Pneumatosis cystoides intestinalis – an incidental finding with unpredictable evolution

Abstract

Pneumatosis cystoides intestinalis is an uncommon disease with unknown etiology characterized by the presence of multiple gas-filled cysts within the submucosa or subserosa of the intestinal wall. Pneumoperitoneum and/or intestinal perforation are complications that may be associated with pneumatosis cystoides intestinalis. The patients are often prone to misdiagnosis or mistreatment. We are presenting a case of pneumatosis cystoides intestinalis in a 42 year-old woman affected by peritoneal free air and numerous, diffuse, bubble-like intramural gas collections into the jejunum and ileum, showed in CT-enterography images. The woman had a carcinoid tumor located in jejunum two years ago, treated with enterectomy. Recent complaints of nonspecific symptoms of abdominal discomfort and diarrhea motivated the realization of CT scan, serum chromogranin and urine 5-hidroxindolacetic acid for hypothesis of tumor carcinoid recurrence withdraw. The only change found was the presence of pneumatosis cystoides intestinalis in CT-enterography images without intestinal necrosis, bleeding or evident obstruction. For that reason no surgical procedure was realized and the patient stayed on surveillance. Actually, the patient complaints are sporadic abdominal discomfort, without pneumatosis cystoides intestinalis clinical evidence. Conclusion The treatment plan of patient with PCI depends on underlying cause and clinical condition severity. When conservative treatment is adopted the clinical evolution of pneumatosis cystoides intestinalis is unpredictable and can even disappear in an indeterminate number of patients.

An unusual case of small bowel volvulus due to pneumatosis cystoides intestinalis: Case report and literature review

“…In general, the existence of free gas in the abdominal cavity corresponds to the perforation of hollow viscera [ 8 , 9 ]. Pneumoperitoneum is the presence of gas in the peritoneal cavity, represented by subdiaphragmatic radiolucency, which suggests rupture of hollow viscera in about 85–95 % of cases [ [8] , [9] , [10] ].…”

“…In general, the existence of free gas in the abdominal cavity corresponds to the perforation of hollow viscera [ 8 , 9 ]. Pneumoperitoneum is the presence of gas in the peritoneal cavity, represented by subdiaphragmatic radiolucency, which suggests rupture of hollow viscera in about 85–95 % of cases [ [8] , [9] , [10] ]. This radiological sign, as well as a clinical presentation of abdominal distension, febrile syndrome, absence of leukocytosis, and peritoneal signs, puts pneumatosis cystoid intestinalis as a diagnostic possibility [ [8] , [9] , [10] , [11] , [12] ].…”

“…Pneumoperitoneum is the presence of gas in the peritoneal cavity, represented by subdiaphragmatic radiolucency, which suggests rupture of hollow viscera in about 85–95 % of cases [ [8] , [9] , [10] ]. This radiological sign, as well as a clinical presentation of abdominal distension, febrile syndrome, absence of leukocytosis, and peritoneal signs, puts pneumatosis cystoid intestinalis as a diagnostic possibility [ [8] , [9] , [10] , [11] , [12] ]. Nevertheless, in patients with PCI, pneumoperitoneum could reflect the rupture of one or more cysts without visceral rupture, as in our case, intraoperatively, we don't evidence any sign of hollow visceral perforation; nevertheless, there are changes in the small intestine and in the mesentery that reflects chronic changes.…”

Management of pneumatosis cystoides intestinalis with pneumoperitoneum: 5-years systematic review

“…Some predisposing factors may be involved, including scleroderma, systemic lupus erythematosus, granulomatosis with polyangiitis, amyloidosis, myeloma, dermatomyositis, COPD, Crohn's disease, nutritional imbalances, dysbacteriosis, gastrointestinal dysmotility, alpha-glucosidase inhibitor, glucocorticoid, kinin, immunosuppressant inhibitor, bone marrow transplantation, lung transplantation, graft versus host disease, and use of trichloroethylene 1,2,3,7,11,14,23,28,31,32 . Changes in immune function appear to be among the most significant predisposing factors, considering their relation with post-transplant organ status, corticotherapy, rheumatic disease treatment, and chemotherapy 2,33,34 .…”

“…PCI causes a wide spectrum of mild and nonspecific symptoms, such as changes in bowel habits, nausea, and abdominal discomfort 1,4,12,18,22 . In 3% of cases, it may be associated with complications such as pneumoperitoneum, volvulus, bleeding, intestinal obstruction, and mesenteric ischemia [16][17][18]22,23 . There is still no consensus on the need for diagnostic laparoscopy 24 .…”