Paradoxical vocal cord movement as a Life threatening stridor in Xeroderma Pigmentosum Type-A

Vocal folds dystonia and total vocal folds paralysis in XPA are rare clinical entities that have been recently described in few case reports from Japanese literature. We report an 11 years old boy with XPA and seizures with history of multiple ER visits for Inspiratory stridor which was treated as croup since the age of 10 . The child was admitted as a severe case of croup and he was treated for about two weeks with minimal improvement so Otolaryngology was consulted to investigate other causes of stridor. Flexible rhinolaryngoscopy showed: paradoxical vocal cord movement with glottic closure upon inspiration and limited glottis opening upon expiration which is consistent with laryngeal dystonia. This report represents the history, physical examination, literature review and the follow up findings in this patient and illustrates the importance of proper examination and laryngoscopy in this fragile patients' group to prevent serious complications.