katamenial pneumotoraks

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CATAMENIAL PNEUMOTHORAX

Mayo Clin Proc. May 2005;80(5):677-680 www.mayoclinicproceedings.com 677

Catamenial Pneumothorax

CASE REPORT

TOBIAS PEIKERT, MD; DELMAR J. GILLESPIE, MD, PHD; AND STEPHEN D. CASSIVI, MD, MSC

From the Department of Internal Medicine and Division of Pulmonary andCritical Care Medicine (T.P., D.J.G.) and Division of General Thoracic Surgery(S.D.C.), Mayo Clinic College of Medicine, Rochester, Minn.

Individual reprints of this article are not available. Address correspondence toStephen D. Cassivi, MD, MSc, Division of General Thoracic Surgery, MayoClinic College of Medicine, 200 First St SW, Rochester, MN 55905.

2005 Mayo Foundation for Medical Education and Research

Catamenial pneumothorax is defined as spontaneous pneumotho-rax occurring within 7 2 hours before or after onset of menstrua-tion. Although catamenial pneumothorax is the most commonclinical manifestation of intrathoracic endometriosis, this lattercondit ion is not universally identif ied in women wit h cat amenialpneumothorax and cannot fully explain t he recurrent and c yclicalepisodes of pneumothorax. Therefore, the etiology of this syn-drome is unknown, alt hough many theories have been proposed toexplain it. We describe a 37-year-old woman with recurrent epi-sodes of spontaneous right-sided pneumothorax and chest painthat occurred close to her menstrual periods. The patients condi-t ion did not abate after init ial surgical exploration with abrasivemechanical pleurodesis or after hormonal suppressive therapy atan institut ion elsewhere. The patient was referred to our instit u-tion for further evaluation. A second surgical inspection of thepleural cavity and diaphragm disclosed the presence of multiplediaphragmatic fenestrations that were closed surgically at thatt ime. Postoperatively, the pat ient discontinued hormonal suppres-sive therapy, and menstrual cycles became regular. Six monthsafter surgery, the pat ient remains asymptomatic w ith no evidenceof recurrence of pneumothorax. This case supports recent reportsthat diaphragmatic defects are often present in patients withcatamenial pneumothorax. Surgical exploration to inspect thediaphragm and to close all identified defects should be performedin patients who continue to experience pneumothorax despiteeffective hormonal suppression.

Mayo Clin Proc. 2005;80(5) :677 -680

Recurrent spontaneous pneumothorax associated with

the menstrual cycle was described first by Maurer etal1 in 1958. The term catamenial pneumothorax was estab-

lished subsequently by Lillington et al2 in 1972.

Today, catamenial pneumothorax is defined as recurrent

spontaneous pneumothorax occurring within 72 hours be-

fore 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 pneu-

mothorax and diaphragmatic fenestrations. Her clinical

course and operative findings support recently published

data that show that diaphragmatic fenestrations are com-monly present and involved in the pathophysiology of this

condition.3-5

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.

The patient recovered subsequently and remained well

until similar symptoms occurred at her next menstrual pe-

riod. 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 con-

sidered. Subsequently, the patient experienced monthly, ip-

silateral episodes of pneumothorax, all of which resolved

spontaneously, although initially symptomatic.

Approximately 6 months after the patients original pre-

sentation, she underwent right video-assisted thoracoscopy

during menstruation with inspection of the pleural space,

followed by abrasive mechanical pleurodesis. No intratho-

racic endometriosis was identified. The patients postop-

erative 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 initialsurgical exploration.

Hormonal suppressive therapy with 11.25 mg of

leuprolide administered intramuscularly every 12 weeks

ended the patients 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 hor-

monal 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 unremarkablefindings on physical examination, which included the pel-

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Mayo Clin Proc. May 2005;80(5):677-680 www.mayoclinicproceedings.com678


vis. 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 bleed-

ing. 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 recur-rent symptoms.

The patient underwent a second right video-assisted

thoracoscopy for further diagnosis and therapy. Limited

visualization secondary to adhesions necessitated conver-

sion 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 abra-

sive mechanical pleurodesis was performed. The patient

had an uncomplicated postoperative clinical course and

continues to do well 6 months after surgical closure of herdiaphragmatic fenestrations. She has discontinued hor-

monal suppressive therapy, and her menstrual cycles are

regular; no pneumothorax or chest pain has occurred.

DISCUSSION

Catamenial pneumothorax is believed to be the most fre-

quent clinical manifestation of intrathoracic endometrio-

sis.6 Interestingly, concomitant pelvic endometriosis is

present in only 61% of women with catamenial pneu-

mothorax.6 The mean age at presentation varies between 32

and 37 years in different published series, with a range

from 19 to 54 years.3,4,6 The most common symptoms are

chest pain and dyspnea. This condition occurs predomi-nantly 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.7,8 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.3

Although the clinical association between the develop-

ment 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 con-

genital diaphragmatic channels or fenestrations are more

common in the right hemidiaphragm, it is not surprising

that manifestations of thoracic endometriosis occur pre-

dominantly 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 pleu-ral irritation that causes chest pain and pulmonary air leaks,

resulting in pneumothorax.9 This theory is supported by the

identification of endometrial deposits in the pleural space,

present in 13% to 62.5% of these patients.3,4,6

The hormonal hypothesis proposed by Rossi and

Goplerud10 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 bron-

chospasm, may result in alveolar rupture and pneumotho-

rax. This theory is based on observations of high serum

prostaglandin levels associated with ovulatory cycles and

on documented changes of respiratory epithelium duringthe menstrual cycle.10 However, attempts to use nonsteroi-

dal anti-inflammatory medications to inhibit prostaglandin

synthesis have failed to prevent recurrence of catamenial

pneumothorax.11 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 pa-

tient population.

