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TWO CASE REPORTS OF CHYLOTHORAX DUE TO LYMPHANGIOLEIOMYOMATOSIS
S56
Oral Communications / European Journal of Internal Medicine 19S (2008), S1–S59
The Case: a 50 years old man with type 2 diabetes and severe dilated cardiomyopathy with systolic dysfunction (ejection fraction of left ventricle 20%), NYHA class IV, was admitted in our Hospital for many times in the last years for symptoms related to his chronic congestive cardiac failure. Each time were present dyspnoea at rest with orthopnoea, distal oedema, hepatomegaly, congestion and enlarged cardiac shadow at chest radiogram. Electrocardiogram reported sinus rhythm with Left bundle branch block, always negative cardiac enzymes, arterial blood gases showed a trend to respiratory alkalosis with substantially normal PaO2 value also breathing ambient air. Each admission resulted in a long hospitalisation, with only little improvements due to standard medical treatment including inotropic agents, high doses of diuretics, nitrates, beta blockers, ace inhibitors, oxygen. It seemed to be an end stage heart failure with perhaps a chance of transplantation. During the last admission we observed that the patient developed a periodic breathing with various episodes of nightly and daily central apnoea. In addition to standard aggressive medical therapy we decided to treat the patient with CPAP 7.5 cmH2 O and low FiO2 (28%), with a Venturi-Like Generator Flow (Whisper Flow – Caradine) and a facial mask: the patient performed 10-12 hours/day of CPAP treatment divided into 3-4 cycles of 3-4 hours each, nightly and daily. Since the first day of treatment he reported a significant improvement in sensation of dyspnoea, after few days of treatment the respiratory pattern sowed a decrease of apnoeas and the patient was able to arise and perform personal care (NYHA class III); after 15 days of treatment oedema was lowered, a chest radiogram showed reduction of congestion, echocardiography demonstrated an improvement of ejection fraction of left ventricle (33%). In this patient a long term treatment with nightly electric nasal CPAP at home may be useful in order to improve quality of life and decrease hospitalisation.
Case Report 2: 2 A 42-year-old female patient was admitted to our Division because of acute dyspnoea, cough and chest pain. At history menarche at fourteen years with regular rhythm and flow; hypertension since two years treated initially on enalapril and later on Calcium antagonists, due to onset of cough, suspecting asthma. Moreover since two years dyspnoea for moderate efforts, cough and breathlessness on exertion misdiagnosed for asthma or side effects of ACE inhibitor. abdominal pain, oedema of left leg misdiagnosed for relapsing phlebitis. She appeared very suffering with increasing dyspnoea. Normal BP (110/70 mmHg), EKG, HR (90 b/m), T 37°C. At EGA decrease of pO2 (68%). Respiratory evaluation pointed out hypophonesis in right and left basal pulmonary area with absence of vesicular breathing. Chest-X-ray, urgently executed, showed the presence of bilateral copious pleural effusion. Thoracentesis and laboratory test revealed it was chylous (milky white colour, pH alkaline, Rivalta - - -, proteins 2,8 g/dl, cholesterol 53 mg/dl, triglycerides 200 mg/dl). Laboratory data pointed out increase of LDH (1.253 U/L) and ACE (50/L). Chest and abdomen-HRCT scanning revealed the presence of walled cysts 2-20 mm in diameter distributed through the lung fields, renal angiomyolipomas and uterine fibroids (leiomyomas). The patient underwent to thoracic duct correction, pleurodesis, chest and lymph nodes biopsies with evidence of LAM cells and diagnosis of “lymphangioleiomyomatosis”, total pleurectomy, ovariectomy and