Thoracic Endometriosis: A Cohort Study
Condition: Endometriosis Thoracic; Endometriosis of Lung; Endometriosis of Pleura; Endometriosis-related Pain; Endometriosis
MeSH terms: Endometriosis
Study type: Observational

Summary: 
      Endometriosis is defined as the presence of endometrial glands and stroma outside the uterine
      cavity. It is an estrogen-dependent chronic inflammatory disease. Estimates show that up to
      10% of premenopausal women have endometriosis. The most frequent forms of endometriosis are
      superficial peritoneal implants, ovarian cysts and deep nodules or plaques. However, the
      pelvis is not the exclusive site of endometriotic lesions: endometriosis can also affect the
      diaphragm, the pleura and the lung. All these localizations are included in the spectrum of
      "thoracic endometriosis".

      Thoracic endometriosis may present with cough, wheezing, catamenial pneumothorax, hemothorax,
      hemoptysis and pulmonary nodules. If the diaphragmatic pleura is also involved, catamenial
      periscapular or neck pain may be associated with irritation of the phrenic nerve. The
      symptoms of thoracic endometriosis generally have catamenial onset. Thoracic endometriosis
      rarely occurs isolated. It is considered a progression of pelvic endometriotic disease. Due
      to its varied presentation, diagnosis can be particularly difficult and often only identified
      due to clinical suspicion. The instrumental diagnosis of pneumothorax and catamenial
      hemothorax can be obtained by radiography or computerized axial tomography of the chest.
      Magnetic resonance imaging is to be preferred in case of diaphragmatic involvement. The gold
      standard remains exploratory laparoscopy, possibly accompanied by Video Assisted Thoracic
      Surgery (VATS).

      The first-line treatment is hormone therapy, aimed at suppressing ovulation and also
      preventing the onset of relapses. GnRH analogues are very effective in reducing the painful
      symptoms associated with endometriosis, but are not superior to other first-line treatments
      available. Furthermore, the prolonged hypoestrogenism can cause menopausal symptoms and
      osteoporosis.

      Surgery should be considered a second-line treatment in case of intolerance or
      ineffectiveness of medical therapy. The surgical approach is multidisciplinary and involves
      the endoscopist gynecologist and the thoracic surgeon expert in VATS.

      The principal aim of the study is the retrospective evaluation of thoracic endometriosis
      cases in our clinic over the last 20 years in order to evaluate the effectiveness of medical
      and surgical treatments in terms of satisfaction of patients suffering from this clinical
      condition. The secondary aim is to investigate the pathogenetic aspects of this clinical
      condition.
    

Detailed description: 
      Endometriosis is defined as the presence of endometrial glands and stroma outside the uterine
      cavity. It is an estrogen-dependent chronic inflammatory disease, in which the ectopic
      endometrium grows and proliferate under the action of estradiol. Estimates show that up to
      10% of premenopausal women and 35% to 50% of women with infertility, pelvic pain or both,
      have endometriosis.

      The most frequent forms of endometriosis are superficial peritoneal implants, ovarian cysts
      (endometrioma) and deep nodules or plaques (which can individually involve and infiltrate the
      parametria, Douglas pouch, anterior rectal wall, posterior vaginal fornix, antero-uterine
      pouch, bladder detrusor, ureters and sigmoid colon).

      However, the pelvis is not the exclusive site of endometriotic lesions: endometriosis can
      also be localized outside the pelvis, resulting in the implantation and growth of an ectopic
      endometrium and catamenial symptoms.

      In the thoracic cavity endometriosis can affect the diaphragm, the pleura and, ultimately,
      the lung. All these localizations are included in the spectrum of "thoracic endometriosis".

      Thoracic endometriosis may present with cough, wheezing, catamenial pneumothorax, hemothorax,
      hemoptysis and pulmonary nodules. If the diaphragmatic pleura is also involved, catamenial
      periscapular or neck pain may be associated with irritation of the phrenic nerve.

