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Rationale: Pneumatosis intestinalis (PI) and hepatic portal venous gas (HPVG) are rare but potentially lethal conditions in which gas pathologically accumulates in the portal vein and intestinal wall, respectively. Proposed mechanisms include flatus escaping through an injured intestinal mucosa into the submucosa and thence into the portal venous system, or bacterial translocation (BT) of gas-forming enteric microorganisms from the gut into and through the intestinal wall to other organs. However, there has been no clear histopathological evidence to support these hypotheses., Patient Concerns: A 61-year-old man underwent sigmoidectomy for colonic adenocarcinoma. Postoperatively, he developed paralytic ileus and then had a sudden cardiopulmonary arrest., Diagnoses: PI and HPVG were found at autopsy, presumably caused by the postoperative paralytic ileus and associated with BT of gas-forming organisms., Interventions: Cardiopulmonary resuscitation was unsuccessful., Outcomes: Postmortem imaging indicated the presence of massive PI and HPVG. At autopsy, there was marked intestinal emphysema with diffuse ischemic mucosal necrosis and severe pneumatosis in the stomach and intestine and marked gaseous dilation of the intrahepatic portal veins. Postmortem bacterial cultures revealed enteric bacteria in the peripheral blood and liver tissue., Lessons: Postoperative ileus leading to intestinal mucosal damage may be associated with BT of gas-forming enteric bacteria and the rapid onset of PI and HPVG with a lethal outcome.

Pneumatosis cystoides intestinalis (PCI) refers to the presence of gas within the wall of the small or large intestine. The pathophysiology is incompletely understood and is probably multifactorial in nature. PCI is a known but rare complication of systemic scleroderma, and the aetiology of PCI in patients with scleroderma is not fully understood. We present the case of a patient who was referred to gastroenterology clinic by her general practitioner for investigation of 8 months of weight loss, urgency, diarrhoea, bloating and crampy abdominal pain. Extensive investigations were performed to exclude infective, inflammatory or malignant aetiologies for these symptoms. She was diagnosed with PCI on her colonoscopy and was subsequently screened for secondary causes. Our patient was diagnosed with the limited cutaneous (CREST) variant of systemic scleroderma. This case report illustrates that PCI could be an uncommon presentation of systemic sclerosis, therefore clinicians should be aware of the association between these conditions., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

A 74-year-old Japanese woman was diagnosed with pseudolipomatosis of the cecum and ascending colon. Colonoscopy was performed, which revealed the presence of slightly elevated white lesions, while a magnifying observation showed microbubbles within the mucosa. A month after colonoscopy, the patient was diagnosed with pneumatosis intestinalis. Although the exact pathogenesis is unclear, pneumatosis intestinalis may arise secondary to pseudolipomatosis. This case also indicates that a magnifying observation during colonoscopy may aid in the diagnosis of pseudolipomatosis of the large intestine, since it shows microbubbles within the mucosa, which may be a distinctive feature reflecting the pathology of this disease.

Competing Interests: None

We report a case of a male, term newborn with known left congenital diaphragmatic hernia (CDH) who presented with circulatory compromise requiring maximal inotropic support in the first 24 hours of life. Repeat X-ray at 24 hours showed pneumatosis intestinalis. Emergency laparotomy was performed for suspected necrotising enterocolitis. The terminal ileum to the sigmoid colon were frankly necrotic with multiple perforations. Subtotal colectomy was performed. Although the recovery was protracted, the baby had a favourable outcome with progressive weight gain on follow-up at 3, 6 and 9 months of age.This case of CDH had a postnatal systemic instability that was more severe than predicted, and as well as assessing for persistent pulmonary hypertension of newborn should trigger immediate suspicion for other organ involvement., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI., Aim: Review of our experience of PI in children beyond the early infantile period., Methods: Retrospective review of patient's records and radiological images from 2013 to 2015., Results: Eighteen patients (three girls) had radiologically confirmed PI. The median age was 4.5 years (range 8 months-13 years). Background medical conditions (number): short bowel syndrome (one), congenital heart disease (two), sickle cell disease (one), epilepsy (three), cerebral palsy (six), myotonic dystrophy (four) and peroxisomal biogenesis defect (one).Six children (33%) presented with abdominal distension, four (22%) with abdominal pain, three (17%) with bilious vomiting, two (11%) with diarrhoea and one (6%) with rectal bleeding. Two (11%) were asymptomatic. One had air in portal vein and two had pneumoperitoneum.All patients with symptomatic PI were treated conservatively with successful outcome and complete resolution of PI. None required surgical intervention., Conclusion: PI in children who are not on chemotherapy or immunosuppressant appears to follow a benign course and is responsive to conservative management. In contrast to adults, portal venous gas and pneumoperitoneum do not predict the need for surgical intervention., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Mesenteric ischaemia is relatively uncommon, but is an important diagnosis to remember as it can lead to significant morbidity and mortality. Improvements in clinical imaging technology have made rapid diagnosis and treatment possible, but this relies on awareness of the condition. This article summarizes current best practice in diagnosis and management of mesenteric ischaemia to guide the clinician.

