Catamenial pneumothorax: a rare cause of chest pain in the emergency

department

月經性氣胸：在急診胸痛罕見的原因

H Hasman, O Cinar, E Cevik, L Jay

Catamenial pneumothorax (CP) is defined as recurrent spontaneous pneumothorax occurring within 72hours before or after onset of menstruation. The aetiology of this syndrome is unknown, although manytheories have been proposed to explain it. A 43-year-old female presented to our emergency room with acomplaint of left sided chest pain. She had no dyspnoea or haemoptysis during the admission. She had ahistory of pelvic endometriosis without any complaints for three years. Pneumothorax or pneumonia wasnot detected on chest X-ray. Blood tests and electrocardiogram findings did not support any cardiac orinfectious cause. Computed tomography showed a pneumothorax on the mediastinal side of left hemithorax.The patient was referred to a thoracic surgeon. A thoracoscopy for histological confirmation was performedand the diagnosis of CP was made with a histological intervention. (Hong Kong j.emerg.med. 2012;19:353-356)

月經性氣胸是指月經來臨前或後72小時內發生的經常性自發性氣胸。本綜合徵的病因是未知的，雖然已經提出了許多理論來解釋它。一個43歲的女性，因左側胸痛來到我們的急診室。入院她沒有呼吸困難或咯血。她曾患有盆腔子宮內膜異位症，三年沒有任何症狀。X射線檢測並未發現氣胸或肺炎。血液化驗和心電圖檢查結果不支援任何心臟或感染的原因。電腦斷層掃描顯示左側縱隔氣胸。病人被轉介給胸外

科醫生。進行胸腔鏡的組織和細胞病理診斷。

Keywords: Endometriosis, female, hemothorax, menstruation, recurrence

關鍵詞：子宮內膜異位症、女性、血胸、月經、復發

Correspondence to:Hakan Hasman, MD

Gulhane Military Faculty of Medicine, Emergency Department,General Tevfik Saglam Street, 06010, Etlik, Kecioren, Ankara,TurkeyEmail: hakan_has@yahoo.com

Orhan Cinar, MD

Erdem Cevik, MD

University of Utah, School of Medicine, Emergency Department,Utah, United States of AmericaLoni Jay, MD

Introduction

Catamenial pneumothorax (CP) is a recurrentcondition, closely related to the menstrual cycle andaffecting women of childbearing age.1-3 Since it is avery rare clinical entity, diagnosis is often delayed,and hence it requires astute clinical acumen on thepart of the emergency department healthcare team. Wepresented a case of a 43-year-old woman diagnosed tohave CP, and the difficulties to make such a diagnosisin setting of emergency department were discussed.

Hong Kong Journal of Emergency Medicine

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Case

A 43-year-old woman (gravida 2, para 2) presented toour emergency room with left-sided chest pain. Thepain occurred on the day before her attendance, andwas sharp and well localised in the cardiac region ofthe chest wall, with no correlation to respiration. Thepatient had no dyspnoea or haemoptysis. She had ahistory of pelvic endometriosis, but remainedasymptomatic for 3 years. The complaint of chest painonly correlated with her previous two menstruations.At presentation, she was on the third day of hermenstrual cycle. She reported that her chest pain wassharper than it had been in the previous months. Fouryears ago, she had taken goserelin acetate (a GnRHagonist) for one year. She has been taking trazodonefor major depression for two years. On presentation,her vital signs were the following: temperature 36.2ºC;arterial blood pressure 112/48 mmHg; pulse rate 104/min; respiratory rate 14/min; oxygen saturation 99%on room air. Pneumothorax or pneumonia was notdetected on chest X-ray (Figure 1). Blood tests andelectrocardiogram findings did not support any cardiacor infectious cause. Computed tomography showed apneumothorax on the mediastinal side of the left thorax(Figure 2). The patient was referred to a thoracic

