Thoracic endometriosis

Last updated March 09, 2023

Thoracic endometriosis is a rare form of endometriosis where endometrial-like tissue is found in the lung parenchyma and/or the pleura. It can be classified as either pulmonary, or pleural, respectively.^[1] Endometriosis is characterized by the presence of tissue similar to the lining of the uterus (the endometrium) forming abnormal growths elsewhere in the body. Usually these growths are found in the pelvis, between the rectum and the uterus, the ligaments of the pelvis, the bladder, the ovaries, and the sigmoid colon. The cause is not known. The most common symptom of thoracic endometriosis is chest pain occurring right before or during menstruation. Diagnosis is based on clinical history and examination, augmented with X-ray, CT scan, and magnetic resonance imaging of the chest. Treatment options include surgery and hormones.

Signs and symptoms

Thoracic endometriosis is characterised by onset of the following clinical symptoms within 24 hours prior to and 72 hours after onset of menses.

Catamenial pneumothorax : this is the most common clinical manifestation, present in 80% of cases. Catamenial pneumothorax is defined as a recurrent pneumothorax that occurs within the first 72 hours after menstruation. It may not necessarily occur with every menstrual cycle and in most cases is one-sided and on the right side. There are cases of catamenial pneumothorax on the left side, and on very rare occasions there may be a bilateral catamenial pneumothorax. Symptoms are the same as for other types of pneumothorax: chest pain, cough and breathlessness. Symptoms are usually mild but there may be severe presentations.^[2]
Catamenial hemothorax: this is a rare manifestation of thoracic endometriosis, occurring in 14% of cases. In almost all cases, the right side is affected but has been one case of a bilateral catamenial hemothorax documented. The most common presenting symptoms are nonspecific and include cough, chest pain and shortness of breath. In some cases, signs may mimic pulmonary embolism. The quantity of blood loss varies, but severe anemia is possible. In almost all cases, chest X-ray shows the presence of pleural effusion without specific characteristics. A CT scan may show additional features such as nodular lesions of the pleura, multiloculated effusions, or bulky pleural masses.^[2]
Cyclic haemoptysis: haemoptysis during menstruation is extremely rare, with about 30 case reports in medical literature.^[3] Currently, there have been no reports of massive haemoptysis or death. Cyclic haemoptysis is a sign of pulmonary parenchymal endometriosis; ectopic endometrial tissue in the lung responds to cyclical hormonal variation, bleeding along with the normal endometrium located in the uterus.^[2]
Pulmonary nodules: nodules are common radiological features in patients with thoracic endometriosis; most cases are associated with catamenial haemoptysis.

A woman with thoracic endometriosis may also have dysmenorrhoea and irregular menses.^[4]

Complications

Pneumothorax and haemothorax are rarely life-threatening. The most common complication is progressive tissue damage or scarring related to inflammation, and in extremely rare cases malignant transformation of the endometrial-like tissue.^[1]

Cause

The cause of thoracic endometriosis is unknown.^[5] Those with previous surgeries are more prone to developing thoracic endometriosis due to the surgical manipulation that can cause embolisation of the endometrial tissue into the thoracic cavity. Some thoracic endometriosis patients have been described as having a congenital defect in the diaphragm. There is also an association between thoracic and pelvic endometriosis.^{[ medical citation needed ]}

Pathophysiology

The endometrium, the tissue that normally lines the female uterus, undergoes changes with each menstrual cycle. At the end of each cycle and after the lining has thickened in preparation for hosting a fertilised ovum, it sloughs off, detaches, and is expelled through the cervix and vagina in the process of menstruation. In endometriosis, some endometrial-like tissue is found in other parts of the body; most often the pelvis and abdomen, the central nervous system, the nasal passages, skin and thorax. At these other 'ectopic' sites, endometrium tissue still responds to hormones with normal cyclical changes - bleeding roughly every 28 days.^{[ medical citation needed ]}

Theories explaining distant ectopic endometriosis include:

Retrograde menstruation: Retrograde menstruation proposes that endometrial cells within menstrual blood travel backward through the fallopian tubes (instead of flowing out of the body through the vagina) into the peritoneal cavity, where they can adhere to peritoneal surfaces and grow. This is supported by the idea that the flow of peritoneal fluid containing endometrial cells to the left hemidiaphragm is obstructed by the falciform and phrenicocolic ligaments, subsequently only reaching the right hemidiaphragm, and the observation that endometriosis is nine times more likely to occur on the right hemidiaphragm than on the left.^[6]
Vasculogenesis: Up to 37% of the microvascular endothelium of ectopic endometrial tissue originates from endothelial progenitor cells, which result in de novo formation of microvessels by the process of vasculogenesis rather than the conventional process of angiogenesis.^[7]^{[ clarification needed ]}
Lymphatic spread: endometrial fragments travel through the thoracic duct and hilar lymph nodes, reaching the chest cavity and causing pulmonary or pleural endometriosis.^[8]
Coelomic metaplasia theory: the pleura and peritoneum share the same embryological origin, both derived from mesothelium. A pathological stimulus could be responsible for inducing precursor cells (mesothelial stem cells) of the pleura or peritoneum in order to differentiate into endometrial cells.^[8]
Vascular embolisation: endometrial fragments are taken into the venous system, travel through the right side of the heart, and are deposited in the pulmonary circulation. The endometrial tissue settles in the lung parenchyma or pleura.^[8]

A review of autopsy data showed that patients with endometriosis have bilateral pulmonary lesions, which supports the vascular embolisation theory. The pleural and/or diaphragmatic lesions were always found on the left side, which supports the theory of coelomic metaplasia.^{[ medical citation needed ]}

Diagnosis

The diagnosis of thoracic endometriosis is primarily based on clinical history and examination, augmented with non-invasive studies such as X-ray, CT scan, and magnetic resonance imaging of the chest. Pelvic ultrasound is also useful to determine if the patient has any degree of pelvic or abdominal endometriosis (indicated by the presence of free fluid). More invasive methods for obtaining a tissue diagnosis of thoracic endometriosis include video thoracoscopy (for pleural or pulmonary biopsy), or bronchoscopy (for pulmonary or bronchial biopsy, or bronchial lavage).^[2] A case series has been reported in which clinical diagnosis was made in 50% of patients, the rest being diagnosed either via biopsy (25%) or bronchoalveolar lavage (25%).^{[ citation needed ]}

Treatment

Definitive diagnosis is necessary to avoid unnecessary treatment and exclude more serious diagnoses (for example, haemoptysis, pleural effusion or cancer). Overall treatment for pulmonary endometriosis is surgical, with subsegmentectomy. Preserving lung parenchyma is a priority while removing macroscopic signs of pathological tissue.^[5] Medical treatment can include the use of gonadotropin-releasing hormone analogues, which can cause cessation of menstruation. Side effects of this treatment can be decreased libido, as well as a 50% recurrence rate.^[9] Even in the asymptomatic, treatment is recommended to prevent possible complications listed above.

Epidemiology

Thoracic endometriosis affects women aged 15–54, who are between menarche and menopause. It can affect their quality of life, with catamenial pneumothorax being the most common presentation.^[1]

Related Research Articles

The endometrium is the inner epithelial layer, along with its mucous membrane, of the mammalian uterus. It has a basal layer and a functional layer: the basal layer contains stem cells which regenerate the functional layer. The functional layer thickens and then is shed during menstruation in humans and some other mammals, including apes, Old World monkeys, some species of bat, the elephant shrew and the Cairo spiny mouse. In most other mammals, the endometrium is reabsorbed in the estrous cycle. During pregnancy, the glands and blood vessels in the endometrium further increase in size and number. Vascular spaces fuse and become interconnected, forming the placenta, which supplies oxygen and nutrition to the embryo and fetus. The speculated presence of an endometrial microbiota has been argued against.

<span class="mw-page-title-main">Endometriosis</span> Disease of the female reproductive system

Endometriosis is a disease of the female reproductive system in which cells similar to those in the endometrium, the layer of tissue that normally covers the inside of the uterus, grow outside the uterus. Lesions can be found on ovaries, fallopian tubes, tissue around the uterus and ovaries (peritoneum), intestines, bladder, and diaphragm; it may also occur in other parts of the body. Some symptoms include pelvic pain, heavy periods, pain with bowel movements, painful urination, and infertility. Nearly half of those affected have chronic pelvic pain, while in 70% pain occurs during menstruation. Pain during sexual intercourse is also common. Infertility occurs in up to half of affected individuals. About 25% of individuals have no symptoms and 85% of those seen with infertility in a tertiary center have no pain. Endometriosis can have both social and psychological effects.

