[Pneumatosis cystoides intestinalis: a case report and literature review]. / Kistoznyi pnevmatoz tonkoi kishki: klinicheskii sluchai i obzor literatury.

Pneumatosis cystoides was first described by Du Vernay in 1783. This is a fairly rare disease with nonspecific symptoms and CT data on pneumoperitoneum. The authors present pneumatosis intestinalis in a patient with systemic connective tissue disorder. Free gas in abdominal cavity and dilated intestinal loops were an indication for emergency surgery with subsequent resection of intestine due to signs of ischemic damage. A review of clinical cases allows us to conclude that pneumoperitoneum requires careful differential diagnosis. Free gas in abdominal cavity in patients with cystic pneumatosis is an indication for emergency surgery only in case of complicated course of disease.

Low-risk pneumatosis intestinalis in the pediatric surgical population.

BACKGROUND: Pneumatosis intestinalis (PI, presence of air in bowel wall) develops in a variety of settings and due to a variety of insults which is then characterized by varying severity and clinical course. Anecdotally, many of these cases are benign with few clinical sequelae; however, we lack evidence-based guidelines to help guide management of such lower-risk cases. We aimed to describe the clinical entity of low-risk PI, characterize the population of children who develop this form of PI, determine if management approach or clinical outcomes differed depending on the managing physician's field of practice, and finally determine if a shortened course of NPO and antibiotics was safe in the population of children with low-risk PI. METHODS: We performed a retrospective review of all children over age 1 year treated at Children's Hospital Colorado (CHCO), between 2009 and 2019 with a diagnosis of PI who did not also have a diagnosis of cancer or history of bone marrow transplant (BMT). Data including demographic variables, clinical course, and outcomes were obtained from the electronic medical record. Low-risk criteria included no need for ICU admission, vasopressor use, or urgent surgical intervention. RESULTS: Ninety-one children were treated for their first episode of PI during the study period, 72 of whom met our low-risk criteria. Among the low-risk group, rates of complications including hemodynamic decompensation during treatment, PI recurrence, Clostridium difficile colitis, and death did not differ between those who received 3 days or less of antibiotics and those who received more than 3 days of antibiotics. Outcomes also did not differ between children cared for by surgeons or pediatricians. CONCLUSIONS: Here, we define low-risk PI as that which occurs in children over age 1 who do not have a prior diagnosis of cancer or prior BMT and who do not require ICU admission, vasopressor administration, or urgent surgical intervention. It is likely safe to treat these children with only 3 days of antibiotic therapy and NPO. LEVEL OF EVIDENCE: Level III.

Clinical and Endoscopic Features of Pneumatosis Cystoides Intestinalis: A Retrospective Study in 192 Patients.

BACKGROUND/AIMS: Pneumatosis cystoides intestinalis is not well recognized. Clinical features vary in several case reports, and prognosis remains unclear. We aimed to summarize the clinical and endoscopic features of pneumatosis cystoides intestinalis and to explore potential factors associated with lesion size. MATERIALS AND METHODS: We retrospectively collected clinical and endoscopic features of patients diagnosed with pneumatosis cystoides intestinalis from July 2015 to October 2021. Patients were allocated to 2 groups according to lesion size with 2 cm as boundary value. Baseline characteristics were compared between the groups. RESULTS: A total of 192 patients were included in this study with a 1.3:1 male-to-female ratio. About 91 lesions (47.70%) were ≥2 cm and those patients were more likely to have a history of polypectomy or abdominal surgery compared to lesion size <2 cm (P < .05). For 50 patients who received follow-up colonoscopy, 28 cases (56.00%) disappeared spontaneously and 22 cases (44.00%) remained unchanged. No factors have been observed to be connected with prognosis. CONCLUSIONS: Colonoscopy is beneficial to the diagnosis of pneumatosis cystoides intestinalis. Patients with a history of polypectomy or abdominal surgery were more likely to develop lesions <2 cm. Most patients do not need special treatments and have favorable prognosis.

