American Journal of Emergency Medicine xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
A manifestation of endometriosis that will take your breath away: a case report
Abstract Pneumothorax should be included in the differential diagnosis of any patient presenting with sudden onset chest pain or dyspnea, and the diagnosis should be made promptly and accurately. Catamenial pneumothorax, although a rare entity, is a possibility that should be considered in menstruating females presenting with chest pain and/or dyspnea. This case serves to demonstrate the effectiveness of point-of-care ultrasonography in making the prompt diagnosis of a pneumothorax, which in this case was a catamenial pneumothorax. Catamenial pneumothorax (CP) is a rare entity that is often underdiagnosed [2]. It is described as the development of a spontaneous pneumothorax 48 to 72 hours before or after the onset of menses in women of childbearing age. The pathophysiology remains obscure, and a clear understanding of the mechanism still eludes the scientific community today, although it was first described in 1958 [1]. Point-of-care thoracic ultrasound has been proven to be an effective tool in the emergency setting for multiple applications. This modality has been found to be more specific and sensitive than traditional chest radiography for the diagnosis of pneumothorax [3]. This case clearly demonstrates the advantages of point-of-care ultrasound in the case of a rare presentation of pneumothorax. A 37-year-old woman with a history of endometriosis and a large endometrioma presented to the emergency department with a chief complaint of chest pain for 1 day. She was seen in the emergency department 3 days before this presentation for abdominal pain and was discharged home to follow up with her gynecologist, after computed tomography (CT) of the abdomen and pelvis showed no significant or acute findings. On this visit, she states she no longer had any abdominal pain but rather had severe right-sided chest pain and shortness of breath for 1 day. She states the pain began suddenly and that supine positioning exacerbated her symptoms. She denied any fever, nausea, vomiting, or diarrhea. Of note, her last menstrual period began 3 days before this visit. Her vital signs were as follows: pulse, 112 beats per minute; respiratory rate, 30 breaths per minute; and oxygen saturation of 92% on room air. An electrocardiogram revealed uncomplicated sinus tachycardia. The patient was a healthy-appearing young woman in mild respiratory distress, speaking in full clear sentences. Physical examination revealed tachycardia and decreased breath sounds at the right lung base. There was no lower extremity edema or swelling. Her initial upright posterior-anterior chest radiograph demonstrated a small right-sided pleural effusion with no other abnormalities (Fig. 1). This was confirmed with the radiology department. A bedside thoracic
ultrasound was performed by an ultrasound fellowship-trained emergency physician. Sonography using both B mode and motion (M) mode of the right anterior chest wall showed no evidence of lung sliding (Fig. 2a), which raised the suspicion of pneumothorax. The left lung had no sonographic abnormalities. A CT of the chest was performed to further evaluate the contradictory findings, when sonography was compared with radiography. The CT revealed a moderate sized right-sided hemopneumothorax (Fig. 3a and b). Given the findings and the patient's symptoms, thoracostomy tube placement was performed using a 24F catheter chest tube. Two hundred milliliters of blood returned from the tube immediately. A follow-up radiograph 1 hour later showed interval resolution of the hemopneumothorax. The patient was admitted to the thoracic surgery service with obstetrics and gynecology consultation. The chest tube was removed on hospital day 3, and the obstetrics and gynecology service recommended beginning hormonal suppression therapy. The decision was made to forgo surgical exploration of the pleura and diaphragm, as this was the patient's first presentation to health care with a hemopneumothorax. The patient's clinical status improved, and she was discharged home in stable condition on hospital day 5. She was to follow up with obstetrics and gynecology within the week to schedule elective removal of the endometrioma.
Fig. 1. Initial Chest X-ray.
0735-6757/Published by Elsevier Inc.
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
2
G. Patel et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 2. Motion mode ultrasound of right lung showing no lung sliding.
Fig. 4. Pleural Line.
