Successful treatment with hyperbaric oxygen therapy for pneumatosis cystoides intestinalis as a complication of granulomatosis with polyangiitis: a case report.

BACKGROUND: Although gastrointestinal involvement in patients with granulomatosis with polyangiitis is uncommon, it is associated with mild to severe life-threatening complications. We present a case of pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis that was treated successfully with hyperbaric oxygen. CASE PRESENTATION: A 70-year-old Japanese man with a 3-year history of granulomatosis with polyangiitis consulted our hospital with a complaint of severe back pain. Computed tomography showed a large amount of gas located in his bowel wall and mesentery. He underwent urgent exploratory laparotomy, which led to a diagnosis of pneumatosis cystoides intestinalis without intestinal perforation or necrosis. He consequently underwent 13 sessions of hyperbaric oxygen therapy and was discharged from our hospital without complications. CONCLUSIONS: Several previous reports have supported the efficacy of hyperbaric oxygen for treating pneumatosis cystoides intestinalis. The present case, however, is the first in which pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis was successfully treated with hyperbaric oxygen. We therefore suggest that hyperbaric oxygen therapy could be a candidate treatment for pneumatosis cystoides intestinalis in patients with granulomatosis with polyangiitis.

Pneumatosis cystoides intestinalis in scleroderma-related conditions.

AIMS: Pneumatosis cystoides intestinalis (PCI) is a rare life-threatening gastrointestinal complication in the course of connective tissue disease (CTD). PCI is characterised by the appearance of intramural clusters of gas in the small and large bowel wall on X-ray or computed tomography and often is accompanied by free air in the peritoneal cavity. METHODS: We present three cases of PCI in patients with scleroderma-related conditions. A review of the English language literature published on MEDLINE from 1973 to 2008 was conducted using the terms: 'systemic sclerosis', 'connective tissue disease' and 'pneumatosis cystoides intestinalis'. This review focused on clinical features, diagnostic and treatment strategies of PCI in the context of CTD. RESULTS: Symptoms of PCI are non-specific: abdominal pain, vomiting, constipation, bloating and weight loss. Coexistence of PCI with other manifestations of CTD, such as intestinal pseudo-obstruction and/or bacterial overgrowth, complicates the clinical diagnosis. Treatment approach to PCI is mostly conservative: intestinal 'rest', parenteral nutrition, antibiotics, fluids and electrolyte supplementation, and inhaled oxygen. Surgical intervention should be performed only in cases of bowel perforation, ischaemia or necrosis. Patients with PCI have high mortality rates due to PCI itself but also to the severity and variety of basic CTD complications. CONCLUSION: Recognition of PCI, particularly in the context of underlying CTD, is necessary for proper therapeutic application. In patients with underlying CTD and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including rheumatologist, gastroenterologist, imaging specialist and surgeons familiar with intestinal complications of CTD-related conditions, is warranted.

Three cases of pneumatosis intestinalis presenting in autoimmune diseases.

Pneumatosis intestinalis (PI) is a comparatively rare disease characterized by the presence of intramural gas in the gastrointestinal tract. PI is known to be associated with several clinical conditions, such as pulmonary diseases, gastrointestinal diseases, and traumatic injury, as well as autoimmune disorders. In particular, PI is commonly seen in systemic sclerosis (SSc) but rarely in systemic lupus erythematosus and dermatomyositis (DM). In this report, we present three cases of PI presenting in autoimmune diseases, including DM, Sjögren's syndrome, and limited SSc, and further discuss its background characteristics.

Pneumatosis cystoides intestinalis following alpha-glucosidase inhibitor treatment: a case report and review of the literature.

A 69-year-old man was diagnosed as having myasthenia gravis (MG) in September 2004, and treated with thymectomy and prednisolone. He was then diagnosed as having steroid-induced diabetes mellitus, and received sulfonylurea (SU) therapy in May 2005. An alpha-glucosidase inhibitor (alphaGI) was added in March 2006, resulting in good glycemic control. He experienced symptoms of abdominal distention, increased flatus, and constipation in October 2007, and was admitted into our hospital in late November with hematochezia. Plain abdominal radiography revealed small linear radiolucent clusters in the wall of the colon. Computed tomography (CT) showed intramural air in the sigmoid colon. Colonoscopy revealed multiple smooth surfaced hemispherical protrusions in the sigmoid colon. The diagnosis of pneumatosis cystoides intestinalis (PCI) was made on the basis of these findings. As the alphaGI voglibose was suspected as the cause of this patient's PCI, treatment was conservative, ceasing voglibose, with fasting and fluid supplementation. The patient progressed well, and was discharged 2 wk later. Recently, several reports of PCI associated with alphaGI therapy have been published, predominantly in Japan where alphaGIs are commonly used. If the use of alphaGIs becomes more widespread, we can expect more reports of this condition on a global scale. The possibility of PCI should be considered in diabetic patients complaining of gastrointestinal symptoms, and the gastrointestinal tract should be thoroughly investigated in these patients.

Evaluation of submucosal lesions of the large intestine: part 2. Nonneoplastic causes.