isterectomy. Twelve months later she was on permant O2 therapy. Discussion: LAM is a devastating disease with bad prognosis (death within 10 years from onset!). Recently the research has helped to define the genetic and immunohistochemical characteristics of LAM cells and surely it will soon lead to more effective treatments (rapamicin, sirolimus, anti-EGF. . . ), but nowadays, the treatment remains only symptomatic on surgery, hormones, oxygen, pulmonary transplantation with possible relapses. Early diagnosis it is necessary, conditioning a different outcome in these unlikely women
SA-35 TWO CASE REPORTS OF CHYLOTHORAX DUE TO LYMPHANGIOLEIOMYOMATOSIS
SA-36
Giovita A. Piccillo, Aurelio Pantò, Tommaso Nicolosi, Filippo Fraggetta, Enrico G.M. Mondati, Riccardo Polosa, Luca Miele, Giovanni Gasbarrini. Department of Emergency Medicine, Cannizzaro Hospital, Catania, Italy
Giovita A. Piccillo, Aurelio Pantò, Salvatore A. Azzarelli, Enrico G.M. Mondati, Riccardo Polosa, Luca Miele, Giovanni Gasbarrini. Department of Emergency Medicine, Cannizzaro Hospital, Catania, Italy
Introduction: Lymphangioleiomyomatosis (LAM), first described in 1937 by von Stossel, is a rare disease of unknown origin affecting women of childbearing age and patients of both sexes struck by tuberous sclerosis, characterised by hamartomatous proliferation of smooth muscle in the lungs, mediastinum and abdomen. The reported prevalence of LAM is around one per million, although the true prevalence is likely to be greater. LAM is almost exclusively confined to women the mean age of onset being 34 years and presentation after the menopause is very unusual. While LAM is a systemic disease, the main manifestations are pulmonary. In the lungs the abnormal proliferation is seen around airways, blood vessels, and lymphatics. It extends into the alveolar interstitium causing cystic change and into pulmonary veins causing lung haemorrhage. Lymph node involvement leads to chylous effusion. Extrapulmonary features include renal angiomyolipomas and lymphangioleiomyomas. Clinically it is characterised by increasing chest pain, dyspnoea, breathlessness, hemoptysis, chylothorax and pneumothorax. Case Report 1: 1 A 22-year-old female patient was admitted to our Dept for acute dyspnoea, cough and chest pain. The patient appeared very pale and suffering, dyspnoic, polypnoic, extremely anxious, with cramps to upper and lower limbs. Normal BP (110/70 mmHg), EKG, HR (90 b/m), T 37°C. At EGA decrease of pO2 (68%). Respiratory clinical evaluation revealed hypophonesis in left basal pulmonary area coherent with absence of vesicular breathing. Chest-X-ray, immediately executed, pointed out the presence of important left pleural effusion. Thoracentesis revealed milky white fluid in chest cavity which laboratory test confirmed to be chyle (milky white colour, pH alkaline, Rivalta - - -, proteins 2,5 g/dl, cholesterol 65 mg/dl, triglycerides 110 mg/dl). Laboratory data pointed out moderate anaemia (RBC 3.780.000/mmc, Hgb 11.4 g/dl, Hct (30.6%), MCV (80.3 fL)), increase of LDH (1.086 U/L) and increase of ACE (40 micron/L); decrease of total proteins (5.6 g/dL) and Ca2+ (7.6 mg/dL). Chest-high resolution computerised tomography (HRCT) scanning showed the presence of dilated thoracic duct and few thin-walled cysts in mid and lower lungs and abdomen-CT revealed the presence of ovarian polycystosis. On the grounds of these findings, we suspected LAM and submitted our patient to thoracic duct correction, pleurodesis, chest and lymph nodes biopsies with evidence of LAM cells and diagnosed “lymphangioleiomyomatosis”. The patient was treated on total pleurectomy and lymphadenectomy L1-L5 and oral progesterone. Twelve months later she was in good clinical conditions and underwent to ovariectomy.
