Thoracic endometriosis

Case Report:
Thoracic Endometriosis
Student’s NameDepartment of Internal medicine, Division of Pulmonary and Critical care, Good Samaritan hospital.
Wright state University, Dayton, Ohio, USA.Student’s Contact Info.Professor’s Name
Wd Count: 1099
Abstract
Pleural effusion due to Thoracic endometriosis is a rare occurrence. It is characterized by bloody pleural fluid that occurs in women especially in the years when they are reproductive and usually referred to ‘catamenial hemothorax”. It is defined by the occurrence of functional endometrial tissue in pleura. However, the spectrum of Thoracic endometriosis (TE) is vast and can involve, in addition to pleura, the lung parenchyma, tracheobronchial airways, Diaphragm, and pericardium.
Clinical presentation of TE can be a recurrent pneumothorax, hemothorax, hemopneumothorax, hemoptysis or pulmonary nodule.
The primary symptoms include chest pain, dyspnea, and hemoptysis. Diagnosis is usually deferred for several years after the symptoms of the condition start to show. The pleural effusion in TE is predominantly right side.
We described a 33-year-old African American female non –smoker. The female had a history of chronic recurrent pelvic pain and pelvic endometriosis, followed in infertility clinic, and emerged to have recurrent right-sided pleuritic pains in the chest, and worsening dyspnea. She was diagnosed with right-sided bloody pleural effusion. Pleural biopsy revealed the focal involvement of endometrial stromal tissue consistent with Pleural endometriosis.

The patient underwent Pleurectomy and Talc pleurodesis with no recurrence of pleural effusion after six months follow up.
Keywords: Thoracic endometriosis, Pleural effusion, hemothorax
Table of Contents
TOC o “1-3” h z u Abstract PAGEREF _Toc506922091 h 2Introduction PAGEREF _Toc506922092 h 4Case Report PAGEREF _Toc506922093 h 4Discussion PAGEREF _Toc506922094 h 6Learning Points PAGEREF _Toc506922095 h 8References PAGEREF _Toc506922096 h 9
IntroductionRecurrent hemorrhagic pleural effusion in women during their reproductive year can be the clinical presentation of Thoracic endometriosis syndrome (TES) 1. The case report presented is of a recurrent right bloody pleural effusion that observed in a young female who had a history of pelvic endometriosis who was followed in an infertility clinic. The diagnosis of Thoracic endometriosis with Pleural involvement was confirmed with pleural biopsy which revealed a focal involvement of functional endometrial tissue within the Pleura. The patient underwent Pleurectomy and talc pleurodesis successfully without recurrence of the pleural fluid. Thoracic endometriosis should be in the differential diagnosis of hemorrhagic pleural effusion in women of childbearing age, particularly in a patient who has already an established diagnosis of pelvic endometriosis. Hemorrhagic pleural effusion due to Thoracic endometriosis can mimic other conditions such as pulmonary thromboembolism, trauma, malignancy, Tuberculosis, and others2.
Case ReportA 33-year-old African American female non -smoker, brought to her physician, who administers her primary care, with increasing right-sided pleuritic chest pain and worsening dyspnea for the last several days. CXR was done and revealed right pleural effusion Image (A). The treatment that was administered to the patient was with oral antibiotics for presumed pneumonia with parapneumonic effusion and was later referred to chest clinic. She also had a history of pelvic pain and cramps that were recurrent since she was in high school. She was diagnosed with Endometriosis of pelvic peritoneum by Pelvic Laparoscopy 3 years ago and followed in an infertility clinic. Further, she was on an oral contraceptive for ten years prior to her recent marriage.
Later, the patient was admitted for further testing and management at the hospital. Results from a physical exam revealed that she had diminished breath sounds on the right side. CT–angiogram of the chest demonstrated large right pleural effusion. No pulmonary embolism and no mass lesion, image (B). An Ultrasound-guided thoracentesis was performed which resulted in 1500 cc of bloody pleural fluid. The pleural fluid RBC count was 611000 and WBC count 159.The cytology examination of the patient’s pleural fluid tested negative for malignancy, and the Microbiology study was negative including AFB culture. Blood hematological study was healthy, and collagen vascular disease workup was negative.
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Image (A) Image (B)
The patient subsequently underwent repeated Thoracentesis in the next few weeks for recurrent right pleural effusion and eventually underwent diagnostic thoracoscopy which described normal looking parietal pleura with no obvious lesion or abnormality. Random Pleural biopsy revealed focal involvement by endometrial stromal proliferation and the immunohistologic study was positive for the existence of endometrial tissue consistent with Pleural endometriosis images 1-2.

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Image (1) Image (2)
DiscussionThoracic endometriosis (TE) is an occurrence that is rare in women especially in their reproductive age and is defined as functional endometrial tissue within the pulmonary parenchyma including airways, lung tissue, Pleura3. Pericardium and diaphragms involvement were reported as well4. Most patients with TES has associated evidence of pelvic endometriosis5. Presenting symptoms include chest pain, dyspnea and hemoptysis. The clinical presentation of TES includes four different clinical presentations including Pneumothorax, Hemorrhagic pleural effusion (Hemothorax), Hemoptysis and Pulmonary nodule/mass. Pneumothorax due to TES is the commonest form of TES followed by Hemothorax, Hemoptysis and Pulmonary Nodule. Catamenial Pneumothorax and Hemothorax due to TES are almost always confined to the right side where is a lung parenchymal endometriosis can be on either side5. Radiographic findings and presenting symptoms of TES are non- specific. There are three major theories regarding the pathogenesis of TE including implantation by trans-diaphragmatic pass of endometrial tissue that is conducted via abdomen and diaphragmatic fenestration from pelvic endometrial tissue, vascular or lymphatic microembolization, and coelomic metaplasia6. Patient will normally present with catamenial hemoptysis, pneumothorax, pulmonary nodule, and or in our case hemothorax. Patient with Thoracic endometriosis commonly present with a history of infertility and pelvic surgery. Bronchoscopy with BAL and pleural fluid analysis are rarely beneficial for definitive diagnosis of thoracic endometriosis. Patient instead, usually require Thoracotomy or VATS (Video assistant Thoracoscopy) to assist with tissue diagnosis7. Undertaking a medical therapy should always be considered as the first line of treatment after an individual has been diagnosed with TE. Treatment of TE is usually with hormonal therapy (Danazol or GnRH analogs such as Leuprolide) to suppress the endometrial tissue by obstructing estrogen activity. The recurrence rate in medical hormonal therapy is up to 50%. VATS with chemical pleurodesis and or decortication can also be useful for treatment and prevention of further recurrence and is superior to hormonal therapy. A combination of medical hormonal and surgical therapy is the most effective treatment8. In our case, the patient preferred the surgical approach and underwent pleurectomy and Talc pleurodesis without recurrence of pleural effusion in long-term follow-up.
Learning PointsIt is imperative to keep Thoracic endometriosis in the differential diagnosis in young females with histories of infertility and pelvic surgeries who present with catamenial chest pain, dyspnea, hemoptysis, recurrent pleural effusion, pulmonary nodule, or pneumothorax. Diagnosis of TE is frequently delayed few years after presentation. Tissue diagnosis is needed in most cases to confirm TES. Treatment options include suppression of endometrial tissue and prevention of pelvic seeding by hormonal therapy, or surgical and chemical pleurodesis which are superior to hormonal therapy in preventing recurrence. Combination of medical and surgical treatment is needed most in advanced cases.
 
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