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Chronic intestinal pseudoobstruction
Chronic intestinal pseudo-obstruction (CIPO) is a rare gastrointestinal motility disorder characterized by recurring episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders, and without any physical obstruction being detected by X-ray or during surgery. CIPO develops predominantly in children and may be present at birth.
ORPHA:2978Classification level: Disorder
The prevalence remains unknown. The male-to-female ratio is about 1.5:1 to 4:1.
Patients commonly present with severe chronic "obstructive" symptoms: abdominal pain, distension/fullness, nausea/vomiting, diarrhea and/or intractable constipation, malabsorption of nutrients leading to weight loss and/or failure to thrive. Laboratory abnormalities usually reflect the degree of malabsorption and malnutrition. The radiological findings commonly include paralytic ileus or signs of apparent clinical obstruction with dilated loops of bowel. The regions of the gut affected may be isolated (small bowel involvement is the most typical) or diffuse, and sometimes other visceral musculature, such as the urinary bladder, is involved.
The etiology remains unknown. Disorganization or lack of smooth muscle contractility, resulting in disordered peristalsis or aperistalsis, represents the primary pathophysiological dysfunction. Certain authors divide CIPO into myopathic and neuropathic categories. CIPO may be a primary or secondary disorder due to muscular, neurologic, metabolic or endocrine disorders. It also occurs postinfectiously, postoperatively, or may be caused by abdominal radiation, drugs or noxae..
The clinical diagnosis should be confirmed by a combination of gastrointestinal manometric studies, transit time measurements, radiological findings (dilated bowel with air-fluid levels), and histological examination of a full-thickness biopsy of the affected intestine. If CIPO is suspected, mechanical obstruction must be carefully excluded by radiologic and endoscopic examinations.
Management and treatment
Management requires a multidisciplinary approach (pediatric gastroenterologist, pain management specialist, psychologist) and depends on the cause of the disorder, the extent and location of intestine involved, and the severity of symptoms. General measures include dietary changes (nutritional support to prevent malnutrition by oral and/or enteral nutrition), prokinetic agents (metoclopramide, cisapride), treatment of complications such as bacterial overgrowth and severe pain, and specific surgical procedures. The potential role of operative treatment of CIPO is controversial; unnecessary laparotomies should be strictly avoided as they may lead to adhesions and markedly complicate the clinical course. Surgery in CIPO is indicated for vascular access, intestinal biopsy, and palliation surgery, including total enterectomy, venting enterostomy, duodenojejunostomy, and duodenoplasty. Recently, intestinal transplantation has become a therapeutic option for children with severe refractory disease who are dependent on total parenteral nutrition (TNP), and in whom TPN management is failing.
CIPO is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time. Treatment and long-term outcome are often unsatisfactory.