Catamenial pneumothorax is a rare condition, which exclusively affects women during their reproductive years. Although CP has been known to exist for more than a half a century, a clear explanation of its pathophysiology eludes us to this day [4]. It is encountered in 3% to
6% of spontaneous pneumothorax cases in menstruating women, with hemopneumothorax being an even more rare entity accounting for only 14% of those initial 3% to 6% [5]. Maurer et al [1] first described CP in 1958, and his findings suggested a temporal relationship between the onset of pneumothorax and menses, with symptoms typically presenting 24 to 36 hours after the onset of menses. There is no consensus regarding the exact mechanism, but it is thought to involve preexisting or acquired diaphragmatic defects and endometrial implants. Given the relationship between menses and CP, the presence of pelvic endometriosis is of etiologic importance. Pelvic endometriosis is present in 31% to 50% of CP cases [2]. Through further investigation, Maurer et al [1] proposed the main hypothesis to explain this syndrome: a dissolving cervical mucous plug may allow the ascent of air through the fallopian tubes, causing a transient pneumoperitoneum. This free air would subsequently escape through diaphragmatic defects entering into the pleural space, causing CP. The diaphragmatic defects are thought be a result of hormone-regulated sloughing of endometrial tissue during menses on the diaphragm itself [2]. On rare occasions, endometrial tissue can be found in the thoracic cavity itself [1,5]. Catamenial pneumothorax is the most common manifestation of thoracic endometriosis (73%). Nevertheless, pleural endometriosis is found in only half of the patients with CP who were surgically explored [6,7]. The patient in this case patient presented with a hemopneumothorax, which suggests that she likely developed some form of thoracic endometriosis, where the endometrial cells bled into her pleural cavity during menstruation. As with this patient, nearly 90% of CPs occur on the right side [8].
Fig. 3. CT- scan of Chest.
Fig. 5. Normal lung sliding and the "Seashore sign".
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
G. Patel et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 6. No lung sliding and the "Barcode sign".
Our patient's initial chest radiograph did indeed show fluid accumulation above the right hemidiaphragm; however, several clinicians missed the subtle findings of a pneumothorax at first glance. Traditional chest radiography has long been the test of choice for the rapid diagnosis of pneumothorax in the emergency setting. However, it has been widely reported that there are limitations regarding accuracy. Some studies report a 50% miss rate on supine chest radiographs and nearly 30% for occult pneumothorax on upright AP chest radiographs [9]. With rapid accurate diagnosis being of utmost importance in expediting resuscitation, the role of point-of-care thoracic ultrasound has been widely implemented in the emergency setting. The technique involves longitudinal scanning of the anterior chest wall with the patient in a supine position. A high-frequency linear probe is preferred but not mandatory. The probe is placed on the chest between the third and fourth intercostal space [11]. First, the rib acoustic shadows are visualized to find the intercostal plane. The pleural line is located as a hyperechoic line visible between and below 2 ribs (Fig. 4). In the normal subject, this pleural line is usually characterized by “lung sliding,” which is a to-and-fro movement of the visceral pleura synchronized with respiration as visualized in real-time scanning. In the patient with a pneumothorax, this finding is typically absent. More so than direct visualization, the use of M mode allows us to more accurately assess pleural sliding or a lack thereof. In normal lung sliding, M-mode imaging demonstrates a linear, laminar pattern in the tissue superficial to the pleural and a granular or “sandy” appearance deep to the pleural line. This