Skip to main content

Advertisement

SpringerLink
Log in

“Cystamatic” Review: Is Surgery Mandatory for Pneumatosis Cystoides Intestinalis?

  • DDS–SIRC COOPERATIVE CONFERENCES
  • Published:
Digestive Diseases and Sciences Aims and scope Submit manuscript

Abstract

Introduction

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of intramural gas cysts within the small and large intestines. We describe a case of a 70-year-old man admitted to the Emergency Surgery Department for PCI who was treated conservatively.

Areas Covered

We reviewed 60 cases of PCI described in the international literature over the last 5 years. From our analysis, it appears that the etiology of the gas production within the submucosa or the subserosa of the gastrointestinal tract is still unknown. The rupture of the cysts can lead to pneumoperitoneum that can simulate a surgical emergency.

Expert Commentary

For patients with PCI, a conservative approach is the treatment of choice, with surgery mandatory only for complicated disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Original drawings by Samuele Vaccari, MD—July 2019

Abbreviations

CT:

Computed tomography scan

PCI:

Pneumatosis cystoides intestinalis

COPD:

Chronic obstructive pulmonary disease

References

  1. Du Vernoy JG. Aer intestinorum tam sub extima quam intima tunica inclusus: observationes anatomicae: comment. Acad Acient Imp Petropol. 1730;5:213–225.

    Google Scholar 

  2. Rahim H, Khan M, Hudgins J, et al. Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis. World J Gastroenterol. 2013;19:1135–1139.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Rodríguez-Sánchez D, Sáez-Martínez ME, Sánchez-Jiménez RM, et al. Pneumatosis cystoides, CT colonoscopy and endoscopic correlation. Rev Esp Enferm Dig. 2013;105:486–487. https://doi.org/10.4321/S1130-01082013000800007.

    Article  PubMed  Google Scholar 

  4. Wu LL, Yang YS, Dou Y, et al. A systematic analysis of pneumatosis cystoides intestinalis. World J Gastrenterol. 2013;19:4973–4978.

    Article  Google Scholar 

  5. Morris MS, Gee AC, Co SD, et al. Management and outcome of pneumatosis intestinalis. Am J Surg. 2008;195:679–682.

    Article  PubMed  Google Scholar 

  6. Korhonen K, Lovvorn HN, Koyama T, et al. Incidence, risk factors, and outcome of pneumatosis intestinalis in pediatric stem cell transplant recipients. Pediatr Blood Cancer. 2012;58:616–620.

    Article  PubMed  Google Scholar 

  7. Ivanović A, Kovač J, Mašulović D, et al. Education and imaging. Gastrointestinal: the role of multidetector computer tomography in diagnosis of pneumatosis cystoides intestinalis. J Gastrenterol Hepatol. 2012;27:182.

    Article  Google Scholar 

  8. Ooi SM. Pneumoperitoneum in a non-acute abdomen-pneumatosis cystoides intestinalis. Surg Case Rep. 2015;1:44.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Torigoe K, Arai H, Yamashita A, et al. Improvements in pneumatosis cystoides intestinalis an hepatic portal venous gas with conservative therapy in a patient on maintenance dialysis. Inter Med. 2016;55:1735–1738.

    Article  Google Scholar 

  10. Wayne E, Ough M, Wu A, et al. Management algorithm for pneumatosis intestinalis and portal venous gas: treatment and outcome of 88 consecutive cases. J Gastrointest Surg. 2010;14:437–448.

    Article  PubMed  Google Scholar 

  11. Mitsuyoshi Y, Takakura K, Kobayashi T, et al. Chronic intestinal pseudo-obstruction with pneumatosis cystoides intestinalis in a patient with systemic sclerosis: a case report. Medicine. 2019;98:e15480. https://doi.org/10.1097/MD.0000000000015480.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Lin WC, Wang KC. Pneumatosis cystoides intestinalis secondary to use of an α-glucosidase inhibitor. Radiology. 2019;290:619. https://doi.org/10.1148/radiol.2019182186.

    Article  PubMed  Google Scholar 

  13. Takahashi K, Fujiya M, Ueno N, et al. Endoscopic fine-needle aspiration is useful for the treatment of pneumatosis cystoides intestinalis with intussusception. Am J Gastroenterol. 2019;114:13. https://doi.org/10.14309/ajg.0000000000000069.

    Article  PubMed  Google Scholar 

  14. Singh G, Johnson C, Gill R, et al. Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis. BMJ Case Rep. 2018;28:2018. https://doi.org/10.1136/bcr-2018-225175.