Introduction: Broken Heart Syndrome, also called Apical Ballooning Syndrome (ABS), is a clinical entity characterised by transient abnormal wall motion of the mid and apical segments of the left ventricle resulting in the apical ballooning, absence of significant coronary artery disease, and new ST-T electrocardiographic abnormalities more prevalent among women than men (7:1). Japanese Authors prefer the term Tako-Tsubo for this cardiomyopathy, due to the resemblance of the end-systolic left ventricular angiogram to an octopus (tako) trap (tsubo). Moreover this pathology is also known as Stress Cardiomyopathy, since it is often triggered by an emotional or physical stress (sudden accident, death/funeral of a family member, excessive exercise, quarreling, excessive alcohol consumption or great excitation). The ABS mimics an acute coronary syndrome with anginal chest pain, ischemic ekg alterations, increase in creatine kinasi (CPK) and/or troponin (TPN). Case Report: A female 72-year-old woman, without cardiovascular risk factors, was admitted to our Department because of persistent anginal pain after the death of her son due to cancer. Her physical examination was unremarkable; BP 145/95 mmHg; HR 60 beats/min; the EKG showed 1 mm ST segment elevation in II, III, avF, V3-V6 leads and subsequent electrocardiogram showed T wave inversion in the same leads. Chest pain was relieved with sublingual nitrate, aspirin and heparin. Then, she was transferred to the coronary care unit with a diagnosis of suspected acute coronary syndrome. The angiography, perfomed 4 h after starting chest pain, showed the so called “apical ballooning” during systole, in absence of obstructive coronary artery disease. Serial blood samples pointed out the so-called “apical ballooning” during systole, in absence of obstructive coronary disease. Serial blood samples revealed a peak of troponin I of 7.3 ng/dl (normal<0.4 ng/dl); IgM antibody assay against the following microbes influenza type A (H1N1), influenza type A (H3N2), influenza type B, parainfluenza type 1, parainfluenza type 2, cytomegalovirus, coxackie type B1, coxackie type A16, echo type 7, adenovirus type 3, Chlamidia pneumoniae, parotite, Mycoplasma pneumoniae, Borrelia garinii, Borrelia burgdorferi, was negative. The myocardial contrast echocardiogram performed with written consensus at day 6 showed a large perfusion defect in the akinetic apical region of the left ventricle. At 1-month follow-up myocardial perfusion and wall motion became completely normal. At day 8 the patient was discharged home on aspirin and Ace-inhibitor. Three months after she remained asymptomatic.
A CASE REPORT OF BROKEN HEART SYNDROME
Oral Communications / European Journal of Internal Medicine 19S (2008), S1–S59
The Case: a 50 years old man with type 2 diabetes and severe dilated cardiomyopathy with systolic dysfunction (ejection fraction of left ventricle 20%), NYHA class IV, was admitted in our Hospital for many times in the last years for symptoms related to his chronic congestive cardiac failure. Each time were present dyspnoea at rest with orthopnoea, distal oedema, hepatomegaly, congestion and enlarged cardiac shadow at chest radiogram. Electrocardiogram reported sinus rhythm with Left bundle branch block, always negative cardiac enzymes, arterial blood gases showed a trend to respiratory alkalosis with substantially normal PaO2 value also breathing ambient air. Each admission resulted in a long hospitalisation, with only little improvements due to standard medical treatment including inotropic agents, high doses of diuretics, nitrates, beta blockers, ace inhibitors, oxygen. It seemed to be an end stage heart failure with perhaps a chance of transplantation. During the last admission we observed that the patient developed a periodic breathing with various episodes of nightly and daily central apnoea. In addition to standard aggressive medical therapy we decided to treat the patient with CPAP 7.5 cmH2 O and low FiO2 (28%), with a Venturi-Like Generator Flow (Whisper Flow – Caradine) and a facial mask: the patient performed 10-12 hours/day of CPAP treatment divided into 3-4 cycles of 3-4 hours each, nightly and daily. Since the first day of treatment he reported a significant improvement in sensation of dyspnoea, after few days of treatment the respiratory pattern sowed a decrease of apnoeas and the patient was able to arise and perform personal care (NYHA class III); after 15 days of treatment oedema was lowered, a chest radiogram showed reduction of congestion, echocardiography demonstrated an improvement of ejection fraction of left ventricle (33%). In this patient a long term treatment with nightly electric nasal CPAP at home may be useful in order to improve quality of life and decrease hospitalisation.
