The spectrum of pneumatosis intestinalis in the adult.A surgical dilemma

Pneumatosis intestinalis(PI) is a striking radiological diagnosis. Formerly a rare diagnostic finding, it is becoming more frequently diagnosed due to the wider availability and improvement of computed tomography scan imaging. Once associated only with poor outcome, its clinical and prognostic significance nowadays has to be cross-referenced to the nature of the underlying condition.Multiple mechanisms of pathogenesis have been debated and multiple causes have been detected during the years. All this contributes to creating a broad range of clinical and radiological presentations. The management of patients presenting PI is related to the determining cause if it is identified. Otherwise, in particular if an association with portal venous gas and/or pneumoperitoneum is present, the eventual decision between surgery and non-operative management is challenging,even for stable patients, since this clinical condition is traditionally associated to intestinal ischemia and consequently to pending clinical collapse if not treated.Considering the wide variety of origin and outcomes, PI still remains for surgeons a demanding clinical entity. The manuscript is an updated narrative review and gives some suggestions that may help make the decisional process easier,identifying patients who can benefit from surgical intervention and those who can benefit from non-operative management avoiding unnecessary procedures.

Pneumatosis Cystoides Intestinalis Complicated during Chemotherapy for Pulmonary Nontuberculous Mycobacterial Disease

Background: Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of gas in the intestinal wall. Aim: We report two rare cases of PCI that are complicated during the chemotherapy for pulmonary nontuberculous mycobacterial (NTM) disease. Case Presentation: In this report, we described two cases (a 72-year-old woman and a 60-year-old woman) of PCI that appeared during the combined chemotherapy consisting of rifampicin, ethambutol and clarithromycin. Because there were few clinical symptoms and increased inflammatory responses, the diagnosis of PCI was delayed. However, there were fortunately no severe complications in both cases. Conclusion: Respiratory physicians should be aware of the potential development of PCI in patients during the chemotherapy for pulmonary NTM disease. It is important to detect PCI in the early stage through radiological examinations to avoid severe complications.

Prednisolone induced pneumatosis coli and pneumoperitoneum

Pneumatosis intestinalis(PI)is defined as the presence of gas within the submucosal or subserosal layer of the gastrointestinal tract.It is a radiologic sign suspicious for bowel ischemia,hence non-viable bowel must be ruled out in patients with PI.However,up to 15%of cases with PI are not associated with bowel ischemia or acute abdomen.We described an asymptomatic patient with prednisolone-induced PI and modified the Naranjo score to aid in a surgeon’s decision-making for emergency laparotomy vs non-operative management with serial assessment in patients who are immunocompromised due to long-term steroid use.

A systematic analysis of pneumatosis cystoids intestinalis

AIM:To increase the understanding,diagnosis and treatment of pneumatosis cystoides intestinalis(PCI)and to find the characteristics and potential cause of the disease in China.METHODS:We report here one case of PCI in a 70-year-old male patient who received a variety of treatment methods.Then,we systematically searched the PCI eligible literature published from an available Chinese database from May 2002 to May 2012,including CBM,CBMDisc,CMCC,VIP,Wanfang,and CNKI.The key words were pneumatosis cystoides intestinalis,pneumatosis,pneumatosis intestinalis,pneumatosis coli and mucosal gas.The patients' information,histories,therapies,courses,and outcomes were reviewed.RESULTS:The study group consisted of 239 PCI cases(male:female = 2.4:1)from 77 reported incidents.The mean age was 45.3 ± 15.6 years,and the median illness course was 6 mo.One hundred and sixty patients(66.9%)were in high altitude areas.In addition,43.5%(104/239)of the patients had potential PCI-related disease,and 16.3% had complications with intestinal obstruction and perforation.The most common symptom was abdominal pain(53.9%),followed by diarrhea(53.0%),distention(42.4%),nausea and vomiting(14.3%),bloody stool(12.9%),mucous stool(12.0%)and constipation(7.8%).Most multiple pneumocysts developed in the submucosa of the colon(69.9%).The efficacy of the treatments by combined modalities,surgery,endoscopic treatment,conservative approach,oxygen,and antibiotics were 100%,100%,100%,93.3%,68.3% and 26.3%,respectively.CONCLUSION:PCI can be safely managed by conservative treatments,presents more frequently in males,in the large bowel and submucosa,than in females,in the small intestine and subserosa.High altitude residence maybe associated with the PCI etiology.