surgeon. A thoracoscopy for histological confirmationwas performed and the diagnosis of CP was made witha histological intervention. With thoracoscopy guidedg ro s s e x amin a t i on , m u l t i p l e h a emo r r h ag i cdiscolourations were seen on the left lung. No lesionwas identified on the right lung. During thethoracoscopy, wedge biopsies of haemorrhagicdiscoloured lesions were collected for histologicalexamination. All tissue samples were submitted formicroscopic examination, which revealed abnormalformations of ectopic glandular columnar epitheliumsurrounded by spindled stromal cells within lungparenchyma. This histological finding suggestedectopic endometrial tissue in lung parenchyma.

Discussion

Catamenial pneumothorax is defined as recurrentspontaneous pneumothorax occurring within 72 hoursbefore or after onset of menstruation. The right side isaffected in the majority of cases (95%), and it is cyclicin nature.4,5 In a recent prospective study of womenreferred to a single centre for evaluation and treatmentof persistent or recurrent spontaneous pneumothorax(pneumothorax despite previous chest tube drainageor attempted surgical therapy), 25% had a temporalrelationship with onset of menses.6

Figure 1. Posteroanterior chest radiograph of the patient.

Figure 2. Computed tomography shows a pneumothorax on

the mediastinal side of the left hemithorax (white arrow).

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Hasman et al./Catamenial pneumothorax 355

Despite the fact that the clinical association betweenthe development of spontaneous pneumothorax andthe menstrual cycle has been well described, the causalmechanisms are still undetermined.7 Catamenialpneumothorax was regarded as the most frequentclinical manifestation of intrathoracic endometriosis,while concomitant pelvic endometriosis is seen in only61% of women with catamenial pneumothorax.4 Someretrospective analyses suggested that the prevalence ofCP was 1% to 5% among women with spontaneouspneumothorax during the cyclic period.8,9 Anotherstudy reported the occurrence of CP as much morefrequent, with approximately 25% of all spontaneouspneumothorax cases among women referred to surgery.6

The average age at presentation ranges from 32 to 37years in different published series with patient agesfrom 19-54 years.4,6,10

There have been several hypotheses for the pathogenesisof CP, including the following: (1) spontaneousrupture of blebs; (2) alveolar rupture caused byprostaglandin-induced bronchiolar constriction; (3)movement of endometrial implants to visceral pleurawith subsequent air leak; and (4) in the absence of thecervical mucus plug, passage of air from the genitaltract through congenital or acquired diaphragmaticdefects (i.e. due to sloughing of diaphragmaticendometrial foci).4,5 There could be a preferential flowof peritoneal fluids (including air) and endometrialtissue from the pelvis along the right paracolic gutterup to the subphrenic space. The liver covered by theright diaphragm functions as a "piston", while the softand compressible viscera of the left upper quadranthas no similar mechanism. This piston mechanism wasadopted for explaining the right side CP frequency.11

However, in our case the left side was affected.

The history and clinical presentation of the patient isimportant for the diagnosis of CP.12 The diagnosisshould be suspected when one simultaneouslyexperiences thoracic pain (90%), dyspnoea (31%) orhaemoptysis (7%) with the presence of pneumothorax(73%), haemothorax (14%) or pulmonary nodules(6%) at the time of menses. The diagnosis can beconfirmed with a histological intervention.12 In ourpatient, the symptoms occurred 24 to 48 hours after

the onset of each menstruation, with chest pain as thesole presenting feature. The chest pain was less severein her previous two menstruations.

A history of pelvic endometriosis could be useful fordiagnosis. In a study of 110 patients with thoracicendometriosis syndrome, pelvic endometriosis hadbeen documented in 61% of patients with catamenialpneumothorax, 67% of the patients with haemoptysisand all patients with haemothorax and lung nodules.4

A history of pelvic endometriosis was present in ourpatient.