Intestinal obstruction due to pneumatosis intestinalis.

We present the case of an 83-year-old male, with a past medical history of benign pneumoperitoneum secondary to pneumatosis intestinalis which evolved for a number of years with periodic follow-ups. The patient comes to the Emergency Room with sintomatology of intestinal obstruction. Urgent surgical management is decided, an exploratory laparotomy is performed where an intestinal obstruction secondary to pneumatosis intestinalis, with loss of structure of the intestinal wall as visualized in the images, is determined; therefore resection of the affected small intestine segment and primary anastomosis are performed. The pathology report confirms the diagnosis. The patient progresses favorably during the postoperative period and is currently asymptomatic after 12 months.

Current approaches to the management of pneumatosis intestinalis: an American Pediatric Surgical Association membership survey.

PURPOSE: Pneumatosis intestinalis (PI) remains difficult to treat as it can lead to a broad range of clinical sequalae and there are little published data available to guide management. Our aim was to evaluate how pediatric surgeons currently manage children with PI, how treatment varies based on etiology, and to identify opportunities to optimize current PI management strategies. METHODS: We administered a web-based survey of practicing pediatric surgeons in the United States and Canada. The survey was distributed to all members of the American Pediatric Surgical Association. RESULTS: Of 1508 distributed surveys, 333 responses were received (22% response rate); 174 were complete and included in analysis (12% analyzed). For all scenarios, respondents recommended treatment for PI include a median 7 days of bowel rest and 7 days antibiotics. Only 41% reported their approach to PI management was optimal. Ways to optimize care include treatment based on etiology (83%), decreased number of repeat images (64%), shorter NPO course (49%), and shorter antibiotic course (47%). CONCLUSION: Pediatric surgeons manage PI similarly regardless of etiology but most report this is suboptimal. Future work is needed to prospectively evaluate management protocols that consider etiology.

Hepatic Portal Venous Gas: A Potentially Lethal Sign Demanding Urgent Management.

BACKGROUND Hepatic portal venous gas is a rare and concerning finding occasionally seen on computed tomography (CT) scans, and must be emergently managed, often in the operating room. This condition can present in conjunction with bowel distension, pneumatosis intestinalis, and intestinal ischemia, so care must be taken to examine the imaging closely so as not to miss this dire condition. This report summarizes our experience with a patient who had this problem and how urgent management prevented a lethal outcome. CASE REPORT The patient was a 77-year-old morbidly obese man whose complicated hospital course began with admission for abdominal pain evaluation. This led to a flexible sigmoidoscopy for concerning CT findings suggestive of colitis or malignancy, leading to a perforation at the anterior wall of the sigmoid-rectal junction. Urgent sigmoid colectomy and Hartmann's procedure were performed along with pelvic drainage. Blood cultures returned positive for Klebsiella. After 10 days, the patient decompensated, and a CT scan showed pneumatosis intestinalis, hepatic portal venous gas, and diffuse small bowel distension. Rectal stump dehiscence had occurred; therefore, 2 repeat abdominal wash-outs were performed with aggressive intensive care. The patient eventually stabilized and was ultimately discharged to a skilled nursing facility 32 days later. CONCLUSIONS This case illustrates the importance of prompt imaging, medical management, and, if necessary, surgical exploration in the patient with bowel distension and hepatic portal venous gas on a CT scan. Although uncommon, this finding indicates a potentially poor prognosis and must be addressed emergently to prevent bowel ischemia from progressing in patients with underlying abdominal pathology.

Pneumatosis intestinalis in abdominal CT: predictors of short-term mortality in patients with clinical suspicion of mesenteric ischemia.

