Treatment options for CPT can include surgery and/or hormonal therapy
For the CPT patient, it is important to keep in mind that physicians will address treatment with regard to their own medical specialty. Although catamenial pneumothorax manifests itself as a thoracic problem, the underlying cause is hormone related and therefore needs to be addressed by a gynecologist as well. Successful treatment depends upon the gynecologist and thoracic surgeon/pulmonary specialist working together to provide treatment options.
Initial Surgical Procedures
Literature shows that many women are treated for spontaneous pneumothorax (SPT) until a correlation is made between multiple lung collapses and the start of menses (1). A first incident of SPT may be treated with oxygen, observation and rest if the collapse is small. A medium to large collapse may require manual aspiration or a chest tube. A second occurrence may be treated more aggressively with a sclerosing agent (2). Because these treatments don't address the hormonal cause of CPT, their effectiveness is quite limited and reoccurrence of collapse is likely (2, 3).
Aspiration - A catheter is inserted into the pleural space and the air is removed manually, allowing the lung to re-expand. Another version of this involves connecting the catheter to a one-way valve allowing air to escape passively (Heimlich valve).
Thoracostomy - (Chest tube/chest drain) A tube is inserted through the chest wall and into the pleural space. Generally, suction and a water seal are applied, removing the air and causing the lung to re-expand. This procedure requires pain medication and a hospital stay until re-expansion has occurred.
Chemical Sclerosis / Pleurodesis - A chemical is introduced through the chest tube causing inflammation and scarring of the pleural surfaces. This promotes the formation of adhesions between the lung exterior and the chest wall, thereby discouraging future collapses. One important note: this procedure can be extremely painful. Additionally, in the absence of other treatment methods, chemical pleurodesis alone to treat CPT has a significant failure rate (2).
Secondary Non-Surgical Procedures
Once CPT is suspected (a correlation is made between collapses and the menstrual cycle), hormonal treatment is often tried. This includes endometrial suppression therapy using a variety of synthetic hormonal drugs to suspend the menstrual cycle (3, 4).
Progestin Therapy (non-bioidentical) - Oral contraceptives (birth control pills) and other progestins are used to suppress ovulation. When used continuously (no break), progestins can stop the menstrual cycle as well. The use of oral contraceptives alone, without other treatment methods reportedly has a low success rate in stopping collapses (2, 5). There are also significant risks and side effects associated with the use of progestins. Careful consultation with your physician is recommended before starting treatment with progestins.
GnRH Therapy (non-bioidentical) - Gonadotropin Releasing Hormone agonists suppress menstruation and create a state of chemically induced artificial menopause (2, 6). Previously, these were generally used no longer than six months. Current use of these medications can be for a longer duration and may involve add-back hormonal therapy. As with progestins there are side effects with the use of GnRH drugs. These should be discussed with your doctor prior to use. Literature reports that amenorrhea induced by GnRH therapy has a better rate of success in stopping collapses than that of oral contraceptives (2).
Bioidentical Hormone Therapy - Although not widely used, bioidentical progesterone has been used as an alternative to synthetic progestins (7). One corresponding patient reported the use of bioidentical hormone pellet implants. This treatment stopped her collapses and monthly chest pain even though she continued to menstruate (8).
Major Surgical Procedures
Aggressive treatment options include surgical procedures to remove endometrial implants, repair diaphragmatic fenestrations, abrade pleural surfaces, remove the pleura or remove the ovaries to induce surgical menopause.
Thoracoscopy / VATS - Video Assisted Thoracic Surgery - Accessed through small incisions, this procedure utilizes a fiber optic scope to visualize the lung, pleura and diaphragm, so that the surgeon can find endometrial implants or holes in the diaphragm, and repair them. Bleb resection and pleurodesis can also be performed during VATS (9).
Pleural Abrasion (Mechanical Pleurodesis) - This type of pleurodesis involves abrading or "scrubbing" the pleural surfaces so that the resulting inflammation forms adhesions between the lung and chest wall, making it harder for the lung to collapse again if air should enter the pleural space. Sometimes talc is added to encourage adhesion formation. Literature shows that pleurodesis used as a singular treatment, (without other surgical repairs or hormonal therapy) has a high failure rate (10, 11).
