Yellow Nail Syndrome Pleural Entrapment Successfully Treated With Intrapleural Catheter Placement Followed by Pleurodesis

Abstract

INTRODUCTION: Yellow Nail Syndrome (YNS) is a rare cause of pleural effusions. The etiology of YNS remains unknown and treatment focuses on symptomatic management [1]. Therapies for YNS pleural effusions include thoracentesis, tunneled intrapleural catheters (IPC), pleurodesis, and pleurectomy [1,2,3]. Here we present a patient with YNS and bilateral pleural effusions successfully treated with staged IPC followed by video-assisted thoracoscopic surgery (VATS) pleurodesis. CASE PRESENTATION:A 79 year old woman who is a lifelong nonsmoker with a history of type one diabetes mellitus, hyperlipidemia, and hypothyroidism presented with chronic cough, bilateral lower extremity edema, and yellow nail discoloration. Chest computed tomography (CT) scan revealed bilateral lower lobe bronchiectasis and bilateral pleural effusions. A diagnosis of YNS was made recognizing the triad of yellow nails, lymphedema, and pulmonary disease. Thoracentesis revealed a clear-yellow, exudative pleural effusion with a lymphocytic predominance, normal triglyceride level, and negative cultures. Per patient preference, she underwent serial therapeutic thoracenteses. Over the following two years, her pleural effusions progressed requiring increased frequency of drainage, and she developed evidence of bilateral lung entrapment on manual pleural manometry with residual pleural fluid. She then opted to pursue VATS pleurodesis. Her right VATS pleurodesis was preceded by IPC placement, and her lung entrapment improved with serial IPC drainage over two months. She then underwent right VATS mechanical and doxycycline pleurodesis. This same staged procedure was performed on the left side. She has had successful resolution of her pleural effusions without evidence of recurrence at greater than 18 months of follow up.DISCUSSION: YNS is a rare diagnosis with less than 400 cases described and an estimated prevalence of <1:1,000,000 [1]. It is characterized by the triad of yellow discoloration of the nails, lymphedema, and respiratory system involvement [1]. The cause of YNS is unknown, but an anatomic or functional abnormality of lymphatic drainage is proposed as the cause of its clinical manifestations [1]. There are currently no guidelines for the management of YNS, and an individualized approach to the management of YNS pleural effusions should be taken [2,3]. As with other recurrent exudative pleural effusions, serial thoracenteses for YNS may be complicated by pleural entrapment and progression to trapped lung, limiting the efficacy of future pleurodesis.CONCLUSIONS: Our patient's case suggests a role for staged IPC placement followed by VATS pleurodesis in patients with YNS pleural effusions complicated by pleural entrapment. This may also suggest a role for IPC placement in patients who are not currently surgical candidates without sacrificing the potential for more definitive treatment in the future.