PURPOSE: Pneumatosis intestinalis (PI) in the bowel wall demonstrated in computed tomography (CT) of the abdomen is unspecific and its prognostic relevance remains poorly understood. The purpose of this study was to identify predictors of short-term mortality in patients with suspected mesenteric ischemia who were referred to abdominal CT and showed PI. METHODS: In this retrospective, IRB-approved, single-centre study, CT scans and electronic medical records of 540 patients who were referred to abdominal CT with clinical suspicion of mesenteric ischemia were analysed. 109/540 (20%) patients (median age 66 years, 39 females) showed PI. CT findings were correlated with surgical and pathology reports (if available), with clinical and laboratory findings, and with patient history. Short-term outcome was defined as survival within 30 days after CT. RESULTS: PI was found in the stomach (n = 6), small bowel (n = 65), and colon (n = 85). Further gas was found in mesenteric (n = 54), portal (n = 19) and intrahepatic veins (n = 36). Multivariate analysis revealed that PI in the colon [odds ratio (OR) 2.86], elevated blood AST levels (OR 3.00), and presence of perfusion inhomogeneities in other abdominal organs (OR 3.38) were independent predictors of short-term mortality. Surgery had a positive effect on mortality (88% lower likelihood of mortality), similar to the presence of abdominal pain (65% lower likelihood). CONCLUSIONS: Our study suggests that in patients referred for abdominal CT with clinical suspicion of mesenteric ischemia, location of PI in the colon, elevation of blood AST, and presence of perfusion inhomogeneities in parenchymatous organs are predictors of short-term mortality.

Pneumatosis intestinalis and porto-mesenteric venous gas: a multicenter study.

BACKGROUND: Estimating the prognosis of patients with pneumatosis intestinalis (PI) and porto-mesenteric venous gas (PMVG) can be challenging. The purpose of this study was to refine prognostication to improve decision making in daily clinical routine. METHODS: A total of 290 patients with confirmed PI were included in the final analysis. The presence of PMVG and mortality (90d follow-up) were evaluated with regard to the influence of possible risk factors. Furthermore, a linear estimation model was devised combining significant parameters to calculate accuracies for predicting death in patients undergoing surgery by means of a defined operation point (ROC-analysis). RESULTS: Overall, 90d mortality was 55.2% (160/290). In patients with PI only, mortality was 46.5% (78/168) and increased significantly to 67.2% (82/122) in combination with PMVG (median survival: PI: 58d vs. PI and PMVG: 41d; p < 0.001). In the entire patient group, 53.5% (155/290) were treated surgically with a 90d mortality of 58.8% (91/155) in this latter group, while 90d mortality was 51.1% (69/135) in patients treated conservatively. In the patients who survived > 90d treated conservatively (24.9% of the entire collective; 72/290) PMVG/PI was defined as "benign"/reversible. PMVG, COPD, sepsis and a low platelet count were found to correlate with a worse prognosis helping to identify patients who might not profit from surgery, in this context our calculation model reaches accuracies of 97% specificity, 20% sensitivity, 90% PPV and 45% NPV. CONCLUSION: Although PI is associated with high morbidity and mortality, "benign causes" are common. However, in concomitant PMVG, mortality rates increase significantly. Our mathematical model could serve as a decision support tool to identify patients who are least likely to benefit from surgery, and to potentially reduce overtreatment in this subset of patients.

Clinical characteristics of patients with pneumatosis intestinalis.

