How is Thoracic Endometriosis Treated?
This is a difficult question to answer. Part of the problem is a lack of long-term follow-up with patients when hormone suppressants are used. There are numerous case studies which support use of medical management to prevent recurrence. (1,2,3) However, the majority of these studies follow an average of 3-6 months after diagnosis. A few report longer follow-up (2,3) after initiation of solitary medical management or as a post-operative adjunct. Medical management can provide short-term relief of symptoms, with effectiveness in 50% of those with Catamenial Hemoptysis but occurrence of breakthrough symptoms during and return of symptoms following discontinued treatment still occurs. (4,5,6)
Adjunctive hormone suppression after surgery blurs the results of a surgical procedure as effective or not. Large scale studies provide a better perspective of medical and surgical outcomes. A 2017 retrospective analysis of 7 articles (1993-2014) with a collective sample size of 50, concluded that 6/7 publications reported positive association of reduced recurrence risk with adjunctive GnRh use. (3) However, the authors report that:
“when looking at the different types of surgery, diaphragmatic repair showed a potential benefit with 6 recurrences of 29 (20.7%) patients compared with 5 recurrences in 9 (55.6%) patients who did not undergo diaphragmatic repair.” – Garner M (2017)
In layman’s term, intra-operative repair of the diaphragm among those treated w/ adjunctive post-operative medical management may be THE variable, or an additional variable that contributed to better outcomes that could lead to a false-positive conclussion that medical management adjunct is beneficial.
In the early 1970’s when spontaneous pneumothorax at menses was becoming more accepted as probable endometriosis in origin, hormone manipulation for prevention of recurrence was used. Results are similar to today. The management may be effective during its use but recurrence often recurs after discontinuation (7,8). Even following hysterectomy (7).
One hundred and ten (110) persons with Catamenial Pneumothorax were followed for 12 months after conservative medical or operative intervention. At six months follow-up, 50% of those who received medical management had a recurrence of pneumothorax compared to only 5% post-operative. A full year follow-up found 60% recurrence rate among the medical management and 25% recurrence among the post-operative group (9). The findings of this 1996 remain relevant today. All drugs introduced to date are from the same drug classification (hormone suppression) through history. As documented with pregnancy and hormone suppression, recurrent episodes of pneumothorax are possible (10,11,12).
Consideration for why medical management is often ineffective:
The theories of pathogenesis were briefly discussed on the ABC’s of Extra-pelvic Disease page. There, a brief discussion about the tissue collections through the World Endometriosis Research Foundation (WERF) database to collect and begin classification of various phenotypes of lesions is also discussed.
Research like Jagivar et al’s Baboon study (13), which report distinct histological differences between abdominopelvic cavity and thoracic cavity lesions among all of their subjects. Most specific: ALL lesions of the lungs contained TTF-1; ALL lesions in abdominopelvic cavity had NO TTF-1. Jagivar et al’s findings not only support alternate theories of pathogenesis; it provides a histological basis why medical management is often ineffective and poorly prevents recurrent pneuomothorax.
“The immunohistochemical profile of the PPE lesions suggest a different pathogenesis from abdominal endometriosis…The lack of ER/PR staining in the glandular component contradicts the theory of regurgitation and implantation. The presence of deep parenchyma lesions and the occurrence of bilateral lesions cannot be fully explained my Myers’ theory of coelomic metaplasia. The presence of stroma cells in the lymph node supports the embolization theory for extra abdominal endometriosis. The embolization of the stroma component in the lung parenchyma then induces the epithelial component from the local tissue defined stem cell population. The cysts form next due to expansion of the lung during inspiration without fibrosis unlike that seen in abdominal endometriosis where fibrosis is common. Since the lining is not ER/PR positive it might explain why hormonal treatment has failed and recurrences are common with behavior simulating a low grade malignancy with metastasis or benign metastasis. The presence of Thyroid-Transcription factor 1 (TTF-1) present in lesions of the lung supports the theory of coelomic metaplasia.” – Javigar J (2013)
Citation list: How TE treated-Medical Mgmt
“In recent years, treatment of extra-pelvic endometriosis has shifted from medical managment toward a surgical approach as surgery clearly improves disease outcome.” – Jukna A et al. (2014)
If high suspicion of respiratory lesions, it is imperative that an experienced team of gynecologist and cardiothoracic surgeon are considered. The unique ‘landscape’ of the chest cavity (communicating pathways through the chest wall, vascular and lymphatics as well as surface areas inside and out of the lungs), variability of lesion appearance with hormone fluctuation of the female cycle AND numerous presentations (color, shape, texture and more subtle presentations) an experienced team reduces the risk of recurrence and missed disease.
