Case Presentation: Thoracic Endometriosis Syndrome (TES) is a rare condition characterized by the presence of endometrial tissue inside the thoracic cavity. It consists of four distinct clinical entities: catamenial pneumothorax, catamenial hemothorax, hemoptysis, and pulmonary nodules. We report the case of a 47-year-old woman with TES presenting as spontaneous hemopneumothorax. Patient is a 47-year old female with type 2 diabetes and intra-abdominal endometriosis who presented one day after her menstruation onset with shortness of breath of 6 days duration associated with dry cough. The patient had no prior similar symptoms. On physical exam she had decreased breath sounds over the right lower half of the lung. Chest radiograph and chest CT showed right pneumothorax and scattered ground glass opacities in right upper and lower lobes. A chest tube was placed, draining serosanguinous fluid and air. Pleural fluid analysis was consistent with exudative fluid, however, cytology and infectious work-up including AFB were negative. Laboratory results disclosed elevated CA 125. Abdominal CT showed 2.3 cm mass-like structure at the umbilicus, lobulated soft tissue density mass contiguous with the cecum, and adjacent to the terminal ileum. The patient continued to have recurrence of pneumothorax upon clamping of the chest tube and underwent video assisted thoracoscopic surgery (VATS) with wedge resection and manual pleurodesis. Biopsy of diaphragmatic implants revealed endometriosis. She received postoperative gonadotropin-releasing hormone agonist. She failed pleurodesis with persistent small right pneumothorax and underwent repeat VATS with talc pleurodesis with subsequent resolution of pneumothorax.
Discussion: TES is a rare disorder affecting women of reproductive age group. It has varying clinical presentation of which catamenial pneumothorax is the most common type accounting for 74% of cases followed by catamenial hemothorax (14%). Hemopneumothorax due to TES has rarely been reported. Patients present with chest pain, cough and dyspnea occurring within 72 hours from the onset of menstruation. The diagnosis of TES may not be readily suspected after the first event hence high level of clinical suspicion is valuable in the diagnosis. Chest radiograph will reveal pneumothorax and/or hemothorax which in most cases is right-sided but can rarely be left-sided or bilateral. CT and MRI may show evidence of endometrial implants and diaphragmatic defects. Elevated CA-125 is a helpful adjunct to early diagnosis. VATS is the gold standard modality for definitive diagnosis and surgical treatment. Treatment includes hormonal therapy, surgical therapy or a combination of both.
Conclusions: Thoracic endometriosis syndrome should be suspected in women of reproductive age group presenting with spontaneous hemopneumothorax, especially in those who have no underlying lung pathology and with a history of prior uterine surgical procedures or proven pelvic endometriosis. Early diagnosis and multidisciplinary treatment including surgical correction of thoracic defects and postoperative hormonal or surgical therapy leads to reduced recurrence risk.