Catamenial pneumothorax

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Catamenial pneumothorax
Pneumothorax CXR.jpg
Pneumothorax shown on a chest x-ray. Air fills the space between the collapsed left lung and the chest wall.
Specialty Pulmonology
Symptoms Chest or shoulder pain, shortness of breath
Usual onset30-40 years of age
CausesThoracic endometriosis
Risk factors Congenital or acquired diaphragmatic defects allowing entry of endometrial tissue into the pleural space
Diagnostic method VATS procedure
TreatmentSurgical - VATS procedure to remove ectopic endometrial tissue
Pharmacologic - continuous OCP's are favored to suppress menstruation

Catamenial pneumothorax is a spontaneous pneumothorax that recurs during menstruation, within 72 hours before or after the onset of a cycle. [1] It usually involves the right side of the chest and right lung, and is associated with thoracic endometriosis. [2] 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.

Contents

Catamenial pneumothorax is defined as at least two episodes of recurrent pneumothorax corresponding with menstruation. It was first described in 1958 when a woman presented with 12 episodes of right-sided pneumothorax over 1 year, recurring monthly with menstruation. Thoracotomy revealed thoracic endometriosis. [3] Many patients present with chest pain close to their menstrual periods. Surgical exploration can be used in an attempt to visualize the problem; mechanical pleurodesis or hormonal suppressive therapy can also be used. [1] Sometimes, a second surgical look can show fenestrations in the diaphragm. Due to the fact that endometriosis has been attributed to retrograde menstruation, upwards of 90% of women may have an immune deficiency. [2] This prevents clearance of endometrial cells from the peritoneum.

Endometriosis is defined as tissue similar to the endometrial tissue that has implanted outside of the uterus. Mechanisms include retrograde menstruation resulting in abdomino-pelvic spread, blood-borne or lymphatic spread and deposition, and metaplasia. [4]

Thoracic endometriosis is the most common non-abdominal site of involvement and is also the primary risk factor for catamenial pneumothorax. [5] Catamenial pneumothorax is the primary clinical presentation of thoracic endometriosis, and is defined as recurrent episodes of lung collapse within 72 hours before or after menstruation.

Signs and symptoms

Symptoms include chest or shoulder pain, cough, dyspnea, and shortness of breath. [6] Chest and shoulder pain can be radiating out to the shoulder blades. A dry cough can also present and come with a "crackling" sound upon inhaling. [1] Typically, it occurs in women aged 30 to 40 years, but it has been diagnosed in young girls as early as 10 years of age and post menopausal women (exclusively in women of menstrual age), most with a history of pelvic endometriosis. [7] Chest pain requires immediate medical attention.

Pathophysiology

Although the exact cause is not known, a few theories come from metastatic, hormonal, and anatomical possibilities. The metastatic model proposes that endometrial tissue has migrated from the endometrium to the diaphragm or the pleural space, causing small holes in the diaphragm, allowing air into the pleural space. In the hormonal model, it is believed that prostaglandin F2 causes a narrowing of the bronchioles, the small tubes within the lungs. Narrowing of these can cause the alveoli to rupture which may trap air in the pleural space. In the anatomical model, researchers believe that the absence of the cervical mucous plug, which is normally there during the menstrual cycle, allows air to pass from the genital tract to the pleural space through fenestrations in the diaphragm. Another theory is that hormonal changes that come with the menstrual cycle can cause blebs to spontaneously rupture. Blebs are small blisters/pustules filled with air or fluid and can develop on the lungs. [7]

Endometrial tissue attaches within the thoracic cavity, forming chocolate-like cysts. Generally the parietal pleura is involved, but the lung itself, the visceral layer, the diaphragm, and more rarely the tracheobronchial tree may also be afflicted. The mechanism through which endometrial tissue reaches the thorax remains unclear.

