Australas Radio1 1990: 34: 350-352
Fibromuscular Dysplasia of Common Carotid Artery A Case Report R.V.PHADKE,D.M.R.D.,M.D. Assistant Professor,Radiology Sanjay Gana'hi Postgraduate Institute of Medical Sciences Lucknow - 226001 India. (Formerly Reader in Radiology, Govt. Medical College, Nagpur, 440003, India) *K.B. TAORI, D.M.R.D.,M.D. Reader in Radiology (At present Radiologist, Royal Hospital, P.B. 2331. Seeb, Sultanate of Oman) *V.K. DIVEKAR, M.S.,F.R.C.S., D.M.R.D. Senior Lecturer in Radiology *V.R. BHUTADA,D.M.R.E., M.D. Professor and Head of Radiology *Govt.Medical College,Nagpur, 440003, India.
ABSTRACT A case of presumed fibromuscular dysplasia involving the left common carotid artery with formation of fusiform aneurysm of the whole length of the common carotid artery, and a distal web-like lesion, is described in a 25 year old female patient. The angiographic appearance of the lesion is uncommon, as is the involvement of the common carotid artery. The literature is reviewed.
Transfemoral selective angiography showed fusiform dilatation of the left common carotid artery with undulating margins and filling defects suggesting thrombus formation. At the upper end of the common carotid artery a distinct almost circumferential web was noted. Distally the external and internal carotid arteries were normal with good filling of intracranial vessels. Angiography of the right carotid artery and both renal arteries revealed no abnormality.
INTRODUCTION Fibromuscular dysplasia (FMD) is a well recognised non-atheromatous vascular disease entity. First observed in renal arteries in 1938 (Leadbetter and Burkland), FMD has been recognised subsequently in splanchnic, iliac, extracranial internal carotid, vertebral and intracranial arteries. Visceral veins and aorta may also be affected by FMD (So et a1 1981). FMD, generally a disease of young women (20 to 40 yrs) (McCormack et a1 1966), commonly affects renal and extracranial internal carotid vessels. Involvement of the common carotid artery is relatively uncommon. We report a case with involvement of left common carotid artery with formation of a fusiform aneurysm.
CASE REPORT A 25 year old normotensive female patient presented with a diffuse pulsatile swelling in the lower left side of the neck. No bruit was heard over the swelling. The patient was non-diabetic and serological tests for syphilis were negative. She had no relevant history of trauma or infection.
Key wonls: Aneurysm, Carotid Carotid Arteries Fibrodysplasia
Address for correspondence:
FIGURE 1 - AP view, selective left common carotid angiogram showing fusiform aneurysm with filling defects suggestive of thrombus formation.
Dr.R.V. Phadke Assistant Professor, Radiology Sanjay Gandhi Postgraduate Institute of Medical Sciences P.B. 375.Rae Bareli Road, Lucknow (U.P.) India - 226001
350
Submitted for publication on: 15th August, 1989 Resubmitted for publication on: 1st May, 1990 Accepted for publication on: 3rd July, 1990 Australasian Radiology. Vol. X X X N , No. 4 . November. 1990
FIBROMUSCULAR DYSPLASIA The patient had no neurological deficit and declined to undergo any surgical intervention. DISCUSSION There is abundant literature describing various histological types and angiographic ,patterns of FMD. Three main histological types have been identified viz: (i) intima1 fibroplasia, (ii) medial fibroplasia or fibromuscular hyperplasia and (iii) subadventitial hyperplasia (McCormack et a/ 1966, Kincaid et a/ 1968, Harrison and McCormack 197 1, Stanley et a1 1975). Three characteristic angiographic patterns are associated with cephalocervical FMD (Houser and Baker 1968, Bradac 1970, Osbom and Anderson 1977, So e t a / 1981). (i) The “string of beads” appearance is considered most common (80%) and almost pathognomonic. Medial fibroplasia is the most common histological type of FMD associated with this angiographic finding (Osborn and Anderson 1977). (ii) The second and less common angiographic pattern is unifocal o r multifocal tubular stenosis which may be associated with any of the three histological types. (iii) Localised wall involvement with formation of a diverticulum-like outpouching which may later form a saccular aneurysm is described as atypical FMD (Houser and Baker 1968, Houser er al 1971). Web-like or septa1 lesions are uncommon (Rainer and Cramer 1968, Houser er a1 1971, Osbom and Anderson 1977, So et a1 1981). Septa1 FMDs most likely correspond to the point of constrictions along the string of beads, but are non-circumferential dysplastic lesions that proliferate to an exagerrated degree (So et al 1979). Fusiform aneurysmal dilatation is also less frequently seen. Both these types of lesions are usually adjacent to a string of beads abnormality ( O h m and Anderson 1977, So et a1 1981).