phenomenon is known as the “seashore sign [11] (Fig. 5).” As seen in the images of the right chest of this patient, the linear pattern seen in the superficial tissue is also seen below the pleural line. This pattern shows the absence of movement above and below the pleural line suggesting the presence of a pneumothorax. The pattern is known as the “barcode” or “stratosphere sign [11] (Fig. 6).” Several studies have demonstrated high sensitivity and specificity for thoracic ultrasound in the detection of occult pneumothorax in critical care and trauma patients. The studies of Lichtenstein and Menu [3] involved critically ill patients in an intensive care setting. Absence of lung sliding was found in a prospective operator-blinded study to be a
3
useful sign for pneumothorax, with sensitivity of 95.3%, specificity of 91.1%, and negative predictive value of 100%. Kirkpatrick et al [10] studied ultrasound vs chest radiography in the trauma patients. Using chest CT as the criterion standard, chest radiography showed a sensitivity of 75.5% (95% confidence interval [CI], 61.7%-86.2%) and a specificity of 100% (95% CI, 97.1%-100%). The sensitivity for ultrasound was 98.1% (95% CI, 89.9%-99.9%), and the specificity was 99.2% (95% CI, 95.6%99.9%). It should be noted that although the absence of lung sliding suggests the presence of pneumothorax, other entities such pulmonary fibrosis, pleural adhesions, acute respiratory distress syndrome, and those who have undergone pleurodesis might also lack lung sliding [10]. Catamenial pneumothorax as an extremely rare condition that is underrecognized and underdiagnosed (needs reference). Although relatively little is still known about CP, clinicians should have a high suspicion in any female patient of childbearing age with symptoms consistent with pneumothorax. Timely diagnosis and treatment may significantly reduce patient morbidity and mortality; and with the advent of point-of-care ultrasound, clinicians now find themselves with this important tool to aid them in this pursuit. Ultrasound is fast, accurate, and reduces the need for radiation and its cumulative effects. ⁎ Gaurav Patel MD Brendon Stankard PA Robert Gekle MD Steve Park MD Adam Rucker MD Department of Emergency Medicine, North Shore University Hospital 300 Community Drive, Manhasset, NY 11030 ⁎Corresponding author E-mail address: [email protected]
http://dx.doi.org/10.1016/j.ajem.2014.10.051 References [1] Maurer ER, Schaal JA, Mendez FL. Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm. JAMA 1958;168:2013–4. [2] Alifano M, Cancellieri A, Fornelli A, Trisolini R, Boaron M. Endometriosis-related pneumothorax: clinic-pathologic observations from a newly diagnosed case. J Thorac Cardiovasc Surg 2004;127:1219–21. [3] Lichtenstein DA, Menu Y. A bedside ultrasound sign ruling out pneumothorax in the critically ill. Lung sliding. Chest 1995;108:1345–8. [4] Yamazaki S, Ogawa J, Koide S, Shohzu A, Osamura Y. Catamenial pneumothorax associated with endometriosis of the diaphragm. Chest 1980;77:107–9. [5] Nakamura H, Konishiike J, Sugamura A. Epidemiology of spontaneous pneumothorax in women. Chest 1986;89:378–82. [6] Leong AC, Coonar AS, Lang-Lazdunski L. Catamenial pneumothorax: surgical repair of the diaphragm and hormone treatment. Ann R Coll Surg Engl 2006;88: 547–9. [7] Blanco S, Hermano F, Gomez A. Catamenial pneumothorax caused by diaphragmatic endometriosis. J Thorac Cardiovasc Surg 1998;116:179–80. [8] Blaivas M, Lyon M, Duggal S. A prospective comparison of supine chest radiography and bedside ultrasound for the diagnosis of traumatic pneumothorax. Acad Emerg Med 2005;12:844–9. [9] Chan SW. Emergency bedside ultrasound to detect pneumothorax. Acad Emerg Med 2003;10:91–4. [10] Kirkpatrick AW, Ng AKT, Dulchavsky SA. Sonographic diagnosis of a pneumothorax inapperent on plain radiography: confirmation by computed tomography. J Trauma 2001;50:750–2. [11] Wernecke K, Galanski M, Peters PE, Hansen J. Pneumothorax: evaluation by ultrasound—preliminary results. J Thorac Imaging 1987;2(2):76–8.
