International Journal of Health Sciences and Research Vol.11; Issue: 1; January 2021 Website: www.ijhsr.org Case Report ISSN: 2249-9571

Massive Catamenial : Rare Case of Thoracic Syndrome

Lamya Zaher Al Aamri1, Nasser Alawaid2, Adil Al Kindi3, Akhwand Shakeel Ahmed4, G.Rajasekharan Pillal5

1Internal Medicine Specialist, Sultan Qaboos Hospital, Salalah, Oman. 2Consultant Pulmonologist, Head of Department of Medicine, Sultan Qaboos Hospital, Salalah. 3Consultant Cardiothoracic Surgeon of Sultan Qaboos University, Muscat. 4Senior Specialist Medicine, MRCP, Sultan Qaboos Hospital, Salalah 5Senior Consultant, Histopathologist, Sultan Qaboos Hospital, Salalah.

Corresponding Author: Lamya Zaher Al Aamri

ABSTRACT

Spontaneous hemothorax is only rarely due to thoracic endometriosis (TE). TE is presence of ectopic endometrial tissue in thoracic. It is rare phenomena seen in women of childbearing age with predominantly right-sided hemothorax and temporal relationship to menses. We report a thirty -seven -year-old Omani lady who was admitted in Sultan Qaboos hospital, Salalah with Spontaneous hemothorax and dysmenorrhea. She had past history of primary infertility due to endometriosis and she lost follow up. Therefore, TE was suspected and was introduced hormone therapy after chest drain inserted. She had recurrent hemothorax after discharge subsequently, she underwent VATS which help visualization of endometrial lesions with pleurectomy of the involved areas and mechanical decortication and pleurodesis using scratch pad were done. Histopathology confirms diagnosis of TE. She continued hormonal therapy and repeated image showed no recurrence.

Keywords: Spontaneous hemothorax; Thoracic endometriosis; thoracic endometriosis syndrome; Video-assisted thoracoscopic surgery.

INTRODUCTION CASE REPORT Hemothorax is collection of blood A 37 years old nulliparous female with hematocrit >50% in the space between presented to emergency department with the visceral and parietal pleura. Although dysmenorrhea and shortness of breath. Her traumatic or iatrogenic in a vast majority of dyspnea started one month before while cases, it may rarely be spontaneous. menstruating and was worsening. She also Spontaneous hemothorax (SH) is most complained of abdominal distension, nausea commonly due to but may be and poor appetite. She denied any cough, related to malignancy, vascular causes or , or fever. Two years coagulopathy besides various other ago, she was investigated for primary etiologies.1 Thoracic endometriosis (TE) is a infertility and was found to have severe rare cause of SH and should be highly pelvic endometriosis. Surgical treatment suspected in females of reproductive age, was advised, but she declined, and was lost especially with pelvic endometriosis or from follow up. history of pelvic surgery. 1 Such etiology for On clinical examination, her vitals TE is termed Catamenial Hemothorax. were stable, whole right side of chest was We here report a case of a 37 years dull on percussion with diminished breath old female with massive catamenial sounds. Abdomen revealed signs of mild hemothorax due to TE. ascites.

International Journal of Health Sciences and Research (www.ijhsr.org) 209 Vol.11; Issue: 1; January 2021 Lamya Zaher Al Aamri et.al. Massive catamenial hemothorax: rare case of thoracic endometriosis syndrome.

The chest radiograph (CXR) pleural tap was carried out to relieve the demonstrated a large right-sided pleural distress and one liter of dark hemorrhagic effusion with shift of mediastinum to left fluid was removed. Similar fluid was (Figure 1). Following chest ultrasound, obtained on abdominal paracentesis.

