INTRODUCTION: Catamenial hemoptysis and pneumothorax are rare conditions that occur as part of the thoracic endometriosis syndrome (TES). Fewer than 30 cases of catamenial hemoptysis are described in the literature (1). Diagnosis of TES is often made on the basis of the clinical history of symptoms that recur with menses; a histopathologic diagnosis was only established in approximately one-third of reported cases (2). We report a case of a patient with cystic fibrosis (CF) and a history of catamenial pneumothoraces who presented with life-threatening catamenial hemoptysis.
CASE PRESENTATION: Over several months, a 32-year-old female with CF ([DELTA]F508, [DELTA]1507 phenotype) bad experienced several episodes of mild, streaky hemoptysis, and two episodes of spontaneous pnemnothorax, which coincided with the onset of menses. In September 2004, she was assessed following an episode of sudden massive hemoptysis (~500 cc), occurring on the second day of menses, during which she collapsed and required resuscitation. Prior to this event, she had felt relatively well, her baseline lung function had remained stable (FEV1 30-40% predicted), and there were no symptoms to suggest an antecedent infectious exacerbation. She was nonetheless treated with antibiotics and recovered. With the onset of her next menstrual cycle she experienced recurrent streaky hemoptysis, and subsequently underwent bronchial angiography and embolization of bilateral ectatic bronchial arteries. ACT scan obtained at the end of menses demonstrated the presence of pulmonary parenchymal lesions, consistent with hemorrhage, that resolved completely on a follow-up CT obtained midway through her menstrual cycle (Figure 1). Thoracic T2-weighted MRI, obtained at the end of menses, demonstrated hyperintense lesions (consistent with hemorrhage or endometrial tissue) localized to the left apical pleura, and pulmonary parenchymal lesions were also seen corresponding to those visualized on thoracic CT. Bronchoscopy, performed premenstrually, showed at least two friable purplish lesions in her segmental bronchi, which were no longer evident after menses. Bronchial lavage obtained from these areas did not show endometrial cells. Biopsies were not performed. Given the strong clinical suspicion of TES, she was started empirically on danazol and became amenorrheic. She experienced another episode of life-threatening hemoptysis approximately 3 months after starting danazol therapy, coincident with an infectious exacerbation of CF, and subsequently underwent double lung transplantation. Histological evaluation of her native lungs did not demonstrate the presence of endometrial tissue. Three months post-transplant, she has had no further episodes of hemoptysis.
DISCUSSIONS: Although a histopathologic confirmation was not obtained in this patient, the clinical history, as well as the dynamic radiographic and bronchoscopic appearance, strongly suggest a diagnosis of TES. In previous reports, the volume of blood expectorated in the setting of catamenial hemoptysis is usually less than 200 cc, and we propose that the massive nature of the hemoptysis experienced by this patient may be a consequence of the bronchial arterial collateral circulation that had developed due to underlying bronchiectasis. The optimal treatment of TES is uncertain, with surgical resection and ovarian suppression being the most widely reported (3); danazol is reported to be effective, although treatment failures are described (1). In tiffs case, transplantation would be expected to be curative, although the risk of recurrence of pulmonary endometriosis in the lung allograft is unknown.
CONCLUSION: Tills is the first report of catamenial hemoptysis and pneumothoraces occurring in a patient with CF. The clinical presentation is consistent with TES.
(1) Joseph J, Sahn SA. Thoracic endometriosis syndrome: New observations from an analysis of 110 cases. Am J Med 1996; 100:164-170
(2) Wood DJ, Krishna K, Stocks Pet al. Catamenial hemoptysis: a rare cause. Thorax 1993; 48:1048-1049
(3) Ziedalski TM, Sankaranarayanan V, Chitkara RJ. Thoracic endometriosis: A case report and literature review. J Thorac Cardiovasc Surg 2004; 127:1513-1514
DISCLOSURE: Chris Parker, None.
Chris M. Parker MD * Robert Nolan MD M. D. Lougheed MD Queen's University, Kingston, ON, Canada
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