Catamenial pneumothorax: A scourge not to be ignored

Catamenial pneumothorax (CP) is defined as spontaneous pneumothorax occurring within 72 h before or after the onset of menstruation.[] It is the most common form of thoracic endometriosis syndrome (TES), which includes catamenial hemoptysis, catamenial hemothorax, catamenial hemopneumothorax, and endometrial nodular lung mass.[] Recurrent spontaneous pneumothorax associated with the menstrual cycle was first described in 1958 by Maurer et al.[] and the term catamenial pneumothorax was established subsequently by Lillington et al. in 1972.[] It was considered a rare entity,[,] though a prospective study done about a decade ago showed that 25% of women hospitalized in an 18-month period for surgical treatment of spontaneous pneumothorax had this condition.[] PATHOGENESIS The pathogenesis of this condition is not very clear, with the etiology most likely to be multifactorial in origin.[,,,,,,,,,,,,,,,,] Many hypotheses regarding the pathogenesis of CP have been proposed. The air in the pleural space can result from the spontaneous rupture of blebs and alveolar rupture with air leaking into the pleural space or the passage of air from the genital tract through perforations in the diaphragm into the pleural space.[,,,,] Endometrial tissue has been shown to actively invade local tissue that it comes in contact with, and when in the pleural or lung tissue, can result in air leak or even hemoptysis by invading lung tissues.[,,] Hobbs et al. demonstrated that endometrial tissue injected intravenously into rabbits was deposited in the lungs, and subsequently proliferated and sloughed in synchrony with the menstrual cycle.[] Rossi et al. observed in their study a synchronous increase in prostaglandin F2 during menses, with the prostaglandin inducing CP as a result of its potent bronchial and vascular constriction which caused the rupture of preformed subpleural blebs in an otherwise normal lung.[] The theory of trans-fallopian ascent of air is supported by the fact that plication of diaphragmatic perforations and tubal ligation have cured CP.[,,,] It is important to emphasize that the air moves by a diaphragmatic defect which may be congenital or acquired as a result of the sloughing of the diaphragmatic endometrial tissue. However, in what way the endometrial tissue reaches the thoracic cavity remains unknown in spite of the above explanations.[,] Importantly, at menstruation, the dissolving cervical mucus plug may allow the ascent of air through the fallopian tubes. In the same way, the presence of cervical mucus in postpartum and postcoital periods has been reported to have occasionally caused spontaneous pneumothorax.[,,] Currently, the most favored hypothesis suggests endometrial tissues' auto-transplantation to ectopic sites by embolization through the lymphatic or vascular route.[] Although no single theory can explain all of the findings in either TES or CP, there are, however, three extant theories of the pathogenesis that may be relevant to CP:(1) Sampson's theory of retrograde menstruation and implantation,[,,] (2) entry of endometrial cells into the venous system and "metastatic" spread of endometrial tissue,[] and (3) coelomic metaplasia.[] The theories of both implantation and metaplasia were put forward to explain endometriosis.[,,,,] However, reflux of endometrial fragments is common during menstruation,[,,] and proliferating endometrial cells capable of tissue adhesion, invasion, and angiogenesis have been isolated from the peritoneal fluid during menses.[,] Just as stated previously, the air moves into the pleural space from the peritoneum through the fenestrated diaphragm. Thus, for CP to occur, according to one hypothesis, there must be the presence of air moving via the openings. If there is air and no fenestration or fenestration without pneumoperitoneum, there would not be CP, as spontaneous pneumothorax in men with diaphragmatic fenestrations has hitherto not been described.[,,,] However, pneumothorax after therapeutic pneumoperitoneum in men with diaphragmatic lesions has been observed.[] The predominance of the right side CP may also be explained by the piston-like effect of the solid liver bulk, transmitting intraperitoneal pressure spikes across a perforated hemi-diaphragm.[,] The endometrial tissue circulates with the clockwise current of peritoneal fluid in the abdominal cavity - down the left peritoneal gutter, over the pelvic floor, and up the right gutter to the peritoneal surface of the right diaphragm which would explain the preferred occurrence of CP on the right side.[,,] The formation of diaphragmatic bulla was observed to probably represent a stage in the pathology of subsequent diaphragmatic rupture and was therefore considered the commonest cause of spontaneous pneumothorax in cases of pneumoperitoneum existing with such condition.[] Kirschner described the porous diaphragmatic syndrome in which pneumoperitoneum was also related to pneumothorax.[] and it was also noted that these porosity could be created by lymphatics passing through the diaphragm.[] MANAGEMENT STRATEGY A high index of suspicion is entertained when a woman presents with clinical features of catamenial chest pain or catamenial hemoptysis or catamenial pneumothorax.