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Pneumatosis [correction of Pneumocystis] cystoides intestinalis with pneumoperitoneum and pneumoretroperitoneum in a patient with extensive chronic graft-versus-host disease.

Pneumatosis cystoides intestinalis is a rare finding of intramural gasfilled cysts in the bowel wall and sometimes free air in the abdomen. A few conditions are reported to cause this disease, one of them being immunosuppression. We describe a 50-year-old Caucasian male with extensive chronic graft-versus-host disease (GVHD) of the gut and skin who developed PCI with pneumoperitoneum and pneumoretroperitoneum. To our knowledge, this is the first report of PCI occurring in a patient with active chronic GVHD which resolved spontaneously.

Bone Marrow Transplantation↗

Bypass enteropathy. Intestinal and systemic manifestations following small-bowel bypass.

Many manifestations following jejunoileal bypass are due to chronic inflammation of the excluded bowel rather than short bowel malabsorption. Diarrhea, abdominal distention, and gas-fluid levels were common diagnostic features of "bypass enteropathy." Exploration showed the bypassed bowel to be dilated, with serosal inflammation and pneumatosis cystoides intestinalis. The bypassed loops contained a fecal flora and the mucosa demonstrated nonspecific chronic inflammatory changes. Exudative protein losses were noted. Systemic complications of bypass enteropathy were similar to other inflammatory diseases of the bowel. Improvement following treatment with metronidazole or after dismantling of the bypass suggested that bacterial byproducts originating in the excluded bowel were causally related.

Arthritis↗

Benign persistent pneumoperitoneum in scleroderma.

We present a 68-year-old patient who, over a two-year period, was observed to have persistent benign pneumoperitoneum. The free air was present in the abdominal cavity without gastrointestinal perforation or pneumatosis cystoides intestinalis. The persistent pneumoperitoneum was a benign process and was not associated with peritonitis or other ill effects. The source of the free air was not clear. Conceivably, microperforations occurring in colonic diverticula or in distended intestinal wall induced the persistent benign pneumoperitoneum.

Aged↗

Necrotising enterocolitis of the newborn--is it gas-gangrene of the bowel?

Necrotising enterocolitis (N.E.C.) of the newborn is thought to be caused by ischaemia of the bowel. This would favour the conversion of clostridial spores, which can occur very early in the intestinal tract of newborns, to toxin-producing, invading bacilli. The histology of resected gut specimens from 6 of 7 N.E.C. patients who had undergone operation was similar to that in cases of gas-gangrene of the bowel and that in experimentally provoked pneumatosis cystoides intestinalis. In one case Clostridium perfringens type A was cultured in great number by anaerobic technique. The clostridia in these cases may have played an important role in the development of N.E.C.

Clostridium perfringens↗

Small intestinal complications in progressive systemic sclerosis.

A case report of a 33-year-old woman with progressive systemic sclerosis and polymyositis is presented. Admitted with a beta-hemolytic Streptococcus infection of the right knee joint, she had progressive systemic sclerosis with small intestinal involvement, which is rare. This led to more serious complications, malabsorption, pneumoperitoneum, and pneumatosis cystoides intestinalis, which forced a decision to treat her with home total parenteral nutrition. The diagnostic as well as treatment problems encountered in this patient illustrate the importance of nursing care in the overall management of patients with this disease.

Adult↗

Gastrointestinal features of scleroderma.

