INTRODUCTION:Thoracic endometriosis syndrome is a form of extra-pelvic endometriosis associated with the growth of endometrial implants within the airways, lung parenchyma, or pleura. Catamenial pneumothorax, recurrent episodes of pneumothorax associated with menstruation, is the most common manifestation. Thoracic endometriosis typically, but not universally, occurs in association with pelvic endometriosis. We describe a patient with thoracic endometriosis in conjunction with umbilical and diaphragmatic endometriosis but no visualized pelvic involvement.
CASE PRESENTATION:A 44 year old female presented with a history of recurrent right sided pneumothorax. Her first episode occurred 10 years prior and resolved spontaneously. Seven years later she had a second pneumothorax. Despite chest tube drainage, a persistent air leak necessitated thoroscopic exploration of the right chest with mechanical pleurodesis. Thoracoscopy did not demonstrate any parenchymal abnormalities or endometrial pleural implants. It was unclear if the diaphragm was inspected for defects. Thirty-two months later she developed a subsequent right pneumothorax occurring with the onset of menses. Additionally, in the months prior to presentation, she reported intermittent right shoulder pain occurring in conjunction with her irregular menses 2 to 3 times monthly and noted the development of a lump at her navel. She denied dyspnea, fever, cough, or hemoptysis. Physical examination was unremarkable except for decreased breath sounds at the apex of the right lung and pelvic exam revealed an anteflexed uterus with multiple fibroids. Chest radiograph revealed a large right pneumothorax. Pelvic ultrasound revealed multiple uterine fibroids. Laboratory studies, including complete blood count, coagulation profile, and electrolytes were unremarkable. The patient underwent an exploratory laparotomy for evaluation of endometrial disease and total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO) for management of her fibroids. Surgical exploration revealed a nodule abutting the umbilicus. Inspection of the right diaphragm revealed extensive adhesions with brownish blue nodules which bled easily but no obvious structural defects ( Image 1 ). Pathology confirmed the presence of endometriosis involving the umbilicus nodule ( Image 2 ) and from the diaphragm biopsies. Post operative course was uneventful with resolution of the pneumothorax. The patient denied recurrent shoulder pain or pneumothorax at 6 month follow-up.
DISCUSSIONS:Endometriosis affects 5-15% of women in the reproductive age. Thoracic endometriosis is a rare disorder with catamenial pneumothorax the most commonly described manifestation. Menstrual related hemothorax, hemoptysis, pneumomediastinum, pulmonary nodules, and a pleural mass have also been described. The mean age at presentation is 35 years with a range from 15 to 54 years. Many theories have been proposed to explain the mechanism of thoracic endometriosis but the pathophysiology is incompletely elucidated. Most, but not all, patients with thoracic endometriosis have a history of pelvic endometriosis. In a series of 110 patients with thoracic endometriosis 23 of 38 patients who presented with pneumothorax and all 15 patients who presented with hemothorax had evidence of pelvic endmetriosis . Rarely, other sites of extrapelvic endometriosis are seen in conjunction with thoracic disease. Simultaneous umbilical and thoracic endometriosis is rare. Rachagan et al  reported such a case that responded poorly to medical management and required a total abdominal hysterectomy and bilateral salpingo-oophorectomy.
CONCLUSION:An awareness of the syndrome and a high index of suspicion is needed in order to recognize thoracic endometriosis. We report a rare case of catamenial pneumothorax with diaphragmatic and umbilical endometriosis successfully treated with TAH-BSO.
DISCLOSURE:Vichaya Arunthari, No Financial Disclosure Information; No Product/Research Disclosure Information