The pleural cavity, pleural space, or interpleural space is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication between the membranes, and also to create a pressure gradient.

Hemoptysis is the coughing up of blood or blood-stained mucus from the bronchi, larynx, trachea, or lungs. In other words, it is the airway bleeding. This can occur with lung cancer, infections such as tuberculosis, bronchitis, or pneumonia, and certain cardiovascular conditions. Hemoptysis is considered massive at 300 mL. In such cases, there are always severe injuries. The primary danger comes from choking, rather than blood loss.

A pneumothorax is an abnormal collection of air in the pleural space between the lung and the chest wall. Symptoms typically include sudden onset of sharp, one-sided chest pain and shortness of breath. In a minority of cases, a one-way valve is formed by an area of damaged tissue, and the amount of air in the space between chest wall and lungs increases; this is called a tension pneumothorax. This can cause a steadily worsening oxygen shortage and low blood pressure. This leads to a type of shock called obstructive shock, which can be fatal unless reversed. Very rarely, both lungs may be affected by a pneumothorax. It is often called a "collapsed lung", although that term may also refer to atelectasis.

Pleurisy, also known as pleuritis, is inflammation of the membranes that surround the lungs and line the chest cavity (pleurae). This can result in a sharp chest pain while breathing. Occasionally the pain may be a constant dull ache. Other symptoms may include shortness of breath, cough, fever, or weight loss, depending on the underlying cause. Pleurisy can be caused by a variety of conditions, including viral or bacterial infections, autoimmune disorders, and pulmonary embolism.

<span class="mw-page-title-main">Atelectasis</span> Collapse or closure of a lung resulting in reduced or absent gas exchange

Atelectasis is the collapse or closure of a lung resulting in reduced or absent gas exchange. It is usually unilateral, affecting part or all of one lung. It is a condition where the alveoli are deflated down to little or no volume, as distinct from pulmonary consolidation, in which they are filled with liquid. It is often called a collapsed lung, although that term may also refer to pneumothorax.

<span class="mw-page-title-main">Mediastinum</span> Central part of the thoracic cavity

The mediastinum is the central compartment of the thoracic cavity. Surrounded by loose connective tissue, it is an undelineated region that contains a group of structures within the thorax, namely the heart and its vessels, the esophagus, the trachea, the phrenic and cardiac nerves, the thoracic duct, the thymus and the lymph nodes of the central chest.

<span class="mw-page-title-main">Adenomyosis</span> Extension of endometrial tissue into the myometrium

Adenomyosis is a medical condition characterized by the growth of cells that proliferate on the inside of the uterus (endometrium) atypically located among the cells of the uterine wall (myometrium), as a result, thickening of the uterus occurs. As well as being misplaced in patients with this condition, endometrial tissue is completely functional. The tissue thickens, sheds and bleeds during every menstrual cycle.

<span class="mw-page-title-main">Pulmonary sequestration</span> Medical condition

A pulmonary sequestration is a medical condition wherein a piece of tissue that ultimately develops into lung tissue is not attached to the pulmonary arterial blood supply, as is the case in normally developing lung. This sequestered tissue is therefore not connected to the normal bronchial airway architecture, and fails to function in, and contribute to, respiration of the organism.

A hemothorax is an accumulation of blood within the pleural cavity. The symptoms of a hemothorax may include chest pain and difficulty breathing, while the clinical signs may include reduced breath sounds on the affected side and a rapid heart rate. Hemothoraces are usually caused by an injury, but they may occur spontaneously due to cancer invading the pleural cavity, as a result of a blood clotting disorder, as an unusual manifestation of endometriosis, in response to Pneumothorax, or rarely in association with other conditions.

