Chronic intestinal pseudo-obstruction (CIP) is a rare and disabling motility syndrome, yet one that demands an extensive review of digestive motility and peristaltic pathophysiology. Primarily a disorder of the small intestine, CIP was first described by Dudley and colleagues in 1958; it is defined by severe signs and symptoms of intestinal obstruction (abdominal pain and distention, nausea, vomiting, and constipation), in addition to radiographic evidence of dilated bowel in the absence of a true, mechanical obstruction. Symptoms are often slowly progressive and diagnosis requires the presence of symptoms for at least six months.1 A 2013 national survey in Japan estimated the prevalence of CIP at 0.8 to 1.0 per 100,000, with an incidence rate of 0.21 to 0.24 per 100,000. In the same survey, the mean age at diagnosis is 63.1 years for males and 59.2 for females.2 CIP encompasses an extensive differential diagnosis, a complex, multidisciplinary work-up, and a vast array of potential treatment options based in intricate pathophysiology.
RC is a 75-year-old male who presented with recurrent small bowel obstructions (SBOs) between August and September 2014. He has no chronic medical conditions, and his past medical history is significant only for a community-acquired pneumonia and pleural empyema at age 50. His surgical history is significant only for a right inguinal hernia repair at age 7. Initially, his symptoms began in April 2014 and were mild, limited to constipation relieved with over the counter laxatives. After two brief admissions for SBOs that resolved with nasogastric tube decompression, RC presented on September 9, 2014 with a distended, tympanitic abdomen with absence of bowel sounds and minimal tenderness to palpation. A computed tomography (CT) scan demonstrated multiple dilated loops of small bowel with a transition point in the proximal ileum. A nasogastric tube was again placed but the obstruction persisted clinically and on repeat X-Rays. During an exploratory laparoscopy on September 15, 2014, the right colon and entire small bowel were palpated. No transition zone or small bowel abnormality was found and the peritoneal surfaces of all abdominal organs appeared normal. Ultimately, an ileocecectomy was performed and RC underwent a thorough diagnostic workup.
Kalra, MD, Ankush and Dimarino, MD, Anthony
"Chronic Idiopathic Intestinal Pseudo-Obstruction: A Working Diagnosis,"
The Medicine Forum:
Vol. 16, Article 7.
Available at: http://jdc.jefferson.edu/tmf/vol16/iss1/7