Summary:Pneumatosis cystoides intestinalis is a rare finding of intramural gasfilled cysts in the bowel wall and sometimes free air in the abdomen. A few conditions are reported to cause this disease, one of them being immunosuppression. We describe a 50-year-old Caucasian male with extensive chronic graft-versus-host disease (GVHD) of the gut and skin who developed PCI with pneumoperitoneum and pneumoretroperitoneum. To our knowledge, this is the first report of PCI occurring in a patient with active chronic GVHD which resolved spontaneously. Keywords: Pneumatosis cystoides intestinalis; chronic graft-versus-host disease Pneumatosis cystoides intestinalis (PCI) is a rare disorder consisting of multiple intramural gas collections in the bowel wall. Patients are either asymptomatic or present with diarrhea, pain, tenderness, vomiting and flatulence. The pathogenesis of this abnormality is still unknown. It is associated with chronic obstructive pulmonary disease, necrotizing enterocolitis in premature infants, intestinal obstruction, ischemic bowel disorders, bacterial and viral infections 1 and drug therapy. 2 In contrast to other gastrointestinal diseases PCI is a benign finding and usually resolves spontaneously. It responds well to conservative therapy. Rarely, PCI is observed after allogeneic bone marrow transplantation (BMT) where fatalities have been reported in patients receiving immunosuppression.3,4 We describe a patient with myelodysplastic syndrome given an allogeneic marrow graft. He experienced extensive chronic graft-versus-host disease (GVHD) and developed PCI 8 months after BMT.
Case reportA 50-year-old Caucasian male who was diagnosed with myelodysplasia, subtype chronic myelomonocytic leukemia, underwent BMT after conditioning with cyclophosphamide and fractionated total body irradiation (12 Gy) at our institution. He received 3.2 × 10 8 nucleated bone marrow cells/kg body weight from his HLA-identical sibling. GVHD prophylaxis consisted of cyclosporin A and a short course of methotrexate according to the Seattle protocol.
5During aplasia, he experienced fever and Staphylococcus aureus bacteremia. On day +16 the patient developed acute GVHD grade II of the skin that resolved with corticosteroids (2 mg/kg/day). Two months after transplantation, he received pre-emptive gancyclovir therapy because of cytomegalovirus (CMV) reactivation. Five weeks later extensive GVHD of skin required treatment with steroids. Seven months after BMT, herpes encephalitis was diagnosed which resolved with acyclovir therapy. Another course of pre-emptive gancyclovir for CMV reactivation was also administered.One month later the patient presented with diarrhea, slight diffuse abdominal pain and flatulence. Parenteral nutrition and electrolyte and fluid infusions were started. Colonoscopy showed signs of diffuse mucosal irritation. On histological examination biopsy specimens from two regions of the colon showed individual crypt cell necrosis, crypt abscesses with cell flattening and degeneration consistent with GVHD gr...