Case Report 2: 2 A 42-year-old female patient was admitted to our Division because of acute dyspnoea, cough and chest pain. At history menarche at fourteen years with regular rhythm and flow; hypertension since two years treated initially on enalapril and later on Calcium antagonists, due to onset of cough, suspecting asthma. Moreover since two years dyspnoea for moderate efforts, cough and breathlessness on exertion misdiagnosed for asthma or side effects of ACE inhibitor. abdominal pain, oedema of left leg misdiagnosed for relapsing phlebitis. She appeared very suffering with increasing dyspnoea. Normal BP (110/70 mmHg), EKG, HR (90 b/m), T 37°C. At EGA decrease of pO2 (68%). Respiratory evaluation pointed out hypophonesis in right and left basal pulmonary area with absence of vesicular breathing. Chest-X-ray, urgently executed, showed the presence of bilateral copious pleural effusion. Thoracentesis and laboratory test revealed it was chylous (milky white colour, pH alkaline, Rivalta - - -, proteins 2,8 g/dl, cholesterol 53 mg/dl, triglycerides 200 mg/dl). Laboratory data pointed out increase of LDH (1.253 U/L) and ACE (50/L). Chest and abdomen-HRCT scanning revealed the presence of walled cysts 2-20 mm in diameter distributed through the lung fields, renal angiomyolipomas and uterine fibroids (leiomyomas). The patient underwent to thoracic duct correction, pleurodesis, chest and lymph nodes biopsies with evidence of LAM cells and diagnosis of “lymphangioleiomyomatosis”, total pleurectomy, ovariectomy and isterectomy. Twelve months later she was on permant O2 therapy. Discussion: LAM is a devastating disease with bad prognosis (death within 10 years from onset!). Recently the research has helped to define the genetic and immunohistochemical characteristics of LAM cells and surely it will soon lead to more effective treatments (rapamicin, sirolimus, anti-EGF. . . ), but nowadays, the treatment remains only symptomatic on surgery, hormones, oxygen, pulmonary transplantation with possible relapses. Early diagnosis it is necessary, conditioning a different outcome in these unlikely women
SA-35 TWO CASE REPORTS OF CHYLOTHORAX DUE TO LYMPHANGIOLEIOMYOMATOSIS
SA-36
Giovita A. Piccillo, Aurelio Pantò, Tommaso Nicolosi, Filippo Fraggetta, Enrico G.M. Mondati, Riccardo Polosa, Luca Miele, Giovanni Gasbarrini. Department of Emergency Medicine, Cannizzaro Hospital, Catania, Italy
Giovita A. Piccillo, Aurelio Pantò, Salvatore A. Azzarelli, Enrico G.M. Mondati, Riccardo Polosa, Luca Miele, Giovanni Gasbarrini. Department of Emergency Medicine, Cannizzaro Hospital, Catania, Italy
Introduction: Lymphangioleiomyomatosis (LAM), first described in 1937 by von Stossel, is a rare disease of unknown origin affecting women of childbearing age and patients of both sexes struck by tuberous sclerosis, characterised by hamartomatous proliferation of smooth muscle in the lungs, mediastinum and abdomen. The reported prevalence of LAM is around one per million, although the true prevalence is likely to be greater. LAM is almost exclusively confined to women the mean age of onset being 34 years and presentation after the menopause is very unusual. While LAM is a systemic disease, the main manifestations are pulmonary. In the lungs the abnormal proliferation is seen around airways, blood vessels, and lymphatics. It extends into the alveolar interstitium causing cystic change and into pulmonary veins causing lung haemorrhage. Lymph node involvement leads to chylous effusion. Extrapulmonary features include renal angiomyolipomas and lymphangioleiomyomas. Clinically it is characterised by increasing chest pain, dyspnoea, breathlessness, hemoptysis, chylothorax and pneumothorax. Case Report 1: 1 A 22-year-old female patient was admitted to our Dept for acute dyspnoea, cough and chest pain. The patient appeared very pale and suffering, dyspnoic, polypnoic, extremely anxious, with cramps to upper and lower limbs. Normal BP (110/70 mmHg), EKG, HR (90 b/m), T 37°C. At