A: B: Figure1: Massive right sided hemothorax on imaging. (A)Chest radiograph shows complete opacification of right side hemithorax (arrows) and shifting of heart to left side. (B) CT chest showing massive right sided effusion (arrow).

malignancy or infection or acid-fast bacilli (AFB). Serum CA-125 was markedly high (102.2 µ/mL; normal < 35.2 µ/mL). An MRI was carried out to further explore the pelvic mass which demonstrated extensive deep pelvic endometriosis, pelvic adhesions and bilateral adnexal cystic areas engulfing both ovaries. Follow-up CXR showed resolving hemothorax and chest drain was removed. Because pleura effusion cytology analysis and abdomen image, thoracic endometriosis was highly suspected. Therefore, started hormone suppression therapy, levonorgestrel 0.15 mg Figure 2: Grossly hemorrhagic pleural fluid aspirated through the chest drain. and ethinylestradiol 0.03 mg combination pill once daily. Later she developed mild Further evaluation with CT scan of vaginal bleeding and was started on the chest, abdomen and pelvis confirmed Triptorelin injection 3.75mg (Gonadotropin- massive right-sided (Figure releasing hormone agonist) once per month. 1) and gross ascites as well as a complex On a follow-up visit in chest clinic cystic mass in pelvis. Chest drain was after 2 months, her chest image showed re- inserted on right, and 3.5 liters of grossly accumulation of large right-sided pleural hemorrhagic fluid was removed over 48 effusion with mild mediastinal shift to left. hours (Figure 2). Repeat diagnostic pleural aspirate showed The pleural aspirate was grossly hemorrhagic fluid, no malignant cells or hemorrhagic, exudative, negative for acid -fast bacilli and was negative for mycobacterium PCR and sterile on culture. bacterial culture. Cytological examination of the pleural As such, it was decided that she has effusion and ascitic fluid showed failed medical therapy and warranted a hemosiderin-laden macrophage and diagnostic and therapeutic intervention. She mesothelial cells, no evidence of

International Journal of Health Sciences and Research (www.ijhsr.org) 210 Vol.11; Issue: 1; January 2021 Lamya Zaher Al Aamri et.al. Massive catamenial hemothorax: rare case of thoracic endometriosis syndrome. underwent video-assisted thoracoscopy (VATS). There were reddish black deposits on the parietal pleura, in the mid and lower zones, consistent with endometriosis and 1.7 L of tea colored pleural fluid were seen (Figure 3). There was no diaphragmatic defect or fenestrations seen. Pleurectomy of the involved areas and mechanical decortication and pleurodesis using scratch pad were done.

Figure 3: Thoracic cavity visible on VATs showed reddish- black lesions of ectopic endometrial tissue in pleura (arrows).

A: B: Figure 4: Pleura tissue biopsy :(A) Pleural tissue with mesothelial lining on one surface with congestion, hemorrhage and deposition of hemosiderin laden macrophages. Two foci of decidual tissue are seen (arrows). (H&E x 100). (B) Immunohistochemistry. Decidual cells show nuclear positivity for progesterone receptors (arrows). The cells were also positive for estrogen receptors (X 100)

Pleural biopsy showed evidence of thoracic endometriosis (TE).2 While women old and recent hemorrhage with with pelvic endometriosis alone usually hemosiderin-laden macrophages but present at 24-29 years, the manifestations of endometrial glands and stroma were not TE peak 5 years later, mainly at age of 35, seen (Figure 4A). There were foci of around 30-50% of women with inflammatory cell infiltration, fibrosis and endometriosis are infertile.3,4 The symptoms calcification. Small islands of decidual are usually catamenial occurring around reaction were also seen. The nuclei of the time.5 decidual cells where positive for estrogen TE usually affects the pleura, and progesterone receptors (Figure 4B). She (73%) being the discharged home with hormonal therapy. At most common manifestation, followed by follow up chest imaging showed no catamenial hemothorax (14%); while recurrence of pleural effusion. catamenial hemoptysis (7%) and nodules (6%) due to lung parenchymal DISCUSSION involvement are less common.3 Besides the Endometriosis is presence of ectopic four well-known categories mentioned endometrial tissue (stroma and glands) above, the spectrum of thoracic outside the uterine cavity, mostly within the endometriosis syndrome (TES), also pelvic cavity and rarely outside. includes catamenial chest pain and Approximately, 6 to 10% of childbearing endometriosis-related diaphragmatic women are affected, and only 12% of those hernia.2 with endometriosis have extra-pelvic Catamenial hemothorax has involvement, predominantly thorax called nonspecific symptoms of cough, chest pain

