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Address correspondence to Stephen D. Cassivi, MD, MSc, Division of General Thoracic Surgery, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905
Affiliations
Division of General Thoracic Surgery, Mayo Clinic College of Medicine, Rochester, Minn
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
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.
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.
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.
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.
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.
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.
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.
However, attempts to use nonsteroidal anti-inflammatory medications to inhibit prostaglandin synthesis have failed to prevent recurrence of catamenial pneumothorax.
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.
Diaphragmatic fenestrations have been implicated in the pathogenesis of the predominantly right-sided pleural effusions in patients with hepatic hydrothorax and Meigs syndrome.
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.
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).
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.
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).
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.
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.
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
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.
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.
REFERENCES
Maurer ER
Schaal JA
Mendez Jr, FL
Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm.
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