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.
SpecialtyPulmonology
SymptomsChest or shoulder pain, shortness of breath
Usual onset30-40 years of age
CausesThoracic endometriosis
Risk factorsCongenital or acquired diaphragmatic defects allowing entry of endometrial tissue into the pleural space
Diagnostic methodVATS procedure
TreatmentSurgical - VATS procedure to remove ectopic endometrial tissue
Pharmacologic - continuous OCP's are favored to suppress menstruation

Catamenial pneumothorax is a condition of air leaking into the pleural space occurring in conjunction with menstrual periods (catamenial refers to menstruation), and/or during ovulation, caused by the abnormal growth of endometrial tissue in the membrane surrounding the lung and diaphragm.[1]

Catamenial pneumothorax is defined as at least two episodes of recurrent pneumothorax corresponding with menstruation. It was first described in 1858 when a woman presented with 12 episodes of right-sided pneumothorax over 1 year, recurring monthly with menstruation. Thoracotomy revealed thoracic endometriosis.[2]

Endometriosis is defined as 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.[3]

Thoracic endometriosis is the most common non-abdominal site of involvement and is also the primary risk factor for catamenial pneumothorax.[4] 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[edit]

Symptoms include chest or shoulder pain, cough, dyspnea, and shortness of breath.[5] 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.[citation needed]

Pathophysiology[edit]

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.[6] Peritoneal flow occurs in a clockwise pattern, which could likely explain the tendency for catamenial pneumothoraces to be right-sided.[7][8] 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).[9]

Diagnosis[edit]

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.[10]

Classification[edit]

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.[11][12]

Treatment[edit]

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.[13] Surgical removal of the endometrial tissue should be endeavoured during menstruation for optimal visualisation.[14] Pleurodesis may also be helpful.

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.[15]

Epidemiology[edit]

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[edit]

References[edit]

  1. ^ "Definition from mercksource.com (Dorlands Medical Dictionary)". Archived from the original on 2006-07-02. Retrieved 2006-07-01.
  2. ^ Maurer, Elmer R. (1958-12-13). "Chronic Recurring Spontaneous Pneumothorax Due to Endometriosis of the Diaphragm". Journal of the American Medical Association. 168 (15): 2013. doi:10.1001/jama.1958.63000150008012c. ISSN 0002-9955.
  3. ^ Burney, Richard O.; Giudice, Linda C. (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.
  4. ^ Rousset-Jablonski, C.; Alifano, M.; Plu-Bureau, G.; Camilleri-Broet, S.; Rousset, P.; Regnard, J.-F.; Gompel, A. (2011-09-01). "Catamenial pneumothorax and endometriosis-related pneumothorax: clinical features and risk factors". Human Reproduction. 26 (9): 2322–2329. doi:10.1093/humrep/der189. ISSN 0268-1161.
  5. ^ Hirata, Tetsuya; Koga, Kaori; Osuga, Yutaka (2020). "Extra-pelvic endometriosis: A review". Reproductive Medicine and Biology. 19 (4): 323–333. doi:10.1002/rmb2.12340. ISSN 1447-0578. PMC 7542014. PMID 33071634.
  6. ^ Joseph, Jos; Sahn, Steven A. (February 1996). "Thoracic endometriosis syndrome: New observations from an analysis of 110 cases". The American Journal of Medicine. 100 (2): 164–170. doi:10.1016/S0002-9343(97)89454-5.
  7. ^ Meyers, Morton A. (June 1970). "The Spread and Localization of Acute Intraperitoneal Effusions". Radiology. 95 (3): 547–554. doi:10.1148/95.3.547. ISSN 0033-8419.
  8. ^ Drye, J.C. (October 1948). "Intraperitoneal pressure in the human". Surgery, Gynecology, and Obstetrics. 87 (4): 472–475. PMID 18119801.
  9. ^ van der Merwe, E.; Schuurmans, M.M.; de Kock, F.; Siebert, I.; Wright, C.; Bolliger, C.T. (2005). "Bloodstained Pleural Effusion in a 38-Year-Old Non-Smoking Female". Respiration. 72 (1): 101–104. doi:10.1159/000083410. ISSN 0025-7931.
  10. ^ Hirata, Tetsuya; Koga, Kaori; Osuga, Yutaka (2020). "Extra-pelvic endometriosis: A review". Reproductive Medicine and Biology. 19 (4): 323–333. doi:10.1002/rmb2.12340. ISSN 1447-0578. PMC 7542014. PMID 33071634.
  11. ^ Bricelj K; et al. (2017). "A systematic review". Wiener Klinische Wochenschrift. 129 (19–20): 717–726. doi:10.1007/s00508-017-1237-4. PMID 28762057. S2CID 12760621.
  12. ^ 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.
  13. ^ Peikert T, Gillespie DJ, Cassivi SD (May 2005). "Catamenial pneumothorax". Mayo Clinic Proceedings. 80 (5): 677–80. doi:10.4065/80.5.677. PMID 15887438.
  14. ^ Poyraz AS, Kilic D, Hatipoglu A, Demirhan BA (September 2005). "A very rare entity: catamenial pneumothorax". Asian Cardiovascular & Thoracic Annals. 13 (3): 271–3. doi:10.1177/021849230501300317. PMID 16113003. S2CID 46199907.
  15. ^ Hirata, Tetsuya; Koga, Kaori; Osuga, Yutaka (2020). "Extra-pelvic endometriosis: A review". Reproductive Medicine and Biology. 19 (4): 323–333. doi:10.1002/rmb2.12340. ISSN 1447-0578. PMC 7542014. PMID 33071634.
  16. ^ a b 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): 1004–8. doi:10.1378/chest.124.3.1004. PMID 12970030.

External links[edit]

Classification