Catamenial Pneumothorax
2005; Elsevier BV; Volume: 80; Issue: 5 Linguagem: Inglês
10.4065/80.5.677
ISSN1942-5546
AutoresTobias Peikert, Delmar J. Gillespie, Stephen D. Cassivi,
Tópico(s)Intestinal and Peritoneal Adhesions
ResumoCatamenial pneumothorax is defined as spontaneous pneumothorax occurring within 72 hours before or after onset of menstruation. Although catamenial pneumothorax is the most common clinical manifestation of intrathoracic endometriosis, this latter condition is not universally identified in women with catamenial pneumothorax and cannot fully explain the recurrent and cyclical episodes of pneumothorax. Therefore, the etiology of this syndrome is unknown, although many theories have been proposed to explain it. We describe a 37-year-old woman with recurrent episodes of spontaneous right-sided pneumothorax and chest pain that occurred close to her menstrual periods. The patient's condition did not abate after initial surgical exploration with abrasive mechanical pleurodesis or after hormonal suppressive therapy at an institution elsewhere. The patient was referred to our institution for further evaluation. A second surgical inspection of the pleural cavity and diaphragm disclosed the presence of multiple diaphragmatic fenestrations that were closed surgically at that time. Postoperatively, the patient discontinued hormonal suppressive therapy, and menstrual cycles became regular. Six months after surgery, the patient remains asymptomatic with no evidence of recurrence of pneumothorax. This case supports recent reports that diaphragmatic defects are often present in patients with catamenial pneumothorax. Surgical exploration to inspect the diaphragm and to close all identified defects should be performed in patients who continue to experience pneumothorax despite effective hormonal suppression. Catamenial pneumothorax is defined as spontaneous pneumothorax occurring within 72 hours before or after onset of menstruation. Although catamenial pneumothorax is the most common clinical manifestation of intrathoracic endometriosis, this latter condition is not universally identified in women with catamenial pneumothorax and cannot fully explain the recurrent and cyclical episodes of pneumothorax. Therefore, the etiology of this syndrome is unknown, although many theories have been proposed to explain it. We describe a 37-year-old woman with recurrent episodes of spontaneous right-sided pneumothorax and chest pain that occurred close to her menstrual periods. The patient's condition did not abate after initial surgical exploration with abrasive mechanical pleurodesis or after hormonal suppressive therapy at an institution elsewhere. The patient was referred to our institution for further evaluation. A second surgical inspection of the pleural cavity and diaphragm disclosed the presence of multiple diaphragmatic fenestrations that were closed surgically at that time. Postoperatively, the patient discontinued hormonal suppressive therapy, and menstrual cycles became regular. Six months after surgery, the patient remains asymptomatic with no evidence of recurrence of pneumothorax. This case supports recent reports that diaphragmatic defects are often present in patients with catamenial pneumothorax. Surgical exploration to inspect the diaphragm and to close all identified defects should be performed in patients who continue to experience pneumothorax despite effective hormonal suppression. Recurrent spontaneous pneumothorax associated with the menstrual cycle was described first by Maurer et al1Maurer ER Schaal JA Mendez Jr, FL Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm.JAMA. 1958; 168: 2013-2014Crossref PubMed Scopus (174) Google Scholar in 1958. The term catamenial pneumothorax was established subsequently by Lillington et al2Lillington GA Mitchell SP Wood GA Catamenial pneumothorax.JAMA. 1972; 219: 1328-1332Crossref PubMed Scopus (134) Google Scholar in 1972. Today, catamenial pneumothorax is defined as recurrent spontaneous pneumothorax occurring within 72 hours before or after onset of menstruation. Historically, catamenial pneumothorax was believed to be a rare syndrome and was attributed to intrathoracic endometriosis. Even today, many years after the original description, the pathophysiology is poorly understood. We describe a young woman with catamenial pneumothorax and diaphragmatic fenestrations. Her clinical course and operative findings support recently published data that show that diaphragmatic fenestrations are commonly present and involved in the pathophysiology of this condition.