Various nonneoplastic entities may manifest as submucosal abnormalities at colorectal evaluation, and it may be difficult to distinguish between those with an intramural origin and those with an extramural origin on the basis of optical colonoscopy alone. Cross-sectional radiologic imaging, which allows evaluation of the entire bowel wall and the surrounding tissues, plays an important role in the localization and characterization of these abnormalities. However, some superficial submucosal lesions that are initially detected at computed tomographic colonography or barium enema studies may be better characterized with colonoscopy; thus, it is important to recognize the complementary uses of these diagnostic tests. In addition, modalities such as transrectal ultrasonography and magnetic resonance imaging may be useful for the identification and characterization of some abnormalities. For timely and effective management, it is especially important that submucosal neoplasms of the large intestine be accurately distinguished from nonneoplastic entities such as lymphoid polyps, vascular lesions, and cystic lesions, as well as from extracolonic abnormalities (eg, endometriosis, uterine fibroids) and normal extracolonic structures (eg, uterus, vasculature).

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.

Pneumatosis cystoides intestinalis treated with hyperbaric oxygen therapy: usefulness of an endoscopic ultrasonic catheter probe for diagnosis.

A 67-year-old woman was admitted to our hospital with a complaint of abdominal pain. Barium enema examination and colonoscopy showed numerous round polypoid lesions covered with normal mucosa in the area from the ascending colon to the splenic flexure. Endoscopic ultrasound examination with an ultrasonic catheter probe revealed a strong echo with distal acoustic shadowing in the third layer of the diseased colonic wall, which suggested the presence of gas in the submucosa. The gaseous cysts disappeared completely after hyberbaric oxygen therapy at 2 to 3 atmospheres absolute (60 minutes, twice a day) for 30 consecutive days.

Case report: Pneumatosis cystoides intestinalis associated with post-surgical bowel anastomosis: a report of three cases and review of the Japanese literature.

We report three cases of pneumatosis cystoides intestinalis (PCI) occurring in association with post-surgical bowel anastomosis. A 74-year-old man, a 58-year-old woman, and a 62-year-old woman were found to have PCI at the colonic side of a bowel anastomosis at 4 years, 3 years and 1 year after operation, respectively, for right colon carcinoma, although all were asymptomatic. They all had a positive anti-nuclear antibody test and had received postoperative cancer chemotherapy. The clinical features of 123 cases of PCI reported in Japan between 1981 and 1995 were also reviewed. On the basis of the present and previous cases, we propose that post-surgical anastomosis, cancer chemotherapy, and predisposition to collagen vascular disease might be responsible for the damage to intestinal mucosa that leads to the development of PCI.

[Pneumatosis cystoides intestinalis. Diagnostic elements and therapeutic approach]. / La pneumatose kystique intestinale. Eléments diagnostiques et attitudes thérapeutiques.

Pneumatosis cystoides intestinalis is an uncommon condition characterised by multiple gas-filled cysts within the small intestine or colonic wall. Clinical manifestations are unspecific and often found in many other abdominal diseases. To avoid unnecessary laparotomy, radiologic and endoscopic findings are essential to be known. The present case associates symptoms highly suspect of neoplasia like weight loss, rectal mass, bloody stools and tenesmus. Treatment of choice is medical. In the absence of an acute abdomen, surgery is only reserved when it is not responsive to medical treatment.

[Pneumatosis cystoides of the colon. Apropos of 7 cases]. / Pneumatose kystique du côlon. A propos de 7 observations.

Seven cases of pneumatosis cystoides intestinalis affecting the colon (PKC) revealed 5 times by a nonspecific symtomatology and 2 times by an occlusion were reported. In three of the patients the disease was found as a primary idiopathic form free of clinical antecedents. In 4 of the patients the pneumatosis was found to be secondary to a pulmonary disease, a gastric ulcer, a connectivite of a corticotherapy. The PKC was generally diagnosed either by barium enema or by computed tomography and less frequently by colonoscopy with deep biopsy allowing differential diagnosis with colonic polyposis. The mechanism and etiology of the PKC were not fully understood. The illness is a benign condition that often responds to a conservative management--i.e. abstention, oxygenotherapy, diet or antibiotherapy- or exceptionally to surgical colonic resection in case of acute complication.

[Pneumatosis coli vs. deep cystic colitis]. / Pneumatosis coli vs. colitis quística profunda.

We present the case of a 63-year-old female who with no previous history presented with episodes of self-limited diarrhea without fever, general repercussions, abnormal elements or tenesmus. Biochemistry: no abnormality. Parasite and feces culture: negative. Colonoscopy: at 20 to 40 cm from the anal margin were found polypoid lesions covered with normal mucosa. Barium enema confirmed the presence of multiple lacunar defects and possible parietal gas. Biopsy: submucous cysts with foreign body type giant cells. Mixed infiltrate. The differential diagnosis was made between intestinal enterogenous cyst, lymphangioma, retractable mesenteritis and deep cystic colitis, concluding with pneumatosis coli that evolved favorably.

[Cystic pneumatosis affecting the sigmoid. Information provided by colonic mucography (double contrast enema) and endoscopy. One case; diagnostic significance of the "puzzle" appearance (author's transl)]. / Pneumatose kystique à localisation sigmoïdienne. Apports respectifs de la mucographie colique (lavement double contraste) et de l'endoscopie. A propos d'un cas. Valeur sémiologique de l'image "en puzzle".

On the basis of one case of colonic sub-mucosal cystic pneumatosis explored by double contrast enema, the authors emphasise the pathognomic diagnostic value of the "puzzle" appearance. This appearance is made by precise adaptation of the opaque colonic mucosa depressed in the form of a "cupola" over the intraparietal gas bubble which results in the deformity. Endoscopy failed to reveal the disorder in the case reported here.