An 80-year-old woman was brought to our hospital in an ambulance because of signs of peritoneal irritation. Computed tomography revealed volvulus, sigmoid colon dilation, and free air and pneumatosis within the colonic and bowel wall. An emergency laparotomy was performed after the diagnosis of colonic perforation associated with volvulus or pneumatosis. Numerous gas-filled cysts, up to 10mm in diameter, were located in the resected bowel wall. The cells at the inner lining of the cysts tested positive for D2-40 immunoreactivity, seemingly originating from the lymphatic vessels. In many cysts, KP-1 positive small mononuclear cells and/or foreign-body-type giant cells adhered to the D2-40 positive cells were observed. Fibrosis and lymphoplasmacytic infiltration was observed around the cysts. Contrary to the influential mechanical cause theory, in the present case, pneumatosis may have preceded volvulus, occurring as an idiopathic disease. Therefore, the lymphatic vessels were hypothesized to be associated with the pathogenesis of PCI.

Antecedentes: La neumatosis quística intestinal es una condición infrecuente, caracterizada por la formación de quistes de contenido gaseoso debajo de la mucosa y la serosa intestinales., Caso Clínico: Mujer de 84 años, con antecedente de artritis reumatoide en tratamiento inmunosupresor, intervenida hace 2 años por neumoperitoneo, con diagnóstico de diverticulosis yeyunal y enfisema mesentérico, acude con cuadro de dolor abdominal difuso de 2 días de evolución, sin otra sintomatología. En la radiografía de abdomen se observa neumoperitoneo con elevación del hemidiafragma derecho y distensión de asas del intestino delgado. Ante la sospecha de perforación de víscera hueca se decide intervención quirúrgica, en la cual se diagnostica a la paciente de neumatosis quística intestinal., Conclusión: La neumatosis quística intestinal es de causa desconocida, aunque en un elevado porcentaje de pacientes se ha visto asociada a enfermedades pulmonares obstructivas, enfermedades del tejido conectivo, inmunosupresión o enfermedades gastrointestinales. Se localiza más frecuentemente en el colon y el intestino delgado. Se diagnostica principalmente mediante pruebas de imagen (radiografía o tomografía computarizada). El tratamiento es conservador, realizándose intervención quirúrgica si existe sospecha de necrosis intestinal. Es difícil el diagnóstico diferencial con un neumoperitoneo por perforación de víscera hueca., Background: Pneumatosis cystoides intestinalis is an infrequent condition, characterized by the formation of gaseous content cysts under the mucosa and intestinal serous., Clinical Case: 84-year-old woman, with a history of rheumatoid arthritis under immunosuppressive treatment, operated 2 years ago by pneumoperitoneum, where she was diagnosed of jejunal diverticulosis and mesenteric emphysema, with diffuse abdominal pain of 2 days of evolution, without other symptoms. In abdominal radiography: pneumoperitoneum with elevation of right hemidiaphragm and distention of small intestine. Given the suspicion of perforation of the intestinal wall, surgical intervention was decided, in which the patient was diagnosed with pneumatosis cystoides intestinalis., Conclusion: Pneumatosis cystoides intestinalis has an unknown etiology, although in a high percentage of patients it has been associated with obstructive pulmonary pathologies, connective tissue diseases, immunosuppression or gastrointestinal diseases. It is located more frequently in the colon and small intestine. It is diagnosed mainly through imaging tests (radiography or computed tomography). The treatment is conservative, performing surgical intervention if there is suspicion of intestinal necrosis; being difficult the differential diagnosis with a pneumoperitoneum by perforation of hollow viscera.., (Copyright: © 2018 Permanyer.)

