Pneumatosis cystoides intestinalis: a benign differential diagnosis for computed tomographic evidence of pneumoperitoneum in a stable patient

Abstract

We present an 88-year-old man who was reviewed in a regional hospital with nausea, vomiting, generalized abdominal distension and vague pain for 3 days. Computed tomography (CT) was performed, and this was reported as perforated viscus with intramural gas and pneumoperitoneum (Figs 1,2). The patient was urgently transferred to a tertiary referral hospital for an urgent laparotomy.He was assessed in the emergency department following transfer and clinically had a soft, slightly tender and distended abdomen but with no clinical signs of peritonism. However, based on the radiological findings of intramural gas, pneumoperitoneum and suggestions of a partial small bowel obstruction without a transition point, a decision was made to perform a laparotomy. There was no evidence of necrotizing enterocolitis at laparotomy. There was a small bowel obstruction without any ischaemic segment, and careful adhesiolysis was performed. Incidentally, there were numerous segments of jejunal and ileal air-filled cysts and large jejunal diverticula and several small bowel cystic lesions were excised for histology.Post-operatively, the pathologist confirmed the diagnosis of pneumatosis cystoides intestinalis (PCI) (Fig. 3). The cysts ranged in size from 1 to 20 mm in diameter. Microscopically, the specimens consisted of cystic spaces lined by histiocytes and multinucleated foreign body giant cells on a background of dilated and congested blood vessels.PCI is a rare benign condition that has a similar CT appearance to perforated viscus. In the literature, there have been a number of case reports and series on PCI. Most are found incidentally during laparoscopy or laparotomy for other conditions. Fig. 2. Computed tomographic findings suggestive of free locules of gas and pneumoperitoneum.Fig. 3. Intra-operative finding of lobulated cystic structures within small bowel mesentery and attached to jejunum and ileum.Fig. 1. Computed tomographic finding suggestive of pneumatosis intestinalis highly suspicious for necrotizing enterocolitis.

Pneumatosis intestinalis: Not always bowel ischemia

“…However, there are other causes of PI where surgery is not indicated. Clinical correlation is key in order to avoid unnecessary surgical intervention and associated morbidity [7] . A benign etiology should be considered if the patient's clinical status does not reflect a sinister pathology.…”

Neumatosis intestinal: ¿una urgencia abdominal?

“…La clínica de los pacientes con neumatosis secundaria a enfermedad benigna es de dolor abdominal leve o incluso son asintomáticos (2,8,16), mientras que los pacientes con enfermedad que amenaza la vida presentan síntomas agudos (1), como dolor abdominal severo, deposiciones con sangre, distensión abdominal (1) o incluso signos y síntomas de irritación peritoneal (8).…”

“…Por lo general son pacientes asintomáticos o tienen síntomas inespecíficos (1) como disnea o dolor abdominal crónico (1,16), se ha asociado el uso de esteroides y quimioterapia con esta entidad (1).…”

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

“…The pathogenesis of PCI is not well established, and there are 4 main theories that may explain its origin: bacterial, mechanical, biochemical, or pulmonary 2,9,11,12,[18][19][20] . PCI classically affects the digestive tract, but some studies have reported possible mesentery, omentum, and hepatogastric ligament involvement 2,21 .…”