Computed tomography findings of pneumatosis and portomesenteric venous gas in acute bowel ischemia

AIM:To use more representative sample size to evaluate whether computed tomography(CT)scan evidence of the concomitant presence of pneumatosis and portomesenteric venous gas is a predictor of transmural bowel necrosis.METHODS:Data from 208 patients who were referred for a diagnosis of bowel ischemia were retrospectively reviewed.Only patients who underwent a surgical intervention following a diagnosis of bowel ischemia who also had a post-operative histological confirmation of such a diagnosis were included.Patients were split into two groups according to the presence of histological evidence of transmural bowel ischemia(case group)or partial bowel ischemia(control group).CT images were reviewed for findings of ischemia,including mural thickening,pneumatosis,bowel distension,portomesenteric venous gas and arterial or venous thrombi.RESULTS:A total of 248 subjects who underwent surgery for bowel ischemia were identified.Among the208 subjects enrolled in our study,transmural bowel necrosis was identified in 121 subjects(case group),and partial bowel necrosis was identified in 87 subjects(control group).Based on CT findings,including mural thickening,bowel distension,pneumatosis,pneumatosis plus portomesenteric venous gas and presence of thrombi or emboli,there were no significant differences between the case and control groups.The concomitant presence of pneumatosis and porto-mesenteric venous gas showed an odds ratio of 1.95(95%CI:0.491-7.775,P=0.342)for the presence of transmural necrosis.The presence of pneumatosis plus porto-mesenteric venous gas exhibited good specificity(83%)but low sensitivity(17%)in the identification of transmural bowel infarction.Accordingly,the positive and negative predictive values were 60% and 17%,respectively.CONCLUSION:Although pneumatosis plus porto-mesenteric venous gas is associated with bowel ischemia,we have demonstrated that their co-occurrence cannot be used as diagnostic signs of transmural necrosis.

Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis

A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.

Pneumatosis intestinalis with obstructing intussusception:A case report and literature review

Pneumatosis intestinalis(PI) often represents a benign condition that should not be considered as an argument for surgery. We report a patient with PI and obstructing intussusception who underwent urgent colectomy and review the literatures regarding PI with intussusception. A 20-year-old man presented at our hospital with a 3-d intermittent lower abdominal pain history. He underwent steroid therapy for membranoproliferative glomerulonephritis for 4 years. Computed tomography revealed ascending colon intussusception with air within the wall. Intraoperative colonoscopy revealed numerous soft polypoid masses with normal overlying mucosa and right hemicolectomy was performed. Histological examination of colonic wall sections revealed large cysts in the submucosal layer. The pathological diagnosis was PI. Nine cases of intussusception associated with primary PI have been reported. Although primary PI often represents a benign condition that should not be considered as an argument for surgery,if the case involves intussusception and obstruction,emergent laparotomy should be considered.

Symptomatic pneumatosis intestinalis(including portal venous gas) after laparoscopic total colectomy

The development of intramural intestinal gas may indi-cate a serious postoperative complication and thereforeany radiological indication of such "pneumatosis intes-tinalis"(PI) in an unwell patient after surgery shouldput the clinical team on high-alert. However immedi-ate recourse to relook laparotomy may not be alwaysnecessary and, further, in some cases may possiblyaccelerate the deterioration especially if it proves to benon-therapeutic. Careful and close clinical monitoring,as is described in this clinical report, may allow discrimi-native identification of those in whom this finding is infact transient and therefore benign and who thereforecan be successfully treated without operative re-inter-vention. We describe the presenting features and back-ground scenario of PI early after laparoscopic total col-ectomy for medically refractory, severe ulcerative colitisand detail the critical postoperative decision pivots.

Unusual radiological finding of lethal pneumatosis intestinalis and portomesenteric vein gas

Pneumatosis intestinalis and portomesenteric vein gas are rare and potentially severe radiological findings that occur both in pediatric and adult populations.They actually are radiographic signs of underlying intra-abdominal pathology,abnormality or diagnostic medical interference.If combined with other radiological or clinical signs of intestinal ischemia or sepsis,the prognosis is dismal and urgent laparotomy is mandatory.We report two cases of surgical treatment with ominous outcome in an effort to characterize this finding correctly as an absolute surgical indication or as an additional diagnostic criterion that simply marks a further breakdown of the systems in patients with a long list of severe medical conditions.