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar, 4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar, 5Cowl CT Dunn WF Deschamps C Visualization of diaphragmatic fenestration associated with catamenial pneumothorax.Ann Thorac Surg. 1999; 68: 1413-1414Abstract Full Text Full Text PDF PubMed Scopus (37) Google Scholar A previously healthy 37-year-old woman (gravida III, para III) developed right-sided chest pain while swimming. The pain was sharp and localized to the right posterior chest wall and was accentuated on respiration. The patient had mild dyspnea on exertion. Her symptoms were evaluated by her local physician who obtained a chest radiograph, which revealed a right-sided pneumothorax (Figure 1). In the absence of a history of trauma and associated lung disease (documented by computed tomography of the chest), spontaneous pneumothorax was diagnosed and the patient was treated conservatively with observation. The patient recovered subsequently and remained well until similar symptoms occurred at her next menstrual period. Because the symptoms associated with her first episode of pneumothorax had started within 48 hours of onset of her menses, a diagnosis of catamenial pneumothorax was considered. Subsequently, the patient experienced monthly, ipsilateral episodes of pneumothorax, all of which resolved spontaneously, although initially symptomatic. Approximately 6 months after the patient's original presentation, she underwent right video-assisted thoracoscopy during menstruation with inspection of the pleural space, followed by abrasive mechanical pleurodesis. No intrathoracic endometriosis was identified. The patient's postoperative clinical course was complicated by a prolonged air leak requiring extended chest tube drainage. The patient continued experiencing recurrent, monthly, right-sided chest pain and episodes of pneumothorax after this initial surgical exploration. Hormonal suppressive therapy with 11.25 mg of leuprolide administered intramuscularly every 12 weeks ended the patient's menstrual cycles and chest symptoms. She required low-dose hormone supplementation with 1 mg of ethinyl estradiol and 5 mg of norethisteron acetate formulation, along with a serotonin reuptake inhibitor (20 mg of fluoxetine) for the mood swings associated with hormonal suppression. Eight months after receiving leuprolide therapy, the patient developed breakthrough bleeding, which again was associated with right-sided pneumothorax. She was referred to our institution for further evaluation. The patient had normal vital signs and unremarkable findings on physical examination, which included the pelvis. She denied prior medical problems. The patient was receiving leuprolide injections every 12 weeks and was taking oral fluoxetine. The low-dose estrogen replacement had been discontinued after onset of breakthrough bleeding. The patient denied prior dysmenorrhea, dyspareunia, infertility, or history of endometriosis. Her serum estradiol level was suppressed adequately while she was taking leuprolide, and she was noticeably debilitated by her recurrent symptoms. The patient underwent a second right video-assisted thoracoscopy for further diagnosis and therapy. Limited visualization secondary to adhesions necessitated conversion to a limited thoracotomy. No pleural endometriosis was identified. Inspection of the diaphragm revealed the presence of 4 distinct fenestrations (the largest of which was 3 mm in diameter) that were freely communicating with the peritoneal cavity. These fenestrations were closed individually by suture ligation (Figure 2). A second abrasive mechanical pleurodesis was performed. The patient had an uncomplicated postoperative clinical course and continues to do well 6 months after surgical closure of her diaphragmatic fenestrations. She has discontinued hormonal suppressive therapy, and her menstrual cycles are regular; no pneumothorax or chest pain has occurred. Catamenial pneumothorax is believed to be the most frequent clinical manifestation of intrathoracic endometriosis.6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar Interestingly, concomitant pelvic endometriosis is present in only 61% of women with catamenial pneumothorax.6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar The mean age at presentation varies between 32 and 37 years in different published series, with a range from 19 to 54 years.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar, 4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar, 6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar The most common symptoms are chest pain and dyspnea. This condition occurs predominantly on the right side. The disease is believed to be rare, and previous retrospective analyses suggest a prevalence of 1% to 5% among menstruating women with spontaneous pneumothorax.7Nakamura H Konishiike J Sugamura A Takeno Y Epidemiology of spontaneous pneumothorax in women.Chest. 