Hrev_master [page 100] [Emergency Care Journal 2023; 19:11595] Emergency Care Journal 2023; volume 19:11595 Abstract Spontaneous, massive hemothorax due to pulmonary varix is an uncommon, yet catastrophic cause of respiratory distress during pregnancy and postpartum. Presentation is often confused with pul- monary embolism, the treatment of which, worsens the condition. Diagnosis is challenging because of the elusive abnormalities on radiographic and bronchoscopic examination. Currently, there are no treatment guidelines for pulmonary varix. A 23-year-old preg- nant lady in her second trimester, with intra-uterine fetal demise, presented to our emergency with dyspnoea, fever, and right-sided chest pain for three days. Chest X-ray and therapeutic thoracocen- tesis revealed massive right-sided hemothorax and computed tomography suggested pulmonary varix as the cause which was managed with emergency chest tube drainage. Massive, sponta- neous hemothorax due to pulmonary varix in pregnancy is a rare occurrence, but it requires timely, multidisciplinary assessment and management for the betterment of the mother and the fetus. Introduction Spontaneous, massive hemothorax is a rare, but catastrophic emergency condition. It is an uncommon cause of respiratory dis- tress during pregnancy and postpartum. Hemothorax is defined as pleural fluid with a hematocrit greater than 50%.1 Such patients may be asymptomatic or present with chest pain, dyspnea, short- ness of breath, hemoptysis, epistaxis, or chest pain. The definitive diagnosis is made with contrast-enhanced computed tomography (CECT) and pulmonary angiography. Once a diagnosis of a hemothorax is established, almost always, a chest thoracostomy is required to evacuate the blood and clots inside the chest cavity and transfusion of blood products is often necessary. It may result from a variety of etiologies and in some patients, they may even remain unknown despite exploratory thoracotomy.2,3 In any pregnant or postpartum patient with hemothorax, choriocarcinoma must be excluded.4 Video-assisted Thoracoscopic Surgery (VATS) is the standard treatment but specific treatment depends on the cause.5 There are currently no treatment guidelines for pulmonary varix.6 Case Report A 23-year-old pregnant lady presented to the emergency department (ED) at KIST Medical College and Teaching Hospital (MCTH) in June 2023 from Sarlahi, a remote area of Terai, Nepal. She came with chief complaints of sudden onset of shortness of breath, fever, and chest pain for three days. Shortness of breath was gradual in onset and progressive in nature without any positional or diurnal variation. The severity increased from Modified Medical Research Council (MRC) grade 0 to grade 4 in the past three days. It was associated with right-sided chest pain which was dull aching, pressing type, non-radiating, and aggravated by lying down. She gave a history of fever with a maximum temperature recorded at 100.4°F, associated with chills and rigor, continuous type, relieved by taking antipyretic oral medication. She was in her 27th week of gestation, with uneventful antenatal visits. Her past medical and surgical history were insignificant. According to gravida (G), parity (P), and living (L) status, she was a multigravi- da lady, G4, with 3 living issues, P3L3, without any complications in the past pregnancies. On arrival, she was dyspneic and appeared pale and cyanosed. Her initial vitals were pulse rate (PR) of 126 Correspondence: Pratisha Pradhan, Department of General Practice and Emergency Medicine, KIST MCTH, Imadole, Lalitpur, Nepal. Tel.: +9779849256281 E-mail: pratisha.7@gmail.com Key words: case report; pregnancy; pulmonary varix; spontaneous massive hemothorax Contributions: PP, writing of the manuscript and involved in the care and follow-up of the patient; KA, reviewing of the manuscript and follow-up of the patient; BKH, editing the manuscript; ST, editing the manuscript; PS, editing the manuscript. Conflict of interest: the authors declare no potential conflict of inter- est, and all authors confirm accuracy. Ethics approval and consent to participate: no ethical committee approval was required for this case report by the Department, because this article does not contain any studies with human partic- ipants or animals. Informed consent was obtained from the patient included in this study. Patient consent for publication: the patient gave her written consent to use her personal data for the publication of this case report and any accompanying images. Availability of data and materials: all data underlying the findings are fully available. Received: 18 July 2023. Accepted: 11 August 2023 Early view: 8 September 2023. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2023 Licensee PAGEPress, Italy Emergency Care Journal 2023; 19:11595 doi:10.4081/ecj.2023.11595 Publisher's note: all claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher. Right-sided spontaneous, massive hemothorax in a 27-week pregnant lady: a case report Pratisha Pradhan, Alok Kumar, Kabita Hada Batajoo, Trishna Shrestha, Sneha Pradhananga Department of General Practice and Emergency Medicine, KIST MCTH, Imadole, Lalitpur, Nepal Non -co mmerc ial us e o nly beats per minute (bpm), blood pressure (BP) of 110/70 mm of Hg, respiratory rate (RR) of 30 per minute, and saturation (SpO2) of 70% in the room atmosphere. She was categorized as triage II7 and kept in the resuscitation bay, Monitors were attached and high- flow oxygen (O2) was given via face mask at the rate of 15 liters per minute (L/min). Intravenous access with 18 gauge was gained and a pint of normal saline (NS) 500 ml