These different morphologic patterns may represent different stages of development or progression of this arteriopathy. Bragin and Cherkasov (1979) suggest that fibroblastic transformation of smooth muscle cells plays the primary role in the morphogenesis of different patterns of FMD lesions. Hata et al(1976) consider that an elastic tissue defect underlying the disease process results in a reparative response by smooth muscle and fibrous tissue proliferation. Atherosclerosis and post-traumatic aneurysm may be considered in the differential diagnosis of fusiform aneurysm of left common carotid artery. The age of the patient and a past history of trauma are relevant clues to diagnosis. False aneurysm due to penetrating trauma, intravascular injury during angiography or post-operative injury are rarely found. Mycotic aneuryms of the great vessels are usually related to contiguous infections of the soft tissue or lung apex. Syphilitic aneurysms of the aortic arch branches are quite rare though lesions of the innominate and subclavian arteries have been reported (Abrams 1983). Congenital aneurysms (Hammon et al 1972) and those associated with coarctation of aorta (Stem er al 1970) are also described. The web-like lesion adjacent to the aneurysmal dilatation in our young female patient strongly indicates the diagnosis of FMD and resembles a similar observation by others (Osbom and Anderson 1977, So et a1 1981). The fusiform dilatation with undulating margins in our case probably represents an extreme form of or progression of a string of beads lesion.
REFERENCES Abrams HL (ed). Abrams Angiography. Vascular and Interventional Radiology; Boston: Little, Brown and Company, 3rd Edition 1983: 434435.
Bradac GB. Considerations Concerning a Case of Fibromuscular Hyperplasia of the Carotid Arteries. Neuroradiology 1970 1 : 217-219.
Bragin M A and Cherkasov AP. Morphogenesis of Fibromuscular Dysplasia of Renal Arteries, An Ultrastructural Study. Arch Pathol Lab Med 1979; 41 : 46-52. Hammon JW. Silver D and Young WG. Congenital Aneurysms of the Extracranial Carotid Arteries. Ann Surg 1972: 176 : 777-781. Harrison EG and McCormack LJ. Pathologic Classification of Renal Artery Disease in Renovascular Hypertension. Mayo Clin Proc 1971: 46:161-167. Hata J, Hosoda Y and Shimizu K . Flbromuscular Dysplasia: a Histopathological Study. Exp Clin Med 1976; 1 : 67-78. Houser OW and Baker HL Jr. Fibromusculal Dysplasia and Other Uncommon Diseases of Cervical Carotid Artery : Angiographic Aspects. Amer J Roentgenology 1968: 104 : 201-212. Houser OW. Baker HL Jr. Sandok BA and Holley KE. Cephalic Artenal Fibrornuscular Dysplasia. Radiology 1971: 101 : 605-611. Kincaid OW. Davis GD. Hatterman FJ and Hunt JC. Fibromuscular Dysplasia of the Renal Arteries: Arteriographic Features. Classificanon and Observation on Natural History of the Disease. Amer J Roentgenology 1968: 104 : 271-282. Leadbetter WF and Burkland CE. Hypertension in Unilateral Renal Disease. J Urol 1938: 34 : 61 1-626. FIGURE 2 - Lateral view showing the distal web-like lesion and good distal tilling of internal and external carotid arteries. Australasian Radiology. Val. XXXN. No. 4. Nowmber. I990
McComack U,Pouttasse EF, Meaney TF. Noto TJ Jr and Dustan HP. A Pathologic-Arteriographic Correlation of Renal Arterial Direase. Am Heart J 1966: 72 : IXX-19X.
35 1
R.V. PHADKE ef a1 McCormack LJ, Dustan Kp and Meaney W. Selected Pathology of the Renal Altery. Semin. Roentnenol 1967: 2 : 126. Osborn AG-and Anderson Angiographic Spectrum of Cervical and Intracmial Fibromuscular Dysplasia. Stroke 1977; 8 :617-626. Rainer WG, ‘&mer GC and Newby Jp. Fibromuscula HYmlasia of the Carotid Artery Causing Positional Cerebral Ischemia. Ann Surg 1968: 167 :4.44-445. So E, Toole JF, Dalal P and Moody DM. Cephalic Fibromuscular Dysplasia in 32 Patients - Clinical Findings and Radiologic Features. Arch Neurol 1981; 38 :619-622.
&.
