Original papers
EuropeanJournalof
Pediatrics
Eur J Pediatr (1992) 151 : 652-654
9 Springer-Verlag 1992
Convulsions, hemiparesis and central retinal artery occlusion due to left atrial myxoma in child M. Tiinz 1, A. Laske 1, T. Carrel 1, V. da Silva 2, F. Real 3, and M. Turina 1 ~Clinic for Cardiovascular Surgery, University Hospital Ziirich, Ramistrasse 100, CH-8091 Zurich, Switzerland a Children's Hospital Aarau, Switzerland 3Children's University Hospital Zurich, Switzerland
Abstract. M u l t i p l e e m b o l i z a t i o n s w e r e t h e h a l l m a r k of t h e d i s e a s e in an 8 - y e a r - o l d b o y with a left atrial m y x o m a . E m b o l i z a t i o n s o c c u r r e d initially in b o t h h a n d s a n d legs, l a t e r in the b r a i n with g e n e r a l i z e d seizures and hemiparesis, a n d finally in t h e left e y e with occlusion of the c e n t r a l r e t i n a l a r t e r y a n d c o n s e c u t i v e s e v e r e visual impairment. Echocardiography demonstrated the tumour which was r e m o v e d w i t h o u t c o m p l i c a t i o n s . Key words: A t r i a l m y x o m a - N e u r o l o g i c a l c o m p l i c a t i o n s - Child - Surgery
Introduction M y x o m a s , the m o s t c o m m o n i n t r a c a r d i a c t u m o u r s in adults, a r e e x t r e m e l y r a r e in children. S y m p t o m s arise m o s t l y f r o m the friable s t r u c t u r e of the t u m o u r , which
Correspondence to: M. T6nz
l e a d s to s y s t e m i c a n d in p a r t i c u l a r c e r e b r a l e m b o l i z a tions in 70% o f p a t i e n t s [20]. W e p r e s e n t an 8 - y e a r - o l d b o y with m u l t i p l e c e r e b r a l , r e t i n a l a n d p e r i p h e r a l emb o l i z a t i o n s d u e to a left atrial m y x o m a .
Case report In September 1990, an 8-year-old boy was admitted for generalized seizures. During the previous 2 years the otherwise healthy child presented with recurrent red spots on both hands and feet with pain in the calves. Physical examination at the time of admission showed no cardiac abnormalities. A chest X-ray film and ECG were not performed. Haematological examinations were negative exept for elevated plasma globulins (IgG 26,2g/1, IgE 1834~tg/1) a n d sedimentation rate (30mm/h). MRI (Fig. la) revealed multiple cerebral lesions which were interpreted as granulomas. Investigations for infections (viruses, protozoans, fungal diseases and parasites) or demyelinisating processes were negative. Within a few days the patient recovered completely and was dismissed without a specific diagnosis. Six months later he suffered a right hemiparesis and motor aphasia. While recovering from aphasia, a sudden severe visual impairment of the left eye with concomitant strabismus developed
Fig. 1. a Initial MRI Scan (TR SE 2200) showing bilateral hyperintensive foci in the centrum semiovale (arrowheads). b The MRI 6 months later (TR SE 716) demonstrates a new low signal intensity area in the left putamen (arrow). All lesions are consistent with infarctions due to cerebral emboli
653
Fig. 2. Cross-sectional echocardiogram (left parasternal long axis view) showing a large left atrial myxoma (arrow) protruding in the left ventricle during diastole. 3//, myxoma; LA, left auricle; LV, left ventricle; AO, aorta
after several days. On admission 4 weeks later, physical examination showed a weak and spastic right arm and leg with reduced sensibility and a right facial palsy. Fundoscopy of the left eye revealed an occlusion of the central retinal artery: a pale disc with a splinter haemorrhage, extreme narrowing of the arteries and a focus of chorioretinal degeneration in the periphery. The visual field was reduced to a tiny temporal rest. There were no abnormal findings in the right eye. Besides a peristent elevation of plasma globulins and sedimentation rate (23 ram/h), total blood count and blood chemistries were within normal limits. No auto-immune-antibodies were detected. The second MRI (Fig. lb) showed a new parenchymal defect in the left putamen consistent with an ischaemic lesion. Cardiac examinations revealed a third heart sound and a high pitched holosystolic and diastolic murmur without any evidence of heart failure. The ECG and chest X-ray film showed left atrial enlargement. Echocardiography (Fig. 2) demonstrated a pedunculated left atrial tumour, fixed to the interatrial septum, which almost filled the whole atrium by diastolic protrusion through