BACKGROUND: Several theories explaining the development of pneumatosis intestinalis (PI) have been reported, but a substantial portion of cases have been idiopathic. Additionally, predictors of bowel ischaemia in PI have not been fully investigated, while PI with bowel ischaemia has deteriorated overall outcomes of PI. METHODS: Sixty-four patients diagnosed with PI (2009-2019) were allocated to two groups: with (group 1; n = 15 (23%)) and without (group 2; n = 49 (77%)) bowel ischaemia. Fourteen patients underwent emergency surgery, and bowel ischaemia was identified in nine (64%). Six patients in group 1 were diagnosed with bowel ischaemia, and were treated palliatively. On medical charts, we determined underlying conditions of PI, compared the characteristics and outcomes between the groups, and identified the predictors of bowel ischaemia. RESULTS: Group 1 patients more commonly showed abdominal pain, lower base excess, higher C-reactive protein concentrations, higher white blood cell counts and higher neutrophil-to-lymphocyte ratios, and more frequent comorbid ascites, free air and hepatic portal vein gas. Of nine bowel ischaemia surgery patients, three (33%) died; all because of anastomotic leak. All except three patients in group 2, who presented with aspiration pneumonia, responded to treatment. Only one patient had an unknown cause (1/64, 1.6%), and various underlying conditions in secondary PI were confirmed. CONCLUSION: Idiopathic PI may be identified rarely using current imaging and knowledge, but outcomes in PI patients with bowel ischaemia remain unsatisfactory. Earlier identification of bowel ischaemia by various specialists in accordance with predictors of bowel ischaemia could improve overall outcomes in PI patients.

Machine learning for the prediction of pathologic pneumatosis intestinalis.

BACKGROUND: The radiographic finding of pneumatosis intestinalis can indicate a spectrum of underlying processes ranging from a benign finding to a life-threatening condition. Although radiographic pneumatosis intestinalis is relatively common, there is no validated clinical tool to guide surgical management. METHODS: Using a retrospective cohort of 300 pneumatosis intestinalis cases from a single institution, we developed 3 machine learning models for 2 clinical tasks: (1) the distinction of benign from pathologic pneumatosis intestinalis cases and (2) the determination of patients who would benefit from an operation. The 3 models are (1) an imaging model based on radiomic features extracted from computed tomography scans, (2) a clinical model based on clinical variables, and (3) a combination model using both the imaging and clinical variables. RESULTS: The combination model achieves an area under the curve of 0.91 (confidence interval: 0.87-0.94) for task I and an area under the curve of 0.84 (confidence interval: 0.79-0.88) for task II. The combination model significantly (P < .05) outperforms the imaging model and the clinical model for both tasks. The imaging model achieves an area under the curve of 0.72 (confidence interval: 0.57-0.87) for task I and 0.68 (confidence interval: 0.61-0.74) for task II. The clinical model achieves an area under the curve of 0.87 (confidence interval: 0.83-0.91) for task I and 0.76 (confidence interval: 0.70-0.81) for task II. CONCLUSION: This study suggests that combined radiographic and clinical features can identify pathologic pneumatosis intestinalis and aid in patient selection for surgery. This tool may better inform the surgical decision-making process for patients with pneumatosis intestinalis.

Pneumatosis Intestinalis Due to COVID-19 Infection in Kidney Transplant Recipient: A Case Report.

BACKGROUND: Pneumatosis intestinalis (PI) is a rare condition usually occurring among adults who have undergone solid organ transplant and are taking steroid therapy. The coronavirus disease 2019 (COVID-19) virus uses angiotensin-converting enzyme 2 in gastrointestinal epithelium as a receptor for entry process. Due to the steroid intake, the COVID-19 virus is present in the patient's gastrointestinal tract for extended period of time. It may therefore increase the possibility of PI in such patients. It is usually asymptomatic, with a clinical spectrum ranging from indolent to life-threatening. Unfortunately, there are no algorithms concerning diagnosis and treatment of PI. AIM OF STUDY: The aim of this study is to highlight the problem of PI induced by COVID-19, especially in high-risk groups such as solid organs recipients. CONCLUSION: On the basis of the presented case of a severe course of COVID-19-induced PI, we conclude that laparotomy with bowel resection can be a feasible and a safe option for treatment.

Asymptomatic pneumoperitoneum in the setting of pneumatosis intestinalis: a benign entity or surgical emergency?

Pneumatosis intestinalis is a potential cause of asymptomatic pneumoperitoneum without peritonitis. The disease can be managed conservatively and presents a clinical scenario where pneumoperitoneum does not necessitate surgical management. This case illustrates the importance of acknowledging the condition and its variable presentation, allowing for increased awareness and avoidance of invasive procedures when not indicated.

