Pneumatosis cystoides intestinalis in scleroderma‐related conditions

Abstract

Recognition of PCI, particularly in the context of underlying CTD, is necessary for proper therapeutic application. In patients with underlying CTD and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including rheumatologist, gastroenterologist, imaging specialist and surgeons familiar with intestinal complications of CTD-related conditions, is warranted.

Combination therapy for systemic sclerosis-associated pneumatosis intestinalis

“…The increased non-oxygen PaO 2 in the cysts causes the gas to diffuse out of the high-pressure cyst into the blood stream. Cyst resolution subsequently follows as oxygen reabsorption occurs for use in cellular metabolism 19. In addition to high-flow inhalation oxygen therapy, hyperbaric oxygen therapy has also been put forward as a management option for patients with PI, with a similar mechanism of action and is a subject of recent reviews and case reports,19 20 the obvious limitation being availability of hyperbaric facilities.…”

Professor Witold Nowicki – a greatly spirited pathologist

“…Pneumatosis cystoides can develop on the basis of various disorders of the gut, such as enteritis, inflammatory bowel disease, neonatal necrotizing enterocolitis, infectious colitis, disorders of connective tissue, diverticulosis as well endoscopic interventions, autoimmune diseases, pulmonary diseases such as cystic fibrosis, chronic obstructive lung disease, etc. [10,11,12,13].…”

Management of Gastrointestinal Involvement in Scleroderma

“…It is usually of no consequence, but sometimes can be life threatening in the event of a pneumoperitoneum [128]. Usually these cysts do not require surgery; yet, it is important to be aware of this rare condition.…”