1
Introduction The primary cardiac tumor (PCT) is a rare disease in children, with a frequency between 0.03-0.32 %. Eighty percent are benign tumors. Usually they are asymptomatic and discovered fortuitously. Conclusion Children's cardiac tumors are usually benign. The severity is related to functional consequences to which depends the support. Surgery is the best treatment with an excellent prognosis. Discussion Fetal primary cardiac tumors (FPCT) are uncommon and discovered by widely use of prenatal echocardiography[1]. Benign tumors are the most frequent[2,3] in children as well as in fetuses. cardiac rhabdomyomas, teratomas, and fibromas remained the three most common types.[4] Rhabdomyoma is the most common PCT in fetal life and childhood. It involve the left and right ventricles and ventricular septum. They often grow into the intracavity of the cardiac chambers this is the case of patients 1 and 2. They are associated with tuberous sclerosis in up to 50% of cases [6,7] wich may be the case of youssef who showed convulsions type bending spasms. Usually PCT are asymptomatic and discovered fortuitously but they may cause intracardiac flow obstruction, heart valve insufficiency, arrhythmia [8], heart failure, and hydrops fetalis, or even sudden fetal death[4]. This joins our results since 2/4 patients are asymptomatic. Cardiac ultrasound is paramount in the prenatal diagnosis but there is misdiagnosis. Recalling the echogenic foci in the ventricles, particularly those in the papillary muscles, can be hypertrophic and may sometimes mimic rhabdomyomas, according to Yuan S-M.So ,cardiac MRI is an important complementary modality for characterization of the mass and effect on cardiac function [9]. Histological study of the tumor (biopsy or surgical specimen) remains the best way to confirm the Diagnosis[3]. Therapeutic management of PCT depends on its location, size,number, and complications. Surgery is the solution for tumors resonating on the haemodynamic state. A clinical follow-up may be prescribed in case of rhabdomyoma which may regress spontaneously [11]. Everolimus an mTOR inhibitors which proved its effectiveness for the treatment of different clinical manifestations of tuberosis sclerosis remains the therapeutic future in case of rabdomyoma[12]. Cardiac tumors in newborn: five case report Ayari F ,Bensmail T. , Boussetta A , Ksibi I ,Cheour M , Ben Amara M , Kacem S Neonatology Care Unit Center of Tunis, Tunisia Objectives To recall the clinical and sonographic features of primitive and benign cardiac tumors in children To cite complications through observations of 5 newborns. Observations Here is a table summarizing the clinical and ultrasound characteristics of our patients: Patient medical background Prenatal ultrsound Postnatal Ultrsound resounding others Leith (1) -At term -by vaginal route -eutrophic -good Apgar CT evoking a left intraventricula r rhabdomyoma In the aorta which partially obstructed Asymptomatic Taher (2) -At term -by vaginal route -eutrophic -good Apgar Biventricular Rhabdomyoma (Figure 1) Multiple biventricular whose largest was opposite to the large mitral valve Supraventricul ar tachycardia quick to 300 bpm (Figure 4) Youssef (3) -At term -by vaginal route -eutrophic -good Apgar right ventricular fibroma (Figure 2) In the trabecular room of the right ventricle Asymptomatic Bending spasms evoking a tuberous sclerosis Khaled (4) -At term -by vaginal route -eutrophic -good Apgar two hyperechoic spots 2 mm each depends on the valvular pillars in the left ventricle Nothing Eya (5) -caesarean section -intrauterine growth retardation tumor taking all the right ventricle Aspect of trabeculation Death by refractory hypoxemia (Autopsy: figure 3) total agenesis of the corpus callosum. Figure 1:Three-dimensional echocardiograms using a matrix array transducer: Large right and left ventricular rhabdomyomas Figure 2 :Spin-echo MRI from a child with a large right ventricular fibroma Figure 3: Microscopic features of the Tumor in the autopsy: spider cells Figure 4: Supra Ventricular tachycardia in electrocardiogram References: 1. Mun˜oz H, Sherer DM, Romero R, Sanchez J, Hernandez I, Diaz C. Prenatal sonographic findings of a large fetal cardiac fibroma. J Ultrasound Med 1995;14:479e81. 2. Uzun Orhan, Wilson DG, Vujanic GM, Parsons JM, Giovanni JV. Cardiac tumours in children. Orph J of Rare Diseases 2007;2 (11):1-14. 3. Castro FJ, Escudero. Tumores cardíacos. Protocolos diagnósticos y terapêuticos en Cardiología Pediátrica. Available at:http://www.aepe.es/. 5. Carrilho MC, Tonni G, Araujo Ju´nior E. Fetal cardiac tumors: prenatal diagnosis and outcomes. Rev Bras Cir Cardiovasc 2015;30:VIeVII. 4. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004;25:252e73. 6. Yu K, Liu Y, Wang H, Hu S, Long C. Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact CardioVasc Thorac Surg 2007;6:636-9. 7. Cruz ER, Maldonado RMC. Cardiac Tumors. Available at: http://www.emedicine.com/. 8. Ka Myers, KK Wong, M tipple, S sanatani. Benign cardiac tumours, malignant arrhythmias. Can J Cardiol 2010;26(2):e58-e61. 9. Ghadmi Mahani M, Lu JC, Rigsby CK, Krishnamurthy R, Dorfman AL, Agarwal PP. MRI of pediatric cardiac masses. AJR Am J Roentgenol. 2014 May;202(5):971-81. doi: 10.2214/AJR.13.10680. 10.Yuan S-M, Fetal Primary Cardiac Tumors During Perinatal Period, Pediatrics and Neonatology (2016), http://dx.doi.org/10.1016/j.pedneo.2016.07.004. 11. Liu X, Hong H, Zhang H, Xu Z, Liu J, Qiu L. Treatment Strategies for Primary Tumors of the Heart in Children: A 10-Year Experience. Ann thorac surg. 2015 Nov;100(5):1744-9. doi: 10.1016/j.athoracsur.2015.06.030. Epub 2015 Sep 11. 12. Mohamed I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-205138.

