Primary Pneumatosis Cystoides Intestinalis Research Articles

Overview of occupational diseases induced by trichloroethylene and associated basic research

To provide an overview of the pathogenesis of pneumatosis cystoides intestinalis (PCI) and hypersensitivity syndrome (HS) caused by trichloroethylene (TCE) and the basic research into their toxicity. We reviewed previously published research articles. PCI clustered in Japan in the 1980s is a rare disease characterized by cyst-like distention of gas in the intestinal wall, which can be secondary or primary. No TCE users were found in the former group, whereas approximately 71% of the latter group were TCE users, suggesting the involvement of TCE exposure in primary PCI. However, the pathogenesis was unclear. TCE is metabolized by the drug-metabolizing enzyme CYP2E1, and intermediate immunocomplexes with CYP2E1 may be involved in hepatotoxicity. HS clustered in the southern part of China since early 2000 is a systemic skin-liver disorder involving anti-CYP2E1 autoantibodies and HLA-B*13:01 polymorphisms, with elevated cytokines and reactivation of Human Herpesvirus 6. PCI and HS, occupational diseases caused by TCE, were clustered in Japan and southern China, respectively. HS was mediated by immune system disorders and genetic polymorphisms, whereas their relevance to PCI occurrence remained unknown.

1642 Pelvic Congestion Syndrome: An Unusual Manifestation of Sigmoid Pneumatosis Cystoides Intestinalis

INTRODUCTION: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal subserosa and submucosa. We present a rare case of primary PCI affecting sigmoid colon complicated by pelvic congestion syndrome (PCS). CASE DESCRIPTION/METHODS: A 34-year-old morbidly obese female presented with diffuse lower abdominal pain and intermittent foamy diarrhea without blood or mucus for the past 5 months. CT abdomen showed small bubbles of gas in the peritoneum in the left upper quadrant and large amount of cystic pneumatosis in a loop of sigmoid colon. Lab work was unremarkable. She was given one week course of antibiotics. A repeat CT scan 5 months later showed interval resolution of free intraperitoneal air but pneumatosis bubbles had enlarged in size. A colonoscopy was performed which showed large cystic-appearing air-filled bullae in sigmoid colon causing some luminal narrowing and the scope could not be safely advanced beyond mid-transverse colon. Patient underwent laparoscopic sigmoid colectomy and intraoperatively noted to have dilated and tortuous bilateral gonadal veins. Surgical pathology showed multiple clear fluid-filled cysts throughout the length of the specimen with viable and unremarkable proximal and distal resection margins. Patient recovered well from surgery and was discharged with no further complaints. DISCUSSION: PCI is an unusual condition caused by a myriad of underlying pathophysiological processes that range from benign to life-threatening conditions. Primary PCI is rare (15%), while underlying conditions for secondary PCI can be divided into several groups; traumatic, mechanical, inflammatory, autoimmune, infectious, pulmonary and drug (steroids and chemotherapeutic agents) induced. Conservative management is preferable except in cases of clinical deterioration. This case is unique because even after the resolution of free intraperitoneal air the patient continued to have lower abdominal and pelvic pain with worsening of pneumatosis on repeat CT scan several months later. It is possible that sigmoid anatomic changes due to pneumatosis resulted in chronic intermittent venous obstruction of pelvic veins leading to gonadal veins dilation and tortuosity and hence secondary PCS which resulted in ongoing lower abdominal pain. This case highlights the fact that persistent PCI, with ongoing abdominal symptoms even in the absence of any obvious etiology warrants laparoscopic exploration to rule out any underlying disease process.

