{"paper_id":"c6f1ef45-9915-4114-97c4-5e0d92a14523","body_text":"INTRODUCTION\nEndometriosis is a common gynaecological \ncondition where endometrium-like tissue \nis located outside the uterine cavity, \npredominantly inside the pelvis, causing \nsymptoms such as dysmenorrhoea and \ndyspareunia. 1  However, extrapelvic \nendometriosis can also occur, often leading \nto delay in diagnosis that results in chronic \nsymptoms and progression of the disease \nwith possible lifelong consequences (for \nexample, infertility). 1–3  The current case \nreport illustrates a rare example of \nextrapelvic endometriosis and catamenial \npneumothorax (that is, pneumothorax \noccurring during the perimenstrual period). \nThe aim of this article is therefore to improve \nendometriosis awareness among GPs. The \nkey message from this case report is that \nGPs should be aware of endometriosis \nwhen women present with symptoms that \nfluctuate with their menstrual cycle.\nCASE REPORT\nA 34-year-old woman was referred by her \nGP because of coughing, painful breathing, \nand dyspnoea. \nA right-sided \npneumothorax  \nwas diagnosed \nand successfully \ndrained. After \n5 months, a second \npneumothorax  \noccurred, for which \na thoracoscopic \ntalc pleurodesis \nwas performed \nto obliterate the \npleural space to \nprevent further \nrecurrences. No \nabnormalities  \nwere seen during \nthoracoscopy or \nchest CT scan. \nDuring the follow-\nup visit at the \npulmonologist, the patient indicated that \nshe had noticed that the thoracic pain \nalways started after the beginning of her \nperiod. Moreover, retrospectively, the \nseries of pneumothoraxes started when the \npatient switched from an oral contraceptive \nto a levonorgestrel intrauterine device. A \ncatamenial pneumothorax was considered \nand the patient was referred to the \ngynaecologist. In the past, an oophorectomy \nhad been performed due to recurrent ovarian \ncysts, but no endometriosis was noted. \nNow, she reported minor cyclic abdominal \npain, without dyspareunia, dysmenorrhoea, \nor bladder or bowel dysfunction, and \nphysical examination revealed no \nsigns of endometriosis. Despite ovarian \nsuppression by a continuous progestin, the \npatient developed another pneumothorax \nand a video-assisted thoracoscopic surgery \n(VATS) was performed with a partial \npleurectomy. No abnormalities were \nseen during the procedure; moreover, no \nindications of endometriosis were spotted \n(for example, bullae or fenestrations in the \ndiaphragm). Pathological examination of \nDelay in diagnosis of endometriosis:\na case report of catamenial pneumothorax\nMarije G Hierink, Felix Poppelaars, Ellen R Klinkert, Hester van der Vaart \nand J Marinus van der Ploeg\nClinical Intelligence\n626  British Journal of General Practice, December 2019\nMG Hierink, MD; JM van der Ploeg, MD, PhD, \nDepartment of Obstetrics and Gynaecology, \nMartini Hospital, Groningen. F Poppelaars, \nMD, PhD, Department of Obstetrics and \nGynaecology, Martini Hospital, Groningen; \nDepartment of Internal Medicine, Division \nof Nephrology, University of Groningen, \nUniversity Medical Center Groningen, \nGroningen. ER Klinkert, MD, PhD, Department \nof Obstetrics and Gynaecology, University \nof Groningen, University Medical Center \nGroningen, Groningen. H van der Vaart, MD, \nPhD, Department of Pulmonology, University \nof Groningen, University Medical Center \nGroningen, Groningen. \nAddress for correspondence\nJM van der Ploeg, Martini Hospital, Department \nof Obstetrics and Gynaecology, PO Box 30.033, \n9700 RM, Groningen, the Netherlands.\nEmail: PloegJM@mzh.nl\nSubmitted: 19 April 2019; Editor’s response:  \n28 May 2019; final acceptance: 21 June 2019.\n©British Journal of General Practice 2019; \n69: 626–627.\nDOI: https://doi.org/10.3399/bjgp19X707045\nFigure 1. Endometriosis with extensive scarring in the pelvis (A); endometriosis \nspots (B); fenestration (C); and scarring on the diaphragm (D).\n\nBritish Journal of General Practice, December 2019  627\nthe pleural tubes demonstrated no signs of \nendometriosis. After the VATS, the patients \nsuffered from three more recurrences \nof a pneumothorax. After the sixth right-\nsided pneumothorax, a laparoscopy was \nperformed to search for endometriosis and/\nor fenestrations in the diaphragm. During \nlaparoscopy, peritoneal endometriosis \nand endometriotic lesions with extensive \nscarring and fenestrations were seen at \nthe diaphragm (Figure 1). Three years later, \na partial pleurectomy with pleurodesis \n(that is, obliteration of the pleural space) \nwas finally performed because continuous \nhormonal suppression was unsuccessful. \nAt present, there has been no recurrence of \na pneumothorax; nevertheless, once every \nfew months the patient still has a pulling \nand painful sensation in her thorax.\nDISCUSSION\nEndometriosis can affect various organ \nsystems and symptoms depending on the \nlocation, resulting in complaints of the \ngynaecological, urinary, gastrointestinal, \nor pulmonary tract (Box 1). 3,4  A common \nmisconception among GPs is that \nendometriosis is rare in young women; \nhowever, endometriosis can also occur \nin teenagers and adolescents. 5  Principal \nsymptoms include dysmenorrhoea, chronic \n(menstrual or continuous) pelvic pain, \ndyspareunia, and abnormal bleeding. 1  \nEndometriosis located in the urinary tract \ncan lead to symptoms of frequency, urgency, \nhaematuria, and painful micturition. 6,7  \nSymptoms of bowel involvement include \ndyschezia, constipation, diarrhoea, \nor rectal bleeding, and can be wrongly \ndiagnosed as irritable bowel syndrome \n(IBS). 2,8  Catamenial complaints of chest \npain, pneumothorax, haemothorax, and \nhaemoptysis, can take place in women with \nthoracic endometriosis. 3,9,10  Endometriosis \ncan also occur in the abdominal wall and \nin scar tissue after a caesarean section \nor laparoscopy. 11  The clinical presentation \nis diverse, and symptoms that are \nexacerbated during the menstrual cycle \ncould be a manifestation of endometriosis. \nThe importance of the GP in the diagnosis \nof endometriosis is emphasised by the fact \nthat women with endometriosis consult \nprimary care more frequently, offering a \nwindow of opportunity. 12\nPhysical examination might reveal \nabnormalities (for example, pain, adnexal \nmasses, or vaginal nodules), but normal \nfindings do not rule out endometriosis. 12  \nPatients with suspected endometriosis \nshould be referred to a gynaecologist \nfor examination with ultrasound, MRI, \nor laparoscopy, because histological \nexamination is required for the definitive \ndiagnosis. 3,13  In cases where symptoms \nare difficult to treat, a combination of \nhormonal and surgical resection seems to \ngive the best outcome. 14  In case of doubt, \nempirical management by hormonal \nsuppression therapy with the combined oral \ncontraceptive pill or progestins only can be \nstarted by the GP. 1  Reduced symptoms \nafter treatment support the presumptive \ndiagnosis of endometriosis. 5\nCONCLUSION\nIn conclusion, extrapelvic endometriosis is \na rare presentation of endometriosis that \ndoes not have a standardised treatment \nprotocol. In women who develop recurrent \ncomplaints with a catamenial character \nin the fertile age, endometriosis should \nalways be considered. Better recognition \nwill ultimately lead to more insight into the \nunderlying pathology and treatment options. \nThe importance of timely recognition \nand treatment is illustrated by the case \npresented here, with a prolonged and \nburdensome process that is unfortunately \nnot uncommon for these patients.\nProvenance\nFreely submitted; externally peer reviewed.\nDiscuss this article\nContribute and read comments about this \narticle: bjgp.org/letters\nREFERENCES\n1. Giudice LC, Kao LC. Endometriosis. Lancet  \n2004; 364(9447): 1789–1799. \n2. Burton C, Iversen L, Bhattacharya S, et al . \nPointers to earlier diagnosis of endometriosis: \na nested case-control study using primary care \nelectronic health records. Br J Gen Pract  2017; \nDOI: https://doi.org/10.3399/bjgp17X693497. \n3. Machairiotis N, Stylianaki A, Dryllis G, et al . \nExtrapelvic endometriosis: a rare entity or an \nunder diagnosed condition? Diagn Pathol  2013; \n8: 194. \n4. Davis AC, Goldberg JM. Extrapelvic \nendometriosis. Semin Reprod Med  2017; 35(1):  \n98–101.\n5. Dunselman GA, Vermeulen N, Becker C, et \nal . ESHRE guideline: management of women \nwith endometriosis. Hum Reprod  2014; 29(3):  \n400–412. \n6. Gabriel B, Nassif J, Trompoukis P, et al . \nPrevalence and management of urinary \ntract endometriosis: a clinical case series. \nUrology  2011; 78(6): 1269–1274. http://dx.doi.\norg/10.1016/j.urology.2011.07.1403.\n7. Leone Roberti Maggiore U, Ferrero S, Candiani \nM, et al . Bladder endometriosis: a systematic \nreview of pathogenesis, diagnosis, treatment, \nimpact on fertility, and risk of malignant \ntransformation. Eur Urol  2017; 71(5): 790–807. \n8. Nezhat C, Li A, Falik R, et al . Bowel \nendometriosis diagnosis and management. \nAm J Obstet Gynecol  2018; 218(6): 549–562. \nhttps://doi.org/10.1016/j.ajog.2017.09.023.\n9. Marshall MB, Ahmed Z, Kucharczuk JC, et al . \nCatamenial pneumothorax: optimal hormonal \nand surgical management. Eur J Cardiothorac \nSurg  2005; 27(4): 662–666.\n10. Lillington GA, Mitchell SP, Wood GA. \nCatamenial pneumothorax. JAMA  1972; \n219(10): 1328–1332. \n11. Andolf E, Thorsell M, Källén K. Caesarean \nsection and risk for endometriosis: a \nprospective cohort study of Swedish registries. \nBJOG  2013; 120(9): 1061–1065.\n12. Johnston JL, Reid H, Hunter D. Diagnosing \nendometriosis in primary care: clinical \nupdate. Br J Gen Pract  2015; DOI: https://doi.\norg/10.3399/bjgp15X683665.\n13. Pugsley Z, Ballard K. Management of \nendometriosis in general practice: the pathway \nto diagnosis. Br J Gen Pract  2007; 57(539):  \n470–476.\n14. Bulun SE. Endometriosis. N Engl J Med  2009; \n360(3): 268–279. \nBox 1. Possible location of endometrioses for women presenting \nwith cyclic symptoms\nGynaecological tract Menarche, abnormal bleeding, infertility, pregnancies, dysmenorrhoea, \ndyspareunia, pelvic pain\nPulmonary tract Chest pain, pneumothorax, haemothorax, haemoptysis\nGastrointestinal tract Dyschezia, constipation, diarrhoea, rectal bleeding\nUrinary tract Frequency, urgency, haematuria, dysuria","source_license":"CC0","license_restricted":false}