Use of Extracorporeal Membrane Oxygenation in idiopathic capillary leak syndrome: a case report

Authors

  • Florencia Ortega Hospital Italiano de Buenos Aires
  • Indalecio Carboni Bisso Hospital Italiano de Buenos Aires https://orcid.org/0000-0002-4834-4676
  • Ignacio Fernandez Ceballos Hospital Italiano de Buenos Aires
  • Ana Montserrat Rivera Hospital Italiano de Buenos Aires
  • Manuel Tisminetzky Hospital Italiano de Buenos Aires
  • José Dianti Hospital Italiano de Buenos Aires https://orcid.org/0000-0003-2016-7003
  • Eduardo San Román Hospital Italiano de Buenos Aires
  • Sonia Villarroel Hospital Italiano de Buenos Aires
  • Sabrina Di Stefano Hospital Italiano de Buenos Aires
  • Marcos Las Heras Hospital Italiano de Buenos Aires

DOI:

https://doi.org/10.31053/1853.0605.v77.n3.26960

Keywords:

capillary leak syndrome, Clarkson disease, extracorporeal membrane oxygenation, critical care

Abstract

Introduction
Idiopathic systemic capillary leak syndrome (ISCLS) or Clarkson's disease is unusual but potentially lethal, characterized by recurrent shock incidents and anasarca secondary to idiopathic increase of capillary permeability. In such a context, the use of venoarterial Extracorporeal Membrane Oxygenation (ECMO) as cardiorespiratory support is a rescue action that seeks hemodynamic stability generation until spontaneous disappearance of the capillary occurs with the objective of surpassing the complications of resorption phase. 

Case Report
A 42 year old patient presented ISCLS and required ECMO as hemodynamic support for 8 days. She remained 20 days in Intensive Care Unit and was given hospital release after 43 days.

Conclusions
The use of ECMO in the reported case was a useful strategy in the ISCLS management as a bridge to recovery both in the leak stage and the fluid resorption phase. Notwithstanding its indication is limited to thoroughly selected patients and requires further debate between specialists about its risks and benefits. 

Downloads

Download data is not yet available.

References

Baloch NU, Bikak M, Rehman A, Rahman O. Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review. Expert Rev Cardiovasc Ther. 2018 May;16(5):331-340. doi: 10.1080/14779072.2018.1456920.

Pineton de Chambrun M, Luyt CE, Beloncle F, Gousseff M, Mauhin W, Argaud L, Ledochowski S, Moreau AS, Sonneville R, Verdière B, Merceron S, Zappella N, Landais M, Contou D, Demoule A, Paulus S, Souweine B, Lecomte B, Vieillard-Baron A, Terzi N, Azoulay E, Friolet R, Puidupin M, Devaquet J, Mazou JM, Fedun Y, Mira JP, Raphalen JH, Combes A, Amoura Z; EurêClark Study Group. The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. Crit Care Med. 2017 Jul;45(7):1216-1223. doi: 10.1097/CCM.0000000000002496.

Siddall E, Khatri M, Radhakrishnan J. Capillary leak syndrome: etiologies, pathophysiology, and management. Kidney Int. 2017 Jul;92(1):37-46. doi: 10.1016/j.kint.2016.11.029.

Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, Nelson C, Young M, Parikh SM, Druey KM. Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). J Clin Cell Immunol. 2014;5:1000213. doi: 10.4172/2155-9899.1000213.

Xie Z, Kuhns DB, Gu X, Otu HH, Libermann TA, Gallin JI, Parikh SM, Druey KM. Neutrophil activation in systemic capillary leak syndrome (Clarkson disease). J Cell Mol Med. 2019 Aug;23(8):5119-5127. doi: 10.1111/jcmm.14381. Epub 2019 Jun 18.

Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol. 2017 Sep;140(3):663-670. doi: 10.1016/j.jaci.2016.10.042.

Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med. 1960 Aug;29:193-216. doi: 10.1016/0002-9343(60)90018-8.

Kapoor P, Greipp PT, Schaefer EW, Mandrekar SJ, Kamal AH, Gonzalez-Paz NC, Kumar S, Greipp PR. Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. Mayo Clin Proc. 2010 Oct;85(10):905-12. doi: 10.4065/mcp.2010.0159.

Leung KKY, Rosa Duque JS, Yu KM, Cheong KN, Chong PC, Ho MH, Chow PC. Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome. BMC Pediatr. 2019 Jan 21;19(1):28. doi: 10.1186/s12887-019-1401-2.

Le Gall A, Follin A, Cholley B, Mantz J, Aissaoui N, Pirracchio R. Veno-arterial-ECMO in the intensive care unit: From technical aspects to clinical practice. Anaesth Crit Care Pain Med. 2018 Jun;37(3):259-268. doi: 10.1016/j.accpm.2017.08.007.

Downloads

Published

2020-08-21

How to Cite

1.
Ortega F, Carboni Bisso I, Fernandez Ceballos I, Montserrat Rivera A, Tisminetzky M, Dianti J, San Román E, Villarroel S, Di Stefano S, Las Heras M. Use of Extracorporeal Membrane Oxygenation in idiopathic capillary leak syndrome: a case report. Rev Fac Cien Med Univ Nac Cordoba [Internet]. 2020 Aug. 21 [cited 2024 Jul. 17];77(3):208-10. Available from: https://revistas.unc.edu.ar/index.php/med/article/view/26960

Issue

Vol. 77 No. 3 (2020)

Section

Case Report

License

Copyright (c) 2020 Universidad Nacional de Córdoba

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

The Faculty of Medical Sciences Journal (RFCM) subscribes to the Open Access policy and does not charge authors fees for publishing, nor does it charge readers fees for accessing published articles (APC).