Pneumatosis cystoides intestinalis associated with toxic epidermal necrolysis: A case report

Toxic epidermal necrolysis(TEN) is a severe adverse drug reaction, which is characterized by erythema, blisters, and/or erosions of the mucous membranes and skin, but intestinal involvement is rare. In contrast, pneumatosis cystoides intestinalis(PCI) is a rare condition associated with a wide variety of underlying diseases, but to date no patient has presented with PCI associated with TEN. A 55-year-old man was admitted to intensive care unit for treatment of TEN caused by phenobarbital. On day 8 after admission, he presented with progressive abdominal distention and hypotension. Computed tomography(CT) showed gas in the superior mesenteric vein and air filled cysts in the walls of the small intestine. He was suspected of having septic shock due to PCI. As there were no indications of bowel ischemia or necrosis, the patient was managed conservatively with antibiotics and oxygen therapy. On day 10 after admission, he was weaned off catecholamines, with CT on day 11 showing complete resolution of gas in the superior mesenteric vein and air filled cysts. To our knowledge, this article describes the first patient presenting with PCI associated with TEN.

Pneumatosis intestinalis and pneumoperitoneum on computed tomography:Beware of non-therapeutic laparotomy

Pneumatosis intestinalis(PI) is defined as gas within the gastrointestinal wall and is associated with a variety of disorders.As a concurrent occurrence with pneumoperitoneum,it can easily to be mistaken for bowel ischemia with perforated peritonitis.In fact,air dissection or rupture from subserosal cysts may be the cause of intraperitoneal and intraluminal free air,with clinical symptoms such as abdominal pain and fullness occurring as a result.We hereby report a case of an 82-year-old male with a history of chronic obstructive pulmonary disease who was diagnosed with bowel ischemia and received emergency laparotomy because of the appearance of PI and pneumoperitoneum on abdominal computed tomography scan.However,no perforated hollow organ or necrotic bowel segment was found,only diffusely distributed massive intraperitoneal air and PI of gastrointestinal tract.The laparotomy seemed non-therapeutic for this patient.This is significant warning for clinicians to differentiate the associated conditions of PI,and to evaluate whether or not emergency surgery is necessary.

Incidental finding of esophageal pneumatosis

Pneumatosis of the gastrointestinal tract is a rare condition characterized by the presence of air filled cavities in the gastrointestinal tract wall.Its occurrence has been described throughout the gastrointestinal tract from the esophagus to the rectum,however it is most commonly reported in the small intestine.Despite multiple case reports in literature,its pathogenesis still remains unclear.Pneumatosis may be idiopathic or associated with a variety of disorders namely peptic ulcer disease,jejunoileal bypass,intestinal obstruction and non-gastrointestinal disorders like asthma,chronic obstructive pulmonary disease,systemic lupus erythematosus,infectious enteritis,etc.We here present a rare case of pneumatosis of the esophagus diagnosed incidentally at an esophagogastroduodenoscopy(EGD).A 78-year-old asymptomatic woman underwent EGD and colonoscopy at our hospital for evaluation of anemia.Few months prior to EGD,she had undergone excision of laryngocele at our hospital.EGD revealed extensive submucosal blebs distributed throughout the esophagus,otherwise unremarkable stomach and duodenum.Colonoscopy showed a tubular adenomatous polyp.Since our patient was asymptomatic she did not require any surgical intervention.Management of pneumatosis depends on the underlying cause.

Pneumatosis intestinalis after systemic chemotherapy for colorectal cancer:A case report

BACKGROUND Pneumatosis intestinalis(PI),also known as intramural gas in the small intestine,is a rare condition encountered by patients with cancer after receiving chemotherapy.CASE SUMMARY A 78-year-old man with a history of colorectal cancer developed epigastric pain and diarrhea after receiving combination chemotherapy of fluorouracil,leucovorin,irinotecan,and cetuximab.Abdomen radiography revealed in-tramural air in the small intestinal wall.A computed tomography scan of the abdomen revealed the features of PI with air expanding into the mesentery.After surgery,the patient remained symptom-free throughout a 9 mo follow-up period during which he received chemotherapy of fluorouracil,leucovorin,and irinotecan.CONCLUSION Although chemotherapy-induced PI is rare among patients with cancer,the differential diagnosis of PI and fulminant complications(such as ischemia,infarction,and perforation of the gastrointestinal tract)should be conducted,in which case an urgent surgical intervention is required.

