Is surgery required for Relapsing Polychondritis
When does surgery play a role in the management of Relapsing Polychondritis?
Tracheostomy may be required in patients with airway collapse unresponsive to night time positive pressure ventilation. In such patients, extensive imaging of the entire respiratory tree is critical as severe distal involvement may be contributing primarily to symptoms and hence, tracheostomy would not be expected to aid symptoms. Caution should be taken prior to any surgical intervention as tissue disruption holds the potential for disease activation; adequate control of the inflammatory disease should occur prior to surgery if possible.
Airway obstruction caused by tracheal stenosis or tracheomalacia may require surgical resection. Endoscopic laser ablation has been described in the treatment of focal lesions. Intrabronchial stent placement has been reported as a potential remedy for dynamic airway collapse, although reports of stent-related complications are not uncommon. Aortic insufficiency may require valve replacement. In many cases, aortic insufficiency is associated with ascending aortic dilation, and improved outcomes are obtained with combined aortic valve plus aortic root/ascending aorta graft replacement (modified Bentall procedure), similar to patients with Takayasu’s arteritis and Behçet’s. Postgraft dehiscence has been described in roughly 10% of cases unfortunately.
Surgical reconstruction of nasal septal collapse using a bone graft has been successfully described in a patient with quiescent disease, but experience in this area is limited. Cochlear implants can be used for patients with sensorineural hearing loss.