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
A manifestation of endometriosis that will take your breath away: a case report
Abstract Pneumothorax should be included in the differential diagnosis of any patient presenting with sudden onset chest pain or dyspnea, and the diagnosis should be made promptly and accurately. Catamenial pneumothorax, although a rare entity, is a possibility that should be considered in menstruating females presenting with chest pain and/or dyspnea. This case serves to demonstrate the effectiveness of point-of-care ultrasonography in making the prompt diagnosis of a pneumothorax, which in this case was a catamenial pneumothorax. Catamenial pneumothorax (CP) is a rare entity that is often underdiagnosed [2]. It is described as the development of a spontaneous pneumothorax 48 to 72 hours before or after the onset of menses in women of childbearing age. The pathophysiology remains obscure, and a clear understanding of the mechanism still eludes the scientific community today, although it was first described in 1958 [1]. Point-of-care thoracic ultrasound has been proven to be an effective tool in the emergency setting for multiple applications. This modality has been found to be more specific and sensitive than traditional chest radiography for the diagnosis of pneumothorax [3]. This case clearly demonstrates the advantages of point-of-care ultrasound in the case of a rare presentation of pneumothorax. A 37-year-old woman with a history of endometriosis and a large endometrioma presented to the emergency department with a chief complaint of chest pain for 1 day. She was seen in the emergency department 3 days before this presentation for abdominal pain and was discharged home to follow up with her gynecologist, after computed tomography (CT) of the abdomen and pelvis showed no significant or acute findings. On this visit, she states she no longer had any abdominal pain but rather had severe right-sided chest pain and shortness of breath for 1 day. She states the pain began suddenly and that supine positioning exacerbated her symptoms. She denied any fever, nausea, vomiting, or diarrhea. Of note, her last menstrual period began 3 days before this visit. Her vital signs were as follows: pulse, 112 beats per minute; respiratory rate, 30 breaths per minute; and oxygen saturation of 92% on room air. An electrocardiogram revealed uncomplicated sinus tachycardia. The patient was a healthy-appearing young woman in mild respiratory distress, speaking in full clear sentences. Physical examination revealed tachycardia and decreased breath sounds at the right lung base. There was no lower extremity edema or swelling. Her initial upright posterior-anterior chest radiograph demonstrated a small right-sided pleural effusion with no other abnormalities (Fig. 1). This was confirmed with the radiology department. A bedside thoracic
ultrasound was performed by an ultrasound fellowship-trained emergency physician. Sonography using both B mode and motion (M) mode of the right anterior chest wall showed no evidence of lung sliding (Fig. 2a), which raised the suspicion of pneumothorax. The left lung had no sonographic abnormalities. A CT of the chest was performed to further evaluate the contradictory findings, when sonography was compared with radiography. The CT revealed a moderate sized right-sided hemopneumothorax (Fig. 3a and b). Given the findings and the patient's symptoms, thoracostomy tube placement was performed using a 24F catheter chest tube. Two hundred milliliters of blood returned from the tube immediately. A follow-up radiograph 1 hour later showed interval resolution of the hemopneumothorax. The patient was admitted to the thoracic surgery service with obstetrics and gynecology consultation. The chest tube was removed on hospital day 3, and the obstetrics and gynecology service recommended beginning hormonal suppression therapy. The decision was made to forgo surgical exploration of the pleura and diaphragm, as this was the patient's first presentation to health care with a hemopneumothorax. The patient's clinical status improved, and she was discharged home in stable condition on hospital day 5. She was to follow up with obstetrics and gynecology within the week to schedule elective removal of the endometrioma.
Fig. 1. Initial Chest X-ray.
0735-6757/Published by Elsevier Inc.
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
2
G. Patel et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 2. Motion mode ultrasound of right lung showing no lung sliding.
Fig. 4. Pleural Line.