Cardiac tumors in newborn: five case report · Introduction The primary cardiac tumor (PCT) is a rare disease in children, with a frequency between 0.03-0.32 %. Eighty percent are

  • Upload
    others

  • View
    0

  • Download
    0

Embed Size (px)

Citation preview

Page 1: Cardiac tumors in newborn: five case report · Introduction The primary cardiac tumor (PCT) is a rare disease in children, with a frequency between 0.03-0.32 %. Eighty percent are

Introduction The primary cardiac tumor (PCT) is a rare disease in

children, with a frequency between 0.03-0.32 %. Eighty percent are benign tumors. Usually they are

asymptomatic and discovered fortuitously.

Conclusion Children's cardiac tumors are usually benign.

The severity is related to functional consequences to which depends the support. Surgery is the best

treatment with an excellent prognosis.

Discussion

•Fetal primary cardiac tumors (FPCT) are uncommon and discovered by widely use of prenatal echocardiography[1]. • Benign tumors are the most frequent[2,3] in children as well as in fetuses. • cardiac rhabdomyomas, teratomas, and fibromas remained the three most common types.[4]

Rhabdomyoma is the most common PCT in fetal life and childhood. It involve the left and right ventricles and ventricular septum. They often grow into the intracavity of the cardiac chambers this is the case of patients 1 and 2. They are associated with tuberous sclerosis in up to 50% of cases [6,7] wich may be the case of youssef who showed convulsions type bending spasms. Usually PCT are asymptomatic and discovered fortuitously but they may cause intracardiac flow obstruction, heart valve insufficiency, arrhythmia [8], heart failure, and hydrops fetalis, or even sudden fetal death[4]. This joins our results since 2/4 patients are asymptomatic. Cardiac ultrasound is paramount in the prenatal diagnosis but there is misdiagnosis. Recalling the echogenic foci in the ventricles, particularly those in the papillary muscles, can be hypertrophic and may sometimes mimic rhabdomyomas, according to Yuan S-M.So ,cardiac MRI is an important complementary modality for characterization of the mass and effect on cardiac function [9]. Histological study of the tumor (biopsy or surgical specimen) remains the best way to confirm the Diagnosis[3]. Therapeutic management of PCT depends on its location, size,number, and complications. Surgery is the solution for tumors resonating on the haemodynamic state. A clinical follow-up may be prescribed in case of rhabdomyoma which may regress spontaneously [11]. Everolimus an mTOR inhibitors which proved its effectiveness for the treatment of different clinical manifestations of tuberosis sclerosis remains the therapeutic future in case of rabdomyoma[12].

