Catamenial haemothorax-A rare cause of pleural effusion

A 43-year-old female housewife presented to the ED with severe breathlessness for 7 days. She gave a history of intermittent, right-sided chest pain associated with chest tightness and breathlessness during the last 3 years. She was evaluated in a local hospital, and based on the X-ray findings, she was referred to our hospital. She used to have severe bleeding during her periods for the last 3 years. Each time the bleeding lasted for 5–7 days. She developed anaemia and pelvic workup revealed a left ovarian cyst. There was no history of cough or haemoptysis. Clinically she had moderate pleural effusion on the right side which was confirmed by chest X-ray [ Figure 1 a]. Routine blood examination revealed anaemia (Hb–8.3 gm/dL), normal total (7.600/mm 3 ) and differential WBC counts (N-71, E-04, B-00, L-22, M-03), and mild increase of ESR (28 mm/h). She underwent therapeutic as well as diagnostic thoracentesis. Physical observation of pleural fluid revealed a deep brownish-red haemorrhagic fluid, closely resembling blood [ Figure 1 b]. Haematocrit of pleural fluid was more than 50% of blood. Total cell count of pleural fluid was 650/mm 3 , and 60% were lymphocytes, with plenty of RBCs. It was a low ADA lymphocytic effusion. There were no malignant cells or AFB detected in the fluid. Contrast-enhanced CT thorax and abdomen were taken. CT thorax showed moderate right-sided pleural effusion without any evident parenchymal lesions, pleural lesions or mediastinal lymphadenopathy [ Figure 2 a]. CT abdomen revealed a large cystic lesion in the left ovary (27 × 24 mm) [ Figure 2 b] and a left adnexal mass of 37 × 29 mm abutting the left ovary and uterus [ Figure 2 b]. There were moderate ascites [ Figure 2 b]. (a) Chest X-ray PA view showing moderate pleural effusion on the right side. (b) Aspirated pleural fluid which is hemorrhagic shows plenty of RBCs (a) CECT thorax showing moderate pleural effusion on the right side. There are no pleural or parenchymal nodules. (b) CECT abdomen showing left ovarian cyst (red arrow) and a large adnexal mass close to the uterus and left ovarian cyst Serum CEA was normal, and CA 125 was only mildly elevated. Medical thoracoscopy with semirigid thoracoscopy was performed which showed moderate hemorrhagic pleural fluid in the pleural cavity. Diaphragmatic pleura and mediastinal pleura showed multiple hemorrhagic cysts, brownish nodules and gunshot lesions suggestive of pleural endometriosis [ Figure 3 a and b]. All the cysts were broken, and biopsy was taken from the nodules. Chest was closed after putting in an intercostal drainage tube. Histopathological examination of the biopsy specimen showed foci of endometrial glands lined by pseudostratified columnar epithelium with surrounding cellular endometrial stroma [ Figure 4 a and b]. (a) showing thoracoscopic view of right diaphragmatic pleura. Chocolate-coloured cysts are seen on the background of hemorrhagic pleural effusion. (b) showing brownish-black nodular lesion on the pleural surface referred to as ‘gunshot lesions’ (a) Low power view and (b) high power view of biopsy showed foci of endometrial glands lined by pseudostratified columnar epithelium with surrounding cellular endometrial stroma Considering the clinical features, results of various investigations and histopathology, diagnosis of pleural endometriosis were confirmed. Gynaecology opinion was sought, and they suggested hysterectomy with bilateral salpingo-oophorectomy as an elective procedure. Till then menstruation will be suppressed with hormonal therapy. Total hysterectomy and bilateral salpingo-oophorectomy were done as an elective procedure, Histopathology of resected specimens showed corpus luteum and endometriosis in left ovary, corpus luteum and cystic follicles in right ovary and secretory endometrium in the uterus. Hence, this patient had pelvic and thoracic endometriosis.

Endometriosis is a common gynaecological disorder, affecting 10–15% of women of reproductive age. It is considered to be due to extrauterine growth of endometrial tissue, including endometrial glands and stroma. The ectopic tissue is typically located in the peritoneal cavity, most often in the pelvis, but endometriosis has been reported in nearly all body compartments.[ 1 ] Although rare, the thoracic involvement is the most frequent extra-abdominopelvic site of endometriosis.[ 2 ] Thoracic endometriosis syndrome (TES) is the term used to denote various clinical manifestations resulting from the presence of functional endometrial tissue in a thoracic structure. Endometrial tissue is seeded in visceral or parietal pleura, lung parenchyma, airways or diaphragm.[ 2 ] Up to 80% of women with TES present with concomitant pelvic endometriosis. Clinical manifestations are more likely to occur during menses, because of the hormonal responsiveness of ectopic endometrial tissue.[ 2 ] TES includes five well-recognized clinical entities grouped into two forms, namely the pleural forms with catamenial pneumothorax (CP), non-catamenial endometriosis-related pneumothorax (NCP) and catamenial haemothorax (CHt); and the pulmonary forms with catamenial haemoptysis (CH) and lung nodules.[ 2 3 ] Approximately 90% of patients with TES experience catamenial thoracic pain and different entities may be associated.[ 2 ] CHt is a rare cause of haemorrhagic pleural effusion, which recurs during each menstrual cycle. This extremely rare condition is difficult to diagnose, unless direct correlation with the menstrual cycle is established. The right hemithorax is involved in more than 80% of cases.[ 2 ] Symptoms are typically cyclical and recurrent, with a right-sided predominance. Computed tomography (CT) is the first-line imaging method, but is poorly specific. Its main role is to rule out other pulmonary diseases. However, in women with typical clinical history, some key CT findings may help to confirm this syndrome. MRI can also assist with the diagnosis, by showing signal changes typical of haemorrhage within diaphragmatic or pleural lesions. Medical thoracoscopy is the preferred technique to detect endometriotic lesions in the pleura and also help in collecting samples for histopathological diagnosis.

