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.

Gastric pneumatosis intestinalis associated with malignancy:An unusual case report

Pneumatosis intestinalis(PI)is an uncommon disease defined as gas-filled cysts that are found in the wall of the gastrointestinal(GI)tract.The exact causes of PI are still unclear,but it may associated with coexisting diseases,such as some GI disorders,connective tissue disease,some medication and drugs,and rarely malignancy.The most common localization is the small intestine.Gastric PI secondary to malignancy has been rarely documented.We report on a 94-year- old man with gastric PI associated with inoperable adenocarcinoma localized in the duodenum.Following the gastrojejunostomy and choledochojejunostomy bypass,his general condition improved and PI disappeared,but he died due to poor performance status and malignancy 6 mo later.We suggest that in patients presenting with PI,malignancy should be considered in the differential diagnosis.

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.

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.

Acute gastric volvulus combined with pneumatosis coli rupture misdiagnosed as gastric volvulus with perforation:A case report

BACKGROUND Acute gastric volvulus represents a rare form of surgical acute abdomen,which makes it difficult to establish an early diagnosis.As the disease progresses,it can lead to gastric ischemia,necrosis,and other serious complications.CASE SUMMARY This paper reports a 67-year-old female patient with a history of abdominal distension and retching for 1 day.After admission,a prompt and thorough exami-nation was performed to confirm the diagnosis of acute gastric volvulus.Notably,the patient had free air in the abdominal cavity.The first consideration was gastric volvulus with gastric perforation,but the patient had no complaints,such as abdominal pain or signs of peritoneal irritation in the abdomen,and imaging examination revealed no abdominal pelvic effusion.Following endoscopic reduc-tion,the abdominal organs,such as the stomach and spleen,returned to their normal anatomical positions,and the free intraperitoneal air disappeared,su-ggesting a rare case of acute gastric torsion.The source of free air within the abdominal cavity warrants careful consideration and discussion.Combined with the findings from computed tomography,these findings are hypothesized to be associated with the rupture of colonic air cysts.CONCLUSION Patients with gastric torsion combined with free gas in the abdominal cavity should consider nongastrointestinal perforation factors to avoid misdiagnosis.

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.

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 (αGI) 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 αGI 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 αGI therapy have been published,predominantly in Japan where αGIs are commonly used. If the use of αGIs 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.

Pneumatosis cystoides intestinalis:A single center experience

AIM： To share our experience of the management and outcomes of patients with pneumatosis cystoides in- testinalis （PCI）. METHODS： The charts of seven patients who under- went surgery for PCI between 2001 and 2009 were re- viewed retrospectively. Clinical features, diagnoses and surgical interventions of patients with PCI are discussed. RESULTS： Seven patients with PCI （3 males, 4 fe- males; 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 lapa- rotomy, 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 pri- mary PCI when the surgical findings and medical his- tory were assessed together. Gastric atony developed in one case only, as a complication during a postopera- tive follow-up of 5-14 d. CONCLUSION： Although rare, PCl should be consid- ered in the differential diagnosis of acute abdomen. Diagnostic laparoscopy and preoperative radiological tests, including computed tomography, play an impor- tant role in confirming the diagnosis.

Pneumatosis cystoides intestinalis associated with massive free air mimicking perforated diffuse peritonitis

While pneumatosis cystoides intestinalis （PCI） is a rare disease entity associated with a wide variety of gastrointestinal and non-gastrointestinal disorders, PCI associated with massive intra- and retroperitoneal free air is extremely uncommon, and is difficult to diagnose differentially from perforated peritonitis. We present two cases of PCI associated with massive peritoneal free air and/or retroperitoneal air that mimicked perforated peritonitis. These cases highlight the clinical importance of PCI that mimics perforated peritonitis, which requires emergency surgery. Preoperative imaging modalities and diagnostic laparoscopy are useful to make an accurate diagnosis.

Pneumatosis cystoides intestinalis

Pneumatosis cystoides intestinalis(PCI) is a rare condition that may be associated with a variety of diseases.The presenting clinical picture may be very heterogeneous and represent a challenge for the clinician.In the present paper we describe both a common and an uncommon clinical presentation of PCI and review the pertaining literature.Our cases confirm that,apart from asymptomatic cases,the clinical presentation of PCI may be widely different and suggest that a new onset of stipsis might be the presenting symptom.Diagnosis might be suggested by a simple X-ray of the digestive tract showing a change in the characteristics of the intestinal wall in two-thirds of these patients.However,one third of the patients do not have a suggestive X-ray and require a computed tomography(CT) scan/nuclear magnetic resonance that may reveal a thickened bowel wall containing gas to confirm the diagnosis and distinguish PCI from intraluminal air or submucosal fat.CT also allows the detection of additional findings that may suggest an underlying,potentially worrisome cause of PCI such as bowel wall thickening,altered contrast mucosal enhancement,dilated bowel,soft tissue stranding,ascites and the presence of portal air.Our results also point out that clinicians and endoscopists should be aware of the possible presentations of PCI in order to correctly manage the patients affected with this disease and avoid unnecessary surgeries.The increasing number of colonoscopies performed for colon cancer screening makes PCI more frequently casually encountered and/or provoked,therefore the possible endoscopic appearances of this disease should be well known by endoscopists.

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.

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.

Dysplasia in perforated intestinal pneumatosis complicating a previous jejuno-ileal bypass:A cautionary note

We present the case of an elderly woman who developed a bowel perforation related to pneumatosis intestinalis, 33 years after a jejuno-ileal bypass for severe obesity. Final histological examination revealed the presence of dysplasia in the resected specimen. On the basis of our case and a review of the literature, we discuss the etiopathogenesis, the clinical aspects and the treatment of this rare condition.

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.

Portal Gas Embolism with Acute Liver Ischemia： Case Report

Portal gas embolism is a rare entity and considered a sign of poor prognosis in abdominal diseases, its mortality rate is about 75%. This case report is about a 61-year old patient with portal gas embolism and acute liver ischemia, evolving to death within 24 hours. Some cases reported in the literature demonstrate the severity of this dramatic condition. It is necessary to have a higher level of suspicion in the vascular structures of the abdomen in order to allow a timely treatment.