Cardiac tumors in newborn: five case report Ayari F ,Bensmail T. , Boussetta A , Ksibi I ,Cheour M , Ben Amara M , Kacem S

Neonatology Care Unit Center of Tunis, Tunisia

Objectives To recall the clinical and sonographic features of

primitive and benign cardiac tumors in children To cite complications through observations of 5

newborns.

Observations Here is a table summarizing the clinical and ultrasound

characteristics of our patients:

Patient medical background

Prenatal ultrsound

Postnatal Ultrsound

resounding others

Leith (1)

-At term -by vaginal

route -eutrophic

-good Apgar

CT evoking a left

intraventricular

rhabdomyoma

In the aorta

which partially obstructed

Asymptomatic

Taher (2)

-At term -by vaginal

route -eutrophic

-good Apgar

Biventricular

Rhabdomyoma (Figure 1)

Multiple biventricular whose largest

was opposite to the large mitral

valve

Supraventricular tachycardia quick to 300

bpm (Figure 4)

Youssef (3)

-At term -by vaginal

route -eutrophic

-good Apgar

right

ventricular fibroma

(Figure 2)

In the

trabecular room of the

right ventricle

Asymptomatic

Bending spasms

evoking a tuberous sclerosis

Khaled (4)

-At term -by vaginal

route -eutrophic

-good Apgar

two hyperechoic spots 2 mm

each depends on the valvular

pillars in the left ventricle

Nothing

Eya (5)

-caesarean section

-intrauterine growth

retardation

tumor taking all the right

ventricle

Aspect of trabeculation

Death by refractory hypoxemia (Autopsy: figure 3)

total agenesis

of the corpus

callosum.

Figure 1:Three-dimensional echocardiograms using a matrix array

transducer: Large right and left ventricular rhabdomyomas

Figure 2 :Spin-echo MRI from a child with a large right ventricular fibroma

Figure 3: Microscopic features of the Tumor in the autopsy: spider cells

Figure 4: Supra Ventricular tachycardia in electrocardiogram

References: 1. Mun˜oz H, Sherer DM, Romero R, Sanchez J, Hernandez I, Diaz C. Prenatal sonographic findings of a large fetal cardiac fibroma. J Ultrasound Med 1995;14:479e81.

2. Uzun Orhan, Wilson DG, Vujanic GM, Parsons JM, Giovanni JV. Cardiac tumours in children. Orph J of Rare Diseases 2007;2 (11):1-14. 3. Castro FJ, Escudero. Tumores cardíacos. Protocolos diagnósticos y terapêuticos en Cardiología Pediátrica. Available at:http://www.aepe.es/.

5. Carrilho MC, Tonni G, Araujo Ju´nior E. Fetal cardiac tumors: prenatal diagnosis and outcomes. Rev Bras Cir Cardiovasc 2015;30:VIeVII. 4. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004;25:252e73.

6. Yu K, Liu Y, Wang H, Hu S, Long C. Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact CardioVasc Thorac Surg 2007;6:636-9. 7. Cruz ER, Maldonado RMC. Cardiac Tumors. Available at: http://www.emedicine.com/.

8. Ka Myers, KK Wong, M tipple, S sanatani. Benign cardiac tumours, malignant arrhythmias. Can J Cardiol 2010;26(2):e58-e61. 9. Ghadmi Mahani M, Lu JC, Rigsby CK, Krishnamurthy R, Dorfman AL, Agarwal PP. MRI of pediatric cardiac masses. AJR Am J Roentgenol. 2014 May;202(5):971-81. doi:

10.2214/AJR.13.10680. 10.Yuan S-M, Fetal Primary Cardiac Tumors During Perinatal Period, Pediatrics and Neonatology (2016), http://dx.doi.org/10.1016/j.pedneo.2016.07.004.

11. Liu X, Hong H, Zhang H, Xu Z, Liu J, Qiu L. Treatment Strategies for Primary Tumors of the Heart in Children: A 10-Year Experience. Ann thorac surg. 2015 Nov;100(5):1744-9. doi: 10.1016/j.athoracsur.2015.06.030. Epub 2015 Sep 11.

12. Mohamed I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-205138.