44
Moyamoya Disease Associated with Renovascular Hypertension By]. N.]ansen 1, A.]. M. Donker2, w.]. Luth 3 and L. M. E. Smit 1 Free University Hospital, Departments of Ipaediatric Neurology, 2Internal Medicine, 3Radiology, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands
This is areport of a case history of a child with cerebral Moyamoya disease and gradual development of systemic hypertension. Sodium depletion combined with enalapril induced renal failure. A bilateral renal artery stenosis was found. Percutaneous transluminal angioplasty was not successful and was followed by autotransplantation of both kidneys. Histopathological examination of the renal arteries revealed intimal hyperplasia.
Keywords Moyamoya disease - Renal artery stenosis Renovascular hypertension - Converting enzyme inhibition
Introduction Moyamoya disease is the name used to describe the condition of progressive occlusive cerebral arteriopathy with a netlike system of collaterals. It is a rare disorder with a variable picture of neurological symptoms. The term Moyamoya or "puff of smoke" points to the characteristic angiographic picture.
Clinically, Moyamoya disease presents in younger patients with transient repetitive ischaemic episodes and seizures, ultimately resulting in permanent neurological deficit. The aetiology of the disease is not clear and probably heterogeneous. In the idiopathic cases the disease is due to arterial dysplasia. A primary malformation of blood vessels has been suggested by Galligioni et al (3). The association with a history of trauma, infection, brain tumour or radiation suggests there may also be an acquired form (11). The congenital nature of the syndrome is supported by the high incidence of the arteriopathy in infancy, and by the association of abnormalities of the cerebral vasculature together with systemic vasculopathy (1, 7). However, even in infantile cases the diagnosis is seldom made under the age of one year and in most cases there Received August 19, 1988; accepted February 21, 1989 Neuropediatrics 21 (1990) 44-47 © Hippokrates Verlag Stuttgart
is a lack of histopathological confirmation of the vascular dysplasia. We present a case history of a child with occlusive arteriopathy with clinical onset of the neurological symptoms within the first year of life. During subsequent years, renovascular hypertension indicated systemic involvement.
Case report The boy is the second child of healthy, Caucasian parents. During the first three months of pregnancy, the mother suffered from a flu-like illness for which she took trivial drugs. After 34 weeks of amenorrhoea, the delivery took place at horne. Birth weight was 2720 grams. Four hours after birth the child was referred to the local hospital because of mild cyanosis and hypothermia. No cause was found for these transient problems. After discharge the initial development was within normal limits. At the age of 8 months the boy was admitted again because of a hemiconvulsion. Blood pressure was 105/60 mmHg. During the observation period there were repeated convulsions with persisting postictal paresis of the right arm. Laboratory investigations and electro-encephalography (EEG) appeared to be normal. Computer tomography revealed discrete parietal infarction. Triangiography of the cerebral vessels showed the Moyamoya pattern in several tiny collaterals in the lenticulo striate area, due to a stenosis of the supraclinoidal parts of both internal carotid arteries (Fig. 1a). Anti-epileptic drug treatment was started and the function of the right arm improved gradually. In the following years onIy recurrent episodes of headaches occurred, together with a slowly progressive hypertension (150/100 mmHg). A salt-restricted diet had no effect. Laboratory investigations at the age of three years revealed a normal renal clearance and a normal plasma renin concentration. An intravenous pyelography showed no abnormalities. Observations in the outpatient department revealed a psychomotor development within normal limits, although the gross motor function of the right arm was slightly impaired. However, this did not result in a functional deficit with manipulation. The EEG, initially normal, showed an increas( of low frequencies (amplitudes 100-150 JlV) with occipital in· termittent rhythmic delta activity (amplitudes 100-250 JlV and an asymmetry due to paroxysms of the right hemisphere Hyperventilation provocation was not performed.
Downloaded by: University of British Columbia. Copyrighted material.