    Article  Google Scholar 

  15. Asahi Y, Suzuki T, Sawada A, et al. Pneumatosis cystoides intestinalis secondary to sunitinib treatment for gastrointestinal stromal tumor. Case Rep Gastroenterol. 2018;12:432–438. https://doi.org/10.1159/000490657.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Uruga H, Moriguchi S, Takahashi Y, et al. Gefitinib successfully administered in a lung cancer patient with leptomeningeal carcinomatosis after erlotinib-induced pneumatosis intestinalis. BMC Cancer. 2018;18:825. https://doi.org/10.1186/s12885-018-4743-5.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Koysombat K, Capanna MV, Stafford N, et al. Combination therapy for systemic sclerosis-associated pneumatosis intestinalis. BMJ Case Rep. 2018;. https://doi.org/10.1136/bcr-2018-225068.

    Article  PubMed  Google Scholar 

  18. Wang YJ, Wang YM, Zheng YM, et al. Pneumatosis cystoides intestinalis: six case reports and a review of the literature. BMC Gastroenterol. 2018;18:100. https://doi.org/10.1186/s12876-018-0794-y.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Martín-Lagos Maldonado A, Lozano Cejudo C, Sáenz Lozano A. A pneumoperitoneum due to intestinal cystic pneumatosis associated with a tyrosine kinase inhibitor. Rev Esp Enferm Dig. 2018;110:531–532. https://doi.org/10.17235/reed.2018.5588/2018.

    Article  PubMed  Google Scholar 

  20. Suda T, Shirota Y, Wakabayashi T. Pneumatosis cystoides intestinalis. Clin Gastroenterol Hepatol. 2019;17:A33–A34. https://doi.org/10.1016/j.cgh.2018.05.012.

    Article  PubMed  Google Scholar 

  21. Márquez Argente Del Castillo B, Olalla Muñoz JR. Focal Pneumatosis cystoides intestinalis: optic colonoscopy and virtual colonoscopy features. Rev Esp Enferm Dig. 2018;110:411.

    PubMed  Google Scholar 

  22. de la Serna S, Luna A, de la Rosa H. Intestinal pneumatosis and pneumoperitoneum in an oncological scenario: a change of attitude. Rev Esp Enferm Dig. 2018;110:68–69. https://doi.org/10.17235/reed.2017.5333/2017.

    Article  PubMed  Google Scholar 

  23. Mikami T, Kakimoto K, Hirata Y, et al. Pneumatosis cystoides intestinalis in a patient with ulcerative colitis. J Gastrointestin Liver Dis. 2017;26:337. https://doi.org/10.15403/jgld.2014.1121.264.hig.

    Article  PubMed  Google Scholar 

  24. Sugihara Y, Okada H. Pneumatosis cystoides intestinalis. N Engl J Med. 2017;377:2266. https://doi.org/10.1056/NEJMicm1701357.

    Article  PubMed  Google Scholar 

  25. Kunishi Y, Ota M, Kato Y, et al. Pneumatosis cystoides intestinalis: lung window setting on CT. Clin Case Rep. 2017;5:1896–1897. https://doi.org/10.1002/ccr3.1151.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Lee YS, Han JJ, Kim SY, et al. Pneumatosis cystoides intestinalis associated with sunitinib and a literature review. BMC Cancer. 2017;17:732. https://doi.org/10.1186/s12885-017-3744-0.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Kang G. Benign pneumatosis intestinalis: Dilemma for primary care clinicians. Can Fam Physician. 2017;63:766–768.

    PubMed  PubMed Central  Google Scholar 

  28. Nakatani K, Kato T, Okada S, et al. Successful treatment with hyperbaric oxygen therapy for pneumatosis cystoides intestinalis as a complication of granulomatosis with polyangiitis: a case report. J Med Case Rep. 2017;11:263. https://doi.org/10.1186/s13256-017-1421-1.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Rachapalli V, Chaluvashetty SB. Pneumatosis cystoides intestinalis. J Clin Diagn Res. 2017;11:TJ01–TJ02. https://doi.org/10.7860/JCDR/2017/26197.10087.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Jegadeesan R, Sundararajan T, Patel MB, et al. Pneumatosis intestinalis due to extrinsic compression from a large renal cyst. Am J Gastroenterol. 2017;112:983. https://doi.org/10.1038/ajg.2017.64.

    Article  PubMed  Google Scholar 

  31. Liao YH, Lo YP, Liao YK, et al. Pneumatosis cystoides coli related to acarbose treatment. Am J Gastroenterol. 2017;112:984. https://doi.org/10.1038/ajg.2017.88.

