Pneumatosis Cystoides Intestinalis: A Case Report

Background

Pneumatosis intestinal cysticercosis (PCI) is a rare disease wherein cystic gas is observed in the submucosal or serosal tissue of the intestinal wall. It reportedly occurs at all ages, most commonly at 45±15 years of age. Non-surgical treatment results in remission in 70% of patients [1]. However, PCI is often mistaken for other serious diseases due to the associated “severe” imaging findings, which may lead to unnecessary surgical procedures [2]. Here, we present a case of PCI to illustrate the associated key findings and highlight the importance of differentiating PCI from other diseases.

Case report

An 84-year-old woman was referred from a rehabilitation hospital to a tertiary care hospital with abdominal distension and diarrhea that had persisted for 2 weeks. She had an altered state of consciousness and was initially unable to speak; therefore, her subjective symptoms were unknown. Her vital signs were within normal ranges. During the initial physical examination, there was marked abdominal distension without perceptible tenderness. The patient’s medical history included dementia, pneumonia, and cerebral infarction. She was taking multiple medications, including prednisone, lansoprazole, and quetiapine. Blood test results (Table 1) showed that her blood urea nitrogen (10.7 mg/dL, normal: 6 to 24 mg/dL) and creatinine (0.86 mg/dL; normal: 0.7 to 1.3 mg/dL) were within normal limits. Blood gas analysis showed a mildly elevated lactate level (2.1 mmol/L, normal: 0.5–1 mmol/L). Simple abdominal radiography showed the presence of gas in the small bowel (Figure 1). Contrast-enhanced computed tomography (CT) showed free air, copious emphysema in the intestinal wall, and no portal gas or signs of intestinal ischemia (Figures 2, 3). After a multidisciplinary consultation involving surgeons, gastroenterologists, and radiologists, the patient was admitted to the hospital with a diagnosis of PCI with a good prognosis. Surgery was determined to be unnecessary, and the patient was managed with fasting and oral fluids. Oxygen was administered to reduce intestinal emphysema. As there were no signs of infection, no antimicrobials were administered. Three days later, a CT scan revealed that the abdominal distension and intestinal gas had decreased; therefore, oxygen administration was terminated, and food intake was initiated. On day 11 after admission, the patient’s abdominal symptoms improved, and she was discharged.

Discussion

PCI occurs in individuals 50 to 80 years of age, and its incidence is approximately 0.03% [3]. The cause is unknown, but it may involve multiple factors, including mechanical, biochemical, bacterial, and pulmonary disease-related influences [3]. In such cases, the differential diagnosis of other severe gastrointestinal diseases, such as intestinal ischemia, infarction, necrotizing enterocolitis, intestinal perforation, axial torsion, and sepsis, should be excluded by performing blood tests and imaging studies [4]. Table 2 shows the poor prognostic factors associated with PCI. Indicators for surgery include age over 60 years, vomiting, and a white blood cell count >12 000/μL [5].

Although PCI generally has a good prognosis, accurate diagnosis and timely surgery are important. Poor prognostic factors have been proposed, including intrahepatic portal gas, changes in blood test values, and negative predictors such as a pH <7.3, HCO3– level <20 mmol/L, and lactate level >2 mmol/L [6–8]. Despite the patient being elderly, she had normal vital signs and no subjective symptoms except lactate levels; therefore, surgery was not indicated for the patient. There were no findings of wall contrast failure or torsion indicative of intestinal ischemia on CT findings. Based on the diagnostic flowchart for PCI, the vascular risk score was 0 in STEP3 [9] (Figure 4). Therefore, the patient was monitored with follow-ups and imaging tests. The sensitivity and negative predictive value in STEP3 were 100%. In addition to abnormal clinical vital signs and severe abdominal pain, it is important to consider these indicators when planning treatment strategies, including surgery, to rapidly identify and manage severe PCI.

Conclusions

PCI is a rare condition with cystic gas in the intestinal wall and is often mistaken for other severe gastrointestinal diseases; therefore, physicians should carefully differentiate PCI from other severe diseases.