Gastrointestinal involvement occurs in most patients with systemic sclerosis and is subclinical in about one third. Early pathology is characterized by vasculopathy, resulting in tissue ischemia and progressive dysfunction. Noninvasive esophageal studies using semisolid bolus scintigraphy are sensitive but lack specificity. Long-term treatment of reflux with high-dose proton pump inhibitors appears safe and effective for symptom relief and may prevent recurrence of esophagitis and stricture. Dyspepsia may result from gastroparesis and antral distension. Gastric antral vascular ectasia is a vascular manifestation, and bleeding may be controlled endoscopically. Prokinetic agents effective in pseudoobstruction include metoclopramide, domperidone, cisapride, octreotide, and erythromycin. Patients with intestinal neuropathy or response to bolus octreotide are more probable long-term responders. The combination of octreotide and erythromycin may be particularly effective in systemic sclerosis. The combination of cisapride and erythromycin may cause serious cardiac arrhythmia and is contraindicated. Omeprazole may predispose to small intestinal bacterial overgrowth. Malabsorption not responding to antibiotic therapy should be investigated with small-bowel biopsy to rule out more unusual causes. Pneumatosis cystoides intestinalis may be due to excessive hydrogen production by intestinal bacteria altering the partial pressure of nitrogen in the intestinal wall. In selected cases, surgery for intestinal failure is an option with resection or bypass of affected segments or placement of enterostomy tubes for feeding or decompression. Careful preoperative characterization of intestinal segments is required.

Esophageal Diseases↗

Intestinal Strangulation in Clostridium perfringens-Monocontaminated Rats.

Ischemic and hemorrhagic strangulation of closed intestinal segments was studied in germ-free rats individually contaminated with one of seven separate strains of Clostridium perfringens type A. The principal findings were that (i) the monocontaminated rats with ischemic or hemorrhagic intestinal strangulation died at the same rapid rate; (ii) intraintestinal blood did not augment the action of C. perfringens in the presence of ischemic strangulation; (iii) although some strains were more toxic than others, several food-poisoning strains were as rapidly fatal as the classical strains; and (iv) the massive amounts of gas produced by some of these strains probably led to the early rupture of the closed segments and rapid death of the animals. The secondary findings were that (i) the injection of C. perfringens into the lumen of the distal small intestine of the germ-free rat produced the typical lesions of pneumatosis cystoides intestinalis; and (ii) most of the strains of C. perfringens established themselves throughout the gastrointestinal tract of the unoperated germ-free rat.

Journal Article↗

Methane excretion in man--a study of breath, flatus, and faeces.

In this paper aspects of the variability of methane producing status have been examined, and a survey of breath methane excretion in various clinical and control populations is reported. Prevalences of methane excretion were 54% in healthy controls, 53% in non-gastrointestinal patients and 32% in gastrointestinal patients. Patients with Crohn's disease, ulcerative colitis, and pneumatosis cystoides intestinalis had significantly lower prevalences of methane excretion (13%, 15%, and 11% respectively). Faecal constituents and in vitro incubation analysis were similar in breath methane excretors and non-excretors. Several patients did not excrete methane in the breath although methane was present in colonic gas. The results indicate that different gastrointestinal patient groups have different prevalences of breath methane excretion and that all healthy subjects may produce methane but only when the production reaches a threshold does it appear in the breath.

Adolescent↗

Pneumoperitoneum, pneumomediastinum and pneumopericardium following dental extraction.

Pneumoperitoneum, pneumomediastinum, pneumopericardium and subcutaneous emphysema developed in a patient following simple dental extraction. Other causes of this unusual complication, such as pneumatosis cystoides intestinalis, insufflation of fallopian tubes, pulmonary-peritoneal fistula, post-partum knee-chest exercises, laparotomy, paracentesis and peritoneal dialysis should be considered when peritoneal signs are absent so that unnecessary laparotomy can be avoided.

Adipose Tissue↗

[Collagen diseases with gastrointestinal manifestations].