Thoracotomy / Pleurectomy - An incision is made on the side of the chest, between the ribs. The ribs are spread apart, allowing the lung, pleura and diaphragm to be viewed visually by the surgeon. As in VATS, biopsy, bleb resection and pleurodesis can be performed. A pleurectomy can also be performed which involves removal of the pleura to encourage the lung to adhere directly with the chest wall (12).
Diaphragmatic Repair using Polymesh - In this procedure, a Vicryl type mesh is placed over the diaphragm to cover any smaller fenestrations (holes) that may not be seen by the surgeon. Although the Vicryl material is absorbed over time, the mesh allows for tissue ingrowth and the resulting scar tissue obliterates the diaphragm pores (13). As in other thoracic procedures, mechanical pleurodesis or pleural abrasion can also be done. This procedure can be performed during thoracotomy or thoracoscopy, and was first introduced in the literature in The Annuals of Thoracic Surgery in 2003 (3). Since then, it has been performed with success in the United States, although the frequency is unknown. Additional articles strongly advocate use of the mesh in combination with GnRH therapy in order to successfully treat CPT (11).
Tandem Laparoscopic-Thoracoscopic Procedure - It is important that thoracic surgeons and gynecologists understand the complexities of the condition and work together. In California, this tandem approach is being utilized in a dual laparoscopic procedure, whereby both sides of the diaphragm are evaluated for endometrial progression (14, 24). Additionally, more gynecologists are recognizing the value of properly evaluating the underside of the diaphragm and removing endometrial lesions (15).
Hysterectomy / Bilateral Salpingo-Oophorectomy - In some cases both ovaries are removed to limit estrogen production and suppress endometrial tissue by inducing surgical menopause (2, 16, 17). Although the uterus does not produce hormones, it is often (but not always) removed as well (18).
Personal Commentary
In the literature and on various web sites, there are conflicting opinions as to whether or not a "hysterectomy" will effectively "cure" endometriosis/CPT. One reason for this is a difference in the use and meaning of the term "hysterectomy." It should be understood that it is the removal of the ovaries (oophorectomy) that affect estrogen production, not removal of the uterus (hysterectomy). While removing the uterus will stop menstruation, any endometrial implants will still respond to estrogen if the ovaries remain.
Removal of the ovaries has shown to be an effective treatment option for CPT (1, 19, 25), with some qualifiers. One: Both ovaries are removed, and two: Add-back estrogen hormone replacement therapy (HRT) is withheld for several months, as endometriosis can recur with estrogen therapy, even in women who have undergone oophorectomy (19, 25).
CPT patients we've corresponded with have reported that retention of one ovary or estrogen replacement therapy immediately following surgery, have resulted in continuing collapses. Our survey results indicate that a full oophorectomy with no immediate hormone add back therapy is an effective treatment option for CPT. A similar observation is found in the literature (1, 17, 20, 21, 22, 23).
Endometriosis and Hormonal Therapy
Hormones are chemicals produced by glands of the endocrine system which travel through the bloodstream and interact with specific target cells. These cells have receptor sites where the hormones bind like a lock and key mechanism. Many hormones are interactive and interdependent (26), making the endocrine system extremely complex and difficult to fully understand.
As it is widely recognized that estrogen affects the growth of endometriosis, conventional treatment often includes hormonal management to suppress estrogen function (19). Hormonal therapy is also used to treat post-menopausal symptoms (27).
Since the Women's Health Initiative (WHI) Study was published in 2002 (28), there has been significant debate within the medical community about which types of hormones should be available for patients. Much of the argument focuses on the safety and efficacy of pharmacy-compounded bioidentical hormones (BIH) versus traditionally-used pharmaceuticals (29).
Synthetic Hormones
Progestins and gonadotropin-releasing hormone agonists (GnRH) are frequently used to stop menstruation (19). These compounds are synthetic (man-made) drugs, whose chemical structure differs from hormones made in the body (29). Use of these drugs can cause significant side effects which some patients may find difficult to tolerate (29).
Progestins and animal-based estrogens have been used for many years for hormone replacement therapy (HRT) in menopausal women (28). However, since the WHI Study revealed an increased risk of breast cancer, cardiovascular disease and stroke in women taking these compounds, use of these products has declined sharply (27).
