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Pneumatosis cystoides intestinalis. Autopsy study of two fatal cases in adults.

Two cases of pneumatosis cystoides intestinalis (PCI), apparently of different etiologies, were found at autopsy in cancer patients. One case was associated with chronic obstructive lung disease and with emphysema of soft tissues of the mediastinum, retroperitoneum, and mesentery, whereas the other showed pseudomembranous enteritis with bacterial and fungal overgrowths. The latter case supports the role of gas-forming bacteria, while the former provides an anatomical evidence of pulmonary disease as a cause of PCL. Obstruction of the upper gastrointestinal tract may contribute to the occurrence of PCI by a complication of aspiration pneumonitis or disturbance of the normal bacterial flora of the intestine as well as an increase of the intraluminal pressure. In addition to the etiologic considerations, the importance of clinical diagnosis of PCI has been emphasized since both cases in the present report were more or less related to the cause of death.

Aged↗

Development of pneumatosis cystoides intestinalis following transperitoneal renal transplantation in a child.

A 9 1/2-year-old female developed pneumatosis cystoides intestinalis (PCI) which was detected radiographically 4 1/2 months after transperitoneal cadaveric renal transplantation, during a period characterized by recurrent episodes of acute rejection. Radiographic evaluation was prompted by the development of cramping abdominal pain, distention, and tenderness localized to the region of the allograft, which occurred during one such episode. Pneumatosis was localized primarily to an area of colon that lay in direct contact with the allograft. Evaluation of the available clinical and roentgenographic evidence suggested that pneumatosis may have resulted from the development of a sympathetic inflammatory reaction within the bowel wall adjacent to the acutely inflamed allograft. Subsequent stabilization of renal function was associated with resolution of the pneumatosis over the ensuing 8 months without surgical intervention or additional medical therapy.

Child↗

Pneumatosis cystoides intestinalis in intestinal pseudoobstruction. Resolution after therapy with metronidazole.

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.

Aged↗

[Pneumatosis cystoides intestinalis].

Patients with free intraperitoneal air usually undergo emergency surgery. Some of these patients will have no identifiable perforation, for instance those with pneumatosis cystoides intestinalis. This is a rare condition characterized by multiple intramural gas cysts in the gastrointestinal tract. The most common symptoms are meteorism, excessive flatulence, diarrhoea, abdominal pain, passage of mucus per rectum, or rectal bleeding. A case of pneumatosis cystoides intestinalsis is described. Plain abdominal radiographs showed distended bowel with free intraperitoneal air and intramural gas collections. At laparotomy, multiple intramural cysts were found, but no perforation or obstruction. The symptoms resolved after laparotomy, and the patient was discharged after a few days. The aetiology and pathogenesis of pneumatosis cystoides intestinalis are unknown, although deficient hydrogen metabolism and gasforming bacteria that penetrate the mucosal barrier may be involved. If needed, hyperbaric oxygen therapy is the treatment of choice. Surgery is indicated only in fulminant cases.

Aged↗

Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis: treatment with high dose prednisone.

Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynaud's phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.

Adult↗

[Pneumatosis cystoides intestinalis following steroid treatment in a nephrotic syndrome patient: report of a case].

Pneumatosis cystoides intestinalis (PCI) is a relatively rare, benign condition characterized by multiple subserosal or submucosal gas-filled cysts in the bowel wall. The cause and incidence of PCI are uncertain, but the condition is most commonly diagnosed in patients who have chronic obstructive pulmonary disease, gastrointestinal disease (e.g. Crohn's disease, peptic ulcer disease) or collagen disease (e.g. scleroderma, systemic lupus erythematosus). The report of PCI associated with nephrotic syndrome has not be known as far as we have referred. We first experienced a case of PCI with nephrotic syndrome. The patient was a 28-year-old female who had developed nephrotic syndrome in 1977. Although she had been treated by steroid since the onset of the nephrotic syndrome, she was a frequent relapser. She was hospitalized to our hospital on November 1988, due to fourth relapse of the disease. The increasing dosage of steroid (60mg/day) improved general edema and decreased urinary protein, but abdominal pain and fullness occurred seven weeks after the admission. The abdominal radiographs showed air accumulations in the wall of the intestine (probably right sided colon) and retroperitoneum. That finding was confirmed by Barium enema and abdominal computed tomography. We diagnosed the lesions as PCI from the above findings, and high flow oxygen and hyperbaric oxygen therapy improved the symptom of PCI. The etiology of PCI in this case was thought to be mainly a long term steroid treatment.

Adult↗

[Systemic sclerosis with various gastrointestinal problems including pneumoperitoneum, pneumatosis cystoides intestinalis and malabsorption syndrome].

