Monica Onorati1*, Marta Nicola1, Milena Maria Albertoni1, Isabella Miranda Maria Ricotti1, Matteo Viti2, Corrado D’urbano2 and Franca Di Nuovo1
1Pathology Unit, ASST-Rhodense, Garbagnate Milanese, Italy
2Surgical Unit, ASST-Rhodense, Garbagnate Milanese, Italy7>
Received: 09 January, 2017; Accepted: 07 March, 2017; Published: 08 March, 2017
Monica Onorati, MD, Pathology Unit, ASST-Rhodense, v.le Carlo Forlanini, 45, 20024, Garbagnate Milanese (MI), Italy, Tel: 02994302392; Fax: 02994302477; E-mail:
Onorati M, Nicola M, Albertoni MM, Maria Ricotti IM, Viti M, et al. (2017) Pneumatosis Cystoides Intestinalis: Report of a New Case of a Patient with Artropathy and Asthma. Arch Clin Gastroenterol 3(1): 017-020. 10.17352/2455-2283.000031
© 2017 Onorati M, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Pneumatosis cystoides intestinalis (PCI) is an uncommon entity without the characteristics of a disease by itself and it is characterized by the presence of gas cysts within the submucosa or subserosa of the gastrointestinal tract. Its precise etiology has not been clearly established and several hypotheses have been postulated regarding the pathogenesis. Since it was first described by Du Vernoy in autopsy specimens in 1730 and subsequently named by Mayer as Cystoides intestinal pneumatosis in 1825, it has been reported in some studies. PCI is defined by physical or radiographic findings and it can be divided into a primary and secondary forms. In the first instance, no identifiable causal factors are detected whether secondary forms are associated with a wide spectrum of diseases, ranging from life-threatening to innocuous conditions. For this reason, PCI management can vary from urgent surgical procedure to clinical, conservative treatment. The clinical onset may be very heterogeneous and represent a challenge for the clinician. We report the case of a 54-year-old woman with PCI associated with artropathy and asthma and a long-lasting steroid therapy. Our purpose is to underline the correlation of PCI with artropathy and asthma. Moreover we would like to describe the difficulties to diagnose this enity, avoiding a misdiagnosis and therefore an incorrect therapy.
Pneumatosis cystoides intestinalis (PCI) is an uncommon entity characterized by the presence of multiple gas cysts within the submucosa or subserosa of the gastrointestinal tract. The precise etiology has not been clearly established and several hypotheses have been postulated regarding its pathogenesis. It has been divided into two forms: primary or idiophatic and secondary to a wide spectrum of other diseases, ranging from life-threatening to innocuous conditions. The first pathologic description of PCI has been attributed to Du Vernoy, a French pathologist who described it during an autopsy dissection in 1730 . Subsequently it was named by Mayer as Cystoides intestinal pneumatosis in 1825 and Gazin et al. , described the first clinical report in a patient who underwent surgery in 1946. The correlation to asthma has been described only in four papers [3-6]. No previous studies have ever hypothesized a possible association with arthropathies as in our case. This paper describes a case of PCI in a patient who suffered from artropathy and asthma and had a long-lasting history of steroid therapy. Our purpose is to underline the possible correlation between the PCI and these diseases. We would like to stress the difficulties in the correct identification of PCI, avoiding a misdiagnosis and an incorrect therapeutic treatment. It would be desirable to provide guidelines that summarize a multidisciplinary approach to PCI to support a decision-making process. It may help to reduce the rate of benign forms of PCI that are unnecessarily subjected to exploratory surgery, and to shorten the delay in surgical procedures for patients who would profit from early intervention, as in cases with acute complications such as bowel necrosis, perforation, peritonitis, intestinal occlusion and multiple comorbidities like in our case.
A 54-year-old woman suffering from multiple and recurrent intestinal subocclusive episodes, presented to our hospital for severe, generalized abdominal pain, nausea, vomiting, and constipation, without episodes of melena and fever. For several decades her past medical history consisted of hypertension, hyperlipidemia, obesity correlated to hypothyroidism, asthma exacerbated by smoke and artropathy with severe gonarthrosis. Due to the latter diseases, she had been taking a steroid daily therapy for several years (for asthma, inhaled budesonide: a dose of 160 mcg/day; for gonarthrosis, intraarticular methylprednisolone 120 mg/week). On physical examination, abdomen was distended with no bowel sounds and with a generalized pain upon palpation. Test for fecal occult blood was negative. There was evidence of inflammation due to her abdominal symptoms and laboratory signs including elevated serum C-reactive protein levels (20 mg/dl; reference range: 0-0,5mg/dl) and leukocytosis (14x103/ml; reference range: 3-9 x103/ml). Serum calcium (9,3 mg/dl; reference range: 8,80-10,20 mg/dl), kidney (creatinin: 0,8 mg/dl; reference range: 0,5-1,2 mg/dl) and liver function (ALT: 20U/L, reference range: 4-44U/L; AST: 22U/L, reference range: 8-38U/L) were normal. Computed tomography (CT) scan without intravenous contrast medium confirmed, at the splenic flexure, multiple air pockets consisting of isolated and clusters of bubbles thickening the intestinal wall. Moreover air-fluid levels were detected. At colonoscopy, several mucosal polypoid cystic bluish masses, 5 mm to 30 mm in diameter, were noted in the splenic flexure of the colon. These lesions were easily indented with gentle pressure and the overlying mucosa was soft and pinkish. Biopsies were not performed. The other colonic tracts were normal. Since the generalized peritonitis, after a multidisciplinary evaluation, the patient underwent laparoscopic left colectomy to avoid the risk of perforation. At gross examination, the surgical specimen showed numerous bubbles in the wall of the colon. They were single and in clusters and ranged from a few millimetres to 30 mm. in size (Figure 1). Microscopically, several cystic spaces were detected into the mucosa, submucosa and subserosa of the bowel wall. The cysts were partially lined by inflammatory cells including leukocytes, eosinophils, plasma cells, lymphocytes, foreign body cells and macrophages. In some portion of the cystic spaces no lining cells could be appreciated [Figure 2a,b]. Based on these morphological findings, histology was consistent with PCI. Moreover, a strong correlation with the long-lasting steroid therapy for artropathy, severe gonarthrosis and asthma was suspected, inducing clinicians to decrease gradually the dose of drugs. The patients did not require any other specific treatment and she has remained asymptomatic for PCI since two years.
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