Case Report: An unusual case of small bowel volvulus due to appendicitis associated with pneumatosis intestinalis: Review of the literature.

Pneumatosis cystoid intestinalis is a rare disease reported in the literature affecting 0.03% of the population. It has a variety of causes and its manifestation may change widely. It usually presents as a marginal finding resulting from various gastrointestinal pathologies. In the acute complicated form of pneumatosis intestinalis, management is challenging for physicians and surgeons. We present a case of a 60-year-old patient who was admitted to our surgical department with a symptomatology suggestive of small bowel occlusion. Computed tomography demonstrated ileal volvulus associated with parietal signs suffering and pneumoperitoneum. An emergent exploratory laparoscopy followed by conversion was performed demonstrating segmental ileal pneumatosis intestinalis secondary to a small bowel volvulus due to an inflammatory appendix wrapping around the distal ileum. Further, detorsion, retrograde draining, and appendectomy were performed because there were no signs of necrosis and the appendix was pathological. The postoperative course was uneventful. This case is exceedingly rare in the literature, because it was featured by the ileal volvulus due to appendicitis.This case report emphasizes the importance of surgical procedures in the management of symptomatic pneumatosis intestinalis associated to an acute abdomen.

Development and validation of a five-factor score for prediction of pathologic pneumatosis.

BACKGROUND: The significance of pneumatosis intestinalis (PI) remains challenging. While certain clinical scenarios are predictive of transmural ischemia, risk models to assess the presence of pathologic PI are needed. The aim of this study was to determine what patient factors at the time of radiographic diagnosis of PI predict the risk for pathologic PI. METHODS: We conducted a retrospective cohort study examining patients with PI from 2010 to 2016 at a multicenter hospital network. Multivariate logistic regression was used to develop a predictive model for pathologic PI in a derivation cohort. Using regression-coefficient-based methods, the final multivariate model was converted into a five-factor-based score. Calibration and discrimination of the score were then assessed in a validation cohort. RESULTS: Of 305 patients analyzed, 102 (33.4%) had pathologic PI. We identified five factors associated with pathologic PI at the time of radiographic diagnosis: small bowel PI, age 70 years or older, heart rate 110 bpm or greater, lactate of 2 mmol/L or greater, and neutrophil-lymphocyte ratio 10 or greater. Using this model, patients in the validation cohort were assigned risk scores ranging from 0 to 11. Low-risk patients were categorized when scores are 0 to 4; intermediate, score of 5 to 6; high, score of 7 to 8; and very high risk, 9+. In the validation cohort, very high-risk patients (n = 17; 18.1%) had predicted rates of pathologic pneumatosis of 88.9% and an observed rate of 82.4%. In contrast, patients labeled as low risk (n = 37; 39.4%) had expected rates of pathologic pneumatosis of 1.3% and an observed rate of 0%. The model showed excellent discrimination (area under the curve, 0.90) and good calibration (Hosmer-Lemeshow goodness-of-fit, p = 0.37). CONCLUSION: Our score accurately stratifies patient risk of pathologic pneumatosis. This score has the potential to target high-risk individuals for expedient operation and spare low-risk individuals invasive interventions. LEVEL OF EVIDENCE: Prognostic Study, Level III.

Pneumatosis cystoides intestinalis: a rare cause of acute abdomen.

We present the case of an 87-year-old male with Parkinson's disease, admitted to the Emergency Room due to acute abdomen, without leukocytosis or neutrophilia, with PCR of 0.74 and lactate of 2.5. The emergency abdominal computed tomography (CT) scan showed a pneumoperitoneum and significant intestinal pneumatosis on the small bowel loops in the right abdomen. There was a 3.2 cm slight dilation, without portal venous gas and an adequate opacification of the superior mesenteric artery and its main branches. An emergency laparotomy was performed that showed subserosal cysts in the jejunum and ileum, with no signs of transmural perforation. The patient was discharged after a good postoperative evolution.