      Thoracic endometriosis rarely occurs isolated. It is considered a progression of pelvic
      endometriotic disease, due to the coexistence of pelvic localizations in up to 80% of cases
      and its onset at a later age (up to ten years later than pelvic endometriosis).

      The diagnosis of thoracic endometriosis can be misleading, because the related symptoms can
      be very variable, from total asymptomaticity to catamenial pneumothorax (80%), catamenial
      hemothorax (14%), catamenial hemoptysis (5%) or, more rarely , appearance of pulmonary
      nodules.

      The symptoms of thoracic endometriosis generally have catamenial onset (from 24 to 72 hours
      from the onset of menstrual flow), but can also appear remotely, in the event that the
      disease becomes chronic and the symptoms are linked to progressive clinical deterioration.
      Thoracic endometriosis involves the right hemithorax in 92% of cases and the left in 5% of
      cases; bilateral involvement occurs in only 3% of cases. Due to its varied presentation,
      diagnosis can be particularly difficult and often only identified due to clinical suspicion.
      The instrumental diagnosis of pneumothorax and catamenial hemothorax, exactly as for the
      non-catamenial equivalent, can be obtained by radiography or computerized axial tomography of
      the chest. Magnetic resonance imaging is to be preferred in case of diaphragmatic
      involvement; on the other hand, bronchoscopy is often not diagnostic because the
      endometriotic foci are located far from the mucous membranes of the main bronchi. The gold
      standard for the diagnosis of thoracic endometriosis remains exploratory laparoscopy,
      possibly accompanied by Video Assisted Thoracic Surgery (VATS). The evaluation of the
      presence of any diaphragmatic endometriotic implants should be routine during exploratory
      versus operative laparoscopy.

      The first-line treatment is hormone therapy, aimed at suppressing ovulation. Medical therapy
      can also prevent the onset of relapses.

      GnRH analogues are very effective in reducing the painful symptoms associated with
      endometriosis, but are not superior to other first-line treatments available. Furthermore,
      the prolonged hypoestrogenism can cause menopausal symptoms and osteoporosis.

      Surgery should be considered a second-line treatment in case of intolerance or
      ineffectiveness of medical therapy. The surgical approach is multidisciplinary and involves
      the endoscopist gynecologist and the thoracic surgeon expert in minimally invasive surgery
      (VATS). Alternatively or to complete the excisional surgical treatment, in the case of
      pleural involvement, mechanical or chemical pleurodesis can be performed, which can reduce
      the risk of recurrence of pneumothorax by 20-25%.

      The principal aim of the study is the retrospective evaluation of thoracic endometriosis
      cases in our clinic over the last 20 years in order to evaluate the effectiveness of medical
      and surgical treatments in terms of satisfaction of patients suffering from this clinical
      condition. Moreover, the secondary aim is to investigate the pathogenetic aspects of this
      clinical condition.

      This is a case report, retrospective/prospective and monocentric study; it is based on the
      review of medical records and on outpatient follow-up visits of patients in our clinic with
      thoracic endometriosis. This is a study design that it is best suited to a low prevalence of
      disease.
    

Intervention name(s): Satisfaction of patients after medical and surgical treatment for thoracic endometriosis
Intervention type(s): Other
Intervention description(s): It is a retrospectively evaluation of cases of thoracic endometriosis in our clinic over the last 20 years, based on the review of medical records and on outpatient follow-up visits.
The questionnaire are filled in routinely by patients attending the endometriosis center of this institute every 6 months. We will collect data relating to these questionnaires referring prospectively to the study period.
If the questionnaires have not already been filled in by the patient, she will be contacted to fill them in, after signing the informed consent of the study.
Observational model: Cohort
Time perspective: Retrospective
Sampling method: Non-Probability Sample
Study population: 
        All the patients referred to our endometriosis clinic and/or underwent surgery from 1
        January 2000 to 01 March 2022 with a diagnosis of thoracic endometriosis were selected.
      
Keywords: Medical therapy; Surgical treatment; Satisfaction