Pneumatosis cystoides intestinalis

Pneumatosis cystoides intestinalis is a condition in which submucosal or subserosal gas cysts are found in the wall of the small or large bowel. Both fulminant and benign forms exist. Fulminant pneumatosis cystoides intestinalis is associated with an acute bacterial process, sepsis, and necrosis of the bowel, while benign pneumatosis cystoides intestinalis can be totally asymptomatic and observed as an incidental finding at laparotomy. Many different causes of pneumatosis cystoides intestinalis have been proposed, including mechanical and bacterial causes. A review of the literature is presented with emphasis on the etiology of pneumatosis cystoides intestinalis, which probably can be attributed to bacterial and mechanical factors. Diagnosis and different forms of therapy and their efficacy are also discussed.     

A case of pneumatosis cystoides intestinalis in a patient with polymyositis and interstitial pneumonia

We report the case of a 49-year-old man who presented with pneumatosis cystoides intestinalis associated with polymyositis and interstitial pneumonia. Three months after the administration of prednisolone and cyclosporine, he noticed mild abdominal distension, and a radiographic examination showed intraperitoneal free gas and intramural gas, suggestive of pneumatosis cystoides intestinalis (PCI). Additional treatment with a combination of doxycycline and high-dose oxygen therapy was effective in relieving the clinical symptoms and reducing the intramural gas.     

A case of pneumatosis cystoides intestinalis induced by steroid pulse therapy for severe acute hepatitis B

A 26-year-old Japanese woman was admitted to the hospital because of fever and general fatigue. A diagnosis of acute hepatitis B was given because of high levels of transaminase and positivity for HBs-Ag, HBe-Ag and HBc-IgM. On the 2nd day progression to fulminant hepatitis was suspected, and steroid pulse therapy, cyclosporin, entecavir, and interferon-β were started. Her laboratory data improved until transaminase showed an increase on 18th day, and steroid was once again administered. Abdominal CT scan and plain abdominal X-ray showed pneumatosis cystoides intestinalis (PCI) mainly along the ascending colon without any symptoms. After discontinuation of steroid therapy, abnormal gas gradually disappeared. This is a very rare case of PCI, which may have been caused by short-term steroid pulse therapy.     

A case of pneumatosis cystoides intestinalis in a patient with polymyositis and interstitial pneumonia

Abstract

We report the case of a 49-year-old man who presented with pneumatosis cystoides intestinalis associated with polymyositis and interstitial pneumonia. Three months after the administration of prednisolone and cyclosporine, he noticed mild abdominal distension, and a radiographic examination showed intraperitoneal free gas and intramural gas, suggestive of pneumatosis cystoides intestinalis (PCI). Additional treatment with a combination of doxycycline and high-dose oxygen therapy was effective in relieving the clinical symptoms and reducing the intramural gas.     

Pneumatosis cystoides intestinalis in Crohn's disease

Two cases of pneumatosis cystoides intestinalis in patients with Crohn's disease are reported. These cases illustrate both benign and fulminant forms of pneumatosis cystoides intestinalis, and highlight the diagnostic and therapeutic dilemma of patients in whom pneumatosis cystoides intestinalis and Crohn's disease coexist. While therapy is seldom indicated in benign forms of pneumatosis cystoides intestinalis, mortality rates with fulminant pneumatosis cystoides intestinalis are high, regardless of the type of therapy.     

Pneumatosis Cystoides Intestinalis Associated with Refractory Sprue

Pneumatosis cystoides intestinalis is a disease of unknown etiology characterized by gas-filled cysts within the bowel wall. The spectrum of gastrointestinal disorders associated with pneumatosis cystoides intestinalis is expanded by this report of a patient with refractory sprue who developed pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum. Possihie pathogenetic mechanisms and the importance of the clinical diagnosis are discussed.     