Rationale: Pneumatosis cystoids intestinalis (PCI) is a rare disease in which gas develops in the mucosa or submucosa of the digestive tract. The etiology and pathogenesis of this disease, at present, remain unclear, and gastrointestinal malignant neoplasms may be a potentially important cause. Herein, we report a case of mantle cell lymphoma presenting as PCI as well as present a literature review of cases of suspect PCI that was definitively diagnosed as gastrointestinal neoplasms. In doing so, we highlighted cases of neoplastic pathogenesis that present as PCI., Patient Concerns: A 55-year-old man was referred to our gastrointestinal department with complaints of intermittent abdominal pain, distention, diarrhea, and occasional melena that persisted for 2 months. He has a history of nasopharyngeal carcinoma., Diagnoses: Intensive, translucent, grape-like cystoids of the whole colon and small intestine were disguised as PCI upon colonoscopy and capsule endoscopy., Interventions: Right hemicolectomy and ileocecectomy were performed for intussusception and to confirm the diagnosis. Final pathology indicated that the mass was mantle cell lymphoma., Outcomes: After surgery and subsequent chemotherapy, the patient showed good recovery and no abnormal lesions were detected on colonoscopy., Lessons: As shown through this case and a literature review of similar cases of apparent PCI that was definitively diagnosed as gastrointestinal neoplasm, gastrointestinal malignant neoplasms might rarely present as PCI and neoplastic etiologies should also be considered once PCI is detected. Because most patients with malignant PCIs might inevitably experience severe complications, abdominal surgery should be considered and applied timely after unsuccessful resolution by conservative medical therapies and symptomatic treatments., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

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 is a rare condition in which multiple gas-filled cysts are found within the wall of the gastrointestinal tract either in the subserosa or submucosa. Its pathogenesis is uncertain and several pathogenic mechanisms have been proposed to explain its origin. The case of a male patient of 46 years with previous diagnosis of pneumatosis cystic intestinalis, who consulted for abdominal pain, vomiting and fever (39 °C) is presented. By the time of admission ther were signs of peritoneal irritation. The X-ray abdominal reported distension and intestinal hydro-air levels. Exploratory laparotomy was performed and revealed small bowel volvulus with strangulation of some intestinal segment. Histological diagnosis was pneumatosis cystic intestinalis complicated with Infarction trans-mural by intestinal volvulus. The patient evolved satisfactorily.

Background: Our group has previously published a retrospective review defining variables predictive of transmural bowel ischemia in the setting of pneumatosis intestinalis (PI). We hypothesize this prospective study will confirm the findings of the retrospective review, enhancing legitimacy to the predictive factors for pathologic PI previously highlighted., Methods: Data were collected using the Research Electronic Data Capture. Forward logistic regression was utilized to identify independent predictors for pathologic PI. Statistical significance was defined as p ≤ 0.05., Results: During the 3-year study period, 127 patients with PI were identified. Of these, 79 had benign disease, and 49 pathologic PI defined by the presence of transmural ischemia during surgical exploration or autopsy. Laboratory values such as elevated international normalized ratio (INR), decreased hemoglobin, and a lactate value of greater than 2.0 mmol/L were predictive of pathologic PI, as well as clinical factors including adynamic ileus, peritoneal signs on physical examination, sepsis, and hypotension. The location was also a significant factor, as patients with small bowel PI had a higher incidence of transmural ischemia than colonic PI. On multiple logistic regression, lactate value of greater than 2.0 mmol/L (odds ratio, 5.1, 1.3-19.5; p = 0.018), elevated INR (odds ratio, 3.2, 1.1-9.6; p = 0.031), peritonitis (15.0, 2.9-78; p = 0.001), and decreased hemoglobin (0.70, 0.50-0.97, 0.031) remained significant predictors of transmural ischemia (area under the curve, 0.90; 0.83-0.97). A lactate value of 2.0 mmol/L or greater and peritonitis are common factors between the retrospective review and this prospective study., Conclusions: We recommend surgical exploration to be strongly considered for those PI patients presenting also with a lactate greater than 2 mmol/L and/or peritonitis. We suggest strong suspicion for necrosis in those patient with PI and small bowel involvement, ascites on computed tomography scan, adynamic ileus, anemia, and a high INR., Level of Evidence: Prognostic study, level II; therapeutic study, level II.