Catamenial pneumothorax is a rare condition, which exclusively affects women during their reproductive years. Although CP has been known to exist for more than a half a century, a clear explanation of its pathophysiology eludes us to this day [4]. It is encountered in 3% to
6% of spontaneous pneumothorax cases in menstruating women, with hemopneumothorax being an even more rare entity accounting for only 14% of those initial 3% to 6% [5]. Maurer et al [1] first described CP in 1958, and his findings suggested a temporal relationship between the onset of pneumothorax and menses, with symptoms typically presenting 24 to 36 hours after the onset of menses. There is no consensus regarding the exact mechanism, but it is thought to involve preexisting or acquired diaphragmatic defects and endometrial implants. Given the relationship between menses and CP, the presence of pelvic endometriosis is of etiologic importance. Pelvic endometriosis is present in 31% to 50% of CP cases [2]. Through further investigation, Maurer et al [1] proposed the main hypothesis to explain this syndrome: a dissolving cervical mucous plug may allow the ascent of air through the fallopian tubes, causing a transient pneumoperitoneum. This free air would subsequently escape through diaphragmatic defects entering into the pleural space, causing CP. The diaphragmatic defects are thought be a result of hormone-regulated sloughing of endometrial tissue during menses on the diaphragm itself [2]. On rare occasions, endometrial tissue can be found in the thoracic cavity itself [1,5]. Catamenial pneumothorax is the most common manifestation of thoracic endometriosis (73%). Nevertheless, pleural endometriosis is found in only half of the patients with CP who were surgically explored [6,7]. The patient in this case patient presented with a hemopneumothorax, which suggests that she likely developed some form of thoracic endometriosis, where the endometrial cells bled into her pleural cavity during menstruation. As with this patient, nearly 90% of CPs occur on the right side [8].
Fig. 3. CT- scan of Chest.
Fig. 5. Normal lung sliding and the "Seashore sign".
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
G. Patel et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 6. No lung sliding and the "Barcode sign".
Our patient's initial chest radiograph did indeed show fluid accumulation above the right hemidiaphragm; however, several clinicians missed the subtle findings of a pneumothorax at first glance. Traditional chest radiography has long been the test of choice for the rapid diagnosis of pneumothorax in the emergency setting. However, it has been widely reported that there are limitations regarding accuracy. Some studies report a 50% miss rate on supine chest radiographs and nearly 30% for occult pneumothorax on upright AP chest radiographs [9]. With rapid accurate diagnosis being of utmost importance in expediting resuscitation, the role of point-of-care thoracic ultrasound has been widely implemented in the emergency setting. The technique involves longitudinal scanning of the anterior chest wall with the patient in a supine position. A high-frequency linear probe is preferred but not mandatory. The probe is placed on the chest between the third and fourth intercostal space [11]. First, the rib acoustic shadows are visualized to find the intercostal plane. The pleural line is located as a hyperechoic line visible between and below 2 ribs (Fig. 4). In the normal subject, this pleural line is usually characterized by “lung sliding,” which is a to-and-fro movement of the visceral pleura synchronized with respiration as visualized in real-time scanning. In the patient with a pneumothorax, this finding is typically absent. More so than direct visualization, the use of M mode allows us to more accurately assess pleural sliding or a lack thereof. In normal lung sliding, M-mode imaging demonstrates a linear, laminar pattern in the tissue superficial to the pleural and a granular or “sandy” appearance deep to the pleural line. This phenomenon is known as the “seashore sign [11] (Fig. 5).” As seen in the images of the right chest