. 1

Intestinal Transplantation

• Isolated intestinal or multivisceral transplantation is a rescue therapy only for patients with intestinal failure unsuitable for or unable to continue with parenteral nutrition. 4, 5

Common Pitfalls to Avoid

• Do not perform unnecessary surgery, as this is the most critical error in CIPO management and can worsen intestinal function. 1, 4
• Do not continue or prescribe opioids, as they exacerbate dysmotility and can lead to narcotic bowel syndrome. 1
• Do not use cyclizine or anticholinergic drugs, as these worsen intestinal motility. 1
• Do not delay nutritional support in malnourished patients, as this increases morbidity and mortality. 1
• Do not ignore bacterial overgrowth, as it can lead to life-threatening bacterial translocation and sepsis. 1

Monitoring and Follow-up

• Assess nutritional status regularly, aiming for BMI within normal range. 1
• Monitor for complications of bacterial overgrowth including malabsorption, vitamin deficiencies, and sepsis. 1
• If numbness or tingling in feet develops while on antibiotics, stop immediately and contact physician, as this indicates peripheral neuropathy. 2
• Monitor for persistent steatorrhea (fatty, foul-smelling stools). 2