EGA decrease of pO2 (68%). Respiratory clinical evaluation revealed hypophonesis in left basal pulmonary area coherent with absence of vesicular breathing. Chest-X-ray, immediately executed, pointed out the presence of important left pleural effusion. Thoracentesis revealed milky white fluid in chest cavity which laboratory test confirmed to be chyle (milky white colour, pH alkaline, Rivalta - - -, proteins 2,5 g/dl, cholesterol 65 mg/dl, triglycerides 110 mg/dl). Laboratory data pointed out moderate anaemia (RBC 3.780.000/mmc, Hgb 11.4 g/dl, Hct (30.6%), MCV (80.3 fL)), increase of LDH (1.086 U/L) and increase of ACE (40 micron/L); decrease of total proteins (5.6 g/dL) and Ca2+ (7.6 mg/dL). Chest-high resolution computerised tomography (HRCT) scanning showed the presence of dilated thoracic duct and few thin-walled cysts in mid and lower lungs and abdomen-CT revealed the presence of ovarian polycystosis. On the grounds of these findings, we suspected LAM and submitted our patient to thoracic duct correction, pleurodesis, chest and lymph nodes biopsies with evidence of LAM cells and diagnosed “lymphangioleiomyomatosis”. The patient was treated on total pleurectomy and lymphadenectomy L1-L5 and oral progesterone. Twelve months later she was in good clinical conditions and underwent to ovariectomy.
Introduction: Broken Heart Syndrome, also called Apical Ballooning Syndrome (ABS), is a clinical entity characterised by transient abnormal wall motion of the mid and apical segments of the left ventricle resulting in the apical ballooning, absence of significant coronary artery disease, and new ST-T electrocardiographic abnormalities more prevalent among women than men (7:1). Japanese Authors prefer the term Tako-Tsubo for this cardiomyopathy, due to the resemblance of the end-systolic left ventricular angiogram to an octopus (tako) trap (tsubo). Moreover this pathology is also known as Stress Cardiomyopathy, since it is often triggered by an emotional or physical stress (sudden accident, death/funeral of a family member, excessive exercise, quarreling, excessive alcohol consumption or great excitation). The ABS mimics an acute coronary syndrome with anginal chest pain, ischemic ekg alterations, increase in creatine kinasi (CPK) and/or troponin (TPN). Case Report: A female 72-year-old woman, without cardiovascular risk factors, was admitted to our Department because of persistent anginal pain after the death of her son due to cancer. Her physical examination was unremarkable; BP 145/95 mmHg; HR 60 beats/min; the EKG showed 1 mm ST segment elevation in II, III, avF, V3-V6 leads and subsequent electrocardiogram showed T wave inversion in the same leads. Chest pain was relieved with sublingual nitrate, aspirin and heparin. Then, she was transferred to the coronary care unit with a diagnosis of suspected acute coronary syndrome. The angiography, perfomed 4 h after starting chest pain, showed the so called “apical ballooning” during systole, in absence of obstructive coronary artery disease. Serial blood samples pointed out the so-called “apical ballooning” during systole, in absence of obstructive coronary disease. Serial blood samples revealed a peak of troponin I of 7.3 ng/dl (normal<0.4 ng/dl); IgM antibody assay against the following microbes influenza type A (H1N1), influenza type A (H3N2), influenza type B, parainfluenza type 1, parainfluenza type 2, cytomegalovirus, coxackie type B1, coxackie type A16, echo type 7, adenovirus type 3, Chlamidia pneumoniae, parotite, Mycoplasma pneumoniae, Borrelia garinii, Borrelia burgdorferi, was negative. The myocardial contrast echocardiogram performed with written consensus at day 6 showed a large perfusion defect in the akinetic apical region of the left ventricle. At 1-month follow-up myocardial perfusion and wall motion became completely normal. At day 8 the patient was discharged home on aspirin and Ace-inhibitor. Three months after she remained asymptomatic.
A CASE REPORT OF BROKEN HEART SYNDROME