Pneumatosis cystoides intestinalis: A rare complication after hematopoietic stem cell transplantation

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 (GVH...

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Bibliographic Details
Published in:Pediatric transplantation 2021-11, Vol.25 (7), p.e14136-n/a
Main Authors: Arman Bilir, Özlem, Demir, Arzu Meltem, Akçabelen, Yunus Murat, Ok Bozkaya, İkbal, Gürlek Gökçebay, Dilek, Güneş, Altan, Özbek, Namık Yaşar, Yaralı, Neşe
Format: Article
Language:English
Subjects:
Child, Preschool
Colonoscopy
complication
Computed tomography
Fatal Outcome
Graft vs Host Disease - complications
Graft-versus-host reaction
hematopoietic stem cell transplantation
Hematopoietic Stem Cell Transplantation - adverse effects
Hematopoietic stem cells
Histiocytosis
Humans
Intestine
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic - therapy
Male
Mucosa
Octreotide
Patients
pediatric
Peritoneum
pneumatosis cystoides intestinalis
Pneumatosis Cystoides Intestinalis - diagnosis
Pneumatosis Cystoides Intestinalis - etiology
Stem cell transplantation
Tomography, X-Ray Computed
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Summary: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.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.14136