A case of recurrent pneumatosis cystoides intestinalis associated with recurrent pneumoperitoneum]

Pneumatosis cystoides intestinalis is an uncommon condition of unknown etiology, characterized by the presence of multiple gas filled cysts in the gastrointestinal tract. Many different causes of pneumatosis cystoides intestinalis have been proposed, including mechanical, pulmonary, and bacterial causes. Approximately 85% of cases are thought to be secondary to coexisting disorders of the gastrointestinal tract or the respiratory system. The condition has been associated with the therapeutic uses of lactulose, steroids, and various cancer chemotherapeutic regimens. Lactitol is a disaccharide analogue of lactulose which is available as a pure crystalline powder. There are three previous case reports suggestive of lactulose causing pneumatosis intestinalis. We report a case of recurrent pneumatosis cystoides intestinalis associated with benign recurrent pneumoperitoneum developed probably secondary to lactitol therapy.     

Successful management of catastrophic gastrointestinal involvement in polyarteritis nodosa

A patient with polyarteritis nodosa developed necrotizing enterocolitis, as indicated by pneumatosis intestinalis seen on computed tomographic scans of the abdomen. Despite immunosuppressive therapy and concomitant resolution of the intramural and portal venous gas and general clinical improvement, on 2 occasions (between 20 and 30 days later) the patient developed bowel infarctions and perforations that necessitated bowel resection. Leaks developed at anastomotic sites, but were not closed surgically. However, these sites and the lower quadrants of the abdomen were drained, and the patient was given total parenteral nutrition. Over a 2-month period the patient completely recovered from this nearly always fatal gastrointestinal complication of polyarteritis nodosa. The medical, surgical, and radiographic approach we used may be applicable to the management of similar cases in the future.     

Pneumatosis cystoides intestinalis. Presentation as an acute abdomen

It is rare for pneumatosis cystoides intestinalis to give rise to abdominal pain. We report a patient with signs and symptoms of a perforated viscous in whom abdominal exploration was negative, but in whom biopsy of various intraabdominal cystic structures revealed only pneumatosis cystoides intestinalis.     

Pneumatosis cystoides intestinalis: Imaging findings with colonoscopy correlation

We report on a case of pneumatosis cystoides intestinalis in a 32-year-old woman affected by coeliac disease with stool cultures positive for salmonella. After plain film of the abdomen, patient underwent computed tomography and magnetic resonance colonography studies that demonstrated numerous, diffuse bubblelike intramural gas collections into the ascending, transverse and descending colonic wall. CT and MR findings were correlated with colonoscopy.     

Pneumatosis intestinalis in a patient with polyarteritis nodosa

Necrotizing enterocolitis of the small bowel in polyarteritis nodosa is rare. In the proper clinical setting, pneumatosis intestinalis and mesenteric air are characteristic of this entity.     

Pneumatosis Cystoides Intestinalis

A case of pneumatosis cystoides intestinalis following administration of chemotherapy for Hodgkin s disease is presented. Because of a poor response to nonoperative management, a subtotal colectomy was performed.
Although the pathogenesis of pneumatosis cystoides intestinalis is not entirely clear, it seems likely that, in this case, mucosal ulceration secondary to methyl-gag allowed intraluminal air to dissect into the intestinal wall and surrounding retroperitoneal tissues.
Because pneumatosis cystoides intestinalis is often reversible and self-limited, fecal diversion in lieu of bowel resection is entertained as a possible alternative in the operative management of selected cases. In the case presented resection was performed because of extent of the pathologic process.     

Mucosal pseudolipomatosis: an air pressure-related colonoscopy complication

Mucosal pseudolipomatosis is a recently described colonoscopic finding, most likely caused by intramucosal air. We describe four asymptomatic patients with this lesion which completely resolved 3 weeks later on repeat colonoscopy. This is a benign, transient lesion with distinct endoscopic and histologic findings. The similarity between mucosal pseudolipomatosis and pneumatosis cystoides intestinalis is supportive of the mechanical theory for pneumatosis cystoides intestinalis.     