[,] The initial clinical evaluation is done with a chest radiograph to confirm the presence of air of in the pleural space. Subsequently, the accurate search for the presence of endometrial tissues in the aforementioned areas is done; a computerized tomography is obtained to search for intra-thoracic endometrial implants and the air trapped in between the lung lobes.[] This is followed by ensuring lung re-expansion followed by ensuring adequate lung re-expansion by evacuating the pneumothorax and stoppage of further air leakage into the pleural space. To achieve these, the obliteration of the pleural space is done; the obliteration of the diaphragmatic openings with direct closure or with the use of mesh and suppresse the effect of some residual endometrial tissues using hormonal therapy. Therapeutic modalities include medical treatment alone, sequential medical-surgical, surgical-medical, or surgical treatment alone.[] Surgical treatment should be accomplished during menstruation for optimal visualization of pleura-diaphragmatic endometriosis.[] and the Surgery could involve the use of video-assisted thoracoscopy (VAT) or VAT with open surgery. VAT with diaphragmatic resection as well as resection of possible bullae or blebs is probably the best option for the treatment for diaphragmatic perforations, blebs, and for the presence of endometrial deposits. VAT allows for good visualization and enables tissue to be obtained for histological diagnosis, especially to rule out malignancy.[,,] Also, being a minimally invasive procedure, postoperative pain is reduced. Simple suturing of holes rather than resection coverage of defect with mesh has been reported to be associated with a high recurrence rate and it does not provide tissue for diagnosis.[] And, if endometrial implants are left in place, they may lead to perforations and, possibly, further intrathoracic dissemination. Diaphragmatic resection and repair may be achieved by endoscopic stapler devices, provided that the resected surface is small, but should be discouraged in large and multiple defects created by endometrial tissue.[] Despite advances in video-assisted surgical techniques, open surgery remains an important procedure in the treatment of CP, though often reserved for those with previous unsuccessful surgery. With open surgery using mini-thoracotomy, lesions close to the phrenic nerve ccould be cautiously resected.[] Bagan et al. proposed the use of polyglactin mesh to cover the tendinious part of the diaphragm because of the risk of leaving behind occult defects.[] Pleurodesis can be achieved with pleural abrasion or pleurectomy or chemicalpleurodesis.[,] It is important to note that the standard chemical pleurodesis may not be enough and may require the combination of pleurectomy.[] or the use of talc pleurodesis for treatment failure.[] Total abdominal hysterectomy and bilateral salpingo-oophorectomy should also be considered as an adjuvant therapy in those with repeated unsuccessful treatment.[] and those who have completed their family. The basis for the use of hormonal therapy is that the endometrial implants are hormonal dependent and the essential aim of medical treatment therefore is to interrupt the hormonal support to the existing endometrial implants and to prevent further occurrence.[] The drugs that have been tried in this regard include combined oral contraceptives, progestogens, GnRH analogs, and danazol (a derivative of the synthetic steroid ethisterone, a modified progestogen), but no controlled trial has been carried to test the efficacy of these drugs in the management of CP and none has been found to be superior to the other in terms of efficacy.[,] Medical therapy alone is associated with a high recurrence rate and combination with surgery yields better outcome.[] Marshall et al. noted that medical treatment of thoracic endometriosis prevents recurrence in 50% of cases at the most and a sequential medical-surgical or surgical-medical approach may become necessary for patients who do not achieve a satisfactory response to the first treatment option employed.[] In one experience, the estrogen-progesterone treatment was unsuccessful in all cases, but when GnRH agonist therapy was introduced, there seemed to be an improved outcome of treatment of CP.[] The rationale underlying hormonal control is based on the presence of endometrial implants which are hormone dependent. Substances known to suppress the growth of ectopic endometrium would also be active on the pulmonary implants. Hormonal control using danazol has its own risks and side effects such as hirsutism and deepening of voice, which are irreversible changes even when the drugs are stopped, and with GnRH analogs, complications such as postmenopausal symptoms and osteoporosis with rebound effect occur if used for more than 6 months, thus treatment cannot exceed 6 months. Nevertheless, this interval is considered sufficient to avoid recurrences.[,] Standard pleurodesis may not be sufficient to avoid recurrences in CP patients, so subtotal pleurectomy was suggested with chemical pleurodesis on the diaphragm as an initial approach.[,,,]

The etio-pathogenesis of CP is not completely known. However, diagnosis is not difficult in proper settings and requires more proper clinical evaluation, and immediate institution of medical and surgical management will substantially reduce the scourge of this condition.

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