Beyond the Pelvis – A Case of Thoracic Endometriosis Syndrome

Abstract Introduction: Thoracic endometriosis syndrome (TES) is a rare complication of endometriosis that is characterized by the growth of abnormal endometrial gland and stroma in the lungs, pleural space, pericardial space or diaphragm. The clinical manifestations are wide and range from pulmonary nodules to catamenial hemoptysis/ hemothorax or pneumothorax. Here, we present a case of recurrent catamenial pleural effusions secondary to pleural endometriosis despite hormonal therapy. Case Presentation: A 30-year-old African American female with pelvic endometriosis and hypertension had previously been seen at an outside hospital on several occasions for catamenial effusions and was diagnosed with TES by means of video assisted thoracoscopic surgery with right middle lobe wedge biopsy and pleural biopsy. She also underwent diagnostic laparoscopy with lysis of adhesions with findings of severe adnexal disease s/p cauterization and was started on Relugolix, estradiol and norethindrone with successful hormonal suppression of menses. However, four months after being on therapy, she presented to our clinic with increased shortness of breath with chest X ray demonstrating a large right sided pleural effusion. Bedside ultrasound demonstrated significantly thickened pleura, adherent lung and internal septations. She underwent chest tube placement with evacuation of 1400cc of dark exudative fluid from the pleural space. Post procedure, there was radiologic improvement, but she was found to have rapid recurrence. She was offered Lupron, however, deferred it due to risk of medical menopause. Subsequently, she underwent open thoracotomy with talc pleurodesis and decortication with cryoablation. Discussion: TES is a rare disease that affects women in the reproductive age group with 35 years being the mean age on presentation. Due to variability of clinical and radiological presentations, diagnosis and management can be challenging, and high clinical suspicion is required for timely diagnosis. Even with the currently available diagnostic modalities like pleuroscopy and VATS, tissue samples may have inconsistent results due to the appearance and location of the lesions as well as time of sampling. Once the diagnosis has been made, medical management with hormonal therapies such as GnRH analogs, oral contraceptives, and progestins aiming to suppress ovarian function becomes first line. However, there is a high risk of recurrence and therapy failure. Surgery including VATs or open thoracotomy with lysis of adhesions and chemical or pleurodesis remain important management options. In patients presenting with TES, we recommend multidisciplinary approach with specialists in pulmonary medicine, cardiothoracic surgery and obstetrics and gynecology.