the mitral valve. In April 1991, immediately after establishing the diagnosis, the patient was referred to our clinic and the tumour was removed under total cardiopulmonary bypass. Right atriotomy was performed with electrically induced ventricular fibrillation. The incision was carried into the left atrium and the friable, gelatinous tumour was resected with its stalk and a cuff of interatrial septum. Histological examination confirmed the diagnosis of benign papillary myxoma. The postoperative course was uneventful. Hemiparesis and severe visual impairment persisted. Because of the known familial occurrence of cardiac myxomas [7], echocardiography was performed in all family members (mother, father and sister) without positive results. Discussion Primary heart turnouts are very rare in childhood. Simcha et al. [18] found an incidence of 0.08% among all pae-
diatric patients seen in their institution over a 20-year period and Nadas [12] cited a frequency of 0.027% among 11000 paediatric autopsies. Although the majority of reported myxomas occur in the third to sixth decades of life [13], they may be encountered in any age group. The youngest patient reported was a stillborn infant [15]. Familial occurrence is known. It presents either as an independent disease [7] or in combination with endocrine overactivity and spotty pigmentation, known as Swiss syndrome [5, 8]. Our own investigations in the reported case were negative. Myxomas can occur in any cardiac chamber but are most c o m m o n in the left atrium (70%) [22]. The tumour consists of a soft, friable, gelatinous and lobulated mass which usually arises from the interatrial septum by a fibrous stalk [19]. In general clinical symptoms arise from obstruction of the atrioventricular valves or tumour embolizations. Mitral valve obstruction with pulmonary artery hypertension and congestive heart failure is a frequent clinical presentation of a left atrial m y x o m a [1]. When located in the right atrium, right heart failure with fatigue, dyspnoea, hepatomegalia and peripheral o e d e m a are commonly present. H e a r t murmurs, although frequently encountered, are inconstant and may vary from day to day [21]. Small tumours may present without any cardiac symptoms and may be missed on physical examination. In addition systemic reactions of u n k n o w n origin may be encountered. They consist of low grade fever, weight loss, raised sedimentation rate, anaemia, leucocytosis and increased plasma globulins. These findings in combination with signs of peripheral embolizations often lead to the false diagnosis of rheumatic fever or endocarditis. R e p e a t e d embolizations originating from the friable tumour were the hallmark of our case. In the series of Steinke et al. [20] it occurred in 70% of the reported cases, often with simultaneous involvement of the brain and the extremities. Neurological signs show a b r o a d spectrum from behavioral disorders to convulsions, hemiplegia and death. Very often small embolic p h e n o m e n a precede a major neurological event [9, 14]. In addition there is a known association of cardiac myxomas with cerebral aneurysm. Direct infiltration of the vessel wall by embolic myxomatous tissue can lead to aneurysm formation [4]. Cerebral aneurysm may persist, resolve [3] or may appear for the first time long after the removal of the cardiac tumour [17]. The eye represents an extremely rare target organ of myxomatous emboli. The first case of embolism of the central retinal artery due to m y x o m a with consecutive blindness was reported in 1934 by Reichlin [16], but until now only eight have subsequently been reported [6, 10, 11]. Usually central retinal embolism is associated with neurological disorders [6]. Atrial m y x o m a must be considered in the aetiological differential diagnosis of any embolic disease (cerebral, peripheral, retinal). Every child with focal neurological signs should have a thorough cardiac examination. Echocardiography is m a n d a t o r y and can easily "establish the diagnosis. Because of the danger of major cerebral era-
654 bolizations, t u m o u r resection has to be p e r f o r m e d as soon as possible. C h i l d r e n h a v i n g u n d e r g o n e surgery for atrial m y x o m a m u s t be followed closely, b o t h for t u m o u r recurrence [2] and for delayed neurological complications d u e to cerebral a n e u r y s m f o r m a t i o n a n d h a e m o r r h a g e .