The use of Video-Assisted Thoracoscopic Surgery (VATS), which is less invasive and reduced debility during recovery of a full thoracotomy incision, has spurred greater interest and ‘willingness’ to look for disease. Since the utilization of VATS has increased, remarkable gains for awareness of the true prevalence of TE, thoracic-side only diaphragmatic disease and identification of greater disease in color, texture, indurated areas, etc.
In regards to cases of Catamenial Hemoptysis, the use of bronchoscopy and CT imaging was previously discussed in Myth Buster #3 to assist with pre-operative planning to locate lesions most oft located toward the periphery of lung tissue which can be accessed through a VATS.
In our support group, we regularly encounter new members who were treated by a thoracic surgeon who ‘had never seen endometriosis of the chest before, or had little experience with the disease. Unfortunately most, rather than less cases required a second procedure by a experienced team.
A BIG problem exists in our community at the moment. Not only is the dismissal rate very high for those with TE, women can endure progressively intense symptoms for decades before a manifestation occurs (most often lung collapse or hemothorax). Very few thoracic surgeons give consideration (and many insurance companies will not approve) an investigative surgical procedure unless at least ONE confirmed pneumothorax has occurred. In fact, even less surgeons will intervene until with an initial pneumothorax unless it is a ‘tension’ type which reduces the hearts ability to pump adequate blood volume, or if a persistent air leak (does not close) continues (determined number of days differs between surgeons).
“The management of catamenial pneumothorax differs slightly from primary spontaneous pneumothorax. We offer a thoracoscopy already for the initial episode with resection of endometriosis lesions and closure of the diaphragmatic fenestrations.” – Fatma D. and Raoul H. et al. (2016)
There remains the concern for variability in the didactic, clinical and operative skill levels of the thoracic surgeon SPECIFIC to endometriosis that must be addressed. How do we address these concerns?
I can attest that lesions of the diaphragm and parietal pleura (chest wall and chest lining of the diaphragm) can be incapacitating. Most often disease is ADVANCED before a pneumothorax occurs. Identification and Treatment must occur earlier than it currently does. Far to many women diagnosed after disease progression suffer residual effects that could have been prevented with earlier intervention.
(It is important to understand that TE associated lung collapse is considered a Secondary Spontaneous Pneumothorax. This refers to the contribution of a disease process inducing the lung collapse. A Primary Spontaneous Pneumothorax is considered idiopathic (unknow spontaneous rupture without the presence of disease.
“Currently, surgical management of the condition is not standardized. A shortage of physician awareness in the surgical community with personal bias deferring surgery contributes to this lack of standardization, even in the way surgeons treat Primary Spontaneous Pneumothorax.” – Yasser Aljehani (2014)
‘Once and Done’ should be the motto for treatment of women with TE. One surgery (unless bilateral disease exists), to remove all disease and abnormal tissue with recurrence prevention procedures as appropriate. If procedures have occurred to prevent recurrence yet disease remains, symptoms continue and recurrence remains a higher probability. A second surgery becomes more complicated and longer recovery time. It seems feasible and “it has been suggested that catamenial or endometriosis-related pneumothorax needs to be treated in a multidisciplinary setting” (Alifano et al. 2006). Although probability for recurrence may still occur, it will be significantly reduced among surgical teams educated and experienced with recognition and removal of disease and primary chances of recurrence will be reduced to the more ‘difficulty to detect microscopic lesions during surgery” (Katsura D et al – 2016) a possibility.
Yu and Sihoe summarized their experience with a case treated for pneumothorax previously, which they were involved in the follow-up procedure:
“In our patient, it is possible that the failure to treat the underlying endometriosis after the first operation, contributed to both the subsequent recurrence of pneumothorax and the progressive enlargement of the diaphragmatic defect to such a massive degree.” – Yu and Sihoe (2015)
Citations listing: How to Treat TE-Surgical Mgmt TEReferencesHowTreatTESurgicalMgmt
A Serious Concern Regarding TE and Hormone Treatment to prepare for In-vitro Fertilization
This is a very sensitive topic. It is also a very serious topic. When you spend time every-single-day interacting with women who are along the journey with Thoracic Endometriosis. Scenarios appear in which, providers never consider possible or even consider screening questions prior to proceeding with hormone treatments to a person who wishes to undergo in-vitro fertilization. Data base searches located a single case study (1). Interacting with women around the world, every day, we are aware others arrived at their condition in this manner.
Citation: In preparation for In-Vitro Fertilization TEReferencesPrepforInVitro
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