was initiated. On her systemic review of the respiratory system, the chest wall was asymmetrical, decreased movement on the right side and the trachea was shifted to the left. On percussion, the dull note was observed and auscultation revealed decreased air entry on the right side. On abdominal examination, her gravid uterus size was corre- sponding to 26 weeks of gestation (WOG), no tenderness, howev- er, fetal heart sound (FHS) could not be heard. Her cardiovascular and central nervous system examinations were grossly intact. Laboratory investigations sent were complete blood count (CBC), random blood sugar (RBS), renal function test (RFT), pro- thrombin time/international normalized ratio (PT/INR), blood grouping, and d-dimer. Meanwhile, extreme tachycardia at the rate of 185 beats per minute was revealed in bedside electrocardiogram (ECG), and severe metabolic acidosis with pH 7.2, HCO3 8.7 mmol/L, pCO2 20.3 mmHg, pO2 67 mmHg and a lactate of 6.7 mmol/L in arterial blood gas analysis (ABG). Following manage- ment of the abnormal physiologies, in view of suspected septic shock, the empirical injectable broad-spectrum antibiotic of a com- bination of Piperacillin and Tazobactam, 4.5 grams and sodium bicarbonate (NaHCO3) 50 mEq (1 mEq/kg) intravenous were given. On reassessment after 30 minutes of arrival, her BP dropped to 90/50 mmHg. Thus, second wide bore IV access was secured and inotrope (Inj. Noradrenaline) was started at 0.1mcg/kg/minute via syringe pump. Foley’s catheterization was done with an initial output of 200 ml. Inj. Paracetamol 1gm was given for her pain. Ultrasonography of the abdomen and pelvis (USG) showed a single intrauterine pregnancy of 27+3 WOG with no cardiac activi- ty, right-sided pleural effusion, and minimal pericardial effusion with no intraperitoneal collection. Therapeutic tapping revealed bloody pleural fluid suggestive of hemothorax. Further, a portable chest X-ray (CXR) was done which showed homogenous opacity throughout the right lung field as shown in Figure 1. On-duty con- sultants of obstetrics and gynecology (OBG), cardiothoracic and vascular surgery (CTVS), and internal medicine departments were consulted in view of G4P3L3 at 27+3 WOG with intrauterine fetal demise (IUFD) with massive right-sided effusion with severe metabolic acidosis in septic shock with severe anemia with sus- pected amniotic fluid (pulmonary) embolism. A 32-French chest tube was inserted after consent, connected to a water seal drain and placement was confirmed with air column and drainage of bloody pleural fluid. The initial drain was 500 ml in less than 2 minutes and hence the tube was clamped. Thereafter, there was an improvement in her saturation of 94% with 12 L/min O2 via face mask and respiratory rate of 24 per minute. Post-pro- cedural portable CXR was done to confirm the placement of the tube which was observed slightly high up. The patient and her relatives were counseled about the possible need for immediate surgical thoracotomy for the management of the condition. However, due to financial limitations, they denied the surgical approach and opted for conservative management. Therefore, the patient was admitted to the surgical intensive care unit (SICU) and the night of admission was quite uneventful. On the second day of admission, the chest tube was manipulat- ed to locate the tip in the lower zone, and a CT Chest was done which revealed a tortuous and tubular dilated peripheral vein draining to the right superior pulmonary vein (maximum diameter 11 mm) suggestive of pulmonary varix. Also, it showed right-sided gross hemopneumothorax (approx. 680 ccs) with a chest tube in situ, adjacent parenchymal collapse, and features of mediastinal shift towards the left side (Figure 2). The same day, a fresh stillbirth (male baby) was expelled spon- taneously and the placenta was removed manually with a total blood loss of 100 ml. The patient was shifted to the surgical ward on 4th day of admission. Her vitals were stable and CXR was done in series to note improvement with a subsequent decrease in the hemothorax but poor lung expansion was observed with the devel- opment of pneumothorax (Figure 3). A total of 2150 mL of old blood was drained from the right pleural cavity during her entire stay in the hospital. There was no subsequent clinical or imaging evidence of further blood loss. She was advised for bronchoscopy and VATS but refused due to finan- cial issues and on the 12th day of admission she was discharged on request with a chest tube in situ as they finally agreed to visit a government hospital for further management. The patient was called via phone multiple times to know about her progress but she was unreachable thereafter. Discussion Spontaneous, non-traumatic hemothorax could be caused by numerous etiology of which pneumothorax is the most common cause, followed by vascular abnormalities, coagulopathies, neopla- sia, thoracic diseases such as pulmonary infarction due to throm- boembolism, infections or sequestrations.8 Pulmonary arteriovenous malformation (AVM) is a disease with quite similar findings, clini- cally and radiographically. Pulmonary varix should be distinguished from it because their treatment strategies are completely different.6 In pregnancy, diaphragmatic ectopic pregnancy, gestational tro- phoblastic disease (GTD), diaphragmatic endometriosis or rupture of subcapsular hematoma could also lead to spontaneous hemotho- rax. Idiopathic spontaneous hemothorax is a diagnosis of exclusion when every known cause has been investigated.2,5 In our case, CT Case Report Figure 1. CXR showing homogenous opacity throughout the right lung