Case report: an unusal cause of stridor in a post-liver transplant 6-year old

M A Rafferty1, D Devaney, J Russell

  • 1The Children's Hospital, Temple Street, Dublin, Ireland.

Insights

Post-transplant lymphoproliferative disorder can cause upper airway obstruction in children. This case highlights a sub-glottic obstruction in a child with negative Epstein-Barr virus serology.

Area of Science:

  • Pediatric Otolaryngology
  • Hematology/Oncology
  • Transplantation Medicine

Background:

  • Polymorphic lymphoproliferative disorder (LPD) is a known cause of upper airway obstruction in pediatric patients.
  • LPD is frequently associated with immunosuppression therapy and Epstein-Barr virus (EBV) infection, particularly in post-transplant children.
  • The reported prevalence of LPD in post-transplant children ranges from 4% to 13%.

Observation:

  • LPD can manifest in various locations, including the transplanted organ, gastrointestinal tract, head and neck, and notably, the upper airway.
  • Previous cases of upper airway obstruction due to LPD have involved the supraglottis, Waldeyer's ring, glottis, and trachea.
  • This report details a unique case of LPD presenting as acute upper airway obstruction in the sub-glottic region.

Findings:

  • The patient presented with acute upper airway obstruction due to sub-glottic LPD.
  • Crucially, the patient exhibited negative Epstein-Barr virus (EBV) serology, challenging the typical association.

Implications:

  • This case expands the known anatomical locations of LPD-induced upper airway obstruction.
  • The negative EBV serology suggests that LPD can cause airway compromise even in the absence of detectable EBV infection.
  • Further investigation into non-EBV-associated LPD is warranted for comprehensive understanding and management in pediatric transplant recipients.

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