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Abstract

Introduction:

Respiratory failure is the leading cause of mortality in individuals with congenital spine and rib deformities. We present a case report of a child with Jeune syndrome surviving respiratory failure using extracorporeal membrane oxygenation (ECMO). We also summarize thoracic insufficiency syndrome cases reported in the Extracorporeal Life Support Organization (ELSO) registry.

Case Report:

A two-year-old male with a chest circumference less than a third percentile for age was admitted with influenza pneumonia developing a peak oxygenation index of 103.5. The child survived to baseline pulmonary function after nine days of venous-arterial ECMO support.

Discussion:

The ELSO registry contained 27 individuals with a surrogate diagnosis of thoracic insufficiency (0.05%). There was no significant difference in survival to discharge for thoracic insufficiency patients (52%) compared to a previously healthy population supported with ECMO.

Conclusion:

ECMO is safe and may be effective in supporting individuals with thoracic insufficiency.

Keywords

thoracic insufficiency extracorporeal membrane oxygenation Jeune syndrome pediatrics thoracic surgery

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Extracorporeal membrane oxygenation support in individuals with thoracic insufficiency