Bisphosphonate Therapy for Tracheopathia Osteochondroplastica

Presentation Number: SAT 303
Date of Presentation: April 1st, 2017

Asma Al-Zougbi*1, Swarna Rai2 and Joseph Stephen Dillon2
1University of Iowa Hospitals and Clinics, Iowa City, IA, 2University of Iowa Hospital and Clinics, Iowa City, IA

Abstract

Background: Tracheopathia osteochondroplastica (TO) is a rare non-neoplastic disorder characterized by the development of osseous and/or cartilaginous nodules in the trachea or bronchial walls. Often, the condition is silent, however, it can occasionally cause obstruction of the tracheal passage leading to life-threatening respiratory compromise. Severe TO is traditionally treated surgically. In this report, we report a unique case of severe TO that is being treated conservatively with bisphosphonate therapy

Clinical case: A 47 year old woman with significant history of Crohn’s disease presented to our clinic for the evaluation of TO. In the past, she presented with respiratory distress, stridor and hoarseness. Her imaging at that time demonstrated laryngeal calcification and right vocal cord fixation secondary to heterotopic growth of cartilage and bone. Nuclear bone scan showed uptake at the mid cervical spine, confirming active heterotopic ossification. Her severe condition at the time required surgical intervention via tracheostomy. Her postoperative course was complicated by delayed subglottic involvement in soft tissues immediately cranial to tracheostomy site a few months later. Work up for an autoimmune etiology was negative and she had no improvement of activity on nuclear bone scan after a one year trial of adalimumab (Humira).

TO does not usually require treatment, however may occasionally require tracheostomy or surgical resection of the affected tracheal segment or outgrowths. Owing to the patient’s multifocal disease and the expectation of recurrence, surgical resection was not a good option in our patient. TO is a corollary of calcium deposition, as is heterotopic ossification (HO). Bisphosphonates have been shown to halt primary and secondary progression of HO. In effect, we initiated the treatment of our patient on Zoledronic Acid since bisphosphonates have been shown to halt the progression of HO.

Clinical lessons: While bisphosphonate therapy has been used successfully to treat HO, to our knowledge, this is the first report demonstrating the possible benefit of bisphosphonates in the rare setting of TO.

 

Nothing to Disclose: AA, SR, JSD