International Journal of Health Sciences and Research (www.ijhsr.org) 211 Vol.11; Issue: 1; January 2021 Lamya Zaher Al Aamri et.al. Massive catamenial hemothorax: rare case of thoracic endometriosis syndrome. or shortness of breath that usually occur thoracoscopic surgery (VATS) which help 1 day before to 2–3 days after the onset of visualization of endometrial lesions, scars, menses.5,6 Fifty to eighty percent of women bullae, blebs etc.2 with TES have concomitant pelvic Classically, to diagnose TE, endometriosis.2,7 Approximately, 80 % of identification of both endometrial stroma catamenial hemothorax involved right side.8 and glands is required on histological Our patient was suffering from examination; it is nevertheless considered infertility and was already diagnosed to suggestive if stroma alone or pulmonary have pelvic endometriosis. Her presentation parenchymal hemorrhages or hemosiderin- with shortness of breath during laden macrophages are detected.5 dysmenorrhea, a massive right-sided pleural In clinical practice, it is hard to effusion and a grossly bloody aspirate were detect intact glands and stroma with relatively straight forward for thoracic hormone receptor study because of autolysis endometriosis. and degradation of proliferative ectopic The pathology of thoracic endometrial tissue 48 hours after endometriosis is as yet unexplained and menstruation.6 Only one-third of the cases many theories have been proposed. The of TE have typical histopathological most prominent is Sampson's theory of finding.7 retrograde menstruation: the endometrial Hence, in many cases the diagnosis tissue migrates to right side of pleural cavity is based on a compatible clinical picture, through right paracolic gutter and suggestive imaging studies and a bloody diaphragmatic fenestrations. This theory is pleural aspirate, corroborated with supported by predominant right thoracic characteristic findings on VATS. endometriosis.2,6,9 The coelomic metaplasia In our case, she had right-sided theory suggests transformation of the hemothorax, and VATS showed typical pleural and peritoneal mesothelial cells into blackish pleural lesions. The biopsy of endometrial tissue and is supported by the lesions failed to reveal presence of observation that some cases of TE occur in endometrial glands and stroma, but the the absence of pelvic endometriosis.2,10 finding of hemosiderin-laden macrophages Another theory hypothesizes lymphatic and was suggestive for TE there were also foci haematogenous spread of endometrial tissue of decidual tissue (Deciduosis). as a possible mechanism.6,2 TE treatment includes medical and The radiological abnormalities in TE surgical modalities which can be combined.7 are transient; diagnostic yield is increased Medical approach (Ideal for women who when performed around menses.2 Chest X- wish to preserve fertility) depends on ray and CT images are usually the initial suppression of ovarian estrogen secretion by studies to detect pneumothorax and oral contraceptives, progesterone agonists, hemothorax; however, MRI is more gonadotropin-releasing hormone agonists or sensitive in revealing the endometrial danazol for at least 6-12 months.6,11 lesions (hyper-intense) than CT scan where However, the recurrence rate is more than the lesions are hypo- or iso-attenuating.6,11 50% on stopping the treatment.7 Bronchoscopy is not of much avail Surgery is considered in patients in the diagnosis of TE as the pathologic with refractory or recurrent disease.11 Best changes are mostly peripheral.11 surgical modality is VATS which allows CA-125 may be elevated in serum removal of ectopic endometrial tissue, and pleural fluid but is not specific, and pleurectomy and closure of diaphragmatic likewise pleural fluid cytology is seldom of defects.11 Pleurodesis is an alternative or any help.12 can be considered additionally during The gold standard for diagnosis as VATS.2,11,12 well as therapy is, however, video-assisted