352
So EL, Toole JF,Moody DM and Challa VR. Cerebral Embolism from Septa1 Fibromuscular Dysplasia of the Common Carotid Artery. Ann . . Neurol i979; :75-78. Stanley JC, Gewertz EL, Bove EL, Sottiura V and Fry WJ. Arterial Fibromuscular Dysplasia: Histopathologic Character and Currenr Etiologic concepts. Arch sue 1975: 110 :561-566. Stern W Z , Richardson JO Jr and Wolfe R. Multiple Calcified Aneurysms in Coarctation of Aorta, Radiology 1970: 96 : 331-334.
Australasian Radiology, Vol. XXXN, No. 4, November, 1990
Fibromuscular Dysplasia of Common Carotid Artery A Case Report R.V.PHADKE,D.M.R.D.,M.D. Assistant Professor,Radiology Sanjay Gana'hi Postgraduate Institute of Medical Sciences Lucknow - 226001 India. (Formerly Reader in Radiology, Govt. Medical College, Nagpur, 440003, India) *K.B. TAORI, D.M.R.D.,M.D. Reader in Radiology (At present Radiologist, Royal Hospital, P.B. 2331. Seeb, Sultanate of Oman) *V.K. DIVEKAR, M.S.,F.R.C.S., D.M.R.D. Senior Lecturer in Radiology *V.R. BHUTADA,D.M.R.E., M.D. Professor and Head of Radiology *Govt.Medical College,Nagpur, 440003, India.
ABSTRACT A case of presumed fibromuscular dysplasia involving the left common carotid artery with formation of fusiform aneurysm of the whole length of the common carotid artery, and a distal web-like lesion, is described in a 25 year old female patient. The angiographic appearance of the lesion is uncommon, as is the involvement of the common carotid artery. The literature is reviewed.
Transfemoral selective angiography showed fusiform dilatation of the left common carotid artery with undulating margins and filling defects suggesting thrombus formation. At the upper end of the common carotid artery a distinct almost circumferential web was noted. Distally the external and internal carotid arteries were normal with good filling of intracranial vessels. Angiography of the right carotid artery and both renal arteries revealed no abnormality.
INTRODUCTION Fibromuscular dysplasia (FMD) is a well recognised non-atheromatous vascular disease entity. First observed in renal arteries in 1938 (Leadbetter and Burkland), FMD has been recognised subsequently in splanchnic, iliac, extracranial internal carotid, vertebral and intracranial arteries. Visceral veins and aorta may also be affected by FMD (So et a1 1981). FMD, generally a disease of young women (20 to 40 yrs) (McCormack et a1 1966), commonly affects renal and extracranial internal carotid vessels. Involvement of the common carotid artery is relatively uncommon. We report a case with involvement of left common carotid artery with formation of a fusiform aneurysm.
CASE REPORT A 25 year old normotensive female patient presented with a diffuse pulsatile swelling in the lower left side of the neck. No bruit was heard over the swelling. The patient was non-diabetic and serological tests for syphilis were negative. She had no relevant history of trauma or infection.
Key wonls: Aneurysm, Carotid Carotid Arteries Fibrodysplasia
Address for correspondence:
FIGURE 1 - AP view, selective left common carotid angiogram showing fusiform aneurysm with filling defects suggestive of thrombus formation.
Dr.R.V. Phadke Assistant Professor, Radiology Sanjay Gandhi Postgraduate Institute of Medical Sciences P.B. 375.Rae Bareli Road, Lucknow (U.P.) India - 226001
350
Submitted for publication on: 15th August, 1989 Resubmitted for publication on: 1st May, 1990 Accepted for publication on: 3rd July, 1990 Australasian Radiology. Vol. X X X N , No. 4 . November. 1990
FIBROMUSCULAR DYSPLASIA The patient had no neurological deficit and declined to undergo any surgical intervention. DISCUSSION There is abundant literature describing various histological types and angiographic ,patterns of FMD. Three main histological types have been identified viz: (i) intima1 fibroplasia, (ii) medial fibroplasia or fibromuscular hyperplasia and (iii) subadventitial hyperplasia (McCormack et a/ 1966, Kincaid et a/ 1968, Harrison and McCormack 197 1, Stanley et a1 1975). Three characteristic angiographic patterns are associated with cephalocervical FMD (Houser and Baker 1968, Bradac 1970, Osbom and Anderson 1977, So e t a / 1981). (i) The “string of beads” appearance is considered most common (80%) and almost pathognomonic. Medial fibroplasia is the most common histological type of FMD associated with this angiographic finding (