Pneumatosis cystoides intestinalis (PCI), characterized by a collection of gas-filled cysts in the intestinal wall, is an uncommon but well-known condition in gastroenterology. Abdominal pain is the most frequent symptom associated with PCI. Intussusception represents a potential cause of recurrent abdominal pain or emergency presentation. However, the occurrence of colonic intussusception secondary to PCI is very unusual in adulthood.

A 52-year-old male, known with idiopathic PCI, presented seventeen months after initial diagnosis with a new right upper quadrant pain. A computed tomography-scan demonstrated a colonic intussusception at the hepatic flexure. PCI did not progress compared with initial investigation. The patient underwent an emergency right hemicolectomy.

Resection was recommended in this case because PCI proved to be persisting with no identified curable cause. Surgery allowed to address the underlying pathology, the potential relapse of intussusception, and the likely cause of recurrent abdominal pain, either invagination or PCI itself.

Pneumatosis intestinalis is a radiological finding with incompletely understood pathogenesis. To date, there are no protocols to guide surgical intervention.

A systematic review of literature, according to PRISMA criteria, was performed. Medline and PubMed were consulted to identify articles reporting on the items "emergency surgery, pneumatosis coli, and pneumatosis intestinalis" from January 2010 up to March 2022. This study has not been registered in relevant databases.

A total of 1673 patients were included. The average age was 67.1 ± 17.6 years. The etiology was unknown in 802 (47.9%) patients. Hemodynamic instability (246/1673-14.7% of the patients) was associated with bowel ischemia, necrosis, or perforation (p = 0.019). Conservative management was performed in 824 (49.2%) patients. Surgery was performed 619 (36.9%) times, especially in unstable patients with bowel ischemia signs, lactate levels greater than 2 mmol/L, and PVG (p = 0.0026). In 155 cases, surgery was performed without pathological findings.

Many variables should be considered in the approach to patients with pneumatosis intestinalis. The challenge facing the surgeons is in truly identifying those who really would benefit and need surgical intervention. The watch and wait policy as a first step seems reasonable, reserving surgery only for patients who are unstable or with high suspicion of bowel ischemia, necrosis, or perforation.

Pneumatosis cystoid intestinalis is a rare disease reported in the literature affecting 0.03% of the population. It has a variety of causes and its manifestation may change widely. It usually presents as a marginal finding resulting from various gastrointestinal pathologies. In the acute complicated form of pneumatosis intestinalis, management is challenging for physicians and surgeons. We present a case of a 60-year-old patient who was admitted to our surgical department with a symptomatology suggestive of small bowel occlusion. Computed tomography demonstrated ileal volvulus associated with parietal signs suffering and pneumoperitoneum. An emergent exploratory laparoscopy followed by conversion was performed demonstrating segmental ileal pneumatosis intestinalis secondary to a small bowel volvulus due to an inflammatory appendix wrapping around the distal ileum. Further, detorsion, retrograde draining, and appendectomy were performed because there were no signs of necrosis and the appendix was pathological. The postoperative course was uneventful. This case is exceedingly rare in the literature, because it was featured by the ileal volvulus due to appendicitis.This case report emphasizes the importance of surgical procedures in the management of symptomatic pneumatosis intestinalis associated to an acute abdomen.

Pneumatosis cystoid intestinalis is a rare disease reported in the literature affecting 0.03% of the population. It has a variety of causes and its manifestation may change widely. It usually presents as a marginal finding resulting from various gastrointestinal pathologies. In the acute complicated form of pneumatosis intestinalis, management is challenging for physicians and surgeons. We present a case of a 60-year-old patient who was admitted to our surgical department with a symptomatology suggestive of small bowel occlusion. Computed tomography demonstrated ileal volvulus associated with parietal signs suffering and pneumoperitoneum. An emergent exploratory laparoscopy followed by conversion was performed demonstrating segmental ileal pneumatosis intestinalis secondary to a small bowel volvulus due to an inflammatory appendix wrapping around the distal ileum. Further, detorsion, retrograde draining, and appendectomy were performed because there were no signs of necrosis and the appendix was pathological. The postoperative course was uneventful. This case is exceedingly rare in the literature, because it was featured by the ileal volvulus due to appendicitis.This case report emphasizes the importance of surgical procedures in the management of symptomatic pneumatosis intestinalis associated to an acute abdomen.