1986; 89: 378-382Crossref PubMed Scopus (66) Google Scholar, 8Shearin RPN Hepper NGG Payne WS Recurrent spontaneous pneumothorax concurrent with menses.Mayo Clin Proc. 1974; 49: 98-101PubMed Google Scholar In a recent prospective study of women referred to a single center for evaluation and treatment of persistent or recurrent spontaneous pneumothorax despite previous chest tube drainage or attempted surgical therapy, pneumothorax in 25% had a temporal relationship with onset of menses.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar Although the clinical association between the development of spontaneous pneumothorax and the menstrual cycle has been well characterized, the causal mechanisms remain elusive. Three distinct mechanisms have been proposed based on metastatic, hormonal, and anatomical models. The metastatic model suggests migration of endometrial tissue via the peritoneal cavity through transdiaphragmatic lymphatic channels or diaphragmatic fenestrations or hematogenously into the pleural space. Because these congenital diaphragmatic channels or fenestrations are more common in the right hemidiaphragm, it is not surprising that manifestations of thoracic endometriosis occur predominantly on the right side of the chest. Alternatively, it is postulated that endometrial tissue may be deposited in the chest cavity during embryonal development. Monthly shedding of endometrial tissue is believed to result in pleural irritation that causes chest pain and pulmonary air leaks, resulting in pneumothorax.9Van Schil PE Vercauteren SR Vermeire PA Nackaerts YH Van Marck EA Catamenial pneumothorax caused by thoracic endometriosis.Ann Thorac Surg. 1996; 62: 585-586Abstract Full Text PDF PubMed Scopus (73) Google Scholar This theory is supported by the identification of endometrial deposits in the pleural space, present in 13% to 62.5% of these patients.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar, 4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar, 6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar The hormonal hypothesis proposed by Rossi and Goplerud10Rossi NP Goplerud CP Recurrent catamenial pneumothorax.Arch Surg. 1974; 109: 173-176Crossref PubMed Scopus (95) Google Scholar in 1974 suggests that high serum levels of prostaglandin F2 at ovulation may lead to vasospasm and associated ischemia in the lungs. They speculated that this tissue injury, combined with prostaglandin-induced bronchospasm, may result in alveolar rupture and pneumothorax. This theory is based on observations of high serum prostaglandin levels associated with ovulatory cycles and on documented changes of respiratory epithelium during the menstrual cycle.10Rossi NP Goplerud CP Recurrent catamenial pneumothorax.Arch Surg. 1974; 109: 173-176Crossref PubMed Scopus (95) Google Scholar However, attempts to use nonsteroidal anti-inflammatory medications to inhibit prostaglandin synthesis have failed to prevent recurrence of catamenial pneumothorax.11Dattola RK Toffle RC Lewis MJ Catamenial pneumothorax: a case report.J Reprod Med. 1990; 35: 734-736PubMed Google Scholar Also, the hormonal hypothesis does not explain the preponderance of right-sided occurrences. Similarly problematic for proponents of this theory is the paucity of clinical indicators of bronchospasm in this patient population. The anatomical model is based on the influx of air into the pleural space from the peritoneal cavity via diaphragmatic fenestrations. This model postulates that the loss of the cervical mucous plug during the menstrual cycle results in communication between the environment and the peritoneal cavity, allowing an influx of air into the peritoneal cavity via patent fallopian tube(s) and subsequently into the pleural space via communication through the diaphragm.2Lillington GA Mitchell SP Wood GA Catamenial pneumothorax.JAMA. 1972; 219: 1328-1332Crossref PubMed Scopus (134) Google Scholar, 5Cowl CT Dunn WF Deschamps C Visualization of diaphragmatic fenestration associated with catamenial pneumothorax.Ann Thorac Surg. 1999; 68: 1413-1414Abstract Full Text Full Text PDF PubMed Scopus (37) Google Scholar, 12Kirschner PA Porous diaphragm syndromes.Chest Surg Clin N Am. 1998; 8: 449-472PubMed Google Scholar Diaphragmatic fenestrations have been implicated in the pathogenesis of the predominantly right-sided pleural effusions in patients with hepatic hydrothorax and Meigs syndrome.13Majzlin G Stevens FL Meigs' syndrome: case report and review of literature.J Int Coll Surg. 1964; 42: 625-630PubMed Google Scholar, 14Lieberman FL Hidemura R Peters RL Reynolds TB Pathogenesis and treatment of hydrothorax complicating cirrhosis with ascites.Ann Intern Med. 1966; 64: 341-351Crossref PubMed Scopus (228) Google Scholar In agreement with this observation, it has been hypothesized that intraperitoneal air could migrate similarly into the chest. This phenomenon has been suggested by radiographic evidence of concomitant pneumothorax and intraperitoneal free air.15Roth T Alifano M Schussler O Magdaleinat P Regnard JF Catamenial pneumothorax: chest x-ray sign and thoracoscopic treatment.Ann Thorac Surg. 2002; 74: 563-565Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar Furthermore, recent studies have shown a high prevalence of diaphragmatic defects (50%-62.5%) in patients with catamenial pneumothorax.