References 1. Arciniegas E, Hakimi M, Farooki Z, Truccone N, Green E (1988) Primary cardiac tumors in children. J Thorac Cardiovasc Surg 79 : 582-591 2. Bobo H, Evans O (1987) Intracranial aneurysm in a child with recurrent myxoma. Pediatr Neurol 3 : 230-232 3. Branch C, Laster D, Kelly D (1985) Left atrial myxoma with cerebral emboli. Neurosurgery 16 : 675-680 3. Branch C, Laster D, Kelly D (1985) Left atrial myxoma with cerebral emboli. Neurosurgery 16 : 675-680 4. Burton C, Johnston J (1970) Multiple cerebral aneurysms and cardiac myxoma. N Engl J Med 282: 35-36 5. Carney J, Hruska L, Beauchamp G, Gordon H (1986) Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc 61 : 165-172 6. Cogan D, Wray S (1975) Vascular occlusion in the eye from cardiac myxomas. Am J Ophthalmol 80 : 396-403 7. Crawford F, Selby J, Watson D, Joransen J (1978) Unusual aspects of atrial myxoma. Ann Surg 188 : 240-244 8. Hediger C (1987) Kombination yon Herzmyxomen mit primarer Dysplasie der Nebennierenrinde, fleckfOrmigen Hautpigmentierungen und myxomartigen Tumoren anderer Lokalisation - ein eigenartiger famili~trer Symptomenkomplex ("SwissSyndrome"). Schweiz Med Wochenschr 117:591-594
9. Leonhardt T, KuIlenberg K (1977) Bilateral atrial myxomas with multiple arterial aneurysms - a syndrome mimicking polyarthritis nodosa. Am J Med 62 : 792-794 10. Manschot W (1959) Embolism of the central retinal artery. Am J Ophthalmol 48 : 381-385 11. Matamoros N, BenEzra D (1989) Bilateral retinopathy and encephalopathy. Graefe's Arch Clin Exp Opbthalmol 227: 39-41 12. Nadas A, Ellison C (1968) Cardiac tumors in infancy. Am J Cardiol 21 : 363-366 13. Peters M, Hall R, Cooley D, Leachman R, Garcia E (1974) The clinical syndrome of atrial myxoma. JAMA 230 : 675-701 14. Pridie R (1972) Left atrial myxomas in childhood: Presentation with emboli-diagnosis with ultrasonics. Thorax 27 : 759-763 15. Reddy D, Rao T, Venkaiah K, Gupta K, Naidu N (1956) Congenital myxoma of the heart. Indian J Pediatr 23 : 210-212 16. Reichling W (1934) Embolie in die Zentralarterie und mehrere Gef~sse des Zinnschen Gef~tsskranzes bei Pseudomyxom des Herzens. Ber 50. Zusammenk d D Ophth Ges Heidelberg p 331 17. Roeltgen D, Weimer G, Patterson L (1981) Delayed neurologic complications of left atrial myxoma. Neurology 31:8-13 18. Simcha A, Wells B, Tynan M, Waterston D (1971) Primary cardiac tumors in childhood. Arch Dis Child 46 : 508-514 19. Skillington P, Brawn W, Edis B, Menahem S, Venables A, Goh T, Mee R (1987) Surgical excision of primary cardiac tumors in infancy. Aust NZ J Surg 57: 599-604 20. Steinke W, Perry L, Gold H, McClenathan J, Scott L (1972) Left atrial myxoma in a child. Pediatrics 49 : 580-589 21. Van der Hauwaert L (1968) Cardiac tumors in childhood. In: Watson H (ed) Pediatric cardiology. Lloyd-Luke Ltd, London, pp 773-778 22. Wold L, Lie J (1980) Cardiac myxomas. Am J Pathol 10t : 218233
EuropeanJournalof
Pediatrics
Eur J Pediatr (1992) 151 : 652-654
9 Springer-Verlag 1992
Convulsions, hemiparesis and central retinal artery occlusion due to left atrial myxoma in child M. Tiinz 1, A. Laske 1, T. Carrel 1, V. da Silva 2, F. Real 3, and M. Turina 1 ~Clinic for Cardiovascular Surgery, University Hospital Ziirich, Ramistrasse 100, CH-8091 Zurich, Switzerland a Children's Hospital Aarau, Switzerland 3Children's University Hospital Zurich, Switzerland
Abstract. M u l t i p l e e m b o l i z a t i o n s w e r e t h e h a l l m a r k of t h e d i s e a s e in an 8 - y e a r - o l d b o y with a left atrial m y x o m a . E m b o l i z a t i o n s o c c u r r e d initially in b o t h h a n d s a n d legs, l a t e r in the b r a i n with g e n e r a l i z e d seizures and hemiparesis, a n d finally in t h e left e y e with occlusion of the c e n t r a l r e t i n a l a r t e r y a n d c o n s e c u t i v e s e v e r e visual impairment. Echocardiography demonstrated the tumour which was r e m o v e d w i t h o u t c o m p l i c a t i o n s . Key words: A t r i a l m y x o m a - N e u r o l o g i c a l c o m p l i c a t i o n s - Child - Surgery