field and mediastinal shift to the left. [Emergency Care Journal 2023; 19:11595] [page 101] Non -co mmerc ial us e o nly chest was useful to construct the possible cause of the massive hemothorax, i.e. pulmonary varix. However, due to financial con- straints, neither CECT chest nor pulmonary angiography could be performed on our patient to rule out thromboembolism/ AVM. A pulmonary varix is as a localized aneurysmal dilatation of a segment of a pulmonary vein, also known as a pulmonary venous aneurysm, present at any age, without gender predominance, occurring in isolation or associated with obstruction of the pul- monary veins.9–11 It was originally described in 1843 by Arnett and Patton.12 Only 71 cases have been reported as of 2011 and the inci- dence remains unknown as it has been reported infrequently.13 Pulmonary varices are a rare pulmonary venous disorder whose etiology remains uncertain but can be either congenital or acquired and isolated or associated with varices in other organs as well.14,15 Congenital varices develop during the embryonic period and may coexist with various heart diseases such as pulmonary vein atresia and anomalous pulmonary vein that were not present in our case. Acquired forms are associated with diseases with increased pulmonary vein pressure like mitral valve disease or dis- tal occlusion of the pulmonary veins, liver cirrhosis, or emphyse- ma.15,16 In our case, bedside echocardiography was performed but did not reveal significant findings. It morphologically can be clas- sified into three types: saccular type, tortuous type, and confluent type. The present case had a tortuous dilated vasculature in the right lung and thus was diagnosed with a tortuous type.6 Pulmonary varices are usually asymptomatic and are mostly incidental findings during health check-ups. They may not be vis- ible on CXR or may present as solitary pulmonary nodules. CT chest may show tortuous, serpiginous vessels without a vascular nidus and normal-appearing pulmonary arteries, allowing varices to be distinguished from pulmonary AVM, as in our case. Catheter pulmonary angiography remains the gold standard but due to its Case Report Figure 3. CXRs of days 2, 5, and 10 of admission showing improvement. Figure 2. CT Chest coronal sections, showing pulmonary varix denoted by red arrows. [page 102] [Emergency Care Journal 2023; 19:11595] Non -co mmerc ial us e o nly invasiveness, in recent years, the diagnosis of these diseases has been established using CECT chest and magnetic resonance imag- ing (MRI). Three-dimensional CT has also proven to show similar results.6 The size criteria for diagnosis of a pulmonary vein varix have not yet been established, but an aneurysm is usually defined as an increase in 50% of the normal diameter.10 In hemothorax during pregnancy without evidence of fetal dis- tress, management should be centered on the mother’s pulmonary and hemodynamic status. If there is extensive hemorrhage with mediastinum shifts, it requires emergency treatment with termina- tion of pregnancy. However, in our case, there was fetal demise already and hence management was focused on the mother.16 Treatment is usually unnecessary unless varices rapidly increase in size or complications such as hemoptysis, thromboem- bolic disease, or rupture occurs. They can be treated conservatively with chest tube insertion in most patients if the bleeding persists for less than 24 hours. Surgical intervention is reserved for patients with massive hemorrhage with hemodynamic instability, persistent bleeding for more than 24 hours, or retained blood clot refractory to tube drainage, all of which were present in our case. Before 1990, the surgical approach was usually through a thoracotomy, which has now been gradually replaced by the VATS approach with or without axillary mini-thoracotomy which is an easy, acces- sible, and safe procedure. Furthermore, this minimally invasive procedure is advantageous due to less wound pain, better wound cosmetics, and less post-operative respiratory impairment. Since VATS was unavailable in our hospital setting, conservative man- agement with bed rest, oxygen supplementation, manual aspira- tion, and chest tube drainage were considered.17,18 However, despite proper chest tube insertion and drainage in our case, there was no lung expansion and she developed pneumothorax. A possi- ble cause of lung non-expansion could be a condition called “trapped lung” which is the sequela of remote pleural space inflammation resulting in the development of a mature, fibrous membrane that prevents lung expansion during fluid removal. Thus, there could be defective pleural healing even after inflamma- tion in the pleural space has resolved which is considered as a com- plication of active pleural inflammation, or hemothorax as in our case.19 Follow-up CECT chest could not be performed to support this theory or rule out other causes of non-expandable lungs such as fistula, endobronchial obstruction, or pleural disease. This is, to the best of our knowledge, the first case reported of pulmonary varix leading to massive, spontaneous hemothorax in pregnancy with IUFD, in our hospital. On presumptive diagnosis of pulmonary embolism, many are treated accordingly which could be catastrophic. Hence, it requires timely, multidisciplinary assessment and management for the betterment of the mother and the fetus. References 1. Martinez FJ, Villanueva AG, Pickering R, et al. Spontaneous hemothorax. Report of 6 cases and review of the literature. Medicine (Baltimore) 1992;71:354-68. 2. Doshi HM, Robinson S, Chalhoub T, et al. 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