Pediatr Radiol (1992) 22:527-528
Pediatric Radiology 9 Springer-Verlag 1992
Diagnosis of Moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography T. Rupprecht t, D. Wenzel 1, E. Schmitzer t, M. Hofbeck 1, B. BiJwing t, U. Neubauer 2 1Pediatric Hospital of the University Erlangen-N~nberg, FRG 2Department of Neurosurgery of the University Erlangen-Ntirnberg, FRG Received: 21 July 1992; accepted: 4 August 1992
Abstract. M o y a m o y a disease is a rare vascular anomaly of the cerebral arteries. T h e etiology of the disease has not yet b e e n clearly identified. We report the noninvasive diagnosis of M o y a m o y a disease in a patient with a very early onset of symptoms in infancy The diagnosis was m a d e by colour coded D o p p l e r sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was p e r f o r m e d at the age of 7 months with subsequent slight i m p r o v e m e n t of the neurological deficits. Colour D o p p l e r sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. A t the age of 10 months the patient d e v e l o p e d arterial hypertension caused by left renal artery stenosis. O u r case suggests, that in infancy M o y a m o y a disease can be suspected noninvasively by colour D o p p l e r sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.
M o y a m o y a disease is a progressive cerebrovascular d i s o r d e r with bilateral stenosis or occlusion of the internal carotid arteries, resulting in a ' s m o k e ' like, vascular n e t w o r k at the base of the brain
with formation of collaterals from the choroidal arteries, [2, 1] the basilar artery and the meningeal arteries. A l though M o y a m o y a disease is rare with 200 cases r e p o r t e d until 1975, retrospective analysis of the literature shows, that M o y a m o y a disease accounts for about 6 % of all ischemic insults in childhood [11. B a s e d on the time of onset of symptoms, patients with M o y a m o y a disease can be divided into two age groups: The first p e a k is at the age of 3-5 years, the second p e a k is at the age of 35 years. The clinical symptoms of these two groups seem to be different. Children t e n d to have a sudden onset with an acute hemiplegia, m o t o r disturbance, or transitory ischemic attacks, whereas adults m o r e often present with an intracerebral h e m o r r h a g e [2]. Some authors therefore suspected a different etiology of M o y a m o y a disease in these two groups [1, 2] especially since adults often have evidence of other coexisting or underlying disease (e.g. neurofibromatosis, arteriosclerosis, status post radiation therapy). Until now the diagnosis of M o y a m o y a disease was based on angiography of the cerebral arteries [2,1]. We r e p o r t upon a patient, who presented in infancy with seizures and hemiparesis. T h e diagnosis of M o y a m o y a disease was suspected noninvasively by colour D o p p l e r sonography of the cerbral arteries.
Case report
History Correspondence to:Dr. Th. Rupprecht Klinik mit Poliklinik far Kinder und Jugendliche der Universitfit Erlangen, Loschgestrasse 15, W-8520 Erlangen, FRG
Our patient is the first child of healthy parents. She was delivered after an uneventful pregnancy. Because of seizures, starting on
the first day of life, she was treated with phenobarbital. At the age of 2 and 4 months ischemic insults occurred, resulting in alternating transient right and left hemiparesis. She was referred to our hospital at the age of 5 months.
Examinagon On admission we found a 5-months-old female infant in good general condition. The head circumference was below the 3rd percentile for age. She showed a hemiparesis of the left side, involving the upper and lower limbs as well as a moderate psychomotoric retardation. Transfontanellar colour coded and pulsed Doppler sonography (Acuson 128 system, 5 Mhz transducer) revealed a turbulent and accelerated flow in both internal carotid arteries accompanied by a very small vessel diameter. In contrast the investigation of the anterior cerebral artery showed reduced flow velocities with decreased systolic/diastolic pulsatility (systolic peak flow velocity 0.17 m/s; norm 0.77 m/s [5, 4]). At colour Doppler examination a network of collateral vesselswas seen in the thalamic region and the basal ganglia (Fig. i a, 1 b). Cerebral angiography confirmed the suspected diagnosis of Moymoya disease with a typical network of teleangiectasia of the basal cerebral arteries, and severe stenosis of both internal carotid arteries (Fig. 2).
