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Massive pericardial effusion in a neonate
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 5 ( 2 0 1 5 ) 2 2 8 e2 3 0
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/jicc
Case Report
Massive pericardial effusion in a neonate Jayaprasad Narayanapillai a,*, Suresh Madhavan a, Paul Thomas b a b
Assistant Professor, Department of Cardiology, Govt Medical College, Kottayam, Kerala 686008, India Senior Resident, Department of Cardiology, Govt Medical College, Kottayam, Kerala 686008, India
article info
abstract
Article history:
In the absence of hydrops or sepsis, pericardial effusion is a rare occurrence in a neonate.
Received 19 March 2015
We present the case report of a neonate with massive pericardial effusion managed suc-
Accepted 16 April 2015
cessfully with pericardiocentesis. Further evaluation revealed an unusual presentation of
Available online 6 May 2015
Down syndrome known as transient abnormal myelopoiesis (TAM). Copyright © 2015, Indian College of Cardiology. All rights reserved.
Keywords: Neonatal pericardial effusion Abnormal myelopoiesis Transient leukaemia Down syndrome
1.
Introduction
Pericardial effusion is encountered only very rarely in the neonatal period. Most of the cases occur secondary to foetal hydrops or as complication of central venous catheters. We present a unique syndrome in a neonate presenting with massive pericardial effusion.
2.
Case report
A 4 day old male baby was referred from paediatric department for evaluation of respiratory distress. The baby was
born by full term normal delivery to a 30 year old primi. Mother did not give any history of drug intake or significant illness including infections during the antenatal period. On examination baby was tachypneic. There was no cyanosis. Heart sounds were normal and there were no murmurs. ECG showed sinus tachycardia. Chest radiograph showed mild cardiac enlargement. Two dimensional echocardiogram showed mild pericardial effusion (5 mm) and normal intra cardiac anatomy without any features of tamponade. There was no evidence of cardiac failure or pulmonary hypertension. Baby was treated with supportive measures and kept under follow up. Haematological and biochemical evaluation for the cause of pericardial effusion was advised. After about one week, child developed severe respiratory distress and
* Corresponding author. Tel.: þ91 9446723547. E-mail address: [email protected] (J. Narayanapillai). http://dx.doi.org/10.1016/j.jicc.2015.04.002 1561-8811/Copyright © 2015, Indian College of Cardiology. All rights reserved.
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 5 ( 2 0 1 5 ) 2 2 8 e2 3 0
Fig. 1 e Two dimensional echocardiogram subcostal view showing massive pericardial effusion.
showed normocytic normochromic red blood cells. Thirty per cent of leucocytes were atypical cells with a high nuclear cytoplasmic (N/C) ratio and moderate basophilic cytoplasm suggestive of blast cells (Fig. 2). Platelets were reduced in number. As the child had haematological abnormalities and pericardial effusion karyotyping was advised. Chromosomal analysis (GTG banding with 500 band resolution) revealed male karyotype with trisomy 21 pattern in all the metaphases analysed (Fig. 3). This chromosomal complement confirms the diagnosis of Down syndrome. Echocardiogram was repeated on post natal day 14 and 21 and there was no recurrence of pericardial effusion. Peripheral smear examination on post natal day 28 showed only 2% blasts and baby was asymptomatic. We made a final diagnosis of Down syndrome with transient leukaemia presenting as massive pericardial effusion in this neonate.
3. tachypnoea. Arterial pulse was rapid and thready. Heart sounds were muffled. Chest radiograph revealed massive cardiomegaly. Echo showed massive pericardial effusion with features of cardiac tamponade (Fig. 1). The baby was taken up for emergency pericardiocentesis under sedation. A 7 French Arrow multi lumen access catheter was used to aspirate about 100 ml of straw coloured fluid. Pericardial fluid was exudative with about 400 cells/mm, 60% polymorphs and 40% lymphocytes. Extensive evaluation for the cause of pericardial effusion was done. Renal, hepatic and thyroid function tests were normal. His haemogram revealed leucocytosis and mild thrombocytopenia. Peripheral smear
Fig. 2 e Peripheral smear examination showing normocytic normochromic RBCs and blast cells and reduced number of platelets.
