Introduction
Pneumoperitoneum, defined as air within the peritoneal cavity, is
typically a sign of a perforation of an abdominal viscus for which
emergent surgical management is indicated.1 A rare
cause of pneumoperitoneum is pneumatosis cystoides intestinalis, which
is characterized by subserosal or submucosal cysts of the
gastrointestinal (GI) tract.2 Potential sources of gas
that could lead to pneumatosis intestinalis (PI) include intraluminal
gas, bacterial gas, and pulmonary gas.3-5 Previously,
about 85% of pneumatosis intestinalis cases have been found to be
associated with a known condition, such as mesenteric or intestinal
ischemia,6 infectious etiology, collagen vascular
disease (e.g., scleroderma), iatrogenic etiology (e.g., due to
endoscopic procedure), medications (e.g., corticosteroids, lactulose,
chemotherapeutic agents), autoimmune disease,7,8pulmonary diseases (e.g., chronic obstructive pulmonary disease and
asthma),9 with the remaining being
idiopathic.10 Surgical intervention is typically
reserved for severe cases (e.g., involvement of pneumoperitoneum, bowel
necrosis, obstruction).11
To our knowledge, no cases of PI with concurrent focal pneumatosis
cystoides occurring from outside of the GI tract have been documented.
Further investigation into varying presentations and pathogenesis and
their associated outcomes of surgical vs. nonoperative treatment are
warranted to better characterize this disease. This is a report of a
20-year-old male with a history of congenital malrotation who presented
with pneumoperitoneum secondary to PI of unknown etiology, and focal
diaphragmatic pneumatosis cystoides of unknown significance who
underwent surgical management.