Bioidentical Hormones
These compounds are widely used for post-menopausal HRT (27). Some doctors advocate their use over conventional synthetic preparations, claiming they carry less risk of side effects (29, 30, 31). Bioidential hormones are defined as having the same chemical structure as hormones made in the body (30). They can be prescribed in several forms including pharmacy-compounded transdermal creams and subdermal pellets as well as pharmaceutical preparations of oral pills/capsules and transdermal patches (27).
Personal Commentary
In doing our own research, we've encountered strong opinions on both sides of this argument. On one extreme there are doctors who advocate high levels of bioidentical hormones in a cycling regimen with the goal of inducing a menopausal woman to start cycling again. On the other extreme, there are doctors who insist that an FDA-approved synthetic drug with well-documented side effects is really safer than a bioidentical hormone chemically identical to the molecule made by the ovaries. As with any topic where politics and money are involved, there is a lot to gain and a lot to lose for both sides and the patient is left to sort out for themselves the truth from the hype.
While some of the patients we've corresponded with have had success on synthetic hormonal drugs, others reported adverse side effects and/or continuing collapses. Speaking from our own experience, Lori had severe bleeding and developed anemia and hypertension following use of these drugs, as well as a blocked renal artery which led to the loss of her kidney. Was that the result of her progestin use? We don't know for sure, but blood clots can be a side effect of using progestins. What we do know is that we've done well on bioidentical hormones for several years now, and have far fewer menopausal symptoms than we did without HRT. We have not experienced any adverse side effects and continue to maintain a healthy bone density after being in menopause for over ten years.
The bottom line about hormones is that there is a lot of information out there, some of which is complicated and much of which is conflicting. Each patient must educate herself so she can have an intelligent conversation with her doctor about these issues.
Useful Links and References
Endometriosis.org (Information about pharmaceutical hormonal drugs)
The Endocrine Society (Strongly critical of bioidentical hormones)
Women's International Pharmacy (Advocate of bioidentical hormones)
John Carr MD (Advocate of bioidentical hormones; web site features videos about BIH hormone use; Dr. Carr was one of Lori’s doctors for a short time when she started on HRT pellet implants)
Erika Gray, pharmacist (Advocate of bioidentical hormones and family friend who successfully used progesterone in the treatment of her own endometriosis)
John Lee, MD What Your Doctor May Not Tell You About Pre-menopause, Balance Your Hormones and Your Life from Thirty to Fifty (Advocate of bioidentical hormones)
HysterSisters.com ( An excellent resource for information and support for women who have experienced or are considering a hysterectomy)
The Hormone Jungle (A companion site to HysterSisters.com)
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(2) Guidelines for the diagnosis and treatment of spontaneous pneumothorax. Rivas de Andres JJ, Jimenez Lopez MF, Lopez-Rodo LM, Perez Trullen A, Torres Lanzas J. J Arch Bronconeumol. 2008;44(8):437-48
(3) Catamenial pneumothorax: retrospective study of surgical treatment. Bagan P, Barthes FL, Assouad J, Souilamas R, Riquet M. Ann Thorac Surg. 2003 Feb;75(2):378-381
(4) Medical therapy for recurring catamenial pneumothorax following pleurodesis. Dotson RL, Peterson CM, Doucette RC, Quinton R, Rawson DY, Jones KP. Obstet Gynecol. 1993 Oct;82(4 pt.2):656-658
(5) Catamenial pneumothorax - bilateral occurrence while on suppressive therapy. Wilhelm JL, Scommegna A. Obstet Gynecol. 1977 Aug;50(2):227-231