Prolonged postoperative ileus in a patient with primary pneumatosis cystoides intestinalis: a case report

BackgroundPneumatosis cystoides intestinalis (PCI) is a rare disease characterized by multiple gas-filled cysts in the intestinal wall and is associated with various comorbidities. We report herein a case of intractable paralytic ileus caused by primary PCI.Case presentationA 73-year-old man visited out hospital complaining of abdominal pain and vomiting. He had been hospitalized twice for intestinal obstruction in the past 2 months. Based on his history of appendectomy, mechanical bowel obstruction caused by adhesion was diagnosed, and the patient underwent surgery. However, laparotomy revealed small bowel dilatation despite the absence of obstruction or stenosis. Multiple nodules were found in the wall of the dilated bowel loops. The dilated jejunum was excised. Histological examination revealed that the nodules were small gas-filled cysts, suggesting PCI. We made a diagnosis of ileus with underlying PCI and managed the patient conservatively. A large amount of nasogastric tube drainage continued for a long period postoperatively. The patient underwent relaparotomy 35 days after the first operation. The upper jejunum was markedly dilated, although no mechanical stenosis was found. The atonic, dilated jejunum was excised and the ileal stump was anastomosed to the duodenum in a double tract fashion. The patient underwent hyperbaric oxygen therapy because the ileus persisted postoperatively. His condition gradually improved and he was discharged 53 days after the second operation.ConclusionsNon-operative treatment is recommended for primary PCI of unknown etiology. Surgeons should be mindful of the possibility of primary PCI when considering surgical intervention for patients with bowel obstruction.

A report of 12 cases of pneumatosis cystoides intestinalis

Objective To investigate the characteristics and treatment for pneumatosis cystoides intestinalis (PCI). Methods Data of 12 PCI patients admitted to General Hospital of Tianjin Medical University from 1997 to 2015 were analyzed. The causes, endoscopic characteristics, therapeutic effects and prognosis were evaluated. Results Four cases were primary PCI due to long-term exposure to trichloroethylene or unknown reasons, while 8 other cases were secondary to Behcet′s disease, connective tissue disease, emphysema, diabetes, therioma, etc. Endoscopic findings included bubble-like, linear, cobblestone-like, graped or beaded gas cysts, involving sigmoid in 6 cases, ascending colon in 3, descending colon in 2, duodenum in 2 and rectum in 1. After combination of medicinal and endoscopic treatment, symptoms of 8 patients were relieved, and the condition of 2 patients improved, while 2 patients died of malignancy. Conclusion Pneumatosis intestinalis is a rare disease, usually secondary to other diseases, which can be diagnosed by colonoscopy and ultrasonic endoscopy. Pathogenesis of PCI is still unclear. Single PCI can be cured with endoscopic resection. Multiple PCI can be managed with cyst clipping, antibiotics and intestinal flora regulator. Treatment of the original disease and early detection of complications can improve the prognosis and reduce recurrence of PCI. Key words: Endoscopy; Therapeutic endoscopy; Pneumatosis cystoides intestinalis

Primary pneumatosis cystoides intestinalis (with video)

Primary pneumatosis cystoides intestinalis (with video)

A Case of Pneumatosis Cystoides Intestinalis and Portal Venous Gas Accompanied by Emphysematous Pyelonephritis in Type 2 Diabetes Mellitus

Diabetes mellitus is a major risk factor for urinary tract infection (UTI); emphysematous pyelonephritis (EP), a complication of UTIs, often occurs in patients with underlying, poorly controlled diabetes mellitus. We report the case of an 87-year-old woman with EP in type 2 diabetes mellitus who developed pneumatosis cystoides intestinalis (PCI) with portal venous gas. PCI is a radiographic finding, which is found in a linear or cystic form of gas in the submucosa or subserosa of the bowel wall. PCI has two common presentations. Primary PCI is a benign idiopathic condition. Secondary PCI is associated with a wide variety of gastrointestinal and non- gastrointestinal diseases. PCI with portal venous gas in particular is associated with ischemic gastrointestinal disease. Initial pre-enhanced abdominopelvic computed tomography showed EP in the right kidney without PCI. Newly occurring PCI and hepatic portal venous gas were found in the right ascending colon after EP improved. This is a rare case of PCI accompanied by emphysematous pyelonephritis in type 2 diabetes mellitus. The patient's general condition improved with intravenous antibiotics and fluid therapy without a surgical approach. However, she was discharged without further treatment because the family refused any further evaluations and treatments. (J Korean Diabetes 2013;15:45-50) Abstract 가천대학교 의과대학 내과 유승희, 엄영실, 이동민, 이시훈, 김연선, 이기영, 김병준, 김광원, 박이병