FIGURE 1. Posteroanterior chest radiograph reveals right-sidedpneumothorax (arrows indicate edge of visceral pleura).

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Mayo Clin Proc. May 2005;80(5):677-680 www.mayoclinicproceedings.com 679

The anatomical model is based on the influx of air into

the pleural space from the peritoneal cavity via diaphrag-

matic fenestrations. This model postulates that the loss of

the cervical mucous plug during the menstrual cycle results

in communication between the environment and the perito-

neal 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 dia-

phragm.2,5,12 Diaphragmatic fenestrations have been impli-

cated in the pathogenesis of the predominantly right-sided

pleural effusions in patients with hepatic hydrothorax and

Meigs syndrome.13,14 In agreement with this observation,

it has been hypothesized that intraperitoneal air couldmigrate similarly into the chest. This phenomenon has been

suggested by radiographic evidence of concomitant pneu-

mothorax and intraperitoneal free air.15 Furthermore, recent

studies have shown a high prevalence of diaphragmatic

defects (50%-62.5%) in patients with catamenial pneu-

mothorax.3,4 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).3 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 diaphrag-

matic repair.4 This compares favorably with the largestseries reported in the literature, which found a 47.6% recur-

rence rate in the surgical group who underwent a combina-

tion of surgical techniques (mean follow-up, 22 months).6

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.6

Conversely, it is intriguing that subdiaphragmatic air is

not encountered more frequently on routine chest radio-

graphs of women of reproductive age. Also, with the in-

creasing use of laparoscopic techniques during surgical

procedures of the abdomen, it is interesting that incidental

perioperative pneumothorax is not a more frequent compli-

cation of a surgically induced pneumoperitoneum.16 Recur-

rent catamenial pneumothorax also has been reported after

hysterectomy.8,17

Many questions remain unanswered regarding the

pathogenesis of catamenial pneumothorax. Various mecha-

nisms may contribute in individual patients, and further

investigations are needed. Because of the rarity of thiscondition, no randomized or controlled trials are available

to guide the therapy for women with catamenial pneu-

mothorax. Therapeutic options consist of surgical explora-

tion with resection of endometrial deposits within the

pleural space and/or repair of diaphragmatic defects (if

found) with or without pleurodesis (mechanical or chemi-

cal). 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 diaphrag-

matic surface4 to close occult fenestrations and promoteadhesion 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.6 If diaphragmatic defects are

present, surgical repair combined with postoperative hor-

monal suppression has resulted in excellent outcome.3,4

Whether this belt-and-suspenders approach is necessary

remains unclear.

FIGURE 2. Intraoperative 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.

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CONCLUSION

Catamenial pneumothorax should be suspected in any

menstruating woman presenting with recurrent spontane-

ous pneumothorax. Our patients clinical course and our

review of the literature indicate that catamenial pneu-mothorax is a heterogeneous syndrome. Considering the

high frequency of diaphragmatic abnormalities, early sur-

gical 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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pneumothorax due to endometriosis of the diaphragm.JAMA. 1958;168:2013-2014.

2. Lillington GA, Mitchell SP, Wood GA. Catamenial pneumothorax.JAMA. 1972;219:1328-1332.

3. Alifano M, Roth T, Broet SC, Schussler O, Magdeleinat P, Regnard JF.Catamenial pneumothorax: a prospective study. Chest. 2003;124:1004-1008.

4. Bagan P, Le Pimpec Barthes F, Assouad J, Souilamas R, Riquet M.Catamenial pneumothorax: retrospective study of surgical treatment. AnnThorac Surg. 2003;75:378-381.

5. Cowl CT, Dunn WF, Deschamps C. Visualization of diaphragmaticfenestration associated with catamenial pneumothorax.Ann Thorac Surg. 1999;68:1413-1414.

6. Joseph J, Sahn SA. Thoracic endometriosis syndrome: new observationsfrom an analysis of 110 cases.Am J Med. 1996;100:164-170.

7. Nakamura H, Konishiike J, Sugamura A, Takeno Y. Epidemiology ofspontaneous pneumothorax in women. Chest. 1986;89:378-382.

8. Shearin RPN, Hepper NGG, Payne WS. Recurrent spontaneous pneu-mothorax concurrent with menses.Mayo Clin Proc. 1974;49:98-101.

9. Van Schil PE, Vercauteren SR, Vermeire PA, Nackaerts YH, Van MarckEA. Catamenial pneumothorax caused by thoracic endometriosis.Ann ThoracSurg. 1996;62:585-586.

10. Rossi NP, Goplerud CP. Recurrent catamenial pneumothorax. ArchSurg. 1974;109:173-176.

11. Dattola RK, Toffle RC, Lewis MJ. Catamenial pneumothorax: a casereport.J Reprod Med. 1990;35:734-736.

12. Kirschner PA. Porous diaphragm syndromes. Chest Surg Clin N Am. 1998;8:449-472.

13. Majzlin G, Stevens FL. Meigs syndrome: case report and review ofliterature.J Int Coll Surg. 1964;42:625-630.

14. Lieberman FL, Hidemura R, Peters RL, Reynolds TB. Pathogenesis andtreatment of hydrothorax complicating cirrhosis with ascites.Ann Intern Med.1966;64:341-351.

15. Roth T, Alifano M, Schussler O, Magdaleinat P, Regnard JF. Catamenialpneumothorax: chest x-ray sign and thoracoscopic treatment. Ann Thorac

Surg. 2002;74:563-565.16. Fonseca P. Catamenial pneumothorax: a multifactorial etiology. J

Thorac Cardiovasc Surg. 1998;116:872-873.17. Lee CY, Di Loreto PC, Beaudoin J. Catamenial pneumothorax. Obstet

Gynecol. 1974;44:407-411.

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