Thoracic Endometriosis Syndrome: Causes, Diagnosis, Treatment

Thoracic Endometriosis Syndrome (TES) is a rare but significant condition characterized by the growth of endometrial-like tissue in the thoracic (chest) cavity. It affects approximately 6-10% of reproductive-age women.

If left untreated, TES can cause debilitating symptoms and significantly impact the quality of life of affected individuals. This article describes this disorder, its diagnosis, and treatment.

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What is Thoracic Endometriosis Syndrome?

Endometriosis is marked by the development of endometrial tissue outside of the uterus. In TES, patches of endometrium are found in the ovary, cervix, labia, or vagina. In rare cases, it is also found in the chest cavity.

People with TES commonly experience chest pain, shortness of breath (dyspnea), hemoptysis (coughing up blood), and in severe cases, pneumothorax (collapse of the lung) or pleural effusion (fluid around the lungs). These symptoms tend to occur cyclically, correlating with the menstrual cycle and hormonal fluctuations.

The precise mechanism leading to the development of TES has yet to be discovered. However, hormonal influences, particularly high estrogen levels, may promote the growth and proliferation of this endometrial tissue. Dysregulation of the immune system and a genetic predisposition may also contribute to TES.

TES consists of 4 distinct clinical entities:

• Catamenial (menstrual cycle-related) pneumothorax: A spontaneous pneumothorax that coincides with menstruation. It is one of the most common manifestations of TES, occurring in about 80% of cases, and usually presents with chest pain and shortness of breath during the menstrual period.

• Catamenial hemothorax: Presence of blood in the pleural cavity, correlating with menstruation. Symptoms include chest pain, hemoptysis, and respiratory distress.

• Catamenial hemoptysis: Coughing up blood during menstruation due to endometrial tissue in the lungs or airways. Symptoms correlate with the menstrual cycle.

• Catamenial chest pain: Cyclic chest pain that coincides with menstruation. It may vary in severity and be associated with shortness of breath or coughing.

These clinical entities of TES share a common characteristic of cyclical symptomatology, occurring during the menstrual period due to hormonal fluctuations. Recognition of these clinical entities is essential for accurate diagnosis and appropriate management of TES.

Signs and Symptoms of Thoracic Endometriosis Syndrome

• Chest pain, particularly during menstruation
• Dyspnea (shortness of breath) 
• Hemoptysis (coughing up blood)
• Pneumothorax (collapsed lung)
• Pleural effusion (fluid buildup around the lungs)
• Catamenial pneumothorax

The Root Causes of Thoracic Endometriosis Syndrome

Hormonal Influences

Elevated estrogen levels, or estrogen dominance can promote the growth and proliferation of endometrial-like tissue outside the uterus, including in the thoracic cavity.

Immune System Dysfunction

Dysregulation of the immune system may fail to recognize and eliminate ectopic endometrial tissue, leading to its accumulation and subsequent symptoms.

Other Root Causes

Retrograde menstruation, or the backflow of menstrual blood through the fallopian tubes into the pelvic cavity, can potentially carry endometrial cells to the thoracic cavity.

Lymphatic or vascular dissemination is the spread of endometrial cells via lymphatic or blood vessels to distant sites, including the thoracic cavity.

How to Diagnose Thoracic Endometriosis Syndrome

Evaluation of TES begins with a review of a person's symptoms, pre-existing conditions, medications, and a physical exam, followed by imaging studies. If TES is suspected, invasive diagnostic tests may be necessary.

Clinical Presentation: Symptoms and Initial Assessment

1. During the medical history, inquire about the presence and timing of symptoms, particularly in relation to the menstrual cycle.

• Example:  A woman of reproductive age who presents with chest pain, dyspnea, or cough around the time of her menstrual period should be evaluated for TES.

2. Physical examination: Evaluate for chest tenderness, abnormal breath sounds, or signs of respiratory distress.

• Example:  Diminished breath sounds on the affected side may suggest pleural effusion or a pneumothorax.  

Imaging

1. Chest X-ray: Used to assess for pneumothorax, pleural effusion, or other thoracic abnormalities.
2. Computed tomography (CT) scan: Provides detailed images of the thoracic cavity to identify endometrial lesions, pleural abnormalities, or other associated findings.some text
   • CT imaging usually reveals endometrial implants in the diaphragm as hypo-attenuating areas or solitary nodule(s) may be present.  
3. Magnetic resonance imaging (MRI): Useful for evaluating soft tissue abnormalities and their extent and has less radiation exposure than CT.some text
   • Endometrial implants will be hyper-intense on the MRI.