Pneumatosis cystoides intestinalis: A single center experience

To share our experience of the management and outcomes of patients with pneumatosis cystoides intestinalis (PCI). The charts of seven patients who underwent surgery for PCI between 2001 and 2009 were reviewed retrospectively. Clinical features, diagnoses and surgical interventions of patients with PCI are discussed. Seven patients with PCI (3 males, 4 females; mean age, 50 ± 16.1 years; range, 29-74 years) were analyzed. In three of the patients, abdominal pain was the only complaint, whereas additional vomiting and/or constipation occurred in four. Leukocytosis was detected in four patients, whereas it was within normal limits in three. Subdiaphragmatic free air was observed radiologically in four patients but not in three. Six of the patients underwent an applied laparotomy, whereas one underwent an applied explorative laparoscopy. PCI localized to the small intestine only was detected in four patients, whereas it was localized to the small intestine and the colon in three. Three patients underwent a partial small intestine resection and four did not after PCI was diagnosed. Five patients were diagnosed with secondary PCI and two with primary PCI when the surgical findings and medical history were assessed together. Gastric atony developed in one case only, as a complication during a postoperative follow-up of 5-14 d. Although rare, PCI should be considered in the differential diagnosis of acute abdomen. Diagnostic laparoscopy and preoperative radiological tests, including computed tomography, play an important role in confirming the diagnosis.

A Case of Primary Pneumatosis Cystoides Intestinalis in a Patient with Chronic Abdominal Pain

A Case of Primary Pneumatosis Cystoides Intestinalis in a Patient with Chronic Abdominal Pain

Unusual Case of Intestinal Obstruction Due to Primary Pneumatosis Cystoides Intestinalis

Purpose: Learning Objectives: 1) To discuss the presentation, evaluation and management of an unusual case of primary pneumatosis cystoides intestinalis (PCI). 2) To differentiate primary from secondary PCI. Case presentation: A 58-year-old male with history of pancreatic divisum, pancreatitis and coronary artery disease presented with abrupt onset of nausea, vomiting and diffuse abdominal pain for 2 days. Past history was significant for multiple episodes of intestinal obstruction over the last 3 years with 2 surgeries for bowel perforation. Physical exam revealed a distended, diffusely tender abdomen with absent bowel sounds, but no peritoneal signs and was suggestive of small bowel obstruction. Abdominal x-ray showed dilated intestine with multiple air fluid levels. CT of the abdomen showed extensive PCI of the intestine from the mid jejunum to the distal ileum with sparing of the colon, dilation of the small intestine, multiple air fluid levels and free intraperitoneal air without any clear transition point to suggest adhesions as the cause of obstruction. Investigation for secondary causes of PCI including complete blood counts, electrolytes, liver function tests, serum amylase and lipase, antibodies for connective tissue diseases, HIV, fecal C. difficile PCR, fecal leukocytes and parasites and imaging of the chest did not reveal an etiology. In the absence of an initiating factor, a diagnosis of primary PCI was made. Surgery was deferred as his symptoms improved and if performed, would have required extensive small bowel resection. Conservative management resulted in resolution of symptoms and the patient was referred for hyperbaric oxygen therapy. Discussion PCI is an uncommon condition characterized by the presence of air filled cysts in the intestinal wall. 85% of reported cases of PCI are secondary to causes such as infection, bowel obstruction, ischemia or infarct. Other associations include chronic obstructive lung disease, immunosuppressive drugs, jejuno-ileal bypass and collagen vascular disease. Thus, a through investigation for secondary causes of PCI is imperative in guiding therapy. Idiopathic primary PCI is usually asymptomatic, but rarely manifests as bowel obstruction due to compression, perforation, volvulus or intussusception. Rupture of subserosal cysts results in pneumoperitoneum without peritonitis. This is not indicative of perforation and does not require surgery. Management of PCI is directed towards the clinical presentation and exclusion of an underlying etiology. Asymptomatic patients with primary PCI do not require therapy. Primary PCI patients with obstruction are reported to be successfully treated with nasogastric decompression and hyperbaric oxygen.