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar, 4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar In a prospective study, only 1 of 8 patients who underwent surgical repair of diaphragmatic defects had a recurrence during a mean follow-up period of 6.6 months (range, 2-15 months).3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar A retrospective analysis of 10 patients with catamenial pneumothorax revealed no recurrences during a mean follow-up of 33 months (range, 12-48 months) in the 5 patients treated by diaphragmatic repair.4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar This compares favorably with the largest series reported in the literature, which found a 47.6% recurrence rate in the surgical group who underwent a combination of surgical techniques (mean follow-up, 22 months).6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar These data suggest that surgical closure of diaphragmatic defects is associated with excellent therapeutic outcome. Additional support for this theory comes from the almost exclusive (95%) right-sided occurrence of this rare type of pneumothorax.6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar Conversely, it is intriguing that subdiaphragmatic air is not encountered more frequently on routine chest radiographs of women of reproductive age. Also, with the increasing use of laparoscopic techniques during surgical procedures of the abdomen, it is interesting that incidental perioperative pneumothorax is not a more frequent complication of a surgically induced pneumoperitoneum.16Fonseca P Catamenial pneumothorax: a multifactorial etiology.J Thorac Cardiovasc Surg. 1998; 116: 872-873Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar Recurrent catamenial pneumothorax also has been reported after hysterectomy.8Shearin RPN Hepper NGG Payne WS Recurrent spontaneous pneumothorax concurrent with menses.Mayo Clin Proc. 1974; 49: 98-101PubMed Google Scholar, 17Lee CY Di Loreto PC Beaudoin J Catamenial pneumothorax.Obstet Gynecol. 1974; 44: 407-411PubMed Google Scholar Many questions remain unanswered regarding the pathogenesis of catamenial pneumothorax. Various mechanisms may contribute in individual patients, and further investigations are needed. Because of the rarity of this condition, no randomized or controlled trials are available to guide the therapy for women with catamenial pneumothorax. Therapeutic options consist of surgical exploration with resection of endometrial deposits within the pleural space and/or repair of diaphragmatic defects (if found) with or without pleurodesis (mechanical or chemical). Nonsurgical options include hormonal suppression with gonadotropin-releasing hormone agonists such as leuprolide, oral contraceptives, and bilateral salpingo-oophorectomy. With surgical exploration, some authors recommend systematic use of a polyglactin mesh cover of the diaphragmatic surface4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar to close occult fenestrations and promote adhesion of the basilar surface of the lung to the diaphragm. Surgical therapy appears to be a more definitive approach because it is associated with fewer recurrences compared with hormonal suppression.6Joseph J Sahn SA Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.Am J Med. 1996; 100: 164-170Abstract Full Text PDF PubMed Scopus (333) Google Scholar If diaphragmatic defects are present, surgical repair combined with postoperative hormonal suppression has resulted in excellent outcome.3Alifano M Roth T Broet SC Schussler O Magdeleinat P Regnard JF Catamenial pneumothorax: a prospective study.Chest. 2003; 124: 1004-1008Crossref PubMed Scopus (177) Google Scholar, 4Bagan P Le Pimpec Barthes F Assouad J Souilamas R Riquet M Catamenial pneumothorax: retrospective study of surgical treatment.Ann Thorac Surg. 2003; 75: 378-381Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar Whether this “belt-and-suspenders” approach is necessary remains unclear. Catamenial pneumothorax should be suspected in any menstruating woman presenting with recurrent spontaneous pneumothorax. Our patient's clinical course and our review of the literature indicate that catamenial pneumothorax is a heterogeneous syndrome. Considering the high frequency of diaphragmatic abnormalities, early surgical exploration, with specific attention to possible defects in the diaphragm, should be pursued in such patients. This is especially the case in the absence of pelvic endometriosis.
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