A Case of Treatment-Resistant Catamenial Disease: Insufficiency in Mainstay Treatment Options

Abstract

Introduction: Thoracic endometriosis syndrome (TES) is a rare phenomenon that affects 6-10% of women of reproductive age. It is characterized by presence of ectopic endometrial tissue within the thoracic cavity, mainly the lung parenchyma and pleura. TES presents as catamenial pneumothorax (CP), catamenial hemothorax (CH), pulmonary nodules or hemoptysis. Hormonal therapy is considered first line treatment followed by operative thoracoscopy for removal of ectopic endometrial implants. Our case highlights the limited number of treatment options in a young patient with recurrent catamenial disease.Description: A 37-year-old G4P0040 female presented to the emergency department (ED) with worsening shortness of breath (SOB) for 5 days. Her SOB was sudden in onset, progressively worsening, and associated with cough and right-sided chest pain. Patient was admitted 5 months ago for similar symptoms. She has a past medical history significant for hypertension, obesity, iron deficiency anemia, severe endometriosis and TES manifesting as recurrent, right sided pleural effusions, CPs and CHs requiring surgical treatment. Previous biopsies of the ectopic endometrial tissue revealed dense fibroconnective tissue with focal endometriosis and no indication for malignancy. In the ED, she appeared in distress and had a BP of 166/102. Physical examination revealed tachypnea, accessory muscle usage, respiratory distress and retractions. Breath sounds were decreased in the right lung. EKG revealed normal sinus rhythm, and a COVID-19 test yielded negative results. Chest x-ray and CT were consistent with findings for right tension hemothorax. She was started on BiPAP initially and thoracic surgery was consulted. A tube thoracostomy was performed in the ED and a chest tube was placed. Moreover, a CBC revealed Hb of 5.7g/dl. She was transfused 4 units pRBC and admitted to the ICU for further monitoring. Ob/gyn was consulted and leuprolide was administered to prevent recurrence of a hemothorax. She remained in the hospital for 3 days and was subsequently discharged after resolution of symptoms confirmed by imaging.Conclusion: Catamenial hemothoraces are a late finding of TES. There is mixed data regarding the effectiveness of GnRH agonists on recurrence rates of CH, however subsequent surgery has shown to be preventative in resistant cases. This unique case illustrates the insufficiency in both mainstay treatments. Despite the fact the patient had previous lobectomies using VATS, lysis of adhesions and hormonal therapy, she continued to have recurrent manifestations of TES. Novel treatments in conjunction with a multidisciplinary team approach should be used to effectively treat TES and prevent disease relapse.