    Article  PubMed  Google Scholar 

  32. Suzuki E, Kanno T, Hazama M, et al. Four cases of pneumatosis cystoides intestinalis complicated by connective tissue diseases. Intern Med. 2017;56:1101–1106. https://doi.org/10.2169/internalmedicine.56.7877.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Iida A, Naito H, Tsukahara K, et al. Pneumatosis cystoides intestinalis presenting as pneumoperitoneum in a patient with chronic obstructive pulmonary disease: a case report. J Med Case Rep. 2017;11:55. https://doi.org/10.1186/s13256-017-1198-2.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Romano-Munive AF, Barreto-Zuñiga R. Pneumatosis cystoides intestinalis. Rev Esp Enferm Dig. 2017;109:61.

    PubMed  Google Scholar 

  35. Venkataramani V, Seif Amir Hosseini A, Schulze MH, et al. Intestinal pneumatosis associated with tuberculosis after allogeneic hematopoietic stem cell transplantation. Acta Haematol. 2017;137:51–54. https://doi.org/10.1159/000452436.

    Article  PubMed  Google Scholar 

  36. Faria LD, Anjos CH, Fernandes GD, et al. Pneumatosis intestinalis after etoposide-based chemotherapy in a patient with metastatic small cell lung cancer: successful conservative management of a rare condition. Einstein (Sao Paulo). 2016;14:420–422. https://doi.org/10.1590/S1679-45082016RC3597.

    Article  Google Scholar 

  37. Fujimi A, Sakamoto H, Kanisawa Y, et al. Pneumatosis intestinalis during chemotherapy with nilotinib in a patient with chronic myeloid leukemia who tested positive for anti-topoisomerase I antibodies. Clin J Gastroenterol. 2016;9:358–364.

    Article  PubMed  Google Scholar 

  38. Furihata T, Furihata M, Ishikawa K, et al. Does massive intraabdominal free gas require surgical intervention? World J Gastroenterol. 2016;22:7383–7388. https://doi.org/10.3748/wjg.v22.i32.7383.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Mima A, Nagahara D, Tansho K. Methotrexate induced pneumatosis intestinalis under hemodialysis patient. Hemodial Int. 2017;21:E9–E12. https://doi.org/10.1111/hdi.12459.

    Article  PubMed  Google Scholar 

  40. Torigoe K, Arai H, Yamashita A, et al. Improvements in pneumatosis cystoides intestinalis and hepatic portal venous gas with conservative therapy in a patient on maintenance dialysis. Intern Med. 2016;55:1735–1738. https://doi.org/10.2169/internalmedicine.55.6235.

    Article  PubMed  Google Scholar 

  41. Fraga M, Silva MJND, Lucas M. Idiopathic pneumatosis intestinalis, radiological and endoscopic images. GE Port J Gastroenterol. 2016;23:270–272. https://doi.org/10.1016/j.jpge.2016.01.005.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Kinjo M. Lurking in the wall: pneumatosis cystoides intestinalis with scleroderma. Am J Med. 2016;129:382–383. https://doi.org/10.1016/j.amjmed.2015.12.007.

    Article  PubMed  Google Scholar 

  43. Ksiadzyna D, Peña AS. Segmental pneumatosis cystoides coli: computed tomography-facilitated diagnosis. Rev Esp Enferm Dig. 2016;108:510–513. https://doi.org/10.17235/reed.2015.3790/2015.

    Article  PubMed  Google Scholar 

  44. Helo N, Rhee K, Dugum M. An intriguing case of bright red blood per rectum. Pneumatosis cystoids intestinalis. Gastroenterology. 2015;149:e1–e2. https://doi.org/10.1053/j.gastro.2014.12.037.

    Article  PubMed  Google Scholar 

  45. Castro-Poças F, Araújo T, Pedroto I. Endoscopic ultrasound of pneumatosis cystoides intestinalis. Endoscopy. 2015;47:E274. https://doi.org/10.1055/s-0034-1391873.

    Article  PubMed  Google Scholar 

  46. Kancherla D, Vattikuti S, Vipperla K. Pneumatosis cystoides intestinalis: is surgery always indicated? Cleve Clin J Med. 2015;82:151–152. https://doi.org/10.3949/ccjm.82a.14006.

    Article  PubMed  Google Scholar 

  47. Akasaka H, Wajima N, Kimura A, et al. A case of pneumatosis cystoides intestinalis after neoadjuvant chemotherapy for esophageal cancer. Gan To Kagaku Ryoho. 2014;41:2375–2377. Japanese.

    PubMed  Google Scholar 

  48. Ando T, Sakata J, Maruyama T, et al. A case of pneumatosis cystoides intestinalis secondary to gefitinib therapy for lung adenocarcinoma. Gan To Kagaku Ryoho. 2015;42:847–849. Japanese.