Thoracentesis, also known as thoracocentesis, pleural tap, needle thoracostomy, or needle decompression, is an invasive medical procedure to remove fluid or air from the pleural space for diagnostic or therapeutic purposes. A cannula, or hollow needle, is carefully introduced into the thorax, generally after administration of local anesthesia. The procedure was first performed by Morrill Wyman in 1850 and then described by Henry Ingersoll Bowditch in 1852.

Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.

Catamenial pneumothorax is a spontaneous pneumothorax that recurs during menstruation, within 72 hours before or after the onset of a cycle. It usually involves the right side of the chest and right lung, and is associated with thoracic endometriosis. A third to a half of patients have pelvic endometriosis as well. Despite this association, CP is still considered to be misunderstood as is endometriosis considered to be underdiagnosed. The lack of a clear cause means that diagnosis and treatment is difficult. The disease is believed to be largely undiagnosed or misdiagnosed, leaving the true frequency unknown in the general population.

Subcutaneous emphysema occurs when gas or air accumulates and seeps under the skin, where normally no gas should be present. Subcutaneous refers to the subcutaneous tissue, and emphysema refers to trapped air pockets resembling the pneumatosis seen in pulmonary emphysema. Since the air generally comes from the chest cavity, subcutaneous emphysema usually occurs around the upper torso, such as on the chest, neck, face, axillae and arms, where it is able to travel with little resistance along the loose connective tissue within the superficial fascia. Subcutaneous emphysema has a characteristic crackling-feel to the touch, a sensation that has been described as similar to touching warm Rice Krispies. This sensation of air under the skin is known as subcutaneous crepitation, a form of crepitus.

Pleural disease occurs in the pleural space, which is the thin fluid-filled area in between the two pulmonary pleurae in the human body. There are several disorders and complications that can occur within the pleural area, and the surrounding tissues in the lung.

Tumor-like disorders of the lung pleura are a group of conditions that on initial radiological studies might be confused with malignant lesions. Radiologists must be aware of these conditions in order to avoid misdiagnosing patients. Examples of such lesions are: pleural plaques, thoracic splenosis, catamenial pneumothorax, pleural pseudotumor, diffuse pleural thickening, diffuse pulmonary lymphangiomatosis and Erdheim–Chester disease.

Asbestos-related diseases are disorders of the lung and pleura caused by the inhalation of asbestos fibres. Asbestos-related diseases include non-malignant disorders such as asbestosis, diffuse pleural thickening, pleural plaques, pleural effusion, rounded atelectasis and malignancies such as lung cancer and malignant mesothelioma.

Endometriosis and its complications are a major cause of female infertility. Endometriosis is a dysfunction characterized by the migration of endometrial tissue to areas outside of the endometrium of the uterus. The most common places to find stray tissue are on ovaries and fallopian tubes, followed by other organs in the lower abdominal cavity such as the bladder and intestines. Typically, the endometrial tissue adheres to the exteriors of the organs, and then creates attachments of scar tissue called adhesions that can join adjacent organs together. The endometrial tissue and the adhesions can block a fallopian tube and prevent the meeting of ovum and sperm cells, or otherwise interfere with fertilization, implantation and, rarely, the carrying of the fetus to term.

The pulmonary pleurae are the two opposing layers of serous membrane overlying the lungs and the inside of the surrounding chest walls.

