A Unique Case of Thoracic Endometriosis Syndrome and Pulmonary Langerhans' Cell Histiocytosis: Six Recurrent Pneumothoraces

Background The diagnosis and treatment of spontaneous pneumothorax (SP) in women of reproductive age caused by thoracic endometriosis syndrome (TES) is challenging.

Materials und Methods This is a retrospective case elucidation of a 33-year-old female patient with a history of smoking (cumulative 15 Pack-years), who presented with six recurrent right sided pneumothoraces. The diagnosis and treatment modalities discussed here were used both at our center and elsewhere.

Results In September 2015, our patient was treated conservatively with a chest tube insertion for the first presentation of a right-sided pneumothorax. A recurrence necessitated a right-sided video-assisted thoracoscopic surgery (VATS) wedge resection and partial parietal pleurectomy in January 2016. Due to another recurrence of the right-sided pneumothorax in December 2016, a right-sided VATS was performed with multiple wedge resections and a complete pleurectomy, which revealed a pulmonary Langerhans' cell histiocytosis (PLCH) on histological and immunohistochemical examinations. The patient was advised to quit smoking, and her course was uneventful until May 2019, when she underwent a talc pleurodesis via right- sided VATS due to another right-sided pneumothorax. She recieved a talc slurry pleurodesis over a right- sided chest drain due to yet another recurrence on the right side. A right-sided VATS was performed in March 2020 due to a recurrence, and a blueish nodular lesion found intraoperatively was resected from the diaphragm. A diagnosis of TES was made after a histological test showed endometrial tissue. Since the patient's cycles and the onset of pneumothorax symptoms did not coincide, the final diagnosis of non- catamenial endometriosis-related pneumothorax was created. The patient is now on hormone therapy and continues to abstain from smoking. There hasn't been a recurrence in her case.

Conclusion In clinical practice, it is important not to just relay on the information available to us, but to reevaluate the patient history to uncover new clues leading to a new diagnosis. Furthermore, it isn ́t mandatory for TES to exhibit a relation to menstruation. As illustrated by our case, this could shed light on aspects previously deemed mundane and in turn facilitate an ideal treatment. In closing, there are exceptions to Occam ́s razor.