Nearly 90% of cases occur on the right hemithorax, a phenomenon thought to potentially be due to the direction of flow of retroperitoneal fluid. [8] Peritoneal flow occurs in a clockwise pattern, which could likely explain the tendency for catamenial pneumothoraces to be right-sided. [9] [10] Defects in the diaphragm, which are found often in affected individuals, could provide an entry path, as could microembolization through pelvic veins. Such diaphragmatic defects may be either congenital or acquired.

The cysts can release blood; the endometrial cyst "menstruates" in the lung. Endometrial cells undergo structural changes during the secretory phase of the menstrual cycle, in a process called decidualization. Decidualized pleural endometrial implants can disrupt the pleura and lead to pneumothorax (and hemothorax). [11]

Diagnosis

Diagnosis can be hinted by high recurrence rates of lung collapse in a woman of reproductive age with endometriosis. CA-125 is elevated.

Clinical diagnosis can be made based on history and imaging, while the gold standard for definitive diagnosis remains Video-assisted thoracoscopic surgery, or VATS, which allows not only the visualization of the lesion, but also surgical treatment via cauterization of the ectopic endometrial tissue. Special staining under a microscope or utilizing a cell marker, such as PAX8, can be used to positively identify endometrial stroma. [6]

Classification

Catamenial pneumothorax is the most common form of thoracic endometriosis syndrome, which also includes catamenial hemothorax, catamenial hemoptysis, catamenial hemothorax and endometriosis lung nodules, as well as some exceptional presentations. [12] [13]

Treatment

Pneumothorax can be a medical emergency, as it can become associated with decreased lung function, and if progressed to tension pneumothorax, potentially fatal. In many cases, catamenial pneumothorax will resolve spontaneously and not require immediate intervention. In more severe cases, a chest tube may be required to release air and/or blood and to allow the lung to re-expand.[ citation needed ]

Surgery, hormonal treatments and combined approaches have all been proposed, with variable results in terms of short and long term outcome. [14] These have both been effective, separately and together. Both have been used to treat women with this condition, and specifics depend on each patient and each situation. Surgery may be used to excise endometrial tissue from the lungs and pleural space as well as repair damage and holes in the diaphragm. [1] Surgery may also be used to remove blisters (blebs). An additional procedure involves a mesh that is placed over the diaphragm to block any holes that may have been missed in the first surgery. Hormonal therapies can also be used to suppress ovulation. Surgical removal of the endometrial tissue should be endeavored during menstruation for optimal visualization. [15] Pleurodesis may also be effective in removing the space between the lung and the chest wall; preventing air build up between these layers.

Non-surgical treatment includes pharmacological treatment via continuous oral contraceptive therapy to avoid cyclical bleeding with monthly menstruation. Oral contraceptives appear to be preferable for patients due to fewer negative side effects than treatment with GnRH agonist therapy, which can lead to hypoestrogenic effects including osteoporosis, resulting in discontinuation of therapy and thus a higher likelihood of recurrence of catamenial pneumothorax. [6]

Epidemiology

Some sources claim this entity represents 3 to 6% of pneumothorax in women. [16] In regard of the low incidence of primary spontaneous pneumothorax (i.e. not due to surgical trauma etc.) in women (about 1/100'000/year), [16] this is a very rare condition. Hence, many basic textbooks do not mention it, and many doctors have never heard of it. Therefore, catamenial pneumothorax is probably under-recognized. [17]

See also

Related Research Articles

<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. Most often this is on the ovaries, fallopian tubes, and tissue around the uterus and ovaries; in rare cases it may also occur in other parts of the body. Some symptoms include pelvic pain, heavy periods, pain with bowel movements, 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.

<span class="mw-page-title-main">Pneumothorax</span> Abnormal collection of air in the pleural space

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.

