Thoracic endometriosis is probably one of the most challenging forms of endometriosis in the extrapelvic location. It can be recognized on the dome of the diaphragm, the lung parenchyma (portion of the lung involved in gas transfer), visceral and parietal pleura (serous membrane that covers surface of lung and the outer membrane covering the thoracic cavity respectively), and even the tracheobronchial tree (branching airway passages of the respiratory system). The challenge with these lesions is that they can be the source of catamenial pneumothorax (pneumothorax development in conjunction to one’s period), catamenial hemothorax (blood collection in the pleural space in conjunction to one’s period), catamenial hemoptysis (coughing up blood in conjunction to one’s period) and lung nodules. The other challenging aspect of thoracic endometriosis is unless there is chest pain, difficulty breathing, or other clear respiratory symptoms, it does not come to the attention of doctors much. In addition, many physicians neglect to ask a woman about her period when these symptoms come up and whether they can be in occurrence with the menstrual cycle itself. Thus in cases where patients are symptomatic for thoracic endometriosis, it is important during laparoscopic examination to assess the status of the diaphragm, along with the liver and upper abdomen, to check for signs of possible thoracic endometriosis implants. However, many times if there are lesions on the diaphragm, they can reflect to the pericardium, or to the right side, and thus can be difficult to identify. Therefore it is crucial to perform surgery alongside a thoracic surgeon in order to properly treat these lesions. Lesions can be excised via laparoscopy and suturing. There are even cases where a mesh covering is placed along the diaphragm where there is no obvious hole, but the defects are visible and large. This is done in order to prevent further pneumothorax or a history of pneumothorax and is often performed on patients with recurrent catamenial pneumothorax, along with possible lobectomies, partial lobectomies, and lung resection procedures.
What is the thoracic cavity?
The thoracic cavity, or chest cavity as it is commonly known, is the chamber of the body protected by the rib cage and associated skin, muscle, and tissue. It is most known to contain two highly vital organ systems, the respiratory system (lungs, bronchi, esophagus, etc.) and the cardiovascular system (namely the heart and its blood vessels). It begins with the esophagus, right around the base of the neck, and ends with the diaphragm, which separates the thoracic cavity from the abdominal cavity. The central compartment of the thoracic cavity is mediastinum.
Thoracic endometriosis is defined as the presence of endometrial tissue in or around the thoracic cavity, namely the lungs and diaphragm. In other words, tissue that truly belongs in the uterine cavity is found to be in the thoracic cavity. It is a complex and rare condition, with proper diagnosis often being delayed. This can lead to recurrent hospitalizations and other medical complications.
What areas of the thoracic cavity can be affected by endometriosis?
Lungs: two saclike respiratory organs located on the left and right side of the thoracic cavity. The branching passages of the lungs allow for proper air exchange in body, in which oxygen is absorbed into the blood and carbon dioxide is released.
Pleura: the serous membranes that lines the thoracic cavity and surrounds both lungs. The pleura has both an inner layer (visceral pleura) and out layer (parietal pleura), with an intermembrane space in between the two (pleural space).
Heart: the muscular organ that is responsible for pumping blood through the blood vessels of the circulatory system, providing the body with oxygen and nutrients.
Pericardium: the membrane surrounding the heart, composed of a fibrous outer layer, and inner double serous membrane.
Diaphragm: The long sheet of internal skeletal muscle that extends the bottom of the thoracic cavity. In addition to playing a key role in how our body is able to breathe properly, the diaphragm separates the thoracic cavity from the abdominal cavity.
What is a pneumothorax and how does it relate to endometriosis?
Endometriosis lesions in the thoracic cavity are of a particular concern due to their association with catamenial pneumothorax (a collapsed lung occurring in conjunction with menstrual periods). When there is a small rupture in the lung, a pneumothorax occurs when air leaks out of the lungs, into the space between it and the chest wall (pleural space). The higher pressure this creates against the lung wall (visceral pleura), can in turn cause the lung to partially or totally collapse.