Therapy and follow-up At the age of 7 months the girl underwent a bilateral encephalodurosynangiosis. During the follow-up period of 9 months she showed a slight improvement of the neurological deficits. Postoperative colour coded Doppler sonography demonstrated earlyvascularisation from the temporal grafts into the arachnoid space. At the age of 10 months she developed arterial hypertension. Colour Doppler sonography of the kidneys revealed a distal stenosls of the left renal artery (Fig.3). Under medical treatment with propranolol and nifedipine she is now normotensive.
528
Fig.la, b. Cerebralsonographicimaging. a Transfontanellartransversescan:moderatedilatation of both ventricles and the subdural space. Colour coded Doppler shows abnormal, dilated vessels in the left thalamic region (marked by white arrow ). The internal carotid arteries are not shown in this view. b Right parasagittal scan: 'Smoke' like dilated vessels around the basal ganglia (marked by white arrows ) only recognizeable at low colour Doppler scale settings Fig.2. Cerebralangiography. Right carotid arterylateralprojection (a, anterior) demonstrates typical findings of high degree carotid stenosis (lower arrow) and 'smoke' like dilated collateral "Moyamoya" vessels (marked by upper arrow) Fig. 3. Renal sonogram. Transverse colour and duplex Doppler sonogram at the level of the renal hilum. The sample volume is placed in the left renal artery which shows markedly increased flow velocities, indicating distal renal artery stenosis (continuous wave measurement: systolic peak flow velocity = 3.09 m/s)
vessel suggesting a severe proximal stenosis. In our opinion, M o y a m o y a disease should be excluded in every child presenting with cerebral infarction. (Colour)-Doppler sonography, which can be performed easily in infancy using the transfontanellar approach [4], is a valuable method to detect abnormal cerebral blood flow caused by stenosis of the internal carotid arteries. In elder children and adults diagnosis can be suspected by transcranial Doppler sonography [5]. Both methods may reduce the frequency of angiography in screening for vascular disease in children with stroke. Patients should be screened carefully for involvement of extracranial arteries (especially the renal arteries ) . Our patient showed distal renal artery stenosisas is usually the case with fibromuscular hyperplasia - at the age of 10 month whereas an investigation at admission revealed normal flow velocities in both renal arteries. Encephalodurosynangiosis is a therapeutical option to treat children with severe neurological symptoms [2]. Acknowledgements. We thank Prof. Huk (Department of Neuroradiology) for performing the angiograms.
References Discussion M o y a m o y a disease is a rare, progressive cerebral arterial occlusive disease involving both internal carotid arteries [1, 2]. Subsequently these patients develop a collateral circulation at the base of the brain. Involvement of extracranial arteries in this disease is very rare and has been reported almost exclusively in adults or autopsy studies [3, 2]. Our patient presented with a very early onset of symptoms in infancy. The usual onset of M o y m o y a disease in childhood has been reported beginning at the age of 3-4 years. There are only
very few cases in the literature with an onset of symptoms prior to the age of one year [1, 2]. To the best of our knowledge noninvasive diagnosis of M o y a m o y a disease by colour coded Doppler-sonography has not been previously described. M o y a m o y a disease was suspected in our patient by cerebral Doppler-sonography: Colour coded Doppler sonography revealed a turbulent accelerated flow in both internal carotid arteries as well as abnormal vessels at the base of the brain. Pulsed Doppler sonography of the anterior cerebral artery demonstrated a decreased flow velocity in this
1. Schoenberg, BS, Mellinger JF, Mellinger DG (1978) Moyamoya disease in children. South Med J 71:237-241 2. Suzuki J (1986) Moyamoya disease. Springer, New York Tokyo 3. Hata M, Tachibana M, Baba S, Deguchi N, Tazaki H, Suzuki H, Suruta T (1989) A therapeutic guide for renovascular hypertension with reevaluation of surgical treatment. Hinyokika-Kiyo 35:1035-1040 4. Deeg KH, Rupprecht T (1989) Pulsed Doppler-sonographic measurements of normal values for the flow velocities in the intracranial arteries of healthy newborns. Pediatr Radio119: 71-78 5. Bode H (1988) Pediatric applications of transcranial Doppler sonography. Springer, WienNew York
Pediatric Radiology 9 Springer-Verlag 1992
Diagnosis of Moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography T. Rupprecht t, D. Wenzel 1, E. Schmitzer t, M. Hofbeck 1, B. BiJwing t, U. Neubauer 2 1Pediatric Hospital of the University Erlangen-N~nberg, FRG 2Department of Neurosurgery of the University Erlangen-Ntirnberg, FRG Received: 21 July 1992; accepted: 4 August 1992
Abstract. M o y a m o y a disease is a rare vascular anomaly of the cerebral arteries. T h e etiology of the disease has not yet b e e n clearly identified. We report the noninvasive diagnosis of M o y a m o y a disease in a patient with a very early onset of symptoms in infancy The diagnosis was m a d e by colour coded D o p p l e r sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was p e r f o r m e d at the age of 7 months with subsequent slight i m p r o v e m e n t of the neurological deficits. Colour D o p p l e r sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. A t the age of 10 months the patient d e v e l o p e d arterial hypertension caused by left renal artery stenosis. O u r case suggests, that in infancy M o y a m o y a disease can be suspected noninvasively by colour D o p p l e r sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.
M o y a m o y a disease is a progressive cerebrovascular d i s o r d e r with bilateral stenosis or occlusion of the internal carotid arteries, resulting in a ' s m o k e ' like, vascular n e t w o r k at the base of the brain
with formation of collaterals from the choroidal arteries, [2, 1] the basilar artery and the meningeal arteries. A l though M o y a m o y a disease is rare with 200 cases r e p o r t e d until 1975, retrospective analysis of the literature shows, that M o y a m o y a disease accounts for about 6 % of all ischemic insults in childhood [11. B a s e d on the time of onset of symptoms, patients with M o y a m o y a disease can be divided into two age groups: The first p e a k is at the age of 3-5 years, the second p e a k is at the age of 35 years. The clinical symptoms of these two groups seem to be different. Children t e n d to have a sudden onset with an acute hemiplegia, m o t o r disturbance, or transitory ischemic attacks, whereas adults m o r e often present with an intracerebral h e m o r r h a g e [2]. Some authors therefore suspected a different etiology of M o y a m o y a disease in these two groups [1, 2] especially since adults often have evidence of other coexisting or underlying disease (e.g. neurofibromatosis, arteriosclerosis, status post radiation therapy). Until now the diagnosis of M o y a m o y a disease was based on angiography of the cerebral arteries [2,1]. We r e p o r t upon a patient, who presented in infancy with seizures and hemiparesis. T h e diagnosis of M o y a m o y a disease was suspected noninvasively by colour D o p p l e r sonography of the cerbral arteries.
Case report
History Correspondence to:Dr. Th. Rupprecht Klinik mit Poliklinik far Kinder und Jugendliche der Universitfit Erlangen, Loschgestrasse 15, W-8520 Erlangen, FRG
Our patient is the first child of healthy parents. She was delivered after an uneventful pregnancy. Because of seizures, starting on
the first day of life, she was treated with phenobarbital. At the age of 2 and 4 months ischemic insults occurred, resulting in alternating transient right and left hemiparesis. She was referred to our hospital at the age of 5 months.
Examinagon On admission we found a 5-months-old female infant in good general condition. The head circumference was below the 3rd percentile for age. She showed a hemiparesis of the left side, involving the upper and lower limbs as well as a moderate psychomotoric retardation. Transfontanellar colour coded and pulsed Doppler sonography (Acuson 128 system, 5 Mhz transducer) revealed a turbulent and accelerated flow in both internal carotid arteries accompanied by a very small vessel diameter. In contrast the investigation of the anterior cerebral artery showed reduced flow velocities with decreased systolic/diastolic pulsatility (systolic peak flow velocity 0.17 m/s; norm 0.77 m/s [5, 4]). At colour Doppler examination a network of collateral vesselswas seen in the thalamic region and the basal ganglia (Fig. i a, 1 b). Cerebral angiography confirmed the suspected diagnosis of Moymoya disease with a typical network of teleangiectasia of the basal cerebral arteries, and severe stenosis of both internal carotid arteries (Fig. 2).
Therapy and follow-up At the age of 7 months the girl underwent a bilateral encephalodurosynangiosis. During the follow-up period of 9 months she showed a slight improvement of the neurological deficits. Postoperative colour coded Doppler sonography demonstrated earlyvascularisation from the temporal grafts into the arachnoid space. At the age of 10 months she developed arterial hypertension. Colour Doppler sonography of the kidneys revealed a distal stenosls of the left renal artery (Fig.3). Under medical treatment with propranolol and nifedipine she is now normotensive.
528
Fig.la, b. Cerebralsonographicimaging. a Transfontanellartransversescan:moderatedilatation of both ventricles and the subdural space. Colour coded Doppler shows abnormal, dilated vessels in the left thalamic region (marked by white arrow ). The internal carotid arteries are not shown in this view. b Right parasagittal scan: 'Smoke' like dilated vessels around the basal ganglia (marked by white arrows ) only recognizeable at low colour Doppler scale settings Fig.2. Cerebralangiography. Right carotid arterylateralprojection (a, anterior) demonstrates typical findings of high degree carotid stenosis (lower arrow) and 'smoke' like dilated collateral "Moyamoya" vessels (marked by upper arrow) Fig. 3. Renal sonogram. Transverse colour and duplex Doppler sonogram at the level of the renal hilum. The sample volume is placed in the left renal artery which shows markedly increased flow velocities, indicating distal renal artery stenosis (continuous wave measurement: systolic peak flow velocity = 3.09 m/s)
vessel suggesting a severe proximal stenosis. In our opinion, M o y a m o y a disease should be excluded in every child presenting with cerebral infarction. (Colour)-Doppler sonography, which can be performed easily in infancy using the transfontanellar approach [4], is a valuable method to detect abnormal cerebral blood flow caused by stenosis of the internal carotid arteries. In elder children and adults diagnosis can be suspected by transcranial Doppler sonography [5]. Both methods may reduce the frequency of angiography in screening for vascular disease in children with stroke. Patients should be screened carefully for involvement of extracranial arteries (especially the renal arteries ) . Our patient showed distal renal artery stenosisas is usually the case with fibromuscular hyperplasia - at the age of 10 month whereas an investigation at admission revealed normal flow velocities in both renal arteries. Encephalodurosynangiosis is a therapeutical option to treat children with severe neurological symptoms [2]. Acknowledgements. We thank Prof. Huk (Department of Neuroradiology) for performing the angiograms.
References Discussion M o y a m o y a disease is a rare, progressive cerebral arterial occlusive disease involving both internal carotid arteries [1, 2]. Subsequently these patients develop a collateral circulation at the base of the brain. Involvement of extracranial arteries in this disease is very rare and has been reported almost exclusively in adults or autopsy studies [3, 2]. Our patient presented with a very early onset of symptoms in infancy. The usual onset of M o y m o y a disease in childhood has been reported beginning at the age of 3-4 years. There are only
very few cases in the literature with an onset of symptoms prior to the age of one year [1, 2]. To the best of our knowledge noninvasive diagnosis of M o y a m o y a disease by colour coded Doppler-sonography has not been previously described. M o y a m o y a disease was suspected in our patient by cerebral Doppler-sonography: Colour coded Doppler sonography revealed a turbulent accelerated flow in both internal carotid arteries as well as abnormal vessels at the base of the brain. Pulsed Doppler sonography of the anterior cerebral artery demonstrated a decreased flow velocity in this
1. Schoenberg, BS, Mellinger JF, Mellinger DG (1978) Moyamoya disease in children. South Med J 71:237-241 2. Suzuki J (1986) Moyamoya disease. Springer, New York Tokyo 3. Hata M, Tachibana M, Baba S, Deguchi N, Tazaki H, Suzuki H, Suruta T (1989) A therapeutic guide for renovascular hypertension with reevaluation of surgical treatment. Hinyokika-Kiyo 35:1035-1040 4. Deeg KH, Rupprecht T (1989) Pulsed Doppler-sonographic measurements of normal values for the flow velocities in the intracranial arteries of healthy newborns. Pediatr Radio119: 71-78 5. Bode H (1988) Pediatric applications of transcranial Doppler sonography. Springer, WienNew York