    PubMed  Google Scholar 

  49. Blair HA, Baker R, Albazaz R. Pneumatosis intestinalis an increasingly common radiological finding, benign or life-threatening? A case series. BMJ Case Rep. 2015;. https://doi.org/10.1136/bcr-2014-207234.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Chandola R, Elhenawy A, Lien D, et al. Massive gas under diaphragm after lung transplantation: pneumatosis intestinalis simulating bowel perforation. Ann Thorac Surg. 2015;99:687–689. https://doi.org/10.1016/j.athoracsur.2014.03.047.

    Article  PubMed  Google Scholar 

  51. Aziret M, Erdem H, Ülgen Y, et al. The appearance of free-air in the abdomen with related pneumatosis cystoides intestinalis: three case reports and review of the literature. Int J Surg Case Rep. 2014;5:909–913. https://doi.org/10.1016/j.ijscr.2014.09.031.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Klimova K, Pérez Valderas MD, Merino Rodríguez B, et al. Pneumatosis cystoides intestinalis as an infrequent cause of chronic abdominal pain. Rev Gastroenterol Mex. 2014;79:302–303. https://doi.org/10.1016/j.rgmx.2014.09.001.

    Article  CAS  PubMed  Google Scholar 

  53. Rodrigues-Pinto E, Ramalho R, Baldaia H, et al. Pneumatosis cystoides intestinalis secondary to increased intraluminal air pressure of previous colonoscopies. Endoscopy. 2014;46:E572–E573. https://doi.org/10.1055/s-0034-1377985.

    Article  PubMed  Google Scholar 

  54. Santos-Antunes J, Ramalho R, Lopes S, et al. Asymptomatic pneumatosis cystoides intestinalis diagnosed in the follow-up of a dysplastic polyp. Endoscopy. 2014;46:E425–E426. https://doi.org/10.1055/s-0034-1377435.

    Article  PubMed  Google Scholar 

  55. Yao SY, Seo R, Nagano T, et al. Pneumatosis cystoides intestinalis associated with toxic epidermal necrolysis: a case report. World J Clin Cases. 2014;2:469–473. https://doi.org/10.12998/wjcc.v2.i9.469.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Tseng PY, Tarng DC, Yang WC, et al. Benign pneumatosis intestinalis. Intern Med. 2014;53:1589–1590.

    Article  PubMed  Google Scholar 

  57. Bamakhrama K, Abdulhady L, Vilmann P. Endoscopic ultrasound diagnosis of pneumatosis cystoides coli initially misdiagnosed as colonic polyps. Endoscopy. 2014;46:E195–E196. https://doi.org/10.1055/s-0034-1365152.

    Article  PubMed  Google Scholar 

  58. Ohtake S, Namura K, Fujikawa A, et al. A case of pneumatosis cystoides intestinalis secondary to sunitinib treatment for renal cell carcinoma. Hinyokika Kiyo. 2014;60:75–78. Japanese.

    PubMed  Google Scholar 

  59. Jurado-Román M, Calero-García P, Flores-Garnica LM, et al. Pneumatosis cystoides intestinalis. Uncommon cause of pneumoperitoneum. Rev Esp Enferm Dig. 2014;1060:71–72.

    Article  Google Scholar 

  60. Rodrigues-Pinto E, Pereira P, Macedo G. Primary pneumatosis cystoides intestinalis (with video). Gastrointest Endosc. 2014;79:1008–1010. https://doi.org/10.1016/j.gie.2014.01.027.

    Article  PubMed  Google Scholar 

  61. Cottom H, Ford AC, Rotimi O. Masquerading gastrointestinal polyps: air on the side of caution? Cystic pneumatosis. Gastroenterology. 2014;146:e9–10. https://doi.org/10.1053/j.gastro.2013.11.043.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Departments of Emergency Surgery and Gastroenterology, St. Orsola University Hospital, Bologna, Italy

    M. Brighi, S. Vaccari, A. Lauro, N. Pagano, M. Cervellera & V. Tonini

  2. Department of Surgical Sciences, La Sapienza University, Umberto I Hospital, Rome, Italy

    V. D’Andrea

  3. Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA

    I. R. Marino

Authors
  1. M. Brighi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. S. Vaccari
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. Lauro
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. V. D’Andrea
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. N. Pagano
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. I. R. Marino
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. M. Cervellera
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. V. Tonini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Lauro.

Ethics declarations

Conflict of interest

None of the authors have any conflicts of interest pertaining to this work.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Brighi, M., Vaccari, S., Lauro, A. et al. “Cystamatic” Review: Is Surgery Mandatory for Pneumatosis Cystoides Intestinalis?. Dig Dis Sci 64, 2769–2775 (2019). https://doi.org/10.1007/s10620-019-05767-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10620-019-05767-4

Keywords

Search

Navigation