Presentation
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. [ncbi.nlm.nih.gov]
Entire Body System
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Turkish
A 55-year-old Turkish male presented to the emergency department with complaints of recurrent abdominal pain and vomiting. Free air was detected in abdominal x-ray and abdominal computed tomography (CT). [ncbi.nlm.nih.gov]
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Relapsing Polychondritis
Relapsing polychondritis (RP) is a rare systemic disease characterized by an inflammatory process involving predominantly cartilaginous structures. Pneumatosis cystoides intestinalis (PCI) in RP has not been reported previously. [ncbi.nlm.nih.gov]
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Fulminant Sepsis
From the clinical and histological findings, we hypothesized that PCI initially occurred, and intestinal bacterias invaded into vessels through broken mucosal barrier and developed fulminant sepsis. [ncbi.nlm.nih.gov]
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Camping
Department of Radiology, Marienhospital Herne, Ruhr-University Bochum, Bochum, Hölkeskampring 40, 44625 Herne, Germany (3) Department of Pathology, Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil, Ruhr-University Bochum, Bürkle de la Camp-Platz [jmedicalcasereports.biomedcentral.com]
Gastrointestinal
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Lower Abdominal Pain
A 79-year-old Asian man presented to our emergency department after 2 days of lower abdominal pain with nausea and constipation. [ncbi.nlm.nih.gov]
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Upper Abdominal Pain
A 29-year-old man visited our hospital for right upper abdominal pain. Computed tomography (CT) scan revealed multiple air-filled cysts along the intestinal wall and a pseudokidney sign in the transverse colon. [karger.com]
Workup
X-Ray
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Pneumoperitoneum
PCI with pneumoperitoneum induced by α-glucosidase inhibitor is rare, with only 27 cases (excluding the present case) reported in Japan to date. [ncbi.nlm.nih.gov]
The latter is a sign of pneumoperitoneum. (B) An upright view revealed an air-fluid level and pockets of free air under the diaphragm (arrows). [amjmed.org]
Y.J....Zhang J. 2018 4 Stenotic Ischemic Enteritis with Concomitant Hepatic Portal Venous Gas and Pneumatosis Cystoides Intestinalis: A Case Report. ( 29491303 ) Iwai N....Itoh Y. 2018 5 Surgical treatment of pneumatosis cystoides intestinalis with pneumoperitoneum [malacards.org]
[…] cysts, but when these cysts rupture they may produce pneumoperitoneum and hence complicate the clinical scenario. [transbiomedicine.com]
Other ECG Findings
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Ischemic Changes
However, neither ischemic change in the colonic wall nor ascites could be seen in the CT scan. Then, we performed an enema with gastrographin. [karger.com]
Treatment
The patient started prednisone treatment, but one month later treatment with voglibose, an alpha-glucosidase inhibitor (alpha-GI), was started because of prednisone-induced diabetes mellitus. [ncbi.nlm.nih.gov]
Prognosis
Pneumatosis cystoides intestinalis and hepatic portal venous gas suggests the presence of severe bowel ischemia associated with a very poor prognosis. An appropriate treatment should be performed immediately. top of page [minervamedica.it]
There are 6 cases of PCI with varied causes encountered, in which the etiology, endoscopic features, treatment methods and prognosis of patients were studied. [ncbi.nlm.nih.gov]
[…] mimic the appearance of pneumatosis on conventional radiographs CT will show air in bowel wall on dependent surface in pneumatosis Air in the lumen should rise to the highest non-dependent part of lumen Treatment None is usually needed for primary form Prognosis [learningradiology.com]
Two features of PCI have been described in the literature—cystic circumferential gas is associated with good prognosis, and linear gas is associated with poor prognosis [ 2, 11 ]. [surgicalcasereports.springeropen.com]
Infectious pneumatosis cystoides intestinalis carries a very poor prognosis, with an overall mortality rate as high as 60% to 70%. [medigoo.com]
Etiology
There are 6 cases of PCI with varied causes encountered, in which the etiology, endoscopic features, treatment methods and prognosis of patients were studied. [ncbi.nlm.nih.gov]
General Benign, generally asymptomatic subset of pneumatosis intestinalis. [1] Small or large bowel. [2] Possible etiologies: [3] IBD. Infection. Bowel necrosis. Malignancy. Drugs, e.g. alpha-glucosidase inhibitors. Idiopathic. [librepathology.org]
Epidemiology
Scleroderma epidemiology. (2003) Rheum Dis Clin North Am 29(2): 239-254. 2. Gompels, B.M. Pneumatosis cystoides intestinalis associated with progressive systemic sclerosis. (1969) Br J Radiol 42(501): 701-703 3. [ommegaonline.org]
Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases. ( 27651961 ) Kaneko M....Watanabe T. 2016 28 A Case of Intussusception Associated with Pneumatosis Cystoides Intestinalis. ( 27721738 ) Fujiya T....Tadokoro K. 2016 29 Epidemiological [malacards.org]
They also found no studies on epidemiology or randomised control trials on PCI. [surgicalcasereports.springeropen.com]
Pneumatosis Intestinalis Predictive Evaluation Study (PIPES): A multicenter epidemiologic study of the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg. 2013; 75 (1): 15-23[ DOI ][ PubMed ] 2. Galandiuk S, Fazio VW. [colorectalresearch.com]
Epidemiology and clinical features of Cryptosporidium infection in immunocompromised patients. Clin Microbiol Rev. 2002;15(1):145-154. 19. Marigold JH. Pneumatosis cystoides coli and chloral hydrate. Gut. 1998;42(6):899-900. 20. Florin TH. [consultant360.com]
Pathophysiology
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. [ncbi.nlm.nih.gov]
The pathophysiology is incompletely understood. [nejm.org]
Discussion Although more than a century has passed since the original description by Bang 12 of PCI in a human setting the underlying pathophysiology remains unclear. [nature.com]
Improving knowledge of the natural history, pathophysiology and prognostic factors of SSc among the various specialties involved may help with treatment decisions. [ommegaonline.org]
Prevention
Prevention of bacterial endocarditis. [books.google.com]
Surgery neither resolves PCI secondary to SSc nor does it prevent its recurrence. Several lessons emerge from these two cases. SSc in older adults is rare, but may be more severe and progressive when present. [ommegaonline.org]
It is likely that cysts prevented any increase of intrarectal pressure, particularly which followed the arrival of stools into the rectal ampulla. [em-consulte.com]
Contrast Enhanced Computerised Tomography (CECT) scan of the abdomen is a better modality for diagnosing Pneumatosis cystoides intestinalis (PCI) and it can prevent unnecessary surgeries. [transbiomedicine.com]