<span class="mw-page-title-main">Chest tube</span> Type of surgical drain

A chest tube is a surgical drain that is inserted through the chest wall and into the pleural space or the mediastinum in order to remove clinically undesired substances such as air (pneumothorax), excess fluid, blood (hemothorax), chyle (chylothorax) or pus (empyema) from the intrathoracic space. An intrapleural chest tube is also known as a Bülau drain or an intercostal catheter (ICC), and can either be a thin, flexible silicone tube, or a larger, semi-rigid, fenestrated plastic tube, which often involves a flutter valve or underwater seal.

<span class="mw-page-title-main">Thoracotomy</span> Surgical procedure

A thoracotomy is a surgical procedure to gain access into the pleural space of the chest. It is performed by surgeons to gain access to the thoracic organs, most commonly the heart, the lungs, or the esophagus, or for access to the thoracic aorta or the anterior spine. A thoracotomy is the first step in thoracic surgeries including lobectomy or pneumonectomy for lung cancer or to gain thoracic access in major trauma.

<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">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">Hemothorax</span> Blood accumulation in the pleural cavity

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.

<span class="mw-page-title-main">Pneumonectomy</span> Surgical removal of a lung

A pneumonectomy is a surgical procedure to remove a lung first successfully done in 1933 by Dr. Evarts Graham. This is not to be confused with a lobectomy or segmentectomy, which only removes one part of the lung.

Hemopneumothorax, or haemopneumothorax, is the condition of having both air (pneumothorax) and blood (hemothorax) in the chest cavity. A hemothorax, pneumothorax, or the combination of both can occur due to an injury to the lung or chest.

<span class="mw-page-title-main">Focused assessment with sonography for trauma</span> Fluid accumulation screening

Focused assessment with sonography in trauma is a rapid bedside ultrasound examination performed by surgeons, emergency physicians, and paramedics as a screening test for blood around the heart or abdominal organs (hemoperitoneum) after trauma. There is also the extended FAST (eFAST) which includes some additional ultrasound views to assess for pneumothorax.

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

A pulmonary laceration is a chest injury in which lung tissue is torn or cut. An injury that is potentially more serious than pulmonary contusion, pulmonary laceration involves disruption of the architecture of the lung, while pulmonary contusion does not. Pulmonary laceration is commonly caused by penetrating trauma but may also result from forces involved in blunt trauma such as shear stress. A cavity filled with blood, air, or both can form. The injury is diagnosed when collections of air or fluid are found on a CT scan of the chest. Surgery may be required to stitch the laceration, to drain blood, or even to remove injured parts of the lung. The injury commonly heals quickly with few problems if it is given proper treatment; however it may be associated with scarring of the lung or other complications.

<span class="mw-page-title-main">Subcutaneous emphysema</span> Medical condition

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.

<span class="mw-page-title-main">Diaphragmatic rupture</span> Tear in the thoracic diaphragm, usually caused by physical trauma

Diaphragmatic rupture is a tear of the diaphragm, the muscle across the bottom of the ribcage that plays a crucial role in breathing. Most commonly, acquired diaphragmatic tears result from physical trauma. Diaphragmatic rupture can result from blunt or penetrating trauma and occurs in about 0.5% of all people with trauma.

<span class="mw-page-title-main">Video-assisted thoracoscopic surgery</span>

Video-assisted thoracoscopic surgery (VATS) is a type of minimally invasive thoracic surgery performed using a small video camera mounted to a fiberoptic thoracoscope, with or without angulated visualization, which allows the surgeon to see inside the chest by viewing the video images relayed onto a television screen, and perform procedures using elongated surgical instruments. The camera and instruments are inserted into the patient's chest cavity through small incisions in the chest wall, usually via specially designed guiding tubes known as "ports".

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

Endometrioma is the presence of tissue similar to, but distinct from, the endometrium in and sometimes on the ovary. It is the most common form of endometriosis. Endometrioma is found in 17–44% patients with endometriosis. More broadly, endometriosis is the presence of tissue similar to, but distinct from, endometrial tissue located outside the uterus. The presence of endometriosis can result in the formation of scar tissue, adhesions and an inflammatory reaction. It usually is a benign growth. An endometrioma is most often found in the ovary. This ovarian endometriosis forms dark, fluid-filled cysts. These fluid-filled sacs can vary greatly in size and are known as endometriomas, also called "chocolate cysts". The fluid inside the cysts is thick, dark, old blood, giving it a chocolate-like appearance. It can also develop in the cul-de-sac, the surface of the uterus, and between the vagina and rectum.