A pneumothorax can be related to a variety of different disorders, namely diseases of the respiratory airways (i.e. COPD, severe, asthma, etc.) and infections of the lung (severe pneumonia, tuberculosis, etc.) However, when it comes to patients with endometriosis or a family history of such, there is concern for the pneumothorax being related to one’s menstrual periods (catamenial pneumothorax). Thus if one is diagnosed with a pneumothorax, particularly in recurring cases, it is important to note if these symptoms coincide with one’s monthly menstrual cycle and voice this to one’s physician.
A pneumothorax is often marked by a total or partial collapsing of the lung. The image above shows a left lung pneumothorax (right side of the image).
Why thoracic endometriosis can often go unnoticed?
Endometriosis is a disease that sadly can often go misdiagnosed by the average physician, which is even more the case when dealing with lesions in the thoracic cavity. Unless there is chest pain, difficulty breathing, or other clear respiratory symptoms, thoracic endometriosis will not come to the attention of doctors. More importantly, when these symptoms do come up, rarely is a physician thinking of a possible thoracic endo case, but rather the doctor will only look to rule out cardiovascular and respiratory issues. However, the truth of the matter is no pneumothorax case should be discussed or treated without considering its primary origin, which can be in some cases the pelvis. When this goes unnoticed and in turn misdiagnosed, many of these patients do end up getting repeated thoracic surgeries without the patient or doctor knowing what is happening.
The important question to ask
While it is certainly important in cases of chest pain and shortness of breath to rule out cardiovascular and respiratory disease first, it is important that a physician considers the source of the symptoms. In cases where respiratory and cardiovascular tests are ruled unremarkable, but there are repeated instances of pneumothorax, and particularly when there is a history or family history of endometriosis, thoracic endometriosis should be considered. The key question for both a patient and doctor to ask alike is whether the clinical picture overlaps with the patient’s period. If a pneumothorax is recurrent and/or their is a relationship between the patient’s symptoms and their menstrual cycle, this could point to a cause of uterine origin, such as endometriosis.
What are the symptoms of thoracic endometriosis?
While the common symptoms of endometriosis can certainly be associated with thoracic endometriosis, there are five distinct clinical entities that are of particular concern for endometriosis in the thoracic cavity:
Catamenial pneumothorax: This can be defined as a pneumothorax occurring in conjunction with a woman’s period. In other words, recurrent air leakage out of the lung and into the pleural space, with a particular association with one’s menstrual cycle. This should be of particular concern if a patient keeps gett hospitalized with for a recurrent pneumothorax.
Catamenial hemothorax: A hemothorax is a collection of blood located in the space between the outer lung and the chest wall (pleural space). Thus, a catamenial hemothorax is a hemothorax in conjunction with menstruation. Once again, this is of particular concern to endometriosis patients when there is a recurrent hemothorax with no known cardiovascular or respiratory cause.
Catamenial hemoptysis: Defined as the coughing up blood or blood-strained mucus, this is a common sign of many lung or respiratory infections. If this symptom is recurring around the time of menstruation, this could be yet another sign of the underlying cause of the symptoms or infection being related to thoracic endometriosis.
Pulmonary nodules: These small single masses in the lungs, while benign, can be the cause of other symptoms above such as hemoptysis. However, it is also important to consider the etiology of the nodules themselves, which when coupled with any of the symptoms above, can point towards endometriosis.
Catamenial chest pain and/or shortness of breath: Intermittent chest pain or shortness of breath is often attributed to cardiovascular or respiratory diseases. However, if a patient notes that they find their symptoms recurring monthly or specifically in synch with their menstrual cycle, it is important to alert one’s physician of this, as this can be a key clinical indication of thoracic endometriosis.
Diagnosing & Treating Thoracic Endometriosis
How to properly diagnose Thoracic Endometriosis?