Diagnostic Tests

1. Thoracoscopy: This minimally invasive procedure allows direct visualization and biopsy of thoracic lesions for definitive diagnosis.
2. Thoracentesis: Removal of pleural fluid for analysis, which may reveal the presence of endometrial-like cells or other characteristic features.

How to Treat Thoracic Endometriosis Syndrome

First-line therapy usually consists of suppression of ovarian estrogen secretion, often using oral contraceptives, progesterone, danazol, and gonadotropin-releasing hormone agonists (GnRH).   

Other therapies and treatments include:

Addressing Inflammation

Reducing inflammation in TES can improve symptoms, prevent complications, and enhance the quality of life for affected individuals. It can decrease pain, reduce pleural irritation, and decrease the risk of pneumothorax, which results from inflammation.  Ways to reduce inflammation include:

• Implementing an anti-inflammatory diet  
• Supplementation with omega-3 fatty acids, turmeric, and ginger

Balancing Hormones

Hormonal balance is aimed at lowering estrogen and can be addressed through:

• Dietary modifications, such as reducing intake of estrogen-mimicking foods
• Supplementation with magnesium, B vitamins, and chasteberry

Immune System Support

Supporting immune function can mitigate TES symptoms by reducing the inflammatory response, promoting tissue repair in areas damaged by endometrial tissue, and suppressing ectopic endometrial tissue growth. This can be achieved by:

• Nutritional strategies like incorporating antioxidant-rich foods
• Supplementation with vitamin D, selenium, and zinc

Pharmacological Interventions

• Hormonal therapy: GnRH agonists, oral contraceptives
• Pain management: NSAIDs, analgesics

Surgery

In some cases, surgery is warranted:

• Hysterectomy to reduce estrogen production
• Video-assisted thoracoscopic surgery (VATS) is used to palliate symptoms.  VATS procedures for TES include:some text
  • Removal of the portions of the lung with endometrial implants
  • Closure of diaphragmatic fenestrations 
  • Pleurodesis (chemical) 
• Excision of endometrial lesions

Risks of Untreated TES

Untreated Thoracic Endometriosis Syndrome (TES) poses various risks and complications that may significantly impact an individual's respiratory health, quality of life, and overall well-being.

Respiratory Risks and Complications 

Untreated TES can lead to a range of respiratory complications, including:

• Pneumothorax: Endometrial-like tissue in the pleural cavity can predispose individuals to recurrent episodes of spontaneous pneumothorax, resulting in sudden chest pain and shortness of breath.
• Pleural effusion: Ectopic endometrial tissue may cause inflammation and fluid accumulation in the pleural space, leading to pleural effusion. This can exacerbate respiratory symptoms and impair lung function.
• Hemoptysis
• Pulmonary nodules: Endometrial implants within the lungs can form nodules, which may mimic other pulmonary conditions in imaging studies.

Chronic Pain and Reduced Quality of Life 

TES is often associated with chronic, cyclical chest pain and discomfort, particularly during menstruation. The pain can significantly impact an individual's quality of life and daily functioning. Chronic pain may also lead to psychological distress, including anxiety and depression, further exacerbating the overall burden of the disease.

Other Risks

• Fertility issues: Although TES primarily affects the thoracic cavity, it may coexist with pelvic endometriosis, which can lead to fertility problems.
• Delayed diagnosis: Due to its rarity and nonspecific symptoms, TES is often misdiagnosed or underdiagnosed, leading to delays in appropriate management and the potential progression of the disease.
• Complications of surgical intervention: Surgical treatment may be necessary to remove ectopic endometrial tissue or manage pneumothorax. However, surgery carries inherent risks, including infection, bleeding, and anesthesia-related complications.

The risks of untreated TES emphasize the importance of early recognition, prompt diagnosis, and intervention to minimize respiratory complications, alleviate symptoms, and improve the quality of life for affected individuals.

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Key Takeaways

• Thoracic Endometriosis Syndrome (TES) is a rare but significant condition characterized by the growth of endometrial-like tissue in the thoracic (chest) cavity.
• Common symptoms of TES include chest pain, shortness of breath, hemoptysis, and pneumothorax or pleural effusion in severe cases. These symptoms occur cyclically and correlate with the menstrual cycle.
• Evaluation of TES begins with assessing a person's symptoms, pre-existing medication conditions, medications, and a physical exam, followed by imaging studies (X-ray, CT, MRI). If TES is suspected, invasive diagnostic tests (thoracoscopy or thoracentesis) may be necessary for diagnosis.
• First-line therapy usually consists of suppression of ovarian estrogen secretion, often using oral contraceptives, progesterone, danazol, and GnRH.
• Untreated TES may lead to pneumothorax, hemoptysis, pain, and reduced quality of life.