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Pneumatosis Cystoides Intestinalis


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]
  • We present a case of pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis that was treated successfully with hyperbaric oxygen.[ncbi.nlm.nih.gov]
  • The results of a contrast enema, computed tomography scan, and laparoscopy are presented. The patient had an uneventful hospital course without any specific therapy. Causes and possible therapeutic options are discussed.[ncbi.nlm.nih.gov]
  • Four cases presented here demonstrate the diversity of the associated diseases.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: 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]
Relapsing Polychondritis
  • Abstract 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]
Lymphadenopathy
  • Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31 . Arch Pathol Lab Med . 2003 ;127(3): 341 – 344 . [Abstract] [Google Scholar] 23.[archivesofpathology.org]
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]
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]
Epigastric Pain
  • He arrived at the emergency department with intense non-radiating epigastric pain accompanied with nausea, but with no vomiting or fever.[revistagastroenterologiamexico.org]
  • CASE A 66-year-old woman was admitted to the hospital with a one month history of intermittent epigastric pain.[kjpbt.org]
  • The physical examination revealed an epigastric pain, accompanied with the abdominal distension. No signs of the peritonitis. The other general clinical findings were the dyspnea, chest pain, and no fever.[ccij-online.org]
Recurrent Abdominal Pain
  • 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]
  • Full Text An 81-year-old man with an unremarkable medical history was in gastrointestinal follow-up consultation for constipation and recurrent abdominal pain.[revistagastroenterologiamexico.org]
Chronic Abdominal Pain
  • A 14-year-old girl was referred for evaluation of a several-year history of growth failure, chronic abdominal pain, and intermittent emesis.[consultant360.com]
  • abdominal pain. ( 25453720 ) Klimova K....MenchAcn FernA![malacards.org]
Abdominal Mass
  • The examination must include bowel sounds, any palpable resistance indicative of an abdominal mass, and signs of peritonism. Routine laboratory tests should be performed.[peertechz.com]

Workup

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]
  • Since PCI often results in pneumoperitoneum through rupture of the cysts, it is important to differentiate the benign form of pneumoperitoneum, in which no intervention is indicated, from the life-threatening form with intestinal infarction and/or perforation[ncbi.nlm.nih.gov]
  • PCI is a physical sign characterized by gas-filled cysts of the submucosa or subserosa of the bowel that can lead to pneumoperitoneum.[ncbi.nlm.nih.gov]
  • The patient was found to have an unsymptomatic pneumoperitoneum on plain chest x-ray. The results of a contrast enema, computed tomography scan, and laparoscopy are presented.[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]
Bloody Ascites
  • Edematous dark-brown colored lesions of mucosal surface were discontinuously observed from terminal ileum to sigmoid colon with bloody ascites.[ncbi.nlm.nih.gov]

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]
  • A laparoscopically assisted approach proved to be a good indication in subtotal colectomy in cases of PCI that are non-responsive to standard conservative treatment.[ncbi.nlm.nih.gov]
  • After combination of medicine and endoscopic treatment, the symptoms of five patients were relieved, while one patient died of malignant tumors.[ncbi.nlm.nih.gov]
  • In conclusion, we should consider the possibility of PCI occurring in patients with malignancies during chemotherapy treatment.[ncbi.nlm.nih.gov]
  • Diagnosis should be prudent because it can mimic pneumoperitoneum leading to unnecessary treatment such as surgical exploration.[ncbi.nlm.nih.gov]

Prognosis

  • CASE PRESENTATION: 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]
  • 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]
  • […] 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]
  • Infectious pneumatosis cystoides intestinalis carries a very poor prognosis, with an overall mortality rate as high as 60% to 70%.[medigoo.com]
  • PI preceded by bowel ischemia or graft versus host disease colitis has the worst prognosis, and the presence of portal venous gas and acidosis correlate with poor outcome. Not all patients with pneumoperitoneum require surgical intervention.[pediatrics.aappublications.org]

Etiology

  • CASE PRESENTATION: 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]
  • 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.[ncbi.nlm.nih.gov]
  • Abstract Pneumatosis cystoides intestinalis (PCI), with an unknown etiology, is an uncommon disease characterized by the presence of multiple gas-filled cysts within the submucosa or subserosa of the intestinal wall.[ncbi.nlm.nih.gov]
  • Although various drugs including anti-neoplastic agents have been suggested as etiologies, cases related to sunitinib are sparse. Because of the rarity of this unusual side effect by sunitinib, we report the case report.[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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • In the context of a review of the relevant literature, this case report illustrates the complex underlying pathophysiology, and difficulty in making a differential diagnosis and treating PCI.[ncbi.nlm.nih.gov]
  • The pathophysiology is incompletely understood.[nejm.org]
  • Improving knowledge of the natural history, pathophysiology and prognostic factors of SSc among the various specialties involved may help with treatment decisions.[ommegaonline.org]
  • Disadvantages of normobaric oxygen treatment are that excessive amounts of 100% oxygen are required to be inhaled for prolonged periods and this may produce pulmonary oxygen toxicity Keywords pneumatosis cystoides intestinalis pneumoperitoneum pathophysiology[link.springer.com]
  • The pathophysiology of the disease remains unknown.[em-consulte.com]

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]
  • The chemical theory, named also as nutritional deficiency theory, underlines that malnutrition can prevent the digestion of carbohydrates and increase bacterial fermentation in the intestine, producing large volumes of gas with distention and ischemia[peertechz.com]

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