Abstract

Pneumatosis intestinalis (PI) is a rare disease, which presents a wide range of severity. Numerous etiologies, including trauma, inflammation, infections, autoimmunity, drugs, and mechanical procedures, gave rise to this complication. Abdominal computerized tomography is the preferred diagnostic tool with high sensitivity. The diagnosis depends on free air detection in the intramural portion of the gastrointestinal system and main and intrahepatic branches of the portal venous structures. However, the exact mechanism is unknown. The clinical scenario may vary from benign to life-threatening. One of the etiological factors that cause PI is the chronic graft-versus-host disease (cGVHD), which must be considered in patients with solid organ or hematological malignancies. Especially, patients who received long-term immunosuppressive therapies and were diagnosed with cGVHD are prone to developing a PI. Many patients experience unnecessary operational risks if underdiagnosed. A multidisciplinary approach by the primary physician, general surgeon, and radiology specialist is necessary for the proper treatment of these patients. This case report aimed to discuss the clinical presentation of PI in the course of cGVHD in the intensive care unit.

Keywords: Chronic graft versus host disease, pneumatosis intestinalis, intensive care

References

  1. Read NW, Al-Janabi MN, Cann PA. Is raised breath hydrogen related to the pathogenesis of pneumatosis coli? Gut 1984;25:839-45.
  2. Sakurai Y, Hikichi M, Isogaki J, Furuta S, Sunagawa R, Inaba K, et al. Pneumatosis cystoides intestinalis associated with massive free air mimicking perforated diffuse peritonitis. World J Gastroenterol 2008;14:6753-6.
  3. Wayne E, Ough M, Wu A, Liao J, Andresen KJ, Kuehn D, et al. Management algorithm for pneumatosis intestinalis and portal venous gas: treatment and outcome of 88 consecutive cases. J Gastrointest Surg 2010;14:437-48.
  4. Ho LM, Paulson EK, Thompson WM. Pneumatosis intestinalis in the adult: benign to life-threatening causes. AJR Am J Roentgenol 2007;188:1604-13.
  5. St Peter SD, Abbas MA, Kelly KA. The spectrum of pneumatosis intestinalis. Arch Surg 2003;138:68-75.
  6. Day DL, Ramsay NK, Letourneau JG. Pneumatosis intestinalis after bone marrow transplantation. AJR Am J Roentgenol 1988;151:85-7.
  7. Knechtle SJ, Davidoff AM, Rice RP. Pneumatosis intestinalis. Surgical management and clinical outcome. Ann Surg 1990;212:160-5.
  8. Aslam F, Apostolopoulos A, Zeeshan S. Pneumatosis intestinalis with extensive intrahepatic portal venous gas secondary to intra-abdominal sepsis: a rare occurrence. BMJ Case Rep 2017;2017:bcr2017222865.
  9. Muhammad MN, Sadough M, King R, Singh G. Pneumatosis of the esophagus and intestines with portal venous air: a rare presentation. J Community Hosp Intern Med Perspect 2017;7:255-7.
  10. Filipovich AH, Weisdorf D, Pavletic S, Socie G, Wingard JR, Lee SJ, et al. National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group report. Biol Blood Marrow Transplant 2005;11:945-56.
  11. Bhushan V, Collins RH Jr. Chronic graft-vs-host disease. JAMA 2003;290:2599-603.
  12. Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group report. Biol Blood Marrow Transplant 2015;21:389-401.e1.
  13. Pear BL. Pneumatosis intestinalis: a review. Radiology 1998;207:13-9.
  14. Pieterse AS, Leong AS, Rowland R. The mucosal changes and pathogenesis of pneumatosis cystoides intestinalis. Hum Pathol 1985;16:683-8.
  15. Höer J, Truong S, Virnich N, Füzesi L, Schumpelick V. Pneumatosis cystoides intestinalis: confirmation of diagnosis by endoscopic puncture a review of pathogenesis, associated disease and therapy and a new theory of cyst formation. Endoscopy 1998;30:793-9.

Copyright and license

Copyright © 2021 The Author(s). This is an open access article distributed under the Creative Commons Attribution License (CC BY), which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.

How to cite?

1.
Çalışkan T, Özkök H, Küçük M, Turan Erdoğan B, Uygun V, Savran Y, et al. An Exceptional Etiology of a Rare Disease: Pneumatosis Intestinalis in the Intensive Care Unit due to Chronic Graft-Versus-Host Disease. Turk J Intensive Care. 2021;19(4):200-204. https://doi.org/10.4274/tybd.galenos.2021.55265