(6) Nonsurgical treatment of a catamenial pneumothorax with a Gn-RH analogue. Akal M, Kara M. Respiration. 2002;69(3):275-276
(7) Jerilynn Prior, MD, The Centre for Menstrual Cycle and Ovulation Research (CeMCOR)
(8) Personal communication with CPT patient on the Sottopelle program
(9) Catamenial pneumothorax- a prospective study. Alifano M, Roth T, Camilleri Broet S, Schussler O, Magdeleinat P, Regnard J. Chest. 2003;124:1004-1008
(10) Surgical treatment of catamenial pneumothorax: a single centre experience. Ciriaco P, Negri G, Libretti L, Carretta A, Melloni G, Casiraghi M, Bandiera A, Zannini P. Interact Cardiovasc Thorac Surg. 2009 Mar;8(3):349-52. Epub 2008 Dec 16
(11) Catamenial pneumothorax: surgical repair of the diaphragm and hormone treatment. Leong AC, Coonar AS, Lang-Lazdunski L. Ann R Coll Surg Engl. 2006 Oct;88(6):547-9
(12) Personal communication; Michael Soltero, MD Cardiothoracic and Vascular surgeon
(13) Personal communication; Edward Murphy, MD Cardiothoracic surgeon
(14) Personal communication with CPT patient treated at Stanford University
(15) Endometriosis of the diaphragm: four cases treated with a combination of laparoscopy and thoracoscopy. Nezhat C, Bhagan L, Huang JQ, Bosev D, Hajhosseini B, Beygui RE. J Minim Invasive Gynecol. 2009 Sep-Oct;16(5):573-80
(16) Catamenial pneumothorax. Stern H, Toole AL, Merino M. Chest. 1980 Sep;78(3):480-482
(17) Catamenial pneumothorax. Nwosu EC, Sajjad Y, Barnet A. J Obstet Gynecol. 2000 Sep;20(5):547-548
(18) Personal communication with CPT patients treated with hysterectomy and/or oophorectomy
(19) Endometriosis - morphology, clinical presentations and molecular pathology. Agarwal N, Subramanian A. J Lab Physicians [serial online] 2010 [cited 2012 Jan 21];2:1-9, HERS Research Group. JAMA. 2002 Jul 3;288(1):49-57
(20) Thoracic endometriosis: recurrence following hysterectomy with bilateral salpingo-oophorectomy and successful treatment with talc pleurodesis. Joseph J, Reed CE, Sahn SA. Chest. 1994 Dec;106:1894-96
(21) Catamenial hemoptysis and pulmonary endometriosis: A Case Report. Yu Z, Fleischman JK, Rahman HM, Mesia AF, Rosner F. Mt Sinai J Med. 2002 Sep;69(4):261-263
(22) Laparoscopic surgical management of diaphragmatic endometriosis. Nezhat Ce, Seidman DS, Nezhat F, Nezhat Ca. Fertil Steril. 1998 Jun;69(6):1048-55
(23) Catamenial pneumothorax. Lee CY, Di Loreto PC, Beaudoin J. Obstet Gynecol. 1974 Sep;44(3):407-411
(24) Multidisciplinary treatment for thoracic and abdominopelvic endometriosis. Nezhat C, Main J, Paka C, Nezhat A, Beygui RE. JSLS. 2014 Jul-Sep;18(3)
(25) Catamenial pnuemothorax: a rare phenomenon? Thomas V, Thomas E, Lionel J. J Obstet Gynaecol India. 2013 Dec;63(6):424-425
(26) Human Physiology. Stuart Ira Fox, WCB/McGraw-Hill Companies, Inc. 1999; pgs 290-292
(27) Bioidentical hormone therapy. Files JA, Ko MG, Pruthi S. Mayo Clin Proc. 2011;86(7):673-680
(28) Risks and benefits of estrogen plus progestin in healthy postmenopausal women: principal results from the Women's Health Initiative randomized controlled trial. Rossouw J, et al, Writing Group for the Women's Health Initiative Investigators. JAMA. 2002 Jul 17;288(3):321-333
(29) The bioidentical hormone debate: are bioidentical hormones (estradiol, estriol, and progesterone) safer or more efficacious than commonly used synthetic versions in hormone replacement therapy? Holtorf K. Postgrad Med. 2009 Jan;121(1):73-85
(30) Effectiveness of compounded bioidentical hormone replacement therapy: an observational cohort study. Ruiz AD, Daniels KR, Barner JC, Carson JJ, Frei CR. BMC Women's Health. 2011;11:27
(31) The effects of compounded bioidentical transdermal hormone therapy on hemostatic, inflammatory, immune factors; cardiovascular biomarkers; quality-of-life measures; and health outcomes in perimenopausal and postmenopausal women. Stephenson K, Neuenschwander PF, Kurdowska AK. Int J Pharm Compd. 2013 Jan-Feb;17(1):74-85