Collagen vascular diseases are known to present with a diverse array of gastrointestinal manifestations. These can be classified as: 1) gastrointestinal damage due to the collagen vascular disease itself; 2) adverse events caused by pharmacotherapies; or 3) gastrointestinal infections following immunosuppression due to corticosteroid (CS) administration. The first group includes lupus enteritis and protein-losing gastroenteropathy in systemic lupus erythematosus (SLE), reflux esophagitis, chronic intestinal pseudo-obstruction, and pneumatosis cystoids intestinalis in systemic sclerosis, amyloidosis in rheumatoid arthritis, bowel ulcer and bleeding in rheumatoid vasculitis and microscopic polyangiitis, and ileocecal ulcer in Behcet disease. In particular, colonic ulcers associated with SLE represent refractory lesions resistant to CS. Analysis of reported cases showing colonic lesions with SLE (22 cases in Japan) revealed that mean duration of SLE was 9.9 years and 77% of colonic lesions were observed in the rectum and sigmoid colon. Half of the patients developed intestinal perforation or penetration, and 6 of the 11 patients with perforation died. The second group includes lesions in the small and large intestine due to nonsteroidal anti-inflammatory drugs (NSAIDs) and CSs, in addition to peptic ulcers. As perforation in CS-treated patients displays relatively high incidence with poor prognosis, careful attention to such complications is needed. The third group includes candidal esophagitis and cytomegalovirus (CMV) enteritis. Prompt diagnosis is required to prevent colonic bleeding and perforation due to CMV.

Adrenal Cortex Hormones↗

[Three cases of Chilaiditi's syndrome--hepatodiaphragmatic interposition of the colon].

Three cases of Chilaiditi's syndrome are reported. Case 1: A 56-year-old woman was admitted with dysphagia. She had been suffering from progressive systemic sclerosis for 16 years. Three years before the admission, dysphagia developed and dilatation and hypomotility of the esophagus were observed. Chest and abdominal x-ray films on admission showed severe dilatation of the intestine, pneumatosis cystoides intestinalis, abdominal free air, and Chilaiditi's syndrome. Chilaiditi's syndrome and other signs disappeared after conservative treatment. She died four months later due to cor pulmonale. Case 2: An 87-year-old man was admitted with constipation and left lower abdominal pain. Physical examination showed ascites. Chest and abdominal x-ray examination showed Chilaiditi's syndrome. Cytological examination of ascites revealed adenocarcinoma cells. Diagnosis of peritonitis carcinomatosa due to cancer of pancreatic tail was made. Chilaiditi's syndrome disappeared after removal of ascites. Case 3: A 71-year-old bedridden man who had urinary incontinence developed meterorism. Repeated chest x-ray examinations constantly showed Chilaiditi's syndrome. He died of pneumonia two years later. The pathogenesis of Chilaiditi's syndrome was discussed and the literature was reviewed.

Abdominal Pain↗

Role of endoscopic miniprobe ultrasonography in diagnosis of submucosal tumor of large intestine.

AIM: To evaluate the role of miniprobe ultrasonography under colonoscope in the diagnosis of submucosal tumor of the large intestine, and to determine its imaging characteristics. METHODS: Thirty-five patients with submucosal tumors of the large intestine underwent miniprobe ultrasonography under colonoscope. The diagnostic results of miniprobe ultrasonography were compared with pathological findings of specimens by biopsy and surgical resection. RESULTS: Lipomas were visualized as hyperechoic homogeneous masses located in the submucosa with a distinct border. Leiomyomas were visualized as hypoechoic homogeneous mass originated from the muscularis propria. Leiomyosarcomas were shown with inhomogeneous echo and irregular border. Carcinoids were presented as submucosal hypoechoic masses with homogenous echo and distinct border. Lymphangiomas were shown as submocosal hypoechoic masses with cystic septal structures. Malignant lymphomas displayed as hypoechoic masses from mucosa to muscularis propria, while pneumatosis cystoids intestinalis originated from submucosa with a special sonic shadow. One large leiomyoma was misdiagnosed as leiomyosarcoma. CONCLUSION: Endoscopic miniprobe ultrasonography can provide precise information about the size, layer of origin, border of submucosal tumor of the large intestine and has a high accuracy in the diagnosis of submucosal tumor of the large intestine. Pre-operative miniprobe ultrasonography under colonoscope may play an important role in the choice of therapy for submucosal tumor of the large intestine.