Introduction M y x o m a s , the m o s t c o m m o n i n t r a c a r d i a c t u m o u r s in adults, a r e e x t r e m e l y r a r e in children. S y m p t o m s arise m o s t l y f r o m the friable s t r u c t u r e of the t u m o u r , which
Correspondence to: M. T6nz
l e a d s to s y s t e m i c a n d in p a r t i c u l a r c e r e b r a l e m b o l i z a tions in 70% o f p a t i e n t s [20]. W e p r e s e n t an 8 - y e a r - o l d b o y with m u l t i p l e c e r e b r a l , r e t i n a l a n d p e r i p h e r a l emb o l i z a t i o n s d u e to a left atrial m y x o m a .
Case report In September 1990, an 8-year-old boy was admitted for generalized seizures. During the previous 2 years the otherwise healthy child presented with recurrent red spots on both hands and feet with pain in the calves. Physical examination at the time of admission showed no cardiac abnormalities. A chest X-ray film and ECG were not performed. Haematological examinations were negative exept for elevated plasma globulins (IgG 26,2g/1, IgE 1834~tg/1) a n d sedimentation rate (30mm/h). MRI (Fig. la) revealed multiple cerebral lesions which were interpreted as granulomas. Investigations for infections (viruses, protozoans, fungal diseases and parasites) or demyelinisating processes were negative. Within a few days the patient recovered completely and was dismissed without a specific diagnosis. Six months later he suffered a right hemiparesis and motor aphasia. While recovering from aphasia, a sudden severe visual impairment of the left eye with concomitant strabismus developed
Fig. 1. a Initial MRI Scan (TR SE 2200) showing bilateral hyperintensive foci in the centrum semiovale (arrowheads). b The MRI 6 months later (TR SE 716) demonstrates a new low signal intensity area in the left putamen (arrow). All lesions are consistent with infarctions due to cerebral emboli
653
Fig. 2. Cross-sectional echocardiogram (left parasternal long axis view) showing a large left atrial myxoma (arrow) protruding in the left ventricle during diastole. 3//, myxoma; LA, left auricle; LV, left ventricle; AO, aorta
after several days. On admission 4 weeks later, physical examination showed a weak and spastic right arm and leg with reduced sensibility and a right facial palsy. Fundoscopy of the left eye revealed an occlusion of the central retinal artery: a pale disc with a splinter haemorrhage, extreme narrowing of the arteries and a focus of chorioretinal degeneration in the periphery. The visual field was reduced to a tiny temporal rest. There were no abnormal findings in the right eye. Besides a peristent elevation of plasma globulins and sedimentation rate (23 ram/h), total blood count and blood chemistries were within normal limits. No auto-immune-antibodies were detected. The second MRI (Fig. lb) showed a new parenchymal defect in the left putamen consistent with an ischaemic lesion. Cardiac examinations revealed a third heart sound and a high pitched holosystolic and diastolic murmur without any evidence of heart failure. The ECG and chest X-ray film showed left atrial enlargement. Echocardiography (Fig. 2) demonstrated a pedunculated left atrial tumour, fixed to the interatrial septum, which almost filled the whole atrium by diastolic protrusion through the mitral valve. In April 1991, immediately after establishing the diagnosis, the patient was referred to our clinic and the tumour was removed under total cardiopulmonary bypass. Right atriotomy was performed with electrically induced ventricular fibrillation. The incision was carried into the left atrium and the friable, gelatinous tumour was resected with its stalk and a cuff of interatrial septum. Histological examination confirmed the diagnosis of benign papillary myxoma. The postoperative course was uneventful. Hemiparesis and severe visual impairment persisted. Because of the known familial occurrence of cardiac myxomas [7], echocardiography was performed in all family members (mother, father and sister) without positive results. Discussion Primary heart turnouts are very rare in childhood. Simcha et al. [18] found an incidence of 0.08% among all pae-