Portal vein gas combined with pneumatosis intestinalis and emphysematous cystitis:A case report and literature review

BACKGROUND Portal venous gas(PVG)is a rare clinical condition usually indicative of severe disorders,including necrotizing enterocolitis,bowel ischemia,or bowel wall rupture/infarction.Pneumatosis intestinalis(PI)is a rare illness characterized by an infiltration of gas into the intestinal wall.Emphysematous cystitis(EC)is relatively rare and characterized by intramural and/or intraluminal bladder gas best depicted by cross-sectional imaging.Our study reports a rare case coexistence of PVG presenting with PI and EC.CASE SUMMARY An 86-year-old woman was admitted to the emergency room due to the progressive aggravation of pain because of abdominal fullness and distention,complicated with vomiting and stopping defecation for 4 d.The abdominal computed tomography(CT)plain scan indicated intestinal obstruction with ischemia changes,gas in the portal vein,left renal artery,superior mesenteric artery,superior mesenteric vein,some branch vessels,and bladder pneumatosis with air-fluid levels.Emergency surgery was conducted on the patient.Ischemic necrosis was found in the small intestine approximately 110 cm below the Treitz ligament and in the ileocecal junction and ascending colon canals.This included excision of the necrotic small intestine and right colon,fistulation of the proximal small intestine,and distal closure of the transverse colon.Subsequently,the patient displayed postoperative short bowel syndrome but had a good recovery.She received intravenous fluid infusion and enteral nutrition maintenance every other day after discharge from the community hospital.CONCLUSION Emergency surgery should be performed when CT shows signs of PVG with PI and EC along with a clinical situation strongly suggestive of bowel ischemia.

Rare etiology of colonic intussusception involving an adult with emphysematous cystic enteropathy:A case report and review of literature

BACKGROUND Pneumatosis cystoides intestinalis(PCI),characterized by a collection of gas-filled cysts in the intestinal wall,is an uncommon but well-known condition in gastroenterology.Abdominal pain is the most frequent symptom associated with PCI.Intussusception represents a potential cause of recurrent abdominal pain or emergency presentation.However,the occurrence of colonic intussusception secondary to PCI is very unusual in adulthood.CASE SUMMARY A 52-year-old male,known with idiopathic PCI,presented seventeen months after initial diagnosis with a new right upper quadrant pain.A computed tomographyscan demonstrated a colonic intussusception at the hepatic flexure.PCI did not progress compared with initial investigation.The patient underwent an emergency right hemicolectomy.CONCLUSION Resection was recommended in this case because PCI proved to be persisting with no identified curable cause.Surgery allowed to address the underlying pathology,the potential relapse of intussusception,and the likely cause of recurrent abdominal pain,either invagination or PCI itself.

Midgut neuroendocrine tumor presenting with acute intestinal ischemia

Neuroendocrine tumors represent a heterogeneous group of neoplasms that arise from neuroendocrine cells and secrete various peptides and bioamines. While gastrointestinal neuroendocrine tumors,commonly called carcinoids,account for about 2/3 of all neuroendocrine tumors,they are relatively rare. Small intestine neuroendocrine tumors originate from intestinal enterochromaffin cells and represent about 1/4 of small intestine neoplasms. They can be asymptomatic or cause nonspecific symptoms,which usually leads to a delayed diagnosis. Imaging modalities can aid diagnosis and surgery remains the mainstay of treatment. We present a case of a jejunal neuroendocrine tumor that caused nonspecific symptoms for about 1 year before manifesting with acute mesenteric ischemia. Abdominal X-rays revealed pneumatosis intestinalis and an abdominal ultrasound and computed tomography confirmed the diagnosis. The patient was submitted to segmental enterectomy. Histopathological study demonstrated a neuroendocrine tumor with perineural and arterial infiltration and lymph node metastasis. The postoperative course was uneventful and the patient denied any adjuvant treatment.

Does massive intraabdominal free gas require surgical intervention?

We describe a rare case of an 81-year-old man who presented with severe epigastralgia. A chest radiograph showed massive free gas bilaterally in the diaphragmatic spaces. Computed tomography(CT) scan also showed massive free gas in the peritoneal cavity with portal venous gas. We used a wait-andsee approach and carefully considered surgery again when the time was appropriate. The patient received conservative therapy with fasting, an intravenous infusion of antibiotics, and nasogastric intubation. The patient soon recovered and was able to start eating meals 4 d after treatment; thus, surgical intervention was avoided. Thereafter, colonoscopy examination showed pneumatosis cystoides intestinalis in the ascending colon. On retrospective review, CT scan demonstrated sporadic air-filled cysts in the ascending colon. The present case taught us a lesson: the presence of massive intraabdominal free gas with portal venous gas does not necessarily require surgical intervention. Pneumatosis cystoides intestinalis should be considered as a potential causative factor of free gas with portal venous gas when making the differential diagnosis.