Adult↗

[Five cases of systemic sclerosis with associated with intestinal pseudo-obstruction].

OBJECTIVES: We experienced five cases of systemic sclerosis (SSc) associated with pseudo-obstruction, and evaluated their clinical characteristics and outcome. METHODS: Five patients with SSc were found to have intestinal pseudo-obstruction. We studied the clinical characteristics, treatments and outcome of these five case from medical records. RESULTS: The intestinal symptoms were free air in three and pneumatosis cystoides intestinalis (PCI) in two of the five patients. Cisapride was administered to all patients and octreotide and dinoprost to three patients, but the effects were not adequate and intravenous hyperalimentation (IVH) had to be introduced in four patients. Drug treatment was effective in patients in the early stage of intestinal symptoms, but IVH had to be introduced in patients with a long course. Septicemia occurred in two of the four patients on IVH and an infection occurred at the catheter insertion site in one patient. These two patients died of septicemia. Of five patients we experienced with SSc associated with intestinal pseudo-obstruction, two had PCI and four required IVH at home. Two of the four on home IVH developed septicemia. CONCLUSIONS: Intestinal pseudo-obstruction is one of the poor prognostic factors for SSc.

Aged↗

[Enteritis cystica profunda in a patient with Crohn disease].

We describe a case report of a 58-year-old woman presenting with acute intestinal obstruction in association with Crohn's disease. The terminal ileum was resected and was found to be heavily thickened and stenotic, with multiple, mucinous cysts in the bowel wall. Pathological findings were otherwise consistent with Crohn's disease. Enteritis cystica profunda was diagnosed. Diagnosis and differential diagnoses (mucin producing adenocarcinoma and pneumatosis cystoides intestinalis) are discussed.

Crohn Disease↗

[Radiologic pneumoperitoneum without perforation of a hollow viscus].

About 10% of the radiological pneumoperitoneums occur without hollow viscus perforation. Pseudopneumoperitoneum is defined when the subphrenic lucency does not correspond to free intraperitoneal air: subphrenic fat pad, linear lung atelectasis, abnormal subphrenic shape, Chilaïditi syndrome or subphrenic abscess. True pneumoperitoneum without hollow viscus perforation may result from diffusion of thorax-derived air through a phrenic defect or along sheaths of mediastinal blood vessels. The female genital tract represents another route for intraperitoneal air penetration. Other etiologies include iatrogenic pneumoperitoneum (after abdominal surgery and digestive endoscopy) and pneumatosis cystoides intestinalis, when the subserous intraparietal gaseous bubbles rupture into the peritoneal cavity.

Diagnosis, Differential↗

[Pneumoperitoneum without perforation of the digestive tract].

The authors present an account on 9 patients who were detected in their department during the nine-year period from 1991-2000 with the finding of pneumoperitoneum where perforation of the digestive tract was not proved. In 4 of them (44%) conservative treatment was used, in the remaining 5 patients (56%) the condition called for surgical revision. The latter was made as an acute operation in four patients because of signs of peritoneal irritation, in one only after 4 days because of suspected tumours intestinal disease. As to the site of the assumed source of the non-perforation pneumoperitoneum we included 6 of them into the group abdominal area, (1x pneumatosis cystoides intestinalis, 1x aerogenic infection from a perforated abscess of the right liver lobe, 2x microtraumas of the wall of the cardio-esophageal transition with massive vomiting, 1x microtraumas of the lesser curvature of the stomach during extreme distension of the stomach after incorrect intubation and resuscitation, 1x passage of gas through the inflamed distended colonic wall in colitis), two patients into the thoracic area (1x in conjunction with pneumothorax, 1x with hydrothorax), 1 patient in the urogenital area (by the gynaecological route after repeated coitus and orogenital practices). The authors summarize on the basis of data in the literature and their own experience diagnostic and therapeutic possibilities of this relatively rare finding.

Adult↗