Endosonographic diagnosis of pneumatosis cystoides intestinalis in infancy

We report pneumatosis cystoides intestinalis in a 10-month-old girl who developed bloody diarrhea following chemotherapy for leukemia. The diagnosis was made only by colonic endoscopic ultrasonography, whereas the abdominal plain radiogram and computed tomography failed to elucidate the diagnosis. She was successfully treated with hyperbaric oxygen therapy. Wider application of endoscopic ultrasonography may lead to the more frequent detection of pneumatosis cystoides intestinalis, currently a rare disorder. Received: 3 May 2002 / Accepted: 20 May 2002 Correspondence to H. Kaneko     

Pneumatosis cystoides intestinalis and benign pneumoperitoneum in a patient with antinuclear antibody negative systemic lupus erythematosus

A patient with antinuclear antibody (ANA) negative systemic lupus erythematosus (SLE) developed pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum. The literature of pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum is reviewed, the benignancy of these conditions is emphasized, and particular attention directed to their association with and implications in the collagen vascular diseases. This is the first reported case of pneumatosis cystoides intestinalis and pneumoperitoneum in a patient with ANA negative SLE.     

Pneumatosis cystoides intestinalis: an unexpected finding in intestinal bleeding under therapy with phenprocoumon

Pneumatosis cystoides intestinalis is a rarely observed disorder on plain abdominal X-ray or colonoscopy examination. Although causing few complaints in adults, it can nevertheless lead to gastrointestinal bleeding. In the reported case, gastrointestinal bleeding was observed with coinciding pneumatosis and phenprocoumon therapy. After stopping anticoagulant therapy, pneumatosis vanished completely, thus suggesting that phenprocoumon is probably a further cause of the cystic disease. In the known list of drugs able to provoke pneumatosis, this case adds a new mechanism of affliction different to either immunosuppression or gas production.     

Surgical Treatment for Pneumatosis Cystoides Intestinalis Complicating Jejunoileal By-pass

A case of chronic pneumatosis cystoides intestinalis, complicating jejunoileal by-pass procedure for morbid obesity, is described. Grave clinical symptoms associated with irreversible segmental mucosal atrophy and mural fibrosis resulting from untreated long-standing pneumatosis intestinalis, required resection of the whole by-passed small intestine. It is suggested that this simple and safe surgical procedure is justified in selected cases. Conservative treatment of asymptomatic patients with pneumatosis intestinalis, however, may prevent further surgical intervention in these patients.     

Pneumatosis cystoides intestinalis in intestinal pseudoobstruction

Summary A 66-year-old man with chronic idiopathic intestinal pseudoobstruction was admitted for pneumatosis cystoides intestinalis, complicated by pneumoperitoneum. The latter conditions resolved after treatment with metronidazole. There was no favorable effect of the prokinetic agents cisapride and erythromycin. To the authors' knowledge, this is the first reported case of successful treatment of pneumatosis cystoides intestinalis with metronidazole in primary chronic intestinal pseudoobstruction.     

Pneumoperitoneum Induced by Pneumatosis Cystoides Intestinalis Associated with Volvulus of the Stomach

A rare association of pneumoperitoneum induced by pneumatosis cystoides intestinalis with chronic mesenteroaxial volvulus of an irreducible stomach in a paraesophageal hiatus hernia is described. The importance of accurate radiologic work-up is emphasized. The possible significance of this association to explain the pathogenesis of pneumatosis cystoides intestinalis is discussed.     

Pneumatosis cystoides intestinalis following lupus enteritis and peritonitis

We describe a case of systemic lupus erythematosus (SLE) with enteritis and peritonitis who later developed pneumatosis cystoides intestinalis (PCI). A 35-year-old woman with SLE relapsed with enteritis and peritonitis. Prednisolone (PSL) effectively improved her symptoms. However, 6 weeks later, she developed PCI. Tapering of PSL, administration of intravenous cyclophosphamide, prokinetic agents and antibiotics, bowel rest with intravenous hypernutrition therapy and hyperbaric oxygen therapy successfully improved PCI. Although PCI is a rare complication of SLE, the present case suggests that lupus enteritis could be a risk factor for PCI, and that high-dose PSL could cause additional insult to PCI.