This case series describes our experience in managing 4 children with complex neurodisability, feed intolerance, and pneumatosis coli. In all of the 4 patients, symptoms and feed tolerance were substantially improved by the formation of a laparoscopically assisted defunctioning ileostomy. We describe our present management strategy and believe this is a promising treatment for those patients who can reduce long-term dependence on parenteral nutrition, although we acknowledge that there is a long-term risk of disuse colitits in the defunctioned bowel.

Objectives: To evaluate the clinical significance of pneumatosis intestinalis (PI) including the influence on treatment and outcome., Method and Materials: Two radiologists jointly reviewed MDCT-examinations of 149 consecutive emergency patients (53 women, mean age 64, range 21-95) with PI of the stomach (n = 4), small (n = 68) and/or large bowel (n = 96). PI extension, distribution and possibly associated porto-mesenteric venous gas (PMVG) were correlated with other MDCT-findings, risk factors, clinical management, laboratory, histopathology, final diagnosis and outcome., Results: The most frequent cause of PI was intestinal ischemia (n = 80,53.7 %), followed by infection (n = 18,12.1 %), obstructive (n = 12,8.1 %) and non-obstructive (n = 10,6.7 %) bowel dilatation, unknown aetiologies (n = 8,5.4 %), drugs (n = 8,5.4 %), inflammation (n = 7,4.7 %), and others (n = 6,4 %). Neither PI distribution nor extension significantly correlated with underlying ischemia. Overall mortality was 41.6 % (n = 62), mostly related to intestinal ischemia (p = 0.003). Associated PMVG significantly correlated with underlying ischemia (p = 0.009), as did the anatomical distribution of PMVG (p = 0.015). Decreased mural contrast-enhancement was the only other MDCT-feature significantly associated with ischemia (p p < 0.001). Elevated white blood count significantly correlated with ischemia (p = 0.03)., Conclusion: In emergency patients, ischemia remains the most common aetiology of PI, showing the highest mortality. PI with associated PMVG is an alerting sign. PI together with decreased mural contrast-enhancement indicates underlying ischemia., Key Points: • In emergency patients, PI may be caused by various disorders. • Intestinal ischemia remains the most common cause of PI in acute situations. • PI associated with decreased mural contrast-enhancement indicates acute intestinal ischemia. • PI associated with PMVG should alert the radiologist to possible underlying ischemia.

Background: The significance of pneumatosis intestinalis (PI) and portal venous gas (PVG) is controversial. This retrospective study evaluated the risk factors for bowel necrosis in patients with PI and/or PVG., Methods: Between 2002 and 2015, 52 patients were diagnosed with PI and/or PVG and were included in this study. The patients were classified according to the presence or absence of bowel necrosis in surgical findings or at autopsy. Patient characteristics and clinical findings related to bowel necrosis were investigated., Results: Bowel necrosis was diagnosed in 17 (32.7 %) patients. Amongst these 17, 10 patients received salvage surgical intervention, and seven of those diagnosed with bowel necrosis survived after the operation. The remaining 35 patients received conservative treatment with or without exploratory laparotomy. Between patients with and without bowel necrosis, laboratory data revealed significant differences in the levels of C-reactive protein (P = 0.0038), creatinine (P = 0.0054), and lactate (P = 0.045); clinical findings showed differences in abdominal pain (P = 0.019) and peritoneal irritation signs (P = 0.016); computed tomography detected ascites (P = 0.011) and changes of bowel wall enhancement (P = 0.03) that were significantly higher in patients with bowel necrosis. The rate of PI and/or PVG detected in patients postoperatively was significantly higher in patients with bowel necrosis (P < 0.0001). Multivariate analysis showed that bowel necrosis was significantly more likely when PI or PVG was detected in postoperative patients than in patients who had not had surgery (P = 0.003)., Conclusions: PI and/or PVG, alone, are not automatically indicative of bowel necrosis. However, when these conditions occur postoperatively, they indicate bowel necrosis requiring reoperation.

A 77-year-old man on maintenance dialysis developed hypotension, nausea and abdominal pain one hour after beginning to undergo hemodialysis. Abdominal computed tomography (CT) showed gas shadows in the intrahepatic portal vein and the small intestinal wall, but no signs indicating intestinal necrosis. Three days later, the gas shadows on abdominal CT disappeared by conservative therapy. In cases with both pneumatosis cystoides intestinalis and hepatic portal venous gas, intestinal necrosis should therefore be suspected and surgical therapy should also be considered, particularly in hemodialysis patients with a risk of intestinal ischemia. However, conservative therapy may be an option in cases with no intestinal necrosis.

