Prolonged postoperative ileus in a patient with primary pneumatosis cystoides intestinalis: a case report
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Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by multiple gas-filled cysts in the intestinal wall and is associated with various comorbidities. We report herein a case of intractable paralytic ileus caused by primary PCI.
A 73-year-old man visited out hospital complaining of abdominal pain and vomiting. He had been hospitalized twice for intestinal obstruction in the past 2 months. Based on his history of appendectomy, mechanical bowel obstruction caused by adhesion was diagnosed, and the patient underwent surgery. However, laparotomy revealed small bowel dilatation despite the absence of obstruction or stenosis. Multiple nodules were found in the wall of the dilated bowel loops. The dilated jejunum was excised. Histological examination revealed that the nodules were small gas-filled cysts, suggesting PCI. We made a diagnosis of ileus with underlying PCI and managed the patient conservatively. A large amount of nasogastric tube drainage continued for a long period postoperatively. The patient underwent relaparotomy 35 days after the first operation. The upper jejunum was markedly dilated, although no mechanical stenosis was found. The atonic, dilated jejunum was excised and the ileal stump was anastomosed to the duodenum in a double tract fashion. The patient underwent hyperbaric oxygen therapy because the ileus persisted postoperatively. His condition gradually improved and he was discharged 53 days after the second operation.
Non-operative treatment is recommended for primary PCI of unknown etiology. Surgeons should be mindful of the possibility of primary PCI when considering surgical intervention for patients with bowel obstruction.
KeywordsParalytic ileus Pneumatosis cystoides intestinalis Hyperbaric oxygen therapy Duodenum
Chronic obstructive pulmonary disease
Hyperbaric oxygen therapy
Pneumatosis cystoides intestinalis
Prostaglandin F2 alpha
Port-mesenteric venous gas
Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular gas-filled cysts in the intestinal wall. The etiology of PCI is still unclear, although many theories have been proposed. Secondary PCI, which is associated with bowel obstruction or necrosis, is an indication for urgent surgery, while surgical intervention is usually unnecessary for primary or idiopathic PCI. Despite a considerable number of case reports, primary PCI is still not widely recognized as a disease entity. We report here a case of primary PCI where the patient underwent surgery and had prolonged postoperative ileus.
The patient had prolonged ileus even after the second operation. Prostaglandin F2 alpha and long-acting octreotide were somewhat effective, but the effects were temporary. Endoscopic examination revealed that the passage created by duodenoileostomy was widely open and the fiberscope easily entered the ileal limb. The patient underwent HBOT for 9 days. Thereafter, the incidence of vomiting, which repeatedly occurred, gradually decreased. The patient resumed oral intake and was discharged 53 days after the second operation. Although a cyst-like dilatation of the third portion of the duodenum was seen on a follow-up CT scan conducted 15 months later, the patient’s quality of life is presently good, and he is able to tolerate a normal daily diet.
PCI is a disease that forms gas-filled cysts in the submucosa and subserosa of the intestinal tract . The disease was first reported by Du Vernoi et al. in 1730 . The etiology, however, remains unknown. Four major hypotheses have currently been proposed: (1) the mechanical theory, proposing that gas enters the digestive tract walls through mucosal injury in association with increased luminal pressure ; (2) the bacterial theory, proposing that gas-producing bacteria may cause intramural gas-filled cysts ; (3) the chemical theory, proposing that chronic exposure to chemicals, such as trichloroethylene, alpha-glucosidase inhibitors, or steroids, may impair the integrity of the mucosa ; and (4) the lung theory, proposing that the intramural bowel gas may originate from pneumomediastinum caused by alveolar rupture in cases of chronic obstructive pulmonary disease (COPD) .
The final diagnosis in the present case was primary PCI because surgery showed no mechanical obstruction and the patient had no history of any condition associated with PCI. The patient had previously been diagnosed as having COPD based on honeycomb-like changes seen on pulmonary CT scans. However, the fibrotic changes were limited to the pulmonary bases and the patient had no pulmonary symptoms. Asymptomatic COPD seemed unlikely to have a significant association with PCI.
The patient in this case had prolonged ileus. We believe that the initial laparotomy adversely affected the clinical course. In general, non-surgical treatment including HBOT is preferred for management of primary PCI. Treatment decision-making is not clear cut, however, because PCI sometime leads to life-threatening abdominal emergencies requiring urgent surgery. Treyaud et al.  retrospectively analyzed 149 patients in whom PCI was evident on multidetector CT and found the most frequent cause to be intestinal ischemia (53.7%), followed by infection (12.1%) and bowel obstruction (8.1%). Non-obstructive bowel dilatation was seen in only 6.7% of the patients. The overall mortality in that study was 41.6% and the presence of port-mesenteric venous gas (PVG) and decreased mural contrast enhancement were significant diagnostic radiological findings associated with bowel ischemia. Meanwhile, Morris et al.  analyzed patients with PCI at their institution between 2000 and 2007, and reported that non-operative management was selected for 50 patients (52%), of whom only 3 died. The authors found that a high APACHE II score was a significant risk factor for mortality in their study. Wayne et al.  classified 88 patients who showed PCI and/or PVG into three subgroups: acute mesenteric ischemia, mechanical causes, and benign idiopathic. Each of these subgroups accounted for about a third of the study cohort. The authors developed a scoring algorithm based on the data, taking into account a patient’s history, physical status, laboratory data, and radiological findings, and showed that the algorithm was useful in preventing non-therapeutic laparotomy. There is still no absolute indicator to suggest when urgent laparotomy is necessary for PCI patients. We have to decide whether surgery is indicated after careful consideration of the patient’s history, clinical status, and CT scan findings.
Non-ischemic bowel diseases can also exhibit PCI. Medical treatment, including HBOT, is recommended for primary PCI, where the etiology is unknown. Surgeons should be mindful of the possibility of primary PCI when considering surgical intervention for patients presenting with bowel obstruction and PCI.
The authors received no funding for this study.
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HT, TK, and TC were the attending doctors for the patient. HT and TK drafted this manuscript. UT contributed to the interpretation of CT findings and radiological diagnosis. MN was responsible for the pathological examinations. HK and TW supervised the preparation of the case report. All authors have read and approved the final manuscript.
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- 2.Du Vernoi GJ. Aer intestinorum tam subextima quam intima tunica inclusus. Obsergationac Anatomicae Acad Scient Imp Petoropol. 1730;5:213–5.Google Scholar
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