History and Physical examination: In order to diagnose thoracic endometriosis, a gynecologist must be highly thorough and meticulous in their medical history taking, as well as in their physical examination. It is important for a patient to let their doctor know if they have a history of recurrent pneumothorax, intermittent chest pain, and shortness of breath during their menstrual cycle, and if they have endometriosis or a family history of the disease as well. In terms of physical examination, if a patient has tenderness upon a pelvic and/or rectovaginal exam, with symptoms including catamenial chest pain or shortness of breath, and a history of pneumothorax, these are all signs that can point towards thoracic endometriosis and warrant thoracic cavity inspection.
Imaging testing: One of the common ways a pneumothorax is diagnosed is via a chest x-ray. This is important to note as recurrent pneumothorax, particularly at the time of a woman’s period, is one of they key clinical indications of thoracic endometriosis. In addition, an endovaginal ultrasound is also critical as this can point towards endometriosis within the pelvic cavity, and thus the possible etiology of the catamenial symptoms themselves. A CT scan or MRI are also highly useful imaging tests that an endometriosis specialist may advise for further diagnostic testing.
Surgical diagnosis (laparoscopy & thoracoscopy): While imaging tests and meticulous medical history taking can all point towards inclinations of thoracic endometriosis, laparoscopic and thoracoscopic surgery are the primary ways to actually view lesions themselves and confirm a diagnosis. Both are considered minimally invasive forms of surgery as they simply require small incisions, which a camera (scope) is then inserted through. The surgeon can then use excision clippers to cut out suspected endometriosis lesions, obtain a biopsy, and sent it to a pathology lab for a confirmation of diagnosis. Thus laparoscopic excision and thoracoscopic excision/resection are the gold standards for surgically diagnosing a patient with suspected thoracic endometriosis.
How to treat thoracic endometriosis (Laparoscopy & Thoracoscopically)?
Laparoscopic excision: When it comes to surgically treating endometriosis located in the pelvic and abdominal cavity, laparoscopic excision excision surgery is the gold standard. It removes lesions while preserving healthy surrounding tissue, allowing the surgeon to obtain a biopsy sample to be sent to pathology to confirm a diagnosis. It is also a minimally invasive surgery that ensures minimal use of electricity and heat, ensuring for a quicker recovery time and relief of symptoms. It is important to assess the pelvic and abdominal cavity for suspected thoracic endometriosis cases, as this can provide insight into the cause of the patient’s respiratory symptoms.
Thoracic excision/resection: Suspected endometriosis lesions in the thoracic cavity can also be removed by minimally invasive surgery. Excision of these lesions is done via Video-assisted thoracoscopic surgery (VATs). These lesions will either be excised individually or will require resection of part of the organ or membrane affected.
Why do surgeons have difficulty visualizing thoracic endometriosis?
Thoracic endometriosis is typically more easy to recognize on the dome of the right side of the diaphragm. The caveat here, is visualizing in direct view does not rule out endometriosis lesions that are obscured due to the curve of the liver. Therefore, doctors must use the technique of the 30 degree scope and look behind the liver by pressing down on it and examining the dome of the diaphragm. Many times there can be lesions on the diaphragm that can reflect to the pericardium, even on the right side. Therefore it is crucial to perform surgery alongside a thoracic surgeon in order to properly treat these lesions. The left side of the diaphragm can be performed the same way, but if one reaches the pericardium, it is also key to have a thoracic surgeon onsight.
Thoracic endometriosis remains a challenge for clinicians. When chest pain, shortness of breath, hemothorax or recurrent pneumothorax occur in women of reproductive age, a diagnosis of diaphragmatic or thoracic endometriosis must be considered (Duyos, Lopez-Carrasco et al. 2014). Since there is strong association of thoracic endometriosis with severe pelvic endometriosis and infertility, the necessity of thorough evaluation of the pelvis in patients with thoracic endometriosis to rule out possible pelvic endometriotic lesions is indicated. In many of these patients who were treated via video-assisted thoracoscopic surgery (VATs) by thoracic surgeons, the involvement of and experienced gynecologist is essential. Patients who may desire to conceive should undergo excision of any pelvic endometriotic lesions (Soriano, Schonman et al. 2012).