We describe here an experience of successful treatment of systemic sclerosis (SSc) complicated with various gastrointestinal (GI) problems including pneumoperitoneum, pneumatosis cystoides intestinalis and malabsorption syndrome. A 35-year-old female had developed selerodactyly since February, 1990. She had been treated under the diagnosis of SSc at other hospital. She had required several hospitalizations because of nausea, vomitting and abdominal distension, but her GI symptoms had gradually deteriorated. In April 1993, she was referred to our hospital and admitted for the treatment of her GI problems. On admission, she had systemic cutaneous sclerosis and marked abdominal distension without peritoneal signs was recognized. Chest and abdominal roentgenograms demonstrated massive free air under the diaphragm, marked dilation of small and large bowels, and multiple intestinal cysts (pneumatosis cystoides intestinalis ; PCI). We treated her GI problems with various modalities combined with medications, oxygen breathing, intravenous hyperalimentation and hyperbaric oxygen therapy. Pneumoperitoneum and PCI had disappeared after 8 courses of hyperbaric oxygen therapy and her GI symptoms had been well controled by intravenous hyper alimentation. Thereafter, she has been on intermittent parenteral nutrition through subcutaneous port inplantation. During the courses of this treatment, she developed an episode of Wernicke-Kolsakoff (W-K) syndrome which was considered to associate with malabsorption syndrome. The W-K syndrome had recovered by intravenous administration of vitamin B1.

Adult↗

Pneumatosis cystoides intestinalis coli.

The rare case of a 63-year-old male diagnosed with pneumatosis cystoides intestinalis coli is presented and discussed. The patient was found to have an unsymptomatic pneumoperitoneum on plain chest x-ray. The results of a contrast enema, computed tomography scan, and laparoscopy are presented. The patient had an uneventful hospital course without any specific therapy. Causes and possible therapeutic options are discussed.

Diagnosis, Differential↗

[A case of systemic sclerosis complicated with pneumoperitoneum and pneumatosis cystoides intestinalis].

Here we report a case of systemic sclerosis (SSc) complicated with pneumoperitoneum and pneumatosis cystoides intestinalis (PCI). A 58-year-old woman who had been treated with oxygen therapy for chronic respiratory failure caused by interstitial pneumonia was admitted to Tokyo Metropolitan Ohtsuka Hospital because of abdominal fullness. On admission, laboratory data showed accelerated erythrocyte sedimentation rate, mild anemia, hypoalbuminemia, and elevated level of lactose dehydrogenase (LDH). Anti-nuclear antibodies, anti-RNP antibodies, anti-Topoisomerase I antibodies, anti-Ku antibodies, and rheumatoid factor were positive. Chest and abdominal roentgenograms showed free air under the diaphragm, dilatation of small intestine and colon, and multiple intestinal cysts. The diagnosis of pneumoperitoneum and PCI was made with laboratory findings and radiographic findings. Her symptoms did not respond to medications altering colonic motility and oxygen therapy. Finally, abdominal symptoms and radiographic findings caused by pneumoperitoneum and PCI disappeared three weeks after treatment with 1,500 mg/day of kanamycin sulfate. PCI is rarely associated with SSc. Possible pathogenic relationship between these diseases was discussed.

Female↗

Pneumatosis cystoides intestinalis in children beyond the first year of life: manifestations and management.

Beyond infancy, pneumatosis cystoides intestinalis (PCI) is rare. Data concerning pathogenesis and treatment are limited. Our experience with 12 children was examined to define predisposing factors, presentation, treatment, and outcome. Nine children were immunosuppressed, thus identifying an important etiologic subgroup. Presentation was variable but included abdominal pain, distention, diarrhea and hematochezia. Clostridium difficile was found in 3 patients and cytomegalovirus in 1. Radiographs showed free air in 3. Nine were treated with antibiotics and bowel rest, 1 with bowel rest alone, 1 with oral metronidazole, and 1 with observation. PCI resolved in 7 of 9 treated with antibiotics, although 1 child with leukemia had severe hematochezia secondary to colonic ulceration and required hemicolectomy. No other patient required laparotomy. The free air resolved in 2 of 3. There were 2 deaths, both from sepsis. One had free air on admission but no perforation was found at autopsy. Treatment recommendations remain unclear; however, C difficile and cytomegalovirus are important pathogens that should be identified and treated promptly. In symptomatic patients, bowel rest and antibiotics seem beneficial. Operative intervention should be reserved for patients with peritoneal signs, progressive deterioration, obstruction, or persistent, severe bleeding. Free air alone is not an indication for operative management in children with PCI.

Adolescent↗

Pneumatosis cystoides intestinalis: an unusual complication of jejunoileal bypass.

A case of pneumatosis cytoides intestinalis associated with jejunoileal bypass is reported. The association of increased colonic gas, secondary to fermentation of undigested lactose in the colon, and the reflux of the excessive gas into the excluded segment of small bowel and dissection of the gas into the bowel wall and mesentery are postulated as the causes of pneumatosis cystoides intestinalis in patients with jejunoileal bypass.

Colon, Sigmoid↗

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.

Journal Article↗

Pneumatosis cystoides intestinalis in a patient with polymyositis.

A 29-year-old Japanese woman with polymyositis (PM) developed pneumatosis cystoides intestinalis (PCI). The patient experienced a gradual onset of mild epigastric abdominal pain and abdominal distension. Radiographs revealed pneumatosis intestinalis involving the small bowel, and colonoscopy revealed submucosal cysts. Treatment with antibiotics and high-flow oxygen resulted in remission. This is an extremely rare case of PCI in a patient with PM.

Adult↗