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A Case of Systemic Fibromuscular Dysplasia with Renovascular Hypertension and Superior Mesenteric Arterial Aneurysm

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A Case of Systemic Fibromuscular Dysplasia with Renovascular Hypertension and Superior Mesenteric Arterial Aneurysm 소아과 : 제 40권 제 6 호 1997 1) A Case of Systemic Fibromuscular Dysplasia with Renovascular Hypertension and Superior Mesenteric Arterial Aneurysm Jong-Woon Choi, M.D.+, Sang-Min Yoon, M.D.* and Young-Chae Joo, M.D.** Department of Pediatrics, Department of Urology*, Department of Pathology**, Inha University Hospital, Seongnam, Korea INTRODUCTION mesenteric arterial aneurysm associated with re- novascular hypertension due to fibromuscular Fibromuscular dysplasia is one of the common dysplasia. causes of renal arterial disease, and is the most common cause of renovascular hypertension in CASE REPORT childhood1, 2). It is a systemic disease which can affect other arteries in addition to renal arteries, A 6 month-old male baby was referred for such as carotid, visceral, iliac, subclavian arteries, evaluation of cardiac murmur. The murmur was or even aorta2-4). It causes arterial stenoses and harsh pansystolic murmur of grade IV/VI, which can bring about renovascular hypertension, stroke, was best heard on the left lower sternal border. postprandial abdominal pain, claudication, and The chest roentgenogram showed mild cardiome- weakness in the arms, according to the affected galy, and the electrocardiogram showed sugges- arteries. In addition, arterial aneurysms can also tive findings of left ventricular hypertrophy. A develop with or without stenoses in various ar- small subarterial ventricular septal defect was teries in fibromuscular dysplasia. diagnosed by means of echocardiography, and the Although systemic fibromuscular dysplasia is patient was followed up for one year. At 19 well documented in adults2, 3), no child with sys- months of age, the electrocardiogram showed de- temic fibromuscular dysplasia has been reported finitive findings of left ventricular hypertrophy yet. Regarding aneurysm formation in children and echocardiography revealed hypertrophied left with fibromuscular dysplasia, there are few re- ventricular walls in addition to a small ventricular ports on renal arterial aneurysms5-8) and one septal defect. Blood pressure measured 146/90 report on the dissecting aneurysm of a vertebral mmHg in an arm and 180/100mmHg in a leg, so artery9). However, the superior mesenteric arterial the patient was admitted for evaluation of hyper- aneurysm, to our knowledge, has not been des- tension. Laboratory tests, including thyroid func- cribed in children either. So we report a young tion test and urinary vanillylmandelic acid level, child who was diagnosed as having a superior were normal, and abdominal sonography and in- travenous pyelography revealed no abnormality. 접수일자 : 1996년 9월 2일 Plasma renin activity and serum aldosterone level 승인일자 : 1996년 10월 24일 + Department of Pediatrics, Kangnam Medical Center, were 8.3ng/ml/hr and 86.7ng/dl respectively. Seoul, Korea(currently) - 872 - － A Case of Systemic Fibromuscular Dysplasia with Renovascular Hypertension and Superior Mesenteric Arterial Aneurysm － Fig. 1. Selective angiography revealed (A) the tortuous stenotic left renal artery with a focal aneurysmal dilatation and (B) a huge fusiform aneurysm of the superior mesenteric artery. (C) The caliceal systems of both kidneys were normal, except enlargement of that of the right kidney, which was thought to be due to compensatory hyperfunction. Hydralazine was prescribed and blood pressure pertrophy, and echocardiography revealed a small decreased to 115/70mmHg after ten days. There- ventricular septal defect and hypertrophied left after he did not visit the outpatient clinic, did not ventricle. Cardiac catheterization revealed increa- take any antihypertensive for nine months, and sed left ventricular and aortic pressures and had no problems. normal pulmonary arterial pressure(left ventricle; At 28 months of age, the patient visited our 146/0mmHg, aorta; 138/67mmHg, pulmonary ar- hospital again and blood pressure measured 150/ tery; 22/6mmHg), and pulmonary-to-systemic flow 100mmHg. After three months, blood pressure ratio was 1.33. Selective angiography revealed the measured 170/110mmHg and hydralazine was given tortuous stenotic left renal artery with a focal again for one month, but hypertension was not aneurysmal dilatation(Fig. 1A) and a huge fusi- controlled. So he was admitted for cardiac cathe- form aneurysm of the superior mesenteric artery terization and renal angiography. On physical (Fig. 1B). The caliceal systems of both kidneys examination, harsh pansystolic murmur of grade were normal, except enlargement of the right IV/VI was heard on the left lower sternal border kidney, which was thought to be due to com- and liver was not palpable. Bruit was not heard