of this patient, the linear pattern seen in the superficial tissue is also seen below the pleural line. This pattern shows the absence of movement above and below the pleural line suggesting the presence of a pneumothorax. The pattern is known as the “barcode” or “stratosphere sign [11] (Fig. 6).” Several studies have demonstrated high sensitivity and specificity for thoracic ultrasound in the detection of occult pneumothorax in critical care and trauma patients. The studies of Lichtenstein and Menu [3] involved critically ill patients in an intensive care setting. Absence of lung sliding was found in a prospective operator-blinded study to be a
3
useful sign for pneumothorax, with sensitivity of 95.3%, specificity of 91.1%, and negative predictive value of 100%. Kirkpatrick et al [10] studied ultrasound vs chest radiography in the trauma patients. Using chest CT as the criterion standard, chest radiography showed a sensitivity of 75.5% (95% confidence interval [CI], 61.7%-86.2%) and a specificity of 100% (95% CI, 97.1%-100%). The sensitivity for ultrasound was 98.1% (95% CI, 89.9%-99.9%), and the specificity was 99.2% (95% CI, 95.6%99.9%). It should be noted that although the absence of lung sliding suggests the presence of pneumothorax, other entities such pulmonary fibrosis, pleural adhesions, acute respiratory distress syndrome, and those who have undergone pleurodesis might also lack lung sliding [10]. Catamenial pneumothorax as an extremely rare condition that is underrecognized and underdiagnosed (needs reference). Although relatively little is still known about CP, clinicians should have a high suspicion in any female patient of childbearing age with symptoms consistent with pneumothorax. Timely diagnosis and treatment may significantly reduce patient morbidity and mortality; and with the advent of point-of-care ultrasound, clinicians now find themselves with this important tool to aid them in this pursuit. Ultrasound is fast, accurate, and reduces the need for radiation and its cumulative effects. ⁎ Gaurav Patel MD Brendon Stankard PA Robert Gekle MD Steve Park MD Adam Rucker MD Department of Emergency Medicine, North Shore University Hospital 300 Community Drive, Manhasset, NY 11030 ⁎Corresponding author E-mail address: [email protected]
http://dx.doi.org/10.1016/j.ajem.2014.10.051 References [1] Maurer ER, Schaal JA, Mendez FL. Chronic recurring spontaneous pneumothorax due to endometriosis of the diaphragm. JAMA 1958;168:2013–4. [2] Alifano M, Cancellieri A, Fornelli A, Trisolini R, Boaron M. Endometriosis-related pneumothorax: clinic-pathologic observations from a newly diagnosed case. J Thorac Cardiovasc Surg 2004;127:1219–21. [3] Lichtenstein DA, Menu Y. A bedside ultrasound sign ruling out pneumothorax in the critically ill. Lung sliding. Chest 1995;108:1345–8. [4] Yamazaki S, Ogawa J, Koide S, Shohzu A, Osamura Y. Catamenial pneumothorax associated with endometriosis of the diaphragm. Chest 1980;77:107–9. [5] Nakamura H, Konishiike J, Sugamura A. Epidemiology of spontaneous pneumothorax in women. Chest 1986;89:378–82. [6] Leong AC, Coonar AS, Lang-Lazdunski L. Catamenial pneumothorax: surgical repair of the diaphragm and hormone treatment. Ann R Coll Surg Engl 2006;88: 547–9. [7] Blanco S, Hermano F, Gomez A. Catamenial pneumothorax caused by diaphragmatic endometriosis. J Thorac Cardiovasc Surg 1998;116:179–80. [8] Blaivas M, Lyon M, Duggal S. A prospective comparison of supine chest radiography and bedside ultrasound for the diagnosis of traumatic pneumothorax. Acad Emerg Med 2005;12:844–9. [9] Chan SW. Emergency bedside ultrasound to detect pneumothorax. Acad Emerg Med 2003;10:91–4. [10] Kirkpatrick AW, Ng AKT, Dulchavsky SA. Sonographic diagnosis of a pneumothorax inapperent on plain radiography: confirmation by computed tomography. J Trauma 2001;50:750–2. [11] Wernecke K, Galanski M, Peters PE, Hansen J. Pneumothorax: evaluation by ultrasound—preliminary results. J Thorac Imaging 1987;2(2):76–8.
Please cite this article as: Patel G, et al, A manifestation of endometriosis that will take your breath away: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.10.051