229
Discussion
In the absence of hydrops or sepsis, pericardial effusion is a rare occurrence in the neonate. Neonatal pericardial effusion and cardiac tamponade are uncommon but potentially fatal complications of percutaneous, umbilical and surgically placed central venous catheters.1 Other conditions associated with neonatal pericardial effusion without hydrops include intracardiac and pericardial tumours and viral pericarditis.2 Neonates with Down syndrome can sometimes present with pericardial effusion. Approximately 10% of Down syndrome neonates develop a unique syndrome characterised by transient abnormal myelopoiesis (TAM) or transient leukaemia.3 Although it may occur during foetal life, it mostly presents after birth. During gestation it usually presents with hydrops fetalis.4 After birth presentation may vary from asymptomatic as an incidental finding of circulating blasts in the blood to a severe and potentially lethal illness with hydrops, leucocytosis with circulating blasts, skin infiltrates, exudative effusions (pleural, pericardial and ascites), respiratory distress and hepatomegaly.5 Massive pericardial effusion in a neonate is a potentially fatal complication which needs timely recognition and prompt intervention. Treatment is by percutaneous pericardiocentesis or pericardial window operation. Our patient had trisomy 21 and presented with typical haematological abnormalities and massive pericardial effusion. Patient was treated with pericardiocentesis and there was no recurrence. In most cases, TAM resolves spontaneously within 3 months after birth.6 However, symptomatic babies, especially those with high blast counts or liver dysfunction may benefit from low-dose cytosine arabinoside (10e20 mg/ m2/day for up to 7 days). Despite resolution in most cases of TAM, up to 20% of infants who present to haematology centres still die of disease.5 In our case the abnormalities spontaneously resolved on follow up. The importance of TAM is its potential to transform into an acute leukaemia, known as myeloid leukaemia of Down syndrome which is estimated to
230
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 5 ( 2 0 1 5 ) 2 2 8 e2 3 0
Fig. 3 e Chromosomal analysis (GTG banding with 500 band resolution) showing male karyotype with trisomy 21 pattern suggestive of Down syndrome.
occur in 20e30% of babies with TAM, although the exact frequency is not known.6 2.
Conflicts of interest
3.
All authors have none to declare.
4.
references
1. Beardsall K, White DK, Pinto EM, Kelsall AWR. Pericardial effusion and cardiac tamponade as complications of neonatal
5.
6.
long lines: are they really a problem? Arch Dis Child Fetal Neonatal. 2007;88:F292eF295. Cartagena AM, Levin TL, Issenberg H, Goldman HS. Pericardial effusion and cardiac hemangioma in the neonate. Pediatr Radiol. 1993;23:384e385. Isaacs Jr H. Fetal and neonatal leukemia. J Pediatr Hematol Oncol. 2003;25:348e361. Zipursky A. Transient leukaemiada benign form of leukaemia in newborn infants with trisomy 21. Br J Haematol. 2003;120:930e938. Massey GV, Zipursky A, Chang MN, et al. A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children's Oncology Group (COG) study POG-9481. Blood. 2006;107:4606e4613. Webb David, Roberts Irene, Vyas Paresh. Haematology of Down syndrome. Arch Dis Child Fetal Neonatal. 2007;92:F503eF507.
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/jicc
Case Report
Massive pericardial effusion in a neonate Jayaprasad Narayanapillai a,*, Suresh Madhavan a, Paul Thomas b a b
Assistant Professor, Department of Cardiology, Govt Medical College, Kottayam, Kerala 686008, India Senior Resident, Department of Cardiology, Govt Medical College, Kottayam, Kerala 686008, India
article info
abstract
Article history:
In the absence of hydrops or sepsis, pericardial effusion is a rare occurrence in a neonate.
Received 19 March 2015
We present the case report of a neonate with massive pericardial effusion managed suc-
Accepted 16 April 2015
cessfully with pericardiocentesis. Further evaluation revealed an unusual presentation of
Available online 6 May 2015
Down syndrome known as transient abnormal myelopoiesis (TAM). Copyright © 2015, Indian College of Cardiology. All rights reserved.
Keywords: Neonatal pericardial effusion Abnormal myelopoiesis Transient leukaemia Down syndrome
1.
Introduction
Pericardial effusion is encountered only very rarely in the neonatal period. Most of the cases occur secondary to foetal hydrops or as complication of central venous catheters. We present a unique syndrome in a neonate presenting with massive pericardial effusion.
2.
Case report
A 4 day old male baby was referred from paediatric department for evaluation of respiratory distress. The baby was
born by full term normal delivery to a 30 year old primi. Mother did not give any history of drug intake or significant illness including infections during the antenatal period. On examination baby was tachypneic. There was no cyanosis. Heart sounds were normal and there were no murmurs. ECG showed sinus tachycardia. Chest radiograph showed mild cardiac enlargement. Two dimensional echocardiogram showed mild pericardial effusion (5 mm) and normal intra cardiac anatomy without any features of tamponade. There was no evidence of cardiac failure or pulmonary hypertension. Baby was treated with supportive measures and kept under follow up. Haematological and biochemical evaluation for the cause of pericardial effusion was advised. After about one week, child developed severe respiratory distress and
* Corresponding author. Tel.: þ91 9446723547. E-mail address: [email protected] (J. Narayanapillai). http://dx.doi.org/10.1016/j.jicc.2015.04.002 1561-8811/Copyright © 2015, Indian College of Cardiology. All rights reserved.