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.

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.

<span class="mw-page-title-main">Lung biopsy</span>

A lung biopsy is an interventional procedure performed to diagnose lung pathology by obtaining a small piece of lung which is examined under a microscope. Beyond microscopic examination for cellular morphology and architecture, special stains and cultures can be performed on the tissue obtained.

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. Endometriosis is characterized by the presence of tissue similar to the lining of the uterus 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.

References

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  3. ↑ Maurer ER, Schaal JA, Mendez FL (December 1958). "Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm". Journal of the American Medical Association. 168 (15): 2013–2014. doi:10.1001/jama.1958.63000150008012c. PMID   13598643.
  4. ↑ Burney RO, Giudice LC (September 2012). "Pathogenesis and pathophysiology of endometriosis". Fertility and Sterility. 98 (3): 511–519. doi:10.1016/j.fertnstert.2012.06.029. PMC   3836682 . PMID   22819144.
  5. ↑ Rousset-Jablonski C, Alifano M, Plu-Bureau G, Camilleri-Broet S, Rousset P, Regnard JF, Gompel A (September 2011). "Catamenial pneumothorax and endometriosis-related pneumothorax: clinical features and risk factors". Human Reproduction. 26 (9): 2322–2329. doi: 10.1093/humrep/der189 . PMID   21685141.
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  7. 1 2 Dong B, Wu CL, Sheng YL, Wu B, Ye GC, Liu YF, et al. (April 2021). "Catamenial pneumothorax with bubbling up on the diaphragmatic defects: a case report". BMC Women's Health. 21 (1): 167. doi:10.1186/s12905-021-01318-0. PMC   8059314 . PMID   33879147.
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  9. ↑ Meyers MA (June 1970). "The spread and localization of acute intraperitoneal effusions". Radiology. 95 (3): 547–554. doi:10.1148/95.3.547. PMID   5442658.
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  11. ↑ van der Merwe E, Schuurmans MM, de Kock F, Siebert I, Wright C, Bolliger CT (2005). "Bloodstained pleural effusion in a 38-year-old non-smoking female". Respiration; International Review of Thoracic Diseases. 72 (1): 101–104. doi:10.1159/000083410. PMID   15753644. S2CID   31933812.
  12. ↑ Bricelj K, Srpčič M, Ražem A, Snoj Ž (October 2017). "Catamenial pneumothorax since introduction of video-assisted thoracoscopic surgery : A systematic review". Wiener Klinische Wochenschrift. 129 (19–20): 717–726. doi:10.1007/s00508-017-1237-4. PMID   28762057. S2CID   12760621.
  13. ↑ McCann MR, Schenk WB, Nassar A, Maimone S (September 2020). "Thoracic endometriosis presenting as a catamenial hemothorax with discordant video-assisted thoracoscopic surgery". Radiology Case Reports. 15 (9): 1419–1422. doi:10.1016/j.radcr.2020.05.064. PMC   7334551 . PMID   32642009.
  14. ↑ Peikert T, Gillespie DJ, Cassivi SD (May 2005). "Catamenial pneumothorax". Mayo Clinic Proceedings. 80 (5): 677–680. doi: 10.4065/80.5.677 . PMID   15887438.
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  16. 1 2 radio/563 at eMedicine
  17. ↑ Alifano M, Roth T, Broët SC, Schussler O, Magdeleinat P, Regnard JF (September 2003). "Catamenial pneumothorax: a prospective study". Chest. 124 (3): 1004–1008. doi:10.1378/chest.124.3.1004. PMID   12970030.
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