Pneumatosis Cystoides Intestinalis: Report of a New Case of a Patient with Artropathy and Asthma

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 fi rst 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 defi ned by physical or radiographic fi ndings and it can be divided into a primary and secondary forms. In the fi rst instance, no identifi able 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 diffi culties to diagnose this enity, avoiding a misdiagnosis and therefore an incorrect therapy. Case Report Pneumatosis Cystoides Intestinalis: Report of a New Case of a Patient with Artropathy and Asthma 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, Italy Dates: Received: 09 January, 2017; Accepted: 07 March, 2017; Published: 08 March, 2017 *Corresponding author: Monica Onorati, MD, Pathology Unit, ASST-Rhodense, v.le Carlo Forlanini, 45, 20024, Garbagnate Milanese (MI), Italy, Tel: 02994302392; Fax: 02994302477; E-mail: https://www.peertechz.com


Introduction
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 fi rst pathologic description of PCI has been attributed to Du Vernoy, a French pathologist who described it during an autopsy dissection in 1730 [1]. Subsequently it was named by Mayer as Cystoides intestinal pneumatosis in 1825 and Gazin et al. [2], described the fi rst clinical report in a patient who underwent surgery in 1946. The correlation to asthma has been described only in four papers [3][4][5][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 diffi culties in the correct identifi cation 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 profi t from early intervention, as in cases with acute complications such as bowel necrosis, perforation, peritonitis, intestinal occlusion and multiple comorbidities like in our case.

Results
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.

Conclusions
PCI is an uncommon entity, without the characteristics of a disease by itself, characterized by the following symptoms in decreasing order of frequency: diarrhea, abdominal pain, abdominal distension, bloody stool, constipation, weight loss, and tenesmus but many patients are asymptomatic [3,7]. These clinical presentation is caused by the presence of multiple gas-fi lled cysts within the submucosa or subserosa of the intestinal wall.
It can be divided into a primary or idiopathic (15%) form without identifi able factors and secondary (85%) when associated with predisposing factors [8].
In 1952, Koss observed that 15% of cases were primary or idiopathic PCI, while 75% were considered secondary PCI, and 10% had an unknown underlying disease. The majority of secondary PCI cases can be related to gastrointestinal disorders, followed by pulmonary, connective tissue, and hematological disease, immunosuppressed state, use of steroid and cytotoxic drugs [3,6,[9][10][11][12][13]. PCI can also be observed in patients who undergo liver and bone marrow transplantation [14][15].
The most frequent site of involvement is the ileum, followed by the colon, even if in nearly 20% of cases both of them are affected. Recently the incidence of colonic involvement is increased due to the use of colonoscopy and barium enemas [8]. In our case, a tract of the large bowel was affected; while the small bowel was spared. From a radiological point of view, three patterns of PCI have been described: a bubble-like or cystoid (i.e. bubbles of gas with a cystic appearance), linear (i.e. curvilinear or crescent shape) and circular or circumferential.
In some cases more than one pattern may be present.
Although the exact etiology of PCI remains obscure, clinicians believe that it has a multifactorial etiology that could explain the cyst formation and the abnormal accumulation of gas into them. Several theories have been suggested. The most   important theories are: mechanical, pulmonary, bacterial, chemical and lymphatic. The mechanical theory is thought to be due to increased intraluminal pressure causing the intramural accumulation of gas [16,17]. The pulmonary theory (pulmonary diseases, such as chronic obstructive pulmonary disease, asthma, and interstitial pneumonia) is explained by pulmonary alveolar rupture producing a pneumomediastinum that dissects along the aorta and then along the mesenteric vessels to the bowel wall. In the bacterial theory, the gas is produced by gas-forming bacteria that enter the mucosal barrier through mucosal rents or increased mucosal permeability and produce the gas within the bowel wall. Indirect support for this theory was obtained by the successful treatment of PCI with antibiotics. The chemical theory, named also as nutritional  [19]. In the current case, surgery was the best choice of management because of the recurrent episodes of intestinal sub occlusion worsened by pre-existent comorbidities. The variety of treatments refl ect the poor knowledge regarding the complications of this disease. In the last years, some authors [20][21][22]