Background: Pneumatosis cystoides intestinalis (PCI) is a disorder in which widespread air sacs are present in mucosa, submucosa, subserosa, and intraabdominal area of the intestinal wall. It has a heterogeneous clinical presentation as a rare complication of intestinal graft-versus-host disease (GVHD). Computed tomography is the preferred imaging method for the diagnosis. Since the air sacs could be ruptured spontaneously, the presence of free air in the peritoneal cavity does not confirm intestinal perforation. The conservative treatment approach is sufficient in cases that do not require urgent surgical intervention, such as perforation or obstruction.
Case: Here, we present a 2.5-year-old patient diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH), who underwent allogeneic hematopoietic stem cell transplantation from a matched unrelated donor (MUD) and developed PCI secondary to intestinal GVHD 14th months after HSCT.
Conclusions: Pneumatosis cystoides intestinalis, which is a rare complication, should be kept in mind, especially in patients with intestinal GVHD and receiving intensive immunosuppressive, octreotide, and steroid treatment after HSCT.
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http://dx.doi.org/10.1111/petr.14136 | DOI Listing |
Pneumatosis cystoides intestinalis can present with concurrent diaphragmatic cysts, a previously undocumented phenomenon. Surgical management may be required, but further investigation is needed to understand the pathogenesis and optimize management in atypical and chronic cases, such as this case with a history of corrected intestinal malrotation.
View Article and Find Full Text PDFS Afr J Surg
October 2024
Third Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Greece.
Pneumatosis intestinalis (PI) is characterised by pathological gas infiltration into the submucosa and subserosa of the gastrointestinal tract, sometimes with an unclear pathogenesis. The clinical presentation of PI varies, with the diagnosis established via computed tomography (CT), where PI manifests as linear or bubbly gas patterns within the bowel wall. Management often necessitates surgical intervention to address potential life-threatening causes like mesenteric ischemia or bowel necrosis.
View Article and Find Full Text PDFANZ J Surg
November 2024
Melbourne University, Melbourne, Victoria, Australia.
Int J Surg Pathol
October 2024
PathologyOutlines.com Owner and Founder.
Clin Rheumatol
December 2024
Department of Rheumatology and Immunology, Beijing Friendship Hospital, Capital Medical University, 95 Yong'an Road, Beijing, 100050, China.
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