CHt is a rare, unusual lesion that may be mistaken for various other causes of haemothorax unless the direct relationship with menstrual cycle is established. Diagnosis of CHt is challenging, as these women's symptoms may not immediately be attributed to endometriosis. The key features are the temporal relation with menses, cyclical changes in radiographic abnormalities, and the distinctive posterosuperior location of diaphragmatic lesions. Thorough observation of different symptoms and signs along with appropriate investigations is essential for early diagnosis in such cases. The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Nil. There are no conflicts of interest.

CHt represents the second-most common manifestation of TES, occurring in 14% of cases. It affects the right side about 80% of the time. Wilkins et al .[ 4 ] reported 15 cases of TES presenting with haemothorax, and all of his cases were on the right hemithorax. Concomitant pelvic endometriosis was found in 100% of cases 5. A study on 110 patients showed that the mean age at presentation was 35 ± 0.6 years, with a range from 15 to 54 years. Interestingly, the peak incidence for pelvic endometriosis is between 24 and 29 years, whereas the peak incidence for TES is approximately 5 years later.[ 5 ] In our case, CHt was detected at the age of 43 years and she also had concomitant pelvic endometriosis. Diagnosis is frequently delayed until several episodes have occurred as the patient fails to associate symptoms with menstruation. In our case, also the diagnosis was delayed even though the patient complained of non-specific right-sided chest pain during menstruation for about 3 years. Pleural fluid cytology is usually not helpful. Levels of CA-125 may be elevated in the serum and body cavity fluid of patients with endometriosis.[ 6 ] The concentration of CA-125 correlates with both the severity and the clinical course of the disease.[ 7 ] Symptoms have a catamenial pattern, occurring between 24 hours before and 72 hours after the onset of menses, 4 and typically recurring.[ 3 8 9 ] The right-side predominance of symptoms represents a diagnostic clue. Chest X-rays in cases of CHt usually reveal a pleural effusion 7. Spiral CT may show pleural or diaphragmatic thickening in involved areas.[ 10 ] In the present case, CT revealed a right-sided moderate pleural effusion without any pleural thickening or diaphragmatic lesions. Medical thoracoscopy helps in visualization of pleura, which may show multiple, dark red or blue nodule or cysts distributed mainly on the diaphragmatic pleura. In our case, multiple dark red cysts were seen largest measuring 2 × 1 cm. Most of them were bleeding on touch. Apart from that few blackish indurated nodules characteristically described as gunshot lesions were also seen. Microscopically, typical endometriosis consists of the presence of both endometriotic glands and stroma. The glands usually have an endometrioid appearance ranging from inactive, proliferative to hyperplastic. The endometriotic stroma characteristically resembles eutopic, inactive or proliferative endometrial stroma.[ 11 ] Immunohistochemically, in the series reported by Flieder and associates, most glands showed cytoplasmic positivity with broad-spectrum cytokeratin, cytokeratin 7, BER-EP4, and strong nuclear staining for estrogen and progesterone receptors.[ 9 ] Estrogen and progesterone receptors are present in endometriotic glands and stroma in a lower concentration than in eutopic endometrium.[ 12 13 ] Therapy for CHt includes the suppression of endometrial tissue and the prevention of further pelvic seeding. Medical therapy should be considered as the first line of treatment.[ 14 ] Ghio et al .[ 15 ] reported a case of catamenial pneumothorax with chest pain and used medroxyprogesterone acetate as therapy. According to light,[ 16 ] hormonal therapy (progestational agents, danazol and leuprolide acetate), fails in at least 50% cases. Medical treatment for endometriosis symptoms (with or without surgery) is generally needed for longer periods of time because of the chronic and recurrent nature of the disease. Progestins may be an appropriate alternative for the medical management of endometriosis, as these agents are relatively well-tolerated, have a more limited metabolic impact than other agents, and are also inexpensive.[ 17 ] Treatment with GnRH analogues, such as leuprolide, is limited to only 6 months, because these agents induce a hypoestrogenic state that substantially decreases bone mineral density. Poor tolerability represents the major drawback of danazol as a treatment for endometriosis. This agent has both androgenic and anabolic properties. Pleurodesis may be considered as a means of preventing the recurrence of haemothorax.[ 18 ]