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Catamenial Pneumothorax

      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 al
      • Maurer ER
      • Schaal JA
      • Mendez Jr, FL
      Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm.
      in 1958. The term catamenial pneumothorax was established subsequently by Lillington et al
      • Lillington GA
      • Mitchell SP
      • Wood GA
      Catamenial pneumothorax.
      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.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      • Cowl CT
      • Dunn WF
      • Deschamps C
      Visualization of diaphragmatic fenestration associated with catamenial pneumothorax.

      REPORT OF A CASE

      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.
      Figure thumbnail gr1
      Figure 1Posteroanterior chest radiograph reveals right-sided pneumothorax (arrows indicate edge of visceral pleura).
      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.
      Figure thumbnail gr2
      Figure 2Intraoperative images of the pleural surface of the diaphragm. A, Two diaphragmatic fenestrations. B, Probe passing through a fenestration. C, Fenestration after closure by suture ligation.

      DISCUSSION

      Catamenial pneumothorax is believed to be the most frequent clinical manifestation of intrathoracic endometriosis.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      Interestingly, concomitant pelvic endometriosis is present in only 61% of women with catamenial pneumothorax.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      The mean age at presentation varies between 32 and 37 years in different published series, with a range from 19 to 54 years.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      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.
      • Nakamura H
      • Konishiike J
      • Sugamura A
      • Takeno Y
      Epidemiology of spontaneous pneumothorax in women.
      • Shearin RPN
      • Hepper NGG
      • Payne WS
      Recurrent spontaneous pneumothorax concurrent with menses.
      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.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      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.
      • Van Schil PE
      • Vercauteren SR
      • Vermeire PA
      • Nackaerts YH
      • Van Marck EA
      Catamenial pneumothorax caused by thoracic endometriosis.
      This theory is supported by the identification of endometrial deposits in the pleural space, present in 13% to 62.5% of these patients.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      The hormonal hypothesis proposed by Rossi and Goplerud
      • Rossi NP
      • Goplerud CP
      Recurrent catamenial pneumothorax.
      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.
      • Rossi NP
      • Goplerud CP
      Recurrent catamenial pneumothorax.
      However, attempts to use nonsteroidal anti-inflammatory medications to inhibit prostaglandin synthesis have failed to prevent recurrence of catamenial pneumothorax.
      • Dattola RK
      • Toffle RC
      • Lewis MJ
      Catamenial pneumothorax: a case report.
      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.
      • Lillington GA
      • Mitchell SP
      • Wood GA
      Catamenial pneumothorax.
      • Cowl CT
      • Dunn WF
      • Deschamps C
      Visualization of diaphragmatic fenestration associated with catamenial pneumothorax.
      • Kirschner PA
      Porous diaphragm syndromes.
      Diaphragmatic fenestrations have been implicated in the pathogenesis of the predominantly right-sided pleural effusions in patients with hepatic hydrothorax and Meigs syndrome.
      • Majzlin G
      • Stevens FL
      Meigs' syndrome: case report and review of literature.
      • Lieberman FL
      • Hidemura R
      • Peters RL
      • Reynolds TB
      Pathogenesis and treatment of hydrothorax complicating cirrhosis with ascites.
      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.
      • Roth T
      • Alifano M
      • Schussler O
      • Magdaleinat P
      • Regnard JF
      Catamenial pneumothorax: chest x-ray sign and thoracoscopic treatment.
      Furthermore, recent studies have shown a high prevalence of diaphragmatic defects (50%-62.5%) in patients with catamenial pneumothorax.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      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).
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      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.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      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).
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      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.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      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.
      • Fonseca P
      Catamenial pneumothorax: a multifactorial etiology.
      Recurrent catamenial pneumothorax also has been reported after hysterectomy.
      • Shearin RPN
      • Hepper NGG
      • Payne WS
      Recurrent spontaneous pneumothorax concurrent with menses.
      • Lee CY
      • Di Loreto PC
      • Beaudoin J
      Catamenial pneumothorax.
      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 surface
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      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.
      • Joseph J
      • Sahn SA
      Thoracic endometriosis syndrome: new observations from an analysis of 110 cases.
      If diaphragmatic defects are present, surgical repair combined with postoperative hormonal suppression has resulted in excellent outcome.
      • Alifano M
      • Roth T
      • Broet SC
      • Schussler O
      • Magdeleinat P
      • Regnard JF
      Catamenial pneumothorax: a prospective study.
      • Bagan P
      • Le Pimpec Barthes F
      • Assouad J
      • Souilamas R
      • Riquet M
      Catamenial pneumothorax: retrospective study of surgical treatment.
      Whether this “belt-and-suspenders” approach is necessary remains unclear.

      CONCLUSION

      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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