References

1 2 3 Rojas, J. (2014). Endometriosis pulmonar parenquimal. Rev Soc Peru Med Interna, 27(1).
1 2 3 4 Alifano, Marco; Trisolini, Rocco; Cancellieri, Alessandra; Regnard, Jean François (2006). "Thoracic Endometriosis: Current Knowledge". The Annals of Thoracic Surgery. 81 (2): 761–769. doi:10.1016/j.athoracsur.2005.07.044. PMID 16427904.
↑ McGraw-Hill Medical| AccessMedicine | McGraw-Hill Medical http://accessmedicine.mhmedical.com/content.aspx bookid=331
↑ U.S National Library of Medicine https://www.nlm.nih.gov/medlineplus/spanish/ency/article/000915.htm
1 2 Ucvet, Ahmet; Sirzai, Esra Yamansavci; Yakut, Funda Cansun; Yoldas, Banu; Gursoy, Soner (2014). "Endometriosis pulmonar torácica: Presentación de 2 casos de una enfermedad muy poco frecuente". Archivos de Bronconeumología. 50 (10): 454–455. doi:10.1016/j.arbres.2013.11.018. PMID 24411929.
↑ Camran Nezhat; Steven R. Lindheim; Leah Backhus; Mailinh Vu; Nataliya Vang; Azadeh Nezhat; Ceana Nezhat (2019). "Thoracic Endometriosis Syndrome: A Review of Diagnosis and Management". Journal of the Society of Laparoendoscopic Surgeons. 23 (3): e2019.00029. doi: 10.4293/JSLS.2019.00029 . PMC 6684338 . PMID 31427853.
↑ Laschke MW, Giebels C, Menger MD (2011). "Vasculogenesis: a new piece of the endometriosis puzzle". Human Reproduction Update. 17 (5): 628–36. doi: 10.1093/humupd/dmr023 . PMID 21586449.
1 2 3 Molinar, L; Romero, P; Padilla, MA (2011). "Endometriosis parenquimatosa pulmonar multifocal". Patología Revista Latinoamericana (in Spanish). 49 (4): 262–266.
↑ Chung, Chan Kyung; Jung, Yeon Soo; Kim, Jeong Hyeon; Choi, Young Sik; Seo, Seok Kyo (2012). "Two cases of catamenial pneumothorax". Korean Journal of Obstetrics & Gynecology. 55 (12): 1031. doi: 10.5468/KJOG.2012.55.12.1031 .

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[Endometriosis_pulmonar_parenquimal-1] 1 2 3 Rojas, J. (2014). Endometriosis pulmonar parenquimal. Rev Soc Peru Med Interna, 27(1).

[Thoracic_Endometriosis:_Current_Knowledge-2] 1 2 3 4 Alifano, Marco; Trisolini, Rocco; Cancellieri, Alessandra; Regnard, Jean François (2006). "Thoracic Endometriosis: Current Knowledge". The Annals of Thoracic Surgery. 81 (2): 761–769. doi:10.1016/j.athoracsur.2005.07.044. PMID 16427904.

[McGraw-Hill_Harrison-3] McGraw-Hill Medical| AccessMedicine | McGraw-Hill Medical http://accessmedicine.mhmedical.com/content.aspx bookid=331

[U.S_National_Library_of_Medicine-4] U.S National Library of Medicine https://www.nlm.nih.gov/medlineplus/spanish/ency/article/000915.htm

[Endometriosis_pulmonar_toracica-5] 1 2 Ucvet, Ahmet; Sirzai, Esra Yamansavci; Yakut, Funda Cansun; Yoldas, Banu; Gursoy, Soner (2014). "Endometriosis pulmonar torácica: Presentación de 2 casos de una enfermedad muy poco frecuente". Archivos de Bronconeumología. 50 (10): 454–455. doi:10.1016/j.arbres.2013.11.018. PMID 24411929.

[6] Camran Nezhat; Steven R. Lindheim; Leah Backhus; Mailinh Vu; Nataliya Vang; Azadeh Nezhat; Ceana Nezhat (2019). "Thoracic Endometriosis Syndrome: A Review of Diagnosis and Management". Journal of the Society of Laparoendoscopic Surgeons. 23 (3): e2019.00029. doi: 10.4293/JSLS.2019.00029 . PMC 6684338 . PMID 31427853.

[7] Laschke MW, Giebels C, Menger MD (2011). "Vasculogenesis: a new piece of the endometriosis puzzle". Human Reproduction Update. 17 (5): 628–36. doi: 10.1093/humupd/dmr023 . PMID 21586449.

[Endometriosis_parenquimatosa_pulmonar_multifocal-8] 1 2 3 Molinar, L; Romero, P; Padilla, MA (2011). "Endometriosis parenquimatosa pulmonar multifocal". Patología Revista Latinoamericana (in Spanish). 49 (4): 262–266.

[9] Chung, Chan Kyung; Jung, Yeon Soo; Kim, Jeong Hyeon; Choi, Young Sik; Seo, Seok Kyo (2012). "Two cases of catamenial pneumothorax". Korean Journal of Obstetrics & Gynecology. 55 (12): 1031. doi: 10.5468/KJOG.2012.55.12.1031 .

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