pensatory hypertrophy(Fig. 1C). Diethylenetriami- on the abdomen. Peripheral pulses were well pal- nepentaacetic acid scan of kidneys revealed de- pable and were not bounding. Plasma renin acti- creased blood flow to the left kidney. We con- vity and serum aldosterone level were 4.4ng/ml/hr cluded that the patient had renovascular hyper- and 4.7ng/dl respectively, and other laboratory tension probably due to fibromuscular dysplasia, tests were normal. The chest roentgenogram sho- and left nephrectomy was performed. The re- wed mild cardiomegaly, the electrocardiogram sected left kidney and ureter looked normal, and showed definitive findings of left ventricular hy- the renal artery was dilated and tortuous. Histo- - 873 - － Jong-Woon Choi, Sang-Min Yoon, Young-Chae Joo － and media with medial dissection(Fig. 2B). Elastic staining revealed irregular thickening and thinning of media(Fig. 2C). Histologic examination of renal parenchyme and ureter were normal. After surgery, blood pressure decreased slowly and measured 120/60mmHg without antihyper- tensives two weeks after surgery. Fourteen mon- ths after surgery, the patient was doing well and blood pressure was 100/70mmHg. Heart size was decreased on the chest roentgenogram, the elec- trocardiogram showed suggestive findings of left ventricular hypertrophy, and echocardiography re- vealed regressed left ventricular hypertrophy and a small subarterial(or muscular outlet) ventricular septal defect without aortic valve prolapse or re- gurgitation. We decided not to close surgically the ventricular septal defect yet and to observe the ventricular septal defect and the superior me- senteric arterial aneurysm together. DISCUSSION Fibromuscular dysplasia is a systemic vascular disease which affects renal, internal carotid, ce- liac, superior mesenteric, iliac, femoral, and sub- clavian arteries in order of frequency. Although infrequently, it may affect other visceral, verte- bral, cerebral, coronary arteries, and even aorta. Young women are predominantly affected, but it can occur in any age including early childhood. The principal manifestations of fibromuscular dy- Fig. 2. Histologic examination of the left renal ar- splasia are renovascular hypertension and cere- tery revealed (A) marked medial hyper- brovascular accident. In patients with renovas- plasia with luminal narrowing and (B) irre- gular thickening of intima and media with cular hypertension, fibromuscular dysplasia is the medial dissection(arrows). (H&E stain, ×100) (C) Elastic staining revealed irregular underlying cause in 20 to 50%, and it is the most thickening and thinning of media(Elastic common cause of renovascular hypertension in stain, ×200). children1, 2). logic examination of the renal artery revealed Pathologically, fibromuscular dysplasia is clas- marked medial hyperplasia with luminal narro- sified into three types according to the predo- wing(Fig. 2A) and irregular thickening of intima minant site of dysplasia-1) intimal fibroplasia, 2) - 874 - － A Case of Systemic Fibromuscular Dysplasia with Renovascular Hypertension and Superior Mesenteric Arterial Aneurysm － medial fibromuscular dysplasia, 3) periarterial or splasia, who had renovascular hypertension and a periadventitial fibroplasia. Medial fibromuscular superior mesenteric arterial aneurysm. The dia- dysplasia is further divided into three subtypes - gnosis was made by means of selective angio- a) medial fibroplasia, b) perimedial fibroplasia, c) graphy and confirmed by histologic examination medial hyperplasia. Among them medial fibroplasia following surgery. Hypertension was controlled is the most common type, and intimal fibroplasia after nephrectomy, and the superior mesenteric is also common in children. As complications, arterial aneurysm is under observation now. medial dissection, aneurysms(dissecting aneury- sms or true aneurysms), or arteriovenous fistula ACKNOWLEDGMENT can develop secondarily2, 4). It is known that aneurysm formation in fibro- We acknowledge the contribution of Dr. Mi- muscular dysplasia is rarer in children than in Young Kim, a radiologist of Inha University Hos- adults, and there are only few reports on pe- pital, to the preparation of the photographs of an- 5-8) diatric patients with renal arterial aneurysms giograms. and only one report on a child with an other arterial aneurysm9). Moreover we could refer to REFERENCES neither reports on systemic fibromuscular dys- plasia in children, nor reports on the superior 1) Fry WJ, Ernst CB, Stanley JC, Brink B, Arbor A : Renovascular hypertension in the pediatric mesenteric arterial aneurysm in children with patient. Arch Surg 107:692-698, 1973 fibromuscular dysplasia. In our patient, the patho- 2) Lüscher TF, Lie JT, Stanson AW, Houser OW, logic diagnosis of the left renal arterial lesion was Hollier LH, Sheps SG : Arterial Fibromuscular dysplasia. M ayo Clin Proc 62:931-952, 1987 intimal fibroplasia with
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