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 5 ( 2 0 1 5 ) 2 2 8 e2 3 0
Fig. 1 e Two dimensional echocardiogram subcostal view showing massive pericardial effusion.
showed normocytic normochromic red blood cells. Thirty per cent of leucocytes were atypical cells with a high nuclear cytoplasmic (N/C) ratio and moderate basophilic cytoplasm suggestive of blast cells (Fig. 2). Platelets were reduced in number. As the child had haematological abnormalities and pericardial effusion karyotyping was advised. Chromosomal analysis (GTG banding with 500 band resolution) revealed male karyotype with trisomy 21 pattern in all the metaphases analysed (Fig. 3). This chromosomal complement confirms the diagnosis of Down syndrome. Echocardiogram was repeated on post natal day 14 and 21 and there was no recurrence of pericardial effusion. Peripheral smear examination on post natal day 28 showed only 2% blasts and baby was asymptomatic. We made a final diagnosis of Down syndrome with transient leukaemia presenting as massive pericardial effusion in this neonate.
3. tachypnoea. Arterial pulse was rapid and thready. Heart sounds were muffled. Chest radiograph revealed massive cardiomegaly. Echo showed massive pericardial effusion with features of cardiac tamponade (Fig. 1). The baby was taken up for emergency pericardiocentesis under sedation. A 7 French Arrow multi lumen access catheter was used to aspirate about 100 ml of straw coloured fluid. Pericardial fluid was exudative with about 400 cells/mm, 60% polymorphs and 40% lymphocytes. Extensive evaluation for the cause of pericardial effusion was done. Renal, hepatic and thyroid function tests were normal. His haemogram revealed leucocytosis and mild thrombocytopenia. Peripheral smear
Fig. 2 e Peripheral smear examination showing normocytic normochromic RBCs and blast cells and reduced number of platelets.
229
Discussion
In the absence of hydrops or sepsis, pericardial effusion is a rare occurrence in the neonate. Neonatal pericardial effusion and cardiac tamponade are uncommon but potentially fatal complications of percutaneous, umbilical and surgically placed central venous catheters.1 Other conditions associated with neonatal pericardial effusion without hydrops include intracardiac and pericardial tumours and viral pericarditis.2 Neonates with Down syndrome can sometimes present with pericardial effusion. Approximately 10% of Down syndrome neonates develop a unique syndrome characterised by transient abnormal myelopoiesis (TAM) or transient leukaemia.3 Although it may occur during foetal life, it mostly presents after birth. During gestation it usually presents with hydrops fetalis.4 After birth presentation may vary from asymptomatic as an incidental finding of circulating blasts in the blood to a severe and potentially lethal illness with hydrops, leucocytosis with circulating blasts, skin infiltrates, exudative effusions (pleural, pericardial and ascites), respiratory distress and hepatomegaly.5 Massive pericardial effusion in a neonate is a potentially fatal complication which needs timely recognition and prompt intervention. Treatment is by percutaneous pericardiocentesis or pericardial window operation. Our patient had trisomy 21 and presented with typical haematological abnormalities and massive pericardial effusion. Patient was treated with pericardiocentesis and there was no recurrence. In most cases, TAM resolves spontaneously within 3 months after birth.6 However, symptomatic babies, especially those with high blast counts or liver dysfunction may benefit from low-dose cytosine arabinoside (10e20 mg/ m2/day for up to 7 days). Despite resolution in most cases of TAM, up to 20% of infants who present to haematology centres still die of disease.5 In our case the abnormalities spontaneously resolved on follow up. The importance of TAM is its potential to transform into an acute leukaemia, known as myeloid leukaemia of Down syndrome which is estimated to
230
j o u r n a l o f i n d i a n c o l l e g e o f c a r d i o l o g y 5 ( 2 0 1 5 ) 2 2 8 e2 3 0
Fig. 3 e Chromosomal analysis (GTG banding with 500 band resolution) showing male karyotype with trisomy 21 pattern suggestive of Down syndrome.
occur in 20e30% of babies with TAM, although the exact frequency is not known.6 2.
Conflicts of interest
3.
All authors have none to declare.
4.
references
1. Beardsall K, White DK, Pinto EM, Kelsall AWR. Pericardial effusion and cardiac tamponade as complications of neonatal
5.
6.
long lines: are they really a problem? Arch Dis Child Fetal Neonatal. 2007;88:F292eF295. Cartagena AM, Levin TL, Issenberg H, Goldman HS. Pericardial effusion and cardiac hemangioma in the neonate. Pediatr Radiol. 1993;23:384e385. Isaacs Jr H. Fetal and neonatal leukemia. J Pediatr Hematol Oncol. 2003;25:348e361. Zipursky A. Transient leukaemiada benign form of leukaemia in newborn infants with trisomy 21. Br J Haematol. 2003;120:930e938. Massey GV, Zipursky A, Chang MN, et al. A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children's Oncology Group (COG) study POG-9481. Blood. 2006;107:4606e4613. Webb David, Roberts Irene, Vyas Paresh. Haematology of Down syndrome. Arch Dis Child Fetal Neonatal. 2007;92:F503eF507.