This series details two cases of benign pneumatosis intestinalis (PI) in patients post-lung transplant, which were discovered incidentally on routine surveillance chest radiographs during ambulatory clinic visits. Both patients had uneventful post-transplant recovery and were asymptomatic at presentation. The patients were admitted for observation. Contrast-enhanced abdominal CT scans confirmed the plain film findings. Both cases were managed conservatively with bowel rest, intravenous hydration and serial abdominal examinations. The patients had unremarkable hospital courses and were both discharged in good condition. Our current understanding of benign PI in patients post-transplant is limited to a few case series and case reports. Greater awareness of this entity may decrease unnecessary invasive procedures and improve management of these patients., (2015 BMJ Publishing Group Ltd.)

Pneumatosis cystoides intestinalis (PCI) is a relatively rare disease in which multilocular or linear pneumatic cysts develop under the mucosa or serosa of the intestinal wall. We conducted a retrospective analysis to investigate the clinical characteristics of 68 patients with PCI. Hepatic portal venous gas (HPVG) was present in 9 patients, 8 of which had underlying intestinal tract necrosis. In most patients, PCI was mild and asymptomatic and resolved spontaneously. The main treatment strategy for PCI is conservative therapy. However, in cases complicated by HPVG, the presence of underlying intestinal tract necrosis must be considered in order to promptly determine whether emergency surgery is required.

Hepatic portal venous gas is a very rare radiologic sign which is characterized by gas accumulation in the portal venous circulation. Pneumatosis intestinalis is also very rare and is characterized by multiple air cysts in the serosal or submucosal layers of the gastrointestinal tract walls. These two findings are caused by various pathological conditions and can develop individually or simultaneously. The latter is clinically more significant because it is frequently related to bowel ischemia or necrosis, and represents a poor prognosis. However, prognosis is more influenced by the severity of underlying disease rather than hepatic portal venous gas or pneumatosis intestinalis itself. If bowel ischemia or necrosis is the primary cause, emergency operation is very important to improve patient's prognosis. Herein, we report a case of necrotizing colitis presenting as hepatic portal venous gas and pneumatosis intestinalis which was successfully managed by early surgery.

A 16-year-old girl presented with free gas under the diaphragm after endoscopic balloon dilation for pyloric stenosis. There was no perforation site identified on laparotomy. However, there was massive pneumatosis intestinalis involving the entire small bowel., (2015 BMJ Publishing Group Ltd.)

The management of pneumatosis intestinalis (PI) can vary from urgent surgical procedure to conservative treatment. Uncommonly, it has been reported in association with organ transplantation, and very few cases have been reported after lung transplantation. The transplant physicians should be well versed in differentiating the fatal form of PI from a benign one. Here, we describe a case of PI with pneumoperitoneum mimicking intestinal perforation after bilateral lung transplantation; this case posed a therapeutic dilemma. After discussion with the multidisciplinary teams, conservative management was chosen to which the patient responded successfully. Clinical signs and imaging features in these situations can mimic true bowel perforation. Hence, a correct diagnosis and proper assessment is imperative., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the first reported case of pneumatosis intestinalis (PI) in a pediatric patient with granulomatosis with polyangiitis (GPA) and multiple other risk factors and review the literature for PI in adult and pediatric rheumatologic conditions., Methods: A PubMed search was completed using the search phrase "pneumatosis intestinalis." Searches were limited to humans and the English language, and remaining articles involving patients with rheumatologic diagnoses were identified and included in our discussion., Results: This is the first reported of case of PI in a patient with underlying GPA or antineutrophil cytoplasmic antibody-associated vasculitides. Out of 90 previously reported cases of PI in patients with rheumatologic conditions, 79 cases were in adults and 11 in children. There were 30 patients with systemic sclerosis, 18 with MCTD/overlap syndrome, 18 with dermatomyositis or polymyositis, 16 with SLE, and 8 with other diagnoses. Overall, 81% of the patients were on corticosteroids or other immunosuppressants prior to development of PI. The most common presenting symptom was abdominal pain, and 51% of patients had associated pneumoperitoneum., Conclusions: PI can be associated with a broad spectrum of rheumatic diseases, including GPA, and should be included in the differential diagnosis of patients with rheumatologic conditions and nonspecific gastrointestinal symptoms., (Copyright © 2014 Elsevier Inc. All rights reserved.)