The Duel Compartment Approach
Up to 80% of women with thoracic endometriosis also contain endometriosis lesions in the abdominal/pelvic cavity, and yet the surgical treatment for the disease is typically performed in separate procedures (Camran, Nezhat et al. 2014). Therefore, we believe that when an endometriosis patient shows clinical signs of thoracic endometriosis, many times these patients need to have what we call Dual Compartment Approach. This is a combination of both minimally invasive laparoscopic surgery and video-assisted thoracoscopic surgery (VATs). In other words, a thoracoscopic surgeon should look through the thoracic cavity, while the gynecological surgeon looks through the abdominal and pelvic cavity for any signs of endometriosis lesions. The positive endometriosis findings within the pelvis in patients with a history of recurrent pneumothorax, is an indication for thoracoscopic inspection.
In addition to proper minimally-invasive visualization of both the abdominal/pelvic and thoracic cavity, we at the Seckin Endometriosis Center stress the importance of proper removal of the endometriosis itself. Lesions can be taken out via laparoscopy and suturing, but if there are lesions located within the thoracic cavity, thoracoscopy is needed and the lesions can be removed by staple, which we have done in our practice for many years. We have great experiencing in suturing transmurally through the full thickness of the diaphragm. There are even cases where we may apply a mesh covering even when there is no obvious hole in the diaphragm, but the defects are visible and large, in order to prevent further pneumothorax or a history of pneumothorax. This procedure is often performed on patients with recurrent catamenial pneumothorax, along with possible lobectomies, partial lobectomies, and lung resection procedures.
The Importance in assessing the diaphragm
Finally, in every laparoscopic examination that is done with our endometriosis patients, we ask specific questions to ensure that there is no history of pneumothorax. If there is, we believe that one of the first things that needs to be assessed upon surgery, along with the liver and upper abdomen, is the status of the diaphragm.
If you feel you are suffering from recurrent chest pain and/or shortness of breath, are having recurrent pneumothorax, and have a history of family history of endometriosis, it is important to find the meticulous care and physicians that you both need and deserve to diagnose such a complex disease as thoracic endometriosis.
If you feel you are suffering from recurrent chest pain and/or shortness of breath, are having recurrent pneumothorax, and have a history of family history of endometriosis, it is important to find the meticulous care and physicians that you both need and deserve to diagnose such a complex disease as thoracic endometriosis
The New York Times featured the care of an anonymous patient of the Seckin Endometriosis Center who was diagnosed with thoracic endometriosis. Even after undergoing pleurodesis (mechanical attachment of the lung to its pleural lining) by a thoracic surgeon, the patient continued to suffer from multiple episodes of catamenial pneumothorax. It was not until a dual compartment approach was performed, by both the Seckin Endometriosis Center and the patient’s thoracic surgeon, was the patient able to be properly diagnosed and surgically treated.
You can read more stories of patients with thoracic endometriosis, and other varying stages of endometriosis, in our testimonial section.
Nezhat, Camran, MD et al. “Multidisciplinary Treatment for Thoracic and Abdominopelvic Endometriosis.” Journal of the Society of Laparoendoscopic Surgeons, National Institute of Health. 2014 Jul-Sep; 18(3).
Duyos, Lopez-Carrasco et al. “Management of thoracic endometriosis: Single Institution Experience.” European Journal of Obstetrics & Gynecology and Reproductive Biology. July, 2014 Volume 178, Pages 56-59.
Soriano, David, MD et al. “Thoracic Endometriosis Syndrome is Strongly Associated With Severe Pelvic Endometriosis and Infertility.” The Journal of Minimally Invasive Gynecology. November-December, 2012 Volume 19, Issue 6, Pages 742-748.
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