Successful Hyperbaric Oxygen Therapy of Pneumatosis Cystoides Intestinals : A Report of Two Cases

Abstract: Two patients with pneumatosis cystoides intestinalis (PCI) successfully treated with hyperbaric oxygen are described, The first patient was a 52‐year‐old male who presented with bloody stools and was diagnosed as having primary PCI. The second was a 61‐year‐old male whose occupation entailed prolonged exposure to trichloroethylene. Following hyperbaric oxygen therapy, the colonic gas cysts completely disappeared in both patients, and there has been no evidence of recurrence. The relevant literature from 1980 to 1992 is reviewed, and hyperbaric oxygen therapy for PCI is discussed in detail. The data accumulated thus far suggest that hyperbaric oxygen is superior to high‐flow oxygen breathing in PCI therapy.

腸管嚢腫様気腫発生職場の労働衛生学的考察 とくにトリクロロエチレン暴露との関連

Four cases of primary pneumatosis cystoides intestinalis (PCI) were studied in relation to occupational trichloroethylene (TRI) exposure. Three of the patients were exposed to TRI in the process of washing or polishing of camera lenses. The maximum concentration of TRI in the work environment of two PCI patients often exceeded 50 ppm, though the geometric mean concentrations were below this value. Monitoring of TRI exposure by measuring urinary TRI metabolites suggested that the third patient had been working in the environment with an 8-h time weighted average TRI concentration exceeding 50 ppm. Scrutiny of 19 PCI cases thus far collected by us revealed that i) three groups of patients (7 in total) were working in the same factories, ii) most of the PCI patients were workers in small-scale factories having poor working conditions, and iii) occupational TRI exposure is probably responsible for the development of primary PCI.

A new health problem due to trichloroethylene: pneumatosis cystoides intestinalis.

The relationship between pneumatosis cystoides intestinalis (PCI) and occupation was studied in 66 patients reported in Japan during a 5-yr-period from 1979-1983. Information concerning their occupation was obtained from 37 (56.1%) patients; 16 patients had secondary PCI and 21 had primary PCI. No particular characteristics were noted in the occupations of secondary PCI patients. Primary PCI, occurring more frequently in females (15/21), affected predominantly the large intestine (20/21), locally involving the sigmoid colon in the majority (14) of the patients. Most patients with primary PCI (16/21) were factory workers, of whom 15 (71.4%) were engaged in degreasing of manufacturing products with trichloroethylene. The high percentage of trichloroethylene workers among the patients with primary PCI suggests that occupational exposure to this agent constitutes an etiological factor in the development of this disease.

Negative report on trichloroethylene-induced pneumatosis cystoides intestinalis in rats

In recent years it has been suggested in Japan that occupational trichloroethylene exposure will induce primary pneumatosis cystoides intestinalis coli. A series of two experiments were conducted to ascertain the cause-effect relationship between trichloroethylene and pneumatosis as follows: In the first experiment, 12 male rats of Fischer strain were exposed to trichloroethylene at a nearly lethal concentration for 12 wk. Five out of 12 rats died during the experiment, and body weight gain was extremely depressed. However, neither pneumatosis nor histological changes were observed. No changes were observed in 4 control rats as well. In the second experiment, 38 male rats of Sprague-Dawley strain were exposed to 800, 200 or 50 ppm trichloroethylene over a period of 12 wk. Two out of 13 rats of the 800 ppm group died, and body weight gain was suppressed. Mild suppression in body weight gain was also observed in the 200 ppm group. However, pneumatosis was not observed in any rats of trichloroethylene groups. No pathological changes were observed in 13 rats of the control group. The foregoing results suggest that trichloroethylene cannot induce pneumatosis cystoides intestinalis in rats of these two strains. Inasmuch as rats are not prone to develop pneumatosis, the possibility for trichloroethylene to induce this disease in man cannot be denied.