Spirometrically controlled high-resolution computedtomography (HRCT) providing two or three-dimensional reconstructed images could visualise theabnormal pleural zones, especially when performedduring menses.13 Computed tomography of the patientshowed a pneumothorax on the mediastinal side of leftthorax. The images obtained from HRCT are notspecific for thoracic endometriosis, but can be usefulbefore the thoracoscopy. Histological evaluation oftissue samples provides a definite confirmation of thediagnosis. Pleural endometriosis, diaphragm defects,cysts and blebs were detected in the majority ofpatients during the thoracoscopy and thoracotomy,while there were no abnormalities in one quarter ofthe patients.4,14,15 The treatment of catamenialpneumothorax could not dis t inct from otherpneumothorax. Small asymptomatic or mild CPs(approximately more than 75% of all cases) could betreated in a conservative manner. Pleural drainageshould be applied for cases of serious and symptomaticpneumothorax.

Conclusion

The diagnosis of CP requires high clinical suspicionin patients with recurrent chest pain associated withmenstruation or menarche requires high clinicalsuspicion. Suspected patients should be evaluated forspontaneous pneumothorax in the emergencydepartment, and referred to a thoracic surgeon fordefinitive diagnosis and a complete spectrum oftreatment plans.

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References

1. Augoulea A, Lambrinoudaki I, Christodoulakos G.Thoracic endometriosis syndrome. Respiration 2008;75(1):113-9.

2. Maurer RR, Schall JA, Mendez FL Jr. Chronic recurringspontaneous pneumothorax due to endometriosis of thediaphragm. J Am Med Assoc 1958;168(15):2013-4.

3. Lillington GA, Mitchell SP, Wood GA. Catamenialpneumothorax. JAMA 1972;219(10):1328-32.

4. Joseph J, Sahn S. Thoracic endometriosis syndrome:new observations from an analysis of 110 cases. Am JMed 1996;100(2):164-70.

5. Carter EJ, Ettensohn DB. Catamenial pneumothorax.Chest1990;98(3):713-6.

6. Alifano M, Roth T, Broet SC, Schussler O, MagdeleinatP, Regnard JF. Catamenial pneumothorax: a prospectivestudy. Chest 2003;124(3):1004-8.

7. Peikert T, Gillespie DJ, Cassivi SD. CatamenialPneumothorax. Mayo Clin Proc 2005;80(5):677-80.

8. Nakamura H, Konishiike J, Sugamura A, Takeno Y.Epidemiology of spontaneous pneumothorax in women.Chest 1986;89(3):378-82.

9. Shearin RP, Hepper NG, Payne WS. Recurrentspontaneous pneumothorax concurrent with menses.Mayo Clin Proc 1974;49(2):98-101.

10. Bagan P, Le Pimpec Barthes F, Assouad J, SouilamasR, Riquet M. Catamenial pneumothorax: retrospectivestudy of surgical treatment. Ann Thorac Surg 2003;75(2):378-81.

11. Kirschner PA. Porous diaphragm syndromes. Chest SurgClin N Am 1998;8(2):449-72.

12. Devuea K, Coenyeb K, Verhaeghe W. A case ofcatamenia l pneumothorax caused by thorac icendometriosis. Eur J Emerg Med 2005,12(2):92-4.

13. Kalapura T, Okadigwe C, Fuchs Y, Veloudios A,Lombardo G. Spiral computerized tomography andvideo thoracoscopy in catamenial pneumothorax. AmJ Med Sci 2000;319(3):186-8.

14. Cowl CT, Dunn W, Descamps C. Visualisation ofdiaphragmatic fenestration associated with catamenialpneumothorax. Ann Thorac Surg 1999;68(4):1413-4.

15. Yamashita J, Iwasaki A, Kawahara K, Shiradusa T.Thoracoscopic approach to the diagnosis and treatmentof diaphragmatic disorders. Surg Laparosc Endosc 1996;6(6):485-8.

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