diatric patients seen in their institution over a 20-year period and Nadas [12] cited a frequency of 0.027% among 11000 paediatric autopsies. Although the majority of reported myxomas occur in the third to sixth decades of life [13], they may be encountered in any age group. The youngest patient reported was a stillborn infant [15]. Familial occurrence is known. It presents either as an independent disease [7] or in combination with endocrine overactivity and spotty pigmentation, known as Swiss syndrome [5, 8]. Our own investigations in the reported case were negative. Myxomas can occur in any cardiac chamber but are most c o m m o n in the left atrium (70%) [22]. The tumour consists of a soft, friable, gelatinous and lobulated mass which usually arises from the interatrial septum by a fibrous stalk [19]. In general clinical symptoms arise from obstruction of the atrioventricular valves or tumour embolizations. Mitral valve obstruction with pulmonary artery hypertension and congestive heart failure is a frequent clinical presentation of a left atrial m y x o m a [1]. When located in the right atrium, right heart failure with fatigue, dyspnoea, hepatomegalia and peripheral o e d e m a are commonly present. H e a r t murmurs, although frequently encountered, are inconstant and may vary from day to day [21]. Small tumours may present without any cardiac symptoms and may be missed on physical examination. In addition systemic reactions of u n k n o w n origin may be encountered. They consist of low grade fever, weight loss, raised sedimentation rate, anaemia, leucocytosis and increased plasma globulins. These findings in combination with signs of peripheral embolizations often lead to the false diagnosis of rheumatic fever or endocarditis. R e p e a t e d embolizations originating from the friable tumour were the hallmark of our case. In the series of Steinke et al. [20] it occurred in 70% of the reported cases, often with simultaneous involvement of the brain and the extremities. Neurological signs show a b r o a d spectrum from behavioral disorders to convulsions, hemiplegia and death. Very often small embolic p h e n o m e n a precede a major neurological event [9, 14]. In addition there is a known association of cardiac myxomas with cerebral aneurysm. Direct infiltration of the vessel wall by embolic myxomatous tissue can lead to aneurysm formation [4]. Cerebral aneurysm may persist, resolve [3] or may appear for the first time long after the removal of the cardiac tumour [17]. The eye represents an extremely rare target organ of myxomatous emboli. The first case of embolism of the central retinal artery due to m y x o m a with consecutive blindness was reported in 1934 by Reichlin [16], but until now only eight have subsequently been reported [6, 10, 11]. Usually central retinal embolism is associated with neurological disorders [6]. Atrial m y x o m a must be considered in the aetiological differential diagnosis of any embolic disease (cerebral, peripheral, retinal). Every child with focal neurological signs should have a thorough cardiac examination. Echocardiography is m a n d a t o r y and can easily "establish the diagnosis. Because of the danger of major cerebral era-
654 bolizations, t u m o u r resection has to be p e r f o r m e d as soon as possible. C h i l d r e n h a v i n g u n d e r g o n e surgery for atrial m y x o m a m u s t be followed closely, b o t h for t u m o u r recurrence [2] and for delayed neurological complications d u e to cerebral a n e u r y s m f o r m a t i o n a n d h a e m o r r h a g e .
