Abstract Introduction Post-transplant lymphoproliferative disorder (PTLD) is a known post-transplant complication typically affecting lymphatic tissues in multiple organ sites but rarely involves the airway outside of adenoids and tonsils. The differential for respiratory abnormalities following transplant and subsequent immunosuppression is broad and include opportunistic infections, chronic acid reflux, and airway obstruction. We present a rare case of recurrent stridor and croup with nonspecific epiglottal and arytenoid edema on bronchoscopy that was ultimately diagnosed as non-destructive PTLD causing supraglottic obstruction. Clinical Vignette A 2-year-old female with biliary atresia status post deceased donor whole liver transplant complicated by acute rejection requiring high dose steroids, presented at 10 months post transplant and after her rejection steroid taper with recurrent episodes of voice hoarseness and stridor necessitating multiple admissions, repeated doses of steroids, and positive pressure ventilation. Initial direct laryngoscopy by otolaryngology revealed significant edema of the epiglottis, arytenoids, and vocal cords, with cultures of her airway growing C. albicans and tropicalis. She was started on prolonged anti-fungals for presumed fungal laryngitis without improvement. Respiratory PCR was positive for parainfluenza and rhinovirus leading to a presumptive diagnosis of infectious croup. Head, neck, and chest CT revealed mild reactive lymphadenopathy but no concern for active PTLD on imaging. Both pre-transplant donor and recipient EBV serostatus were positive. EBV PCR in serum during this period had remained relatively low ranging from 73-4570 IU/mL. Due to recurrent hospital admissions for stridor and acute hypoxic respiratory failure resistant to steroids and anti-microbials, repeat laryngoscopy and bronchoscopy was performed 3 months after initial presentation (13 months post-transplant) and revealed extensive redundant arytenoid tissue with cobblestoning appearance and supraglottic stenosis (Figure 1). Supraglottoplasty with biopsy of the tissue confirmed the diagnosis of EBV+ non-destructive plasmacytoma-like PTLD. Notably, EBV PCR of BAL fluid at time of biopsy contained 633,000 IU/mL. The patient’s stridor was resolved with reduction of tacrolimus alongside treatment with high dose dexamethasone and bortezomib. Discussion Our case highlights a rare, late airway complication of immunosuppression following solid organ transplantation. It is important to maintain a broad differential in immunosuppressed patients with upper airway inflammation—including infection, eosinophilic laryngitis, and both EBV-positive and EBV-negative PTLD—especially when symptoms do not respond to standard therapy. Ultimately, a high index of suspicion when standard croup treatment failed prompting repeat endoscopic evaluation with biopsy were essential to establishing the diagnosis. This abstract is funded by: None
Manker et al. (Fri,) studied this question.