{"paper_id":"ccd41e4d-0519-4ce1-b0a8-9d19d6493dc2","body_text":"e120\nSingapore Med J 2015; 56(7): e120-e123 \ndoi: 10.11622/smedj.2015115Case Report\nINTRODUCTION\nThoracic endometriosis (TE) is an uncommon disorder affecting \nwomen of childbearing age. Patients have a mean age of \n35 (range 15–54) years at presentation. (1,2) TE is characterised \nby the presence of functioning, morphologically normal \nendometrial tissues in the pleura, diaphragm, lung parenchyma \nand/or airways. (1-4) Depending on the affected sites, patients \nwith TE typically present with recurrent clinical symptoms \noccurring between 24 hours before and 48–72 hours after the \nonset of menstruation. Typical clinical manifestations consist of \ncatamenial pneumothorax (CP), catamenial haemoptysis (CH), \ncatamenial haemothorax (CHt) and pulmonary nodules. All of \nthese preferentially involve the right hemithorax.(1-6) CP is the most \ncommon clinical manifestation, accounting for approximately \n70%–80% of TE cases. CH and CHt individually account for \n7%–14% of TE.(1-4) CP coinciding with CH is extremely rare.(7-10) \nOnly two cases regarding bilateral CP and CH have been reported \nin the English literature.(7,9)\nHerein, we report clinical and computed tomo graphy (CT) \nfindings of two cases, in which one woman presented with \nCH alone and another woman presented with bilateral CP \ncoinciding with CH, a rare manifestation of TE. The dynamic \nchanges demonstrated on thin-section chest CT performed \nduring and after menses led to accurate localisation and \npresumptive diagnosis of TE in both patients. The role of \nimaging studies in TE is discussed, with an emphasis on the \nappropriate timing and scanning technique of chest CT in \nwomen presenting with CH, potential mechanisms, treatment \nand patient outcomes.\nCASE REPORTS\nCase 1\nIn 2001, a 38-year-old woman presented to our hospital with \na history of recurrent haemoptysis occurring every menstrual \ncycle for five months. Haemoptysis would develop within \nthe first 48 hours of menstruation, last for two days and \nsubside spontaneously. She had a history of two Caesarean \nsections. The second was performed seven years before the \nhaemoptysis episodes. She denied the presence of chest pain, \nfever or other constitutional symptoms. Except for mild anaemia \nfrom haemoglobin E trait, her physical and gynaecological \nexaminations and laboratory tests were normal. Chest \nradiography revealed no abnormality.\nIn 2002, the patient’s initial chest CT performed during the \nintermenstrual period revealed a single 2-mm thin-walled cystic \nlesion in the lateral basal segment of the right lower lobe (Fig. 1a). \nNo other abnormality was found. Follow-up CT obtained during \nthe next menses revealed newly developed ground-glass opacity \n(GGO) around the pre-existing cyst ( Fig. 1b). Bronchoscopy \nperformed within 48 hours following the patient’s subsequent \nepisode of haemoptysis showed no abnormality. Evidence of \nold haemorrhage was found on histopathologic examination of \nthe transbronchial biopsy specimen ( Fig. 2). Results of staining \nfor acid-fast bacilli and a culture of bronchoalveolar lavage fluid \nwere negative.\nThe presumptive diagnosis of TE with CH was made. The \npatient was treated with danazol 400 mg/day and her symptoms \nwere controlled after two months. Danazol was discontinued after \n6 months and CH recurred shortly thereafter. Danazol was restarted \nand discontinued after 16 months, with no recurrence of CH.\nThoracic endometriosis with catamenial haemoptysis \nand pneumothorax: computed tomography findings and \nlong-term follow-up after danazol treatment\nThitiporn Suwatanapongched1, MD, Viboon Boonsarngsuk2, MD, Naparat Amornputtisathaporn2, MD, \nPaisan Leelachaikul3, MD\n ABSTRACT Thoracic endometriosis (TE) is an uncommon disorder affecting women of childbearing age. We herein report \nclinical and thin-section computed tomography (CT) findings of two cases, in which one woman presented with catamenial \nhaemoptysis (CH) alone and another woman presented with bilateral catamenial pneumothoraces (CP) coinciding with \nCH, a rare manifestation of TE. The dynamic changes demonstrated on thin-section chest CT performed during and after \nmenses led to accurate localisation and presumptive diagnosis of TE in both patients. Following danazol treatment, the \npatient with CH alone had a complete cure, while the patient with CP and CH had an incomplete cure and required long-\nterm danazol treatment. We discuss the role of imaging studies in TE, with an emphasis on the appropriate timing and \nscanning technique of chest CT in women presenting with CH, potential mechanisms, treatment and patient outcomes.\nKeywords: catamenial haemoptysis, catamenial pneumothorax, pleural endometriosis, pulmonary endometriosis, thoracic endometriosis\n1Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, 2Division of Pulmonary and Critical Care, Department of Medicine, 3Department of \nPathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand\nCorrespondence: Dr Thitiporn Suwatanapongched, Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine, Ramathibodi \nHospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok 10400, Thailand. ratrspoom@yahoo.com\n\nCase Report\ne121\nCase 2\nIn 2002, a 25-year-old female non-smoker  presented with two \nepisodes of large spontaneous left pneumothorax. She had no \nprior history of obstetric-gynaecologic procedures nor pregnancy. \nBesides symptoms and signs related to pneumothorax, a physical \nexamination and laboratory investigations were unremarkable. \nFollowing left surgical pleurodesis with ligation of the left lung \nblebs, the patient had recurrent bilateral pneumothoraces during \nmenses. She was treated with chest tube drainage and subsequent \nright talc pleurodesis.\nIn 2003, she was referred for CT on her second day of \nmenstruation. During the examination, she coughed up \napproximately 1–2 tablespoons of blood. CT revealed multiple \n1–3-mm thin-walled cystic lesions and multifocal areas of nodular \nGGO ( Fig. 3a). There were no other CT findings to suggest \nemphysema, bronchiectasis, infection, malignancy or vascular \nabnormality. Therefore, the presumptive diagnosis of CP and \nCH secondary to TE was established. Danazol of 200 mg/day \nwas administered. During the first ten months of treatment, she \nhad two recurrent episodes of CP and CH, which ceased after \nthat period of time. After four years of treatment, danazol was \ndiscontinued and she became pregnant. During pregnancy and \ntwo years thereafter, she remained asymptomatic.\nIn 2010, non-massive CH recurred. Follow-up CT performed \nduring the patient’s menses (Fig. 3b) revealed an increase in the \nextent of the multiple ill-defined GGOs and a slight increase in \nthe size and number of the multiple small cystic lesions in both \nlungs. The GGOs resolved after menses ( Fig. 3c). Danazol was \nrestarted and subsequently discontinued in 2012 upon her desire \nfor a second pregnancy. Due to the relapse of CH after pregnancy, \nthe patient was placed on long-term treatment with danazol.\nDISCUSSION\nSince haemoptysis and pneumothorax are common clinical \nproblems and can be caused by various chest diseases, the \ndiagnosis of TE is often delayed, especially when the temporal \nrelationship between recurrent symptoms and menstruation is \nnot recognised. Similar to the present cases, the time interval \nbetween the onset of initial symptoms and diagnosis in most cases \nranges from 8–19 months.(1,3,4) As histopathologic verification of \nendometrial tissues is not always feasible, even on surgically \nresected specimens,(3,4,7,8) imaging studies play an important role \nin the detection, localisation and diagnosis of TE as well as the \nexclusion of other diseases.\nChest radio graph y often reveals normal or nonspecific \nfindings, which include pneumothorax, pleural effusion, \nhydropneumothorax and nodular lesions. (2,3) Unless there is \nendobronchial involvement, bronchoscopic findings in patients \nwith CH are usually negative.(5,7) Occasionally, active bleeding, \nhyperaemia or mucosal lesions are observed.(1-6,8)\nAs shown in a previous study, (11) the utility of transthoracic \nultrasono graphy is limited to peripheral pulmonary lesions. \nPulmonary and bronchial angio graphy have little value in \nestablishing the diagnosis. (2) Magnetic resonance (MR) imaging \nof the chest has been shown to be more accurate than CT in the \ndiagnosis of CP and CHt. MR imaging typically demonstrates \na hyperintense lesion on fat-suppressed T1- and T2-weighted \nimages at the pleura or diaphragm, signifying haemorrhage \ncoinciding with menses. (4,12) MR imaging in both the axial and \ncoronal planes should be performed during breath-holding. \nThe sagittal plane may be acquired in an equivocal case. The \nadministration of gadolinium is generally not required.(4)\nAs MR imaging lacks spatial resolution, CT is considered the \nfirst-line imaging modality for detecting and diagnosing TE in \nwomen presenting with CH. (4,6) Various CT findings, including \nGGO, pulmonary nodules/masses, thin-walled cavities, cysts \nor bullae, and consolidation have been described.(2-10) The most \ncommon CT finding is GGO, either with or without associated \npulmonary nodules or cysts, mimicking the so-called CT halo \nsign in other clinical entities. (13) An unusual presentation with \nthe so-called reversed halo sign can also occur. (14) Despite \nbilateral pulmonary involvement of TE in autopsy data,(2) a single \npulmonary lesion is usually demonstrated on CT.(5,6) Multiple and \nbilateral pulmonary lesions are rare.(7-10,13,15) They can, however, \nbe associated with CH and CP, as in the case of our second \npatient.(7-10)\n1a 1b\nFig. 1 Case 1. A 38-year-old woman presented with catamenial haemoptysis. \n(a) Axial CT image with 1-mm slice thickness and lung-window display, \ntaken during the intermenstrual period, shows a single 2-mm thin-walled \ncystic lesion (arrow) in the lateral basal segment of the right lower lobe. \n(b) Axial CT image taken during menses shows newly developed ground-\nglass opacity around the pre-existing cystic lesion (arrow).\nFig. 2 Case 1. Photomicrograph of a transbronchial biopsy specimen \nfrom the right lower lobe shows fibrin fragments with histiocytes \ncontaining haemosiderin (arrows) and some scattered red blood cells. \nNo endometrial tissue is present (Haematoxylin & eosin, × 40).\n\nCase Report\ne122\nAs CT findings are nonspecific, the key features of TE are \ntemporal changes in the size and morphological appearance \nof the lesions over the menstrual cycle. (2,4-6,8,13) As shown in \nprevious studies(6,7,9,13) and the present case report, these temporal \nchanges suggest that most of the parenchymal abnormalities \ndemonstrated on CT should represent various stages of pulmonary \nhaemorrhage found in histopathologic specimens. The GGO \ncorresponded to parenchymal haemorrhage partially filling alveoli, \nwhile pulmonary nodules or consolidations corresponded to \norganising pneumonia with haemosiderin-laden macrophages \nor haemorrhage completely filling alveoli.(2,4-6,8,13) The thin-walled \ncavities, cysts or bullae represented either partially or completely \nemptied spaces of previous haematoma. Occasionally, endometrial \nimplants were found. Therefore, chest CT should be performed \nduring the first two days of menstruation to discover pulmonary \nhaemorrhage, and with volumetric acquisition technique and thin \n(1-2 mm) sections, because the lesions can be very small (as in \nthe present cases). Repeat CT through the suspected region after \nmenses is also warranted to demonstrate interval disappearance \nor reduction of the parenchymal abnormalities. (5,6,8,13) As shown \nin Case 2, new emerging lesions can be observed. (9) To avoid \nexcessive radiation exposure in young women, CT should be \nperformed using a low-tube current adjusted to the patient’s body \nsize, without intravenous contrast administration, and limited to \nthe region of interest on follow-up.(4)\nThe pathogenesis of TE remains deba table. There are three \nproposed theories, including: (a) celomic pleural metaplasia \nin utero ; (b) lymphatic or haematogenous embolisation of \nendometrial tissues following an obstetric or gynaecologic \nprocedure; and (c) retrograde menstruation through the fallopian \ntubes with subsequent transperitoneal-transdiaphragmatic migration \nvia a diaphragmatic defect into the pleural cavity. However, none \nof them can explain all clinical manifestations.(1,3,4) The first theory \ncannot explain the development of pulmonary endometrial \nimplants and right-sided preference. The second theory can \nexplain pulmonary involvement and the occurrence of bilateral \nor left-sided TE, but not the predominant right-sided pleural \ndisease, which can be explained by the third theory. Finally, the \nlack of a history of prior obstetric-gynaecologic surgery or pelvic \nendometriosis in approximately two-thirds of patients (as in the case \nof our second patient),(1,3) and the recurrence of pneumothorax after \nclosure of diaphragmatic defects or hysterectomy in patients goes \nagainst the third theory. Some authors suggest that the apparent \ndiaphragmatic defect is not a cause of TE, but actually represents \na hole resulting from repeated proliferation and shedding of \ndiaphragmatic endometrial implants in patients with TE.(1,4)\nRegardless of the pathogenesis of TE, CH and CHt could \nresult from bleeding of the ectopic endometrial implants during \nmenses. CP might be due to spontaneous rupture of blebs, \ntransdiaphragmatic passage of intraperitoneal air, air leakage after \nsloughing of pleural endometrial implants, or alveolar rupture \nsecondary to prostaglandin-induced bronchiolar obstruction in \nthe bronchiolar endometrial implant.(2,4)\nAt present, there is no consensus regarding the standard \ntreatment for TE. Although spontaneous regression can occur, \nmedical therapy with gonadotropin-releasing hormone (GnRH) \nanalogue, oral contraceptives or danazol are commonly used \nbecause of good treatment response in approximately half of \nall patients.(2,5,6) Nevertheless, hormonal therapy can be costly. \nIn addition, side effects, treatment failure and recurrence after \ncompletion of hormonal treatment can occur, particularly \nin patients with CP. (2,8,9) The optimal dosage and duration of \nhormonal treatment remain undetermined.(2)\nSurgical resection via video-assisted thoracoscopic surgery \nor standard thoracotomy should be considered in the following \n3a\n3b\n3c\nFig. 3  Case 2. A 25-year-old woman presented with bilateral \ncatamenial pneumothorax and catamenial haemoptysis.  (a) Axial \nCT image with 1-mm slice thickness and lung-window display, taken \nduring the patient’s menses in 2003, shows left pneumothorax with \nmultiple areas of ill-defined ground-glass opacities and 1–3 -mm \nthin-walled cystic lesions scattered in both lungs. There is minimal \nirregular pleural thickening along the anterior aspect of the right \nmiddle lobe, as the changes followed previous surgical pleurodesis. \n(b) Follow-up axial CT image with lung-window display taken during \nmenses in 2010 shows an increase in the extent of the multifocal \nground-glass opacities and a slight increase in the size and number \nof the small cystic lesions in both lungs. (c) Follow-up axial CT image \nwith lung-window display obtained after menses in 2010 shows \ncomplete disappearance of the ground-glass opacities, suggesting \nresolved pulmonary haemorrhage, with persistence of the multiple \nsmall, bilateral lung cysts.\n\nCase Report\ne123\ncircumstances: (a) a single TE lesion is identified; (b) the patient \nwishes to become pregnant; (c) the patient cannot tolerate the side \neffects of hormonal therapy; (d) medication fails; or (e) symptoms \nrecur after hormonal cessation.(2,15) In complicated cases, bilateral \noophorectomies or even lung transplantation may be required.(8)\nCurative treatment with danazol was achieved in the first \npatient who had CH and a single lesion. The second patient had \nan incomplete cure despite being treated with low-dose danazol \nfor several years. This might be attributed to the more extensive \ninvolvement of bilateral hemithoraces. Due to financial limitations \nand the patient’s young age, alternative treatment with GnRH \nanalogue and bilateral oophorectomies were not considered.\nIn conclusion, we have demonstrated the essential role \nof CT in the detection and localisation of lung parenchymal \ninvolvement in two women presenting with CH. The dynamic \nchanges of parenchymal abnormalities demonstrated on low-dose \nthin-section CT performed during and after menses are the key \nfeatures leading to the presumptive diagnosis of TE.\nACKNOWLEDGEMENTS\nWe thank David S Gierada, MD, Anya Khanthavit, PhD, and \nRyratana Rangsitpol, MA, for their constructive suggestions and \nEnglish editing.\nREFERENCES\n1. Channabasavaiah AD, Joseph JV. Thoracic endometriosis: revisiting the \nassociation between clinical presentation and thoracic pathology based \non thoracoscopic findings in 110 patients. Medicine (Baltimore) 2010; \n89:183-8.\n2. Alifano M, Trisolini R, Cancellieri A, Regnard JF. 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