Pneumatosis cystoides intestinalis after organ transplantation

Abstract

Pneumatosis cystoides intestinalis (PCI) is a rare disorder of undetermined etiology which is associated with various medical and gastrointestinal conditions. Fewer than 50 cases of PCI occurring after organ transplantation have been reported in the transplant and surgical literature. All have occurred either after bone marrow, kidney, or liver transplantation. The etiology remains obscure but proposed factors include pretransplantation chemotherapy and radiotherapy, immunosuppressive therapy, graft-versus-host disease, opportunistic enteric infections, and a sympathetic reaction from an adjacent inflamed allograft. The patients are often mildly symptomatic, and asymptomatic pneumoperitoneum is seen not infrequently. If the patient does not have a serious underlying illness, then the prognosis is good, with spontaneous resolution occurring in most cases. We report the first case of PCI occurring after an orthoptic cardiac transplantation, and extensively review the literature of PCI occurring in the setting of organ transplantation. We conclude that PCI is often a benign and self-limited condition in this setting, and morbidity is usually related to another complication of transplantation. Even the presence of pneumoperitoneum usually is not associated with peritonitis, and most cases of PCI can be managed conservatively.