Abstract
Neuropediatrics 21 (1990)
Because of the progression of the hypertension and the frequent complaints of headache, triangiography of the cerebral vessels was repeated at the age of four years. It clearly demonstrated a pattern of stenosed cerebral vessels and the netlike collaterals (Fig. Ib). When compared with the previous triangiography, it was obvious that the process had progressed. After selective visualization of the cerebral vessels, there was a brief rise in blood pressure (240/130 mmHg). An ensuing angiography of renal arteries gave the impression of a stenosis on the right side. Unfortunately, during the Seldinger procedure, failure of perfusion of the left leg occurred. Therefore, the procedure had to be stopped before the left renal artery was visualized. According to these findings, a renal cause for the hypertension was suggested and treatment with propranolol (later labetolol) and hydrochlorothiazide was initiated. However, despite this regimen, blood pressure remained high. At the age of five years blood pressure was 170/105 mmHg and rose to 240/ 180 mmHg during an adenotonsillectomy under general anaesthesia. At the age of six years he was re-admitted to the hospital because of severe headaches and fatigue. Blood pressure was 215/135 mmHg and a soft murrnur was present in the right hypogastrium. The plasma concentration of creatinine amounted to 78 micromoI/L. After a dose of 12.5 mg captopril a renal DTPA scintigraphy was performed. This showed no aberrations or asymmetry of the renal perfusion. However, the renal transit time was increased on both sides. After discontinuation of the antihypertensive medication, enalapril 10 mg b.i.d. was started, resulting in a blood pressure of 160/100 mmHg. After addition of 12.5 mg hydrochlorothiazide, blood pressure dropped to 110/70 mmHg without symptoms.
Fig. 1A Angiography of the left carotid artery at the age of eight months. Stenosis supraclinoidal part of the carotid artery, tiny collaterals in the lenticulo striate area.
One week after discharge the boy was readmitted because of malaise, headache and vomiting. His serum creatinine level was 278 micromoI/l, urea being 38.6 mmol/l. The clearance of creatinine was 19 mI/min./l. 73 m2 . Discontinuation of enalapril resulted in improvement in renal function and disappearance of symptoms. This course suggested bilateral renal artery stenosis. Subsequent angiography revealed that the lumen of both arteries severely narrowed along by one centimetre. The process started some millimetres from the origin of the vessels, no poststenotic dilatation was seen (Fig. 2). After an unsuccessful percutaneous transluminal angioplasty of both renal arteries, blood pressure rose to 250/150 mmHg. The boy developed acute renal insufficiency due to bilateral renal artery occlusion, and he was presented for renovascular surgery. A reconstruction of both renal arteries was performed and both kidneys were autotransplantated. The postoperative hypertension of 170/110 mmHg normalized after enalapril. Six months later and without any medication, his blood pressure was 120/ 80mmHg. Unfortunately, fourteen days postoperatively the boy suffered from a new cerebral ischaemic accident, resulting in anarthria and severe spastic hemiparesis. Intensive therapy in arehabilitation centre only resulted in a mild improvement in neurological functions.
Histopathological examination In this case of radiodiagnostically proven Moyamoya, we had the opportunity to investigate parts of both
Fig. 1B Angiography of the left carotid artery at the age of four years. Stenosed cerebral arteries with net-like collaterals. Progression of the Moyamoya pattern if compared with Fig. lA.
45
Downloaded by: University of British Columbia. Copyrighted material.
Moyamoya Disease Associated with Renovascular Hypertension
Neuropediatrics 21 (1990)
]. N.]ansen et al
scribe 24 children, two of whom were diagnosed in their first year of life (10). One child was three months old, the other one year. Early recognition of the disease is difficult because of the insidious onset with transient symptoms. The recurrent course usually becomes evident during the later years of childhood. At the age of eight months our patient had a short series of hemiconvulsions, with persisting postictal paresis of the right arm. Similar presenting symptoms were reported by Maki and Nakad, who presented a three-month-old child with initial symptoms of convulsions. These cases illustrate the fact that epilepsy may be the first symptom of the disease and that convulsions may precede the ischaemic attacks with neurological deficit by a number of years.
Fig. 2 Renal angiography showing diminished lumen of both arteries by one centimetre. No poststenotic dilatation.
At the age of eight months angiography revealed an occlusive cerebral angiopathy, although at that time the EEG pattern was normal. During the next few years EEG abnormalities became apparent and at the age of four years progression of the disease was confirmed by repeated angiography. Although there were no localized neurological sequelae of the vasculopathy, the hypertension was initially attributed to "ischaemic cerebral dysfunction". The progression of the hypertension stimulated a repeated investigation and ultimately its renovascular origin became apparent.
Maki and Nakada (1) described hypertension in young children with Moyamoya, but in their extensive review, Suzuki and Kodama (11) only mentioned the occurrence of hypertension in adults, not in children. An association of Moyamoya disease and renovascular hypertension in adults has been reported in the literature (6, 13). In our patient the hypertension was caused by bilateral renal artery stenosis. Histopathological examination of the renal vessels showed intimal hyperplasia. Intimal hyperplasia is described by many other authors in the vessels in the intracranial region. In our patient this indicates a systemic rather than a localized vascular disease. A combination of intimal proliferation of extracranial arteries together with proven cerebral Moyamoya (by arteriography or autopsy), has been mentioned by three auFig. 3 Cross section of the wall of the right renal artery. Proliferation of thors. Halonen et al described the autopsy of a child with Moyathe intima with doubling and discontinuation of the internal lamina elasmoya, in whom intima! proliferation was seen not only in a tica. The media contains more than the usual collagen fibres. renal artery, but also in both coronary arteries (8). Yamashita et al reported autopsy findings of fibromuscular intimal thickrenal arteries. Microscopic examination revealed proliferation enings of a renal artery and fibrocellular intimal thickenings of of the intima with local fibrinoid changes including some gran- both coronary arteries in a seven-year-old Japanese girl with ulocytes (Fig. 3). The lamina elastica interna showed doubling Moyamoya disease (12). Kawakita et al mentioned thickenings and discontinuation in its course. The tunica media contained of the intima by loose connective tissue in the carotid, coronary, more than the usual number of collagen fibres while the ad- renal, hepatic and splenic arteries of a twelve-year-old boy with ventitia was thickened locally. Although some changes were Moyamoya disease and tuberculosis (9). possibly the result of the attempt at percutaneous angioplasty, Clinical evidence for extracranial manifestathe proliferation of the intima and the changes in the lamina tions of a vascular disease in patients with cerebral Moyamoya elastica interna led to a histopathological diagnosis of intimal disease was reported several times, but histopathological confirfibroplasia. mation was lacking in these cases (1, 2, 5, 6, 7). Reviewing the literature, we conclude that renal artery stenosis in Moyamoya disease is rare and it is not generally known that hypertension Discussion can occur early in the course.
Clinical course Reports of Moyamoya in infancy are scarce. Some reviewers give ranges of ages, but no specific information about the prevalence in the first year. Maki and Nakada de-
These are a few specific points with regard to the treatment of hypertension in our patient. Renovascular hypertension was suspected and, therefore, a test dose of captopril, an ACE-inhibitor, was administered.
Downloaded by: University of British Columbia. Copyrighted material.
46
Moyamoya Disease Associated with Renovascular Hypertension
After addition of hydrochlorothiazide, however, blood pressure did become normal, but now renal function deteriorated. This led to the diagnosis of bilateral renal artery stenosis, confirmed by subsequent renal angiography. In the case of bilateral renal artery stenosis, renal function is heavily dependent on angiotensin-11 mediated efferent vasoconstriction, and especially so if diuretics are employed (4). The combination of bilateral renal artery stenosis, diuretics and ACE-inhibition leads, characteristically, to oligurie or non-oligurie renal insufficiency which is, naturally, completely reversible after discontinuation of the ACE-inhibitor (4).
Predilection of the disorder in cerebral arteries is apparent, but during the progressive course repeated attention must be paid to extracranial vessels, especially to the renal arteries.
Acknowledgment Authors thank Prof. Dr. F. Eulderink, Laboratorium voor Pathologie, Leiden, The Netherlands, for his comment to the histopathological contribution.
References Ellison, P. H.,]. A. Largent, A.]. Popp: Moyamoya disease associated with renal artery stenosis. Areh. Neurol. 38 (1981) 467 2 Furuta, K., T. Homma,]. Yoshioka, Y. Tamura, H. Hirabayashi, Y. Sasaki, S. Kawa, S. Monno, T. Hara, T. Furata: A ease of Moyamoya disease assoeiated with the stenosis of the right eoronary artery, siek sinus syndrome and hypertrophie eardiomyopathy. Kokyo To Junkan 33 (1985) 1401-1406 3 Galligioni, F., G. C. Andrioli, G. Marin, S. Briani, G. Iraci: Hypoplasia of the internal earotid artery assoeiated with eerebral pseudoangiomatosis. Am. J. Roentgenol. Rad. Ther. Nucl. Med. 112 (1971) 251-262 4 Gans, R. O. B., S.]. Hoorntje, A.]. M. Donker: Renal effeets of angiotensin-I eonverting enzyme inhibitors (review). Neth. J. Med. 32 (1988) 247-264 5 Gilly, R., N. Elbaz,]. Langue,]. Raveau: Stenoses arterielles eerebrales multiples et progressives, stenose de l'artere renale et maladie de Reeklinghausen. Pediatrie 37 (1982) 523-530 6 Godin, M., M. Tadie,]. Dero,]. P. Fillastre, P. Creissard: Maladie de Moyamoya et stenose de l'artere renale. Sem. Höp. Paris 56 (1980) 1257-1261 7 Goldberg, H.: 'Moyamoya' assoeiated with peripheral vaseular oeclusive disease. Areh. Dis. Child. 49 (1974) 964-966 8 Halonen, H., V. Halonen, M. Donner, M. Iivanainen, M. Vuolio,]. Mäkinen: Oeclusive disease of intraeranial main arteries with eollateral networks in ehildren. Neuropädiatrie 4 (1973) 187-206 9 Kawakita, Y., K. Abe, Y. Miyata, S. Horikoshi: Spontaneous thrombosis of the internal earotid artery in ehildren. Folia Psyehiatr. Neurol. Jpn. 19(1965)245-255 10 Maki, Y., Y. Nakada: Clinieal and radioisotopie follow-up study of 'Moyamoya'. Childs Brain 2 (1976) 257-271 11 Suzuki,]., N. Kodama: Moyamoya disease - a review. Stroke 14 (1983) 104-109 12 Yamashiro, Y., H. Takahashi, K. Takahashi: Cerebrovaseular Moyamoya disease. Eur. J. Pediatr. 142 (1984) 44-50 13 Yamashita, M., K. Tanaka, T. Kishikawa, K. Yokota: Moyamoyadisease associated with renovaseular hypertension. Hum. Pathol. 15 (1984) 191-193 1
In general, percutaneous transluminal angioplasty is a good and safe procedure in cases of renal artery stenosis. However, we were doubtful whether percutaneous transluminal angioplasty would be successful in a case of intimal hyperplasia. During the procedure it became apparent that it was not possible to dilate the stenoses. Unfortunately, our efforts led to bilateral renal artery occlusion and autotransplantation of the kidneys had to be performed. Aetiology
None of the various theories about the origin of Moyamoya disease is entirely satisfactory. One may come across cases of Moyamoya disease following exposure to radiation, following trauma, or as part of an autoimmune process. These conditions support the theory that the disease may be acquired. On the other hand, there is a clear association with other abnormalities which supports the view of a congenital disorder. Galligioni et al (3) postulated a primary malformation of blood vessels, supported by the association of other congenital vascular abnormalities such as aneurysms and a pseudoangiomatous pattern of the collateral network. Goldberg et al (7) assumed that the diffuse, widespread nature of the vascular disease, together with an early onset, indicate a congenital basis. Furthermore, Moyamoya disease is seen with trisomy 21, the latter frequently described in combination with other congenital abnormalities (1). Renal artery stenosis is seen in combination with Moyamoya disease. This does not prove a congenital basis, but suggests a process involving several vessels. Our patient's history favours a congenital origin. The process was detected within the first year of life, combining intracranial malformations with systemic vascular abnormalities later on. Histopathological examinations indicate a similar origin with widespread expression. The progression of the abnormalities give rise to the assumption that Moyamoya disease is a congenital vasculopathy with progressive intimal hyperplasia leading to stenosis and occlusion.
47
Dr. L. M. E. Smit University Hospital of the Free University De Boelelaan 111 7 1081 HV Amsterdam The Netherlands
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Subsequent DTPA renal scintigraphy disclosed no decreased filtration on the right side and thus enalapril, a long-acting ACE-inhibitor, was initiated. Blood pressure decreased but did not become normal.
Neuropediatrics 21 (1990)
Moyamoya Disease Associated with Renovascular Hypertension By]. N.]ansen 1, A.]. M. Donker2, w.]. Luth 3 and L. M. E. Smit 1 Free University Hospital, Departments of Ipaediatric Neurology, 2Internal Medicine, 3Radiology, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands
This is areport of a case history of a child with cerebral Moyamoya disease and gradual development of systemic hypertension. Sodium depletion combined with enalapril induced renal failure. A bilateral renal artery stenosis was found. Percutaneous transluminal angioplasty was not successful and was followed by autotransplantation of both kidneys. Histopathological examination of the renal arteries revealed intimal hyperplasia.
Keywords Moyamoya disease - Renal artery stenosis Renovascular hypertension - Converting enzyme inhibition
Introduction Moyamoya disease is the name used to describe the condition of progressive occlusive cerebral arteriopathy with a netlike system of collaterals. It is a rare disorder with a variable picture of neurological symptoms. The term Moyamoya or "puff of smoke" points to the characteristic angiographic picture.
Clinically, Moyamoya disease presents in younger patients with transient repetitive ischaemic episodes and seizures, ultimately resulting in permanent neurological deficit. The aetiology of the disease is not clear and probably heterogeneous. In the idiopathic cases the disease is due to arterial dysplasia. A primary malformation of blood vessels has been suggested by Galligioni et al (3). The association with a history of trauma, infection, brain tumour or radiation suggests there may also be an acquired form (11). The congenital nature of the syndrome is supported by the high incidence of the arteriopathy in infancy, and by the association of abnormalities of the cerebral vasculature together with systemic vasculopathy (1, 7). However, even in infantile cases the diagnosis is seldom made under the age of one year and in most cases there Received August 19, 1988; accepted February 21, 1989 Neuropediatrics 21 (1990) 44-47 © Hippokrates Verlag Stuttgart
is a lack of histopathological confirmation of the vascular dysplasia. We present a case history of a child with occlusive arteriopathy with clinical onset of the neurological symptoms within the first year of life. During subsequent years, renovascular hypertension indicated systemic involvement.
Case report The boy is the second child of healthy, Caucasian parents. During the first three months of pregnancy, the mother suffered from a flu-like illness for which she took trivial drugs. After 34 weeks of amenorrhoea, the delivery took place at horne. Birth weight was 2720 grams. Four hours after birth the child was referred to the local hospital because of mild cyanosis and hypothermia. No cause was found for these transient problems. After discharge the initial development was within normal limits. At the age of 8 months the boy was admitted again because of a hemiconvulsion. Blood pressure was 105/60 mmHg. During the observation period there were repeated convulsions with persisting postictal paresis of the right arm. Laboratory investigations and electro-encephalography (EEG) appeared to be normal. Computer tomography revealed discrete parietal infarction. Triangiography of the cerebral vessels showed the Moyamoya pattern in several tiny collaterals in the lenticulo striate area, due to a stenosis of the supraclinoidal parts of both internal carotid arteries (Fig. 1a). Anti-epileptic drug treatment was started and the function of the right arm improved gradually. In the following years onIy recurrent episodes of headaches occurred, together with a slowly progressive hypertension (150/100 mmHg). A salt-restricted diet had no effect. Laboratory investigations at the age of three years revealed a normal renal clearance and a normal plasma renin concentration. An intravenous pyelography showed no abnormalities. Observations in the outpatient department revealed a psychomotor development within normal limits, although the gross motor function of the right arm was slightly impaired. However, this did not result in a functional deficit with manipulation. The EEG, initially normal, showed an increas( of low frequencies (amplitudes 100-150 JlV) with occipital in· termittent rhythmic delta activity (amplitudes 100-250 JlV and an asymmetry due to paroxysms of the right hemisphere Hyperventilation provocation was not performed.
Downloaded by: University of British Columbia. Copyrighted material.
Abstract
Neuropediatrics 21 (1990)
Because of the progression of the hypertension and the frequent complaints of headache, triangiography of the cerebral vessels was repeated at the age of four years. It clearly demonstrated a pattern of stenosed cerebral vessels and the netlike collaterals (Fig. Ib). When compared with the previous triangiography, it was obvious that the process had progressed. After selective visualization of the cerebral vessels, there was a brief rise in blood pressure (240/130 mmHg). An ensuing angiography of renal arteries gave the impression of a stenosis on the right side. Unfortunately, during the Seldinger procedure, failure of perfusion of the left leg occurred. Therefore, the procedure had to be stopped before the left renal artery was visualized. According to these findings, a renal cause for the hypertension was suggested and treatment with propranolol (later labetolol) and hydrochlorothiazide was initiated. However, despite this regimen, blood pressure remained high. At the age of five years blood pressure was 170/105 mmHg and rose to 240/ 180 mmHg during an adenotonsillectomy under general anaesthesia. At the age of six years he was re-admitted to the hospital because of severe headaches and fatigue. Blood pressure was 215/135 mmHg and a soft murrnur was present in the right hypogastrium. The plasma concentration of creatinine amounted to 78 micromoI/L. After a dose of 12.5 mg captopril a renal DTPA scintigraphy was performed. This showed no aberrations or asymmetry of the renal perfusion. However, the renal transit time was increased on both sides. After discontinuation of the antihypertensive medication, enalapril 10 mg b.i.d. was started, resulting in a blood pressure of 160/100 mmHg. After addition of 12.5 mg hydrochlorothiazide, blood pressure dropped to 110/70 mmHg without symptoms.
Fig. 1A Angiography of the left carotid artery at the age of eight months. Stenosis supraclinoidal part of the carotid artery, tiny collaterals in the lenticulo striate area.
One week after discharge the boy was readmitted because of malaise, headache and vomiting. His serum creatinine level was 278 micromoI/l, urea being 38.6 mmol/l. The clearance of creatinine was 19 mI/min./l. 73 m2 . Discontinuation of enalapril resulted in improvement in renal function and disappearance of symptoms. This course suggested bilateral renal artery stenosis. Subsequent angiography revealed that the lumen of both arteries severely narrowed along by one centimetre. The process started some millimetres from the origin of the vessels, no poststenotic dilatation was seen (Fig. 2). After an unsuccessful percutaneous transluminal angioplasty of both renal arteries, blood pressure rose to 250/150 mmHg. The boy developed acute renal insufficiency due to bilateral renal artery occlusion, and he was presented for renovascular surgery. A reconstruction of both renal arteries was performed and both kidneys were autotransplantated. The postoperative hypertension of 170/110 mmHg normalized after enalapril. Six months later and without any medication, his blood pressure was 120/ 80mmHg. Unfortunately, fourteen days postoperatively the boy suffered from a new cerebral ischaemic accident, resulting in anarthria and severe spastic hemiparesis. Intensive therapy in arehabilitation centre only resulted in a mild improvement in neurological functions.
Histopathological examination In this case of radiodiagnostically proven Moyamoya, we had the opportunity to investigate parts of both
Fig. 1B Angiography of the left carotid artery at the age of four years. Stenosed cerebral arteries with net-like collaterals. Progression of the Moyamoya pattern if compared with Fig. lA.
45
Downloaded by: University of British Columbia. Copyrighted material.
Moyamoya Disease Associated with Renovascular Hypertension
Neuropediatrics 21 (1990)
]. N.]ansen et al
scribe 24 children, two of whom were diagnosed in their first year of life (10). One child was three months old, the other one year. Early recognition of the disease is difficult because of the insidious onset with transient symptoms. The recurrent course usually becomes evident during the later years of childhood. At the age of eight months our patient had a short series of hemiconvulsions, with persisting postictal paresis of the right arm. Similar presenting symptoms were reported by Maki and Nakad, who presented a three-month-old child with initial symptoms of convulsions. These cases illustrate the fact that epilepsy may be the first symptom of the disease and that convulsions may precede the ischaemic attacks with neurological deficit by a number of years.
Fig. 2 Renal angiography showing diminished lumen of both arteries by one centimetre. No poststenotic dilatation.
At the age of eight months angiography revealed an occlusive cerebral angiopathy, although at that time the EEG pattern was normal. During the next few years EEG abnormalities became apparent and at the age of four years progression of the disease was confirmed by repeated angiography. Although there were no localized neurological sequelae of the vasculopathy, the hypertension was initially attributed to "ischaemic cerebral dysfunction". The progression of the hypertension stimulated a repeated investigation and ultimately its renovascular origin became apparent.
Maki and Nakada (1) described hypertension in young children with Moyamoya, but in their extensive review, Suzuki and Kodama (11) only mentioned the occurrence of hypertension in adults, not in children. An association of Moyamoya disease and renovascular hypertension in adults has been reported in the literature (6, 13). In our patient the hypertension was caused by bilateral renal artery stenosis. Histopathological examination of the renal vessels showed intimal hyperplasia. Intimal hyperplasia is described by many other authors in the vessels in the intracranial region. In our patient this indicates a systemic rather than a localized vascular disease. A combination of intimal proliferation of extracranial arteries together with proven cerebral Moyamoya (by arteriography or autopsy), has been mentioned by three auFig. 3 Cross section of the wall of the right renal artery. Proliferation of thors. Halonen et al described the autopsy of a child with Moyathe intima with doubling and discontinuation of the internal lamina elasmoya, in whom intima! proliferation was seen not only in a tica. The media contains more than the usual collagen fibres. renal artery, but also in both coronary arteries (8). Yamashita et al reported autopsy findings of fibromuscular intimal thickrenal arteries. Microscopic examination revealed proliferation enings of a renal artery and fibrocellular intimal thickenings of of the intima with local fibrinoid changes including some gran- both coronary arteries in a seven-year-old Japanese girl with ulocytes (Fig. 3). The lamina elastica interna showed doubling Moyamoya disease (12). Kawakita et al mentioned thickenings and discontinuation in its course. The tunica media contained of the intima by loose connective tissue in the carotid, coronary, more than the usual number of collagen fibres while the ad- renal, hepatic and splenic arteries of a twelve-year-old boy with ventitia was thickened locally. Although some changes were Moyamoya disease and tuberculosis (9). possibly the result of the attempt at percutaneous angioplasty, Clinical evidence for extracranial manifestathe proliferation of the intima and the changes in the lamina tions of a vascular disease in patients with cerebral Moyamoya elastica interna led to a histopathological diagnosis of intimal disease was reported several times, but histopathological confirfibroplasia. mation was lacking in these cases (1, 2, 5, 6, 7). Reviewing the literature, we conclude that renal artery stenosis in Moyamoya disease is rare and it is not generally known that hypertension Discussion can occur early in the course.
Clinical course Reports of Moyamoya in infancy are scarce. Some reviewers give ranges of ages, but no specific information about the prevalence in the first year. Maki and Nakada de-
These are a few specific points with regard to the treatment of hypertension in our patient. Renovascular hypertension was suspected and, therefore, a test dose of captopril, an ACE-inhibitor, was administered.
Downloaded by: University of British Columbia. Copyrighted material.
46
Moyamoya Disease Associated with Renovascular Hypertension
After addition of hydrochlorothiazide, however, blood pressure did become normal, but now renal function deteriorated. This led to the diagnosis of bilateral renal artery stenosis, confirmed by subsequent renal angiography. In the case of bilateral renal artery stenosis, renal function is heavily dependent on angiotensin-11 mediated efferent vasoconstriction, and especially so if diuretics are employed (4). The combination of bilateral renal artery stenosis, diuretics and ACE-inhibition leads, characteristically, to oligurie or non-oligurie renal insufficiency which is, naturally, completely reversible after discontinuation of the ACE-inhibitor (4).
Predilection of the disorder in cerebral arteries is apparent, but during the progressive course repeated attention must be paid to extracranial vessels, especially to the renal arteries.
Acknowledgment Authors thank Prof. Dr. F. Eulderink, Laboratorium voor Pathologie, Leiden, The Netherlands, for his comment to the histopathological contribution.
References Ellison, P. H.,]. A. Largent, A.]. Popp: Moyamoya disease associated with renal artery stenosis. Areh. Neurol. 38 (1981) 467 2 Furuta, K., T. Homma,]. Yoshioka, Y. Tamura, H. Hirabayashi, Y. Sasaki, S. Kawa, S. Monno, T. Hara, T. Furata: A ease of Moyamoya disease assoeiated with the stenosis of the right eoronary artery, siek sinus syndrome and hypertrophie eardiomyopathy. Kokyo To Junkan 33 (1985) 1401-1406 3 Galligioni, F., G. C. Andrioli, G. Marin, S. Briani, G. Iraci: Hypoplasia of the internal earotid artery assoeiated with eerebral pseudoangiomatosis. Am. J. Roentgenol. Rad. Ther. Nucl. Med. 112 (1971) 251-262 4 Gans, R. O. B., S.]. Hoorntje, A.]. M. Donker: Renal effeets of angiotensin-I eonverting enzyme inhibitors (review). Neth. J. Med. 32 (1988) 247-264 5 Gilly, R., N. Elbaz,]. Langue,]. Raveau: Stenoses arterielles eerebrales multiples et progressives, stenose de l'artere renale et maladie de Reeklinghausen. Pediatrie 37 (1982) 523-530 6 Godin, M., M. Tadie,]. Dero,]. P. Fillastre, P. Creissard: Maladie de Moyamoya et stenose de l'artere renale. Sem. Höp. Paris 56 (1980) 1257-1261 7 Goldberg, H.: 'Moyamoya' assoeiated with peripheral vaseular oeclusive disease. Areh. Dis. Child. 49 (1974) 964-966 8 Halonen, H., V. Halonen, M. Donner, M. Iivanainen, M. Vuolio,]. Mäkinen: Oeclusive disease of intraeranial main arteries with eollateral networks in ehildren. Neuropädiatrie 4 (1973) 187-206 9 Kawakita, Y., K. Abe, Y. Miyata, S. Horikoshi: Spontaneous thrombosis of the internal earotid artery in ehildren. Folia Psyehiatr. Neurol. Jpn. 19(1965)245-255 10 Maki, Y., Y. Nakada: Clinieal and radioisotopie follow-up study of 'Moyamoya'. Childs Brain 2 (1976) 257-271 11 Suzuki,]., N. Kodama: Moyamoya disease - a review. Stroke 14 (1983) 104-109 12 Yamashiro, Y., H. Takahashi, K. Takahashi: Cerebrovaseular Moyamoya disease. Eur. J. Pediatr. 142 (1984) 44-50 13 Yamashita, M., K. Tanaka, T. Kishikawa, K. Yokota: Moyamoyadisease associated with renovaseular hypertension. Hum. Pathol. 15 (1984) 191-193 1
In general, percutaneous transluminal angioplasty is a good and safe procedure in cases of renal artery stenosis. However, we were doubtful whether percutaneous transluminal angioplasty would be successful in a case of intimal hyperplasia. During the procedure it became apparent that it was not possible to dilate the stenoses. Unfortunately, our efforts led to bilateral renal artery occlusion and autotransplantation of the kidneys had to be performed. Aetiology
None of the various theories about the origin of Moyamoya disease is entirely satisfactory. One may come across cases of Moyamoya disease following exposure to radiation, following trauma, or as part of an autoimmune process. These conditions support the theory that the disease may be acquired. On the other hand, there is a clear association with other abnormalities which supports the view of a congenital disorder. Galligioni et al (3) postulated a primary malformation of blood vessels, supported by the association of other congenital vascular abnormalities such as aneurysms and a pseudoangiomatous pattern of the collateral network. Goldberg et al (7) assumed that the diffuse, widespread nature of the vascular disease, together with an early onset, indicate a congenital basis. Furthermore, Moyamoya disease is seen with trisomy 21, the latter frequently described in combination with other congenital abnormalities (1). Renal artery stenosis is seen in combination with Moyamoya disease. This does not prove a congenital basis, but suggests a process involving several vessels. Our patient's history favours a congenital origin. The process was detected within the first year of life, combining intracranial malformations with systemic vascular abnormalities later on. Histopathological examinations indicate a similar origin with widespread expression. The progression of the abnormalities give rise to the assumption that Moyamoya disease is a congenital vasculopathy with progressive intimal hyperplasia leading to stenosis and occlusion.
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Dr. L. M. E. Smit University Hospital of the Free University De Boelelaan 111 7 1081 HV Amsterdam The Netherlands
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Subsequent DTPA renal scintigraphy disclosed no decreased filtration on the right side and thus enalapril, a long-acting ACE-inhibitor, was initiated. Blood pressure decreased but did not become normal.
Neuropediatrics 21 (1990)