References 1. Arciniegas E, Hakimi M, Farooki Z, Truccone N, Green E (1988) Primary cardiac tumors in children. J Thorac Cardiovasc Surg 79 : 582-591 2. Bobo H, Evans O (1987) Intracranial aneurysm in a child with recurrent myxoma. Pediatr Neurol 3 : 230-232 3. Branch C, Laster D, Kelly D (1985) Left atrial myxoma with cerebral emboli. Neurosurgery 16 : 675-680 3. Branch C, Laster D, Kelly D (1985) Left atrial myxoma with cerebral emboli. Neurosurgery 16 : 675-680 4. Burton C, Johnston J (1970) Multiple cerebral aneurysms and cardiac myxoma. N Engl J Med 282: 35-36 5. Carney J, Hruska L, Beauchamp G, Gordon H (1986) Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc 61 : 165-172 6. Cogan D, Wray S (1975) Vascular occlusion in the eye from cardiac myxomas. Am J Ophthalmol 80 : 396-403 7. Crawford F, Selby J, Watson D, Joransen J (1978) Unusual aspects of atrial myxoma. Ann Surg 188 : 240-244 8. Hediger C (1987) Kombination yon Herzmyxomen mit primarer Dysplasie der Nebennierenrinde, fleckfOrmigen Hautpigmentierungen und myxomartigen Tumoren anderer Lokalisation - ein eigenartiger famili~trer Symptomenkomplex ("SwissSyndrome"). Schweiz Med Wochenschr 117:591-594
9. Leonhardt T, KuIlenberg K (1977) Bilateral atrial myxomas with multiple arterial aneurysms - a syndrome mimicking polyarthritis nodosa. Am J Med 62 : 792-794 10. Manschot W (1959) Embolism of the central retinal artery. Am J Ophthalmol 48 : 381-385 11. Matamoros N, BenEzra D (1989) Bilateral retinopathy and encephalopathy. Graefe's Arch Clin Exp Opbthalmol 227: 39-41 12. Nadas A, Ellison C (1968) Cardiac tumors in infancy. Am J Cardiol 21 : 363-366 13. Peters M, Hall R, Cooley D, Leachman R, Garcia E (1974) The clinical syndrome of atrial myxoma. JAMA 230 : 675-701 14. Pridie R (1972) Left atrial myxomas in childhood: Presentation with emboli-diagnosis with ultrasonics. Thorax 27 : 759-763 15. Reddy D, Rao T, Venkaiah K, Gupta K, Naidu N (1956) Congenital myxoma of the heart. Indian J Pediatr 23 : 210-212 16. Reichling W (1934) Embolie in die Zentralarterie und mehrere Gef~sse des Zinnschen Gef~tsskranzes bei Pseudomyxom des Herzens. Ber 50. Zusammenk d D Ophth Ges Heidelberg p 331 17. Roeltgen D, Weimer G, Patterson L (1981) Delayed neurologic complications of left atrial myxoma. Neurology 31:8-13 18. Simcha A, Wells B, Tynan M, Waterston D (1971) Primary cardiac tumors in childhood. Arch Dis Child 46 : 508-514 19. Skillington P, Brawn W, Edis B, Menahem S, Venables A, Goh T, Mee R (1987) Surgical excision of primary cardiac tumors in infancy. Aust NZ J Surg 57: 599-604 20. Steinke W, Perry L, Gold H, McClenathan J, Scott L (1972) Left atrial myxoma in a child. Pediatrics 49 : 580-589 21. Van der Hauwaert L (1968) Cardiac tumors in childhood. In: Watson H (ed) Pediatric cardiology. Lloyd-Luke Ltd, London, pp 773-778 22. Wold L, Lie J (1980) Cardiac myxomas. Am J Pathol 10t : 218233
