Pneumatosis cystoides intestinalis: six case reports and a review of the literature

Abstract

Background: Pneumatosis cystoides intestinalis (PCI) is characterized by gas-filled cysts in the intestinal submucosa and subserosa. There are few reports of PCI occurring in duodenum and rectum. Here we demonstrated four different endoscopic manifestations of PCI and three cases with intestinal stricture all were successfully managed by medical conservative treatment.

Case presentation: There are 6 cases of PCI with varied causes encountered, in which the etiology, endoscopic features, treatment methods and prognosis of patients were studied. One case was idiopathic, while the other one case was caused by exposing to trichloroethylene (TCE), and the remaining four cases were secondary to diabetes, emphysema, therioma and diseases of immune system. Of the six patients, all complained of abdominal distention or diarrhea, three (50%) reported muco-bloody stools, two (33.3%) complained of abdominal pain. In four other patients, PCI occurred in the colon, especially the sigmoid colon, while in the other two patients, it occurred in duodenum and rectum. Endoscopic findings were divided into bubble-like pattern, grape or beaded circular forms, linear or cobblestone gas formation and irregular forms. After combination of medicine and endoscopic treatment, the symptoms of five patients were relieved, while one patient died of malignant tumors.

Conclusion: PCI endoscopic manifestations were varied, and radiology combined with endoscopy can avoid misdiagnosis. The primary bubble-like pattern can be cured by endoscopic resection, while removal of etiology combined with drug therapy can resolve majority of secondary cases, thereby avoiding the adverse risks of surgery.

Keywords: Diagnosis; Endoscopy; Pneumatosis Cystoides intestinalis; Therapy.

Fig. 1

Endoscopic features of PCI. Case 1: (a-c); Case 2: (d, e); Case 3: (f-h); Case 4: (i, j); Case 5: (k); Case 6: (l). a Biopsies were done to reveal the nature of the cystic lesions underneath the mucosa; b Scattered bubble-like or cystoid nodules were seen, which needed to be distinguished from polyps; c NBI showed clear texture of intestinal wall vessels; d Grape-like or beaded subepithelial lesions were identified in the colon, some with erythematous mucosa; e The endoscopic ultrasonography showed low echo of cystic below the mucosal layer; f Line or pebble like sessile cysts were distributed around the colon, with normal overlying mucosa; g Irregular forms were disclosed, which needed to be distinguished from malignant tumor; h The pathologic findings revealed submucosal cystic structure; i Irregular forms needed to be distinguished from Crohn’s disease; j NBI showed unclear vascular texture on mucosal surface; k Gastroscope demonstrated duodenal gas cyst, leading to lumen stenosis; l Appearance of air cysts in the rectum, honeycomb-like

Fig. 2

Imaging findings. Case 2: (a, b); Case 3: (c); Case 4: (d); Case 5: (e); Case 6: (f, g). a Abdominal X-ray showed multiple intraluminal gas pockets in the left colon; b Coronal reconstruction confirmed multiple submucosal lesions; c Abdominal CT showed no portal venous gas embolism. d Abdominal CT revealed multiple polypoid lesions of the colon; e Chest CT showed centrilobular emphysema, pulmonary field scattered in small circular distribution. f Abdominal CT showed a large presence of ascites in the abdomen. g Pelvic CT demonstrated primary peritoneal carcinomatosis with balloon like structures in the rectum

Fig. 3

Endoscopic manifestations after treatment. Case 1: (a, b); Case 2: (c, d); Case 3: (e); Case 4: (f, g); Case 5: (h). a Biopsies were performed, with the release of “air” and cyst collapse; b Gas cyst completely disappeared after treatment; c The cyst of the colon disappeared; d NBI demonstrated visible patchy erythema and yellow nodules; e Gas-filled cysts flattened; f The cyst of the colon improved, but mucosal surface redness still existed; g Colonic gas cyst was full recovery; h Duodenal descending presented as stenosis due to gas cyst