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Hysterectomy and bilateral salpingo- 5. Hwang SM, Lee CW, Lee BS, Park JH. oophorectomy is definitive treatment but Clinical features of thoracic endometriosis: causes infertility and disease may recur if A single center analysis. Obstetrics & hormone replacement therapy is used.6, 11.12 Gynecology Science 2015;58(3):223-231. Our patient had received both doi: 10.5468/ogs.2015.58.3.223 6. McCann MR, Schenk WB, Nassar A, medical and surgical treatment, underwent Maimone S. Thoracic endometriosis VATS with pleurectomy and pleurodesis, presenting as a catamenial hemothorax with and received follow-up hormonal treatment. discordant video-assisted thoracoscopic surgery. Radiology Case Reports 15 (2020) CONCLUSION 1419–1422 TE-associated catamenial hemotho- doi: 10.1016/j.radcr.2020.05.064 rax is a rare etiology for spontaneous 7. Nair S.S., Nayar J. Thoracic endometriosis hemothorax. It usually involves the right syndrome: a veritable Pandora’s Box. J clin side. High clinical suspicion for TE is Diagn Res. 2016 Apr; 10(4): QR04–QR08. required in women of childbearing age as doi: 10.7860/JCDR/2016/17668.7700 the relationship with menstruation may not 8. Wilkins SB, Bell-Thomson J, Tyras DH. Hemothorax associated with endometriosis. be immediately obvious. The successful J Thorac Cardiovasc Surg. 1985 Apr; treatment of TE is also as challenging as is 89(4):636-638. doi: 10.1016/S0022- the diagnosis. 5223(19)38771-9 9. Vercellini P, Abbiati A, Viganò P, ACKNOWLEDGEMENT Somigliana ED, Daguati R, Meroni F, We are grateful to Dr Ibrahim Adnan Crosignani PG. Asymmetry in distribution Suleiman, HOD pathology for providing the of diaphragmatic endometriotic lesions: photomicrographs. evidence in favour of the menstrual reflux theory. 2007 Sep; 22(9):2359-67. doi: REFERENCES 10.1093/humrep/dem224 1. Patrini D, Panagiotopoulos N, Gvinianidze 10. Channabasavaiah AD, Joseph JV. Thoracic L, Iqbal Y, Lawrence D. Etiology and endometriosis: revisiting the association management of spontaneous haemothorax. J between clinical presentation and thoracic Thorac Dis.2015Mar;7(3):520- pathology based on thoracoscopic findings 526. doi: 10.3978/j.issn.2072- in 110 patients. Medicine (Baltimore) 2010 1439.2014.12.50 May;89(3):183–188. doi: 2. Nezhat C, Lindheim SR, Backus L, Vu M, 10.1097/MD.0b013e3181df67d5 Vang N, Nezhat A, Nezhat C. Thoracic 11. Azizad-Pinto P, Clarke D. Thoracic Endometriosis Syndrome: A Review of endometriosis syndrome: case report and Diagnosis and Management. JSLS.2019Jul- review of the literature. J Summer Sep: e2019.00029. 2014;18(3):61-5. doi: 10.7812/TPP/13-154 doi: 10.4293/JSLS.2019.0002 12. Bhattacharjee S, Deb J. Saha R, Chakrabarti 3. Joseph J, Sahn SA. Thoracic endometriosis S, Mukherji J, Tapadar SR. Pleural syndrome: new observations from an Endometriosis: An Exceptional Cause of analysis of 110 cases. Am J Med. 1996, Hemorrhagic Pleural Effusion. J Obstet Feb;100(2):164–170. doi: 10.1016/S0002- Gynaecol India. 2014 Dec: 100– 9343(97)89454-5. 104. doi: 10.1007/s13224-012-0313-y 4. Holoch KJ, Lessey BA. Endometriosis and infertility. Clinical Obstetrics and How to cite this article: Lamya Zaher Al Aamri, Gynecology: June 2010 - Volume 53 - Issue Nasser Al Awaid, Adil Al Kindi et.al. Massive 2 - p 429-438 doi: catamenial hemothorax: rare case of thoracic 10.1097/GRF.0b013e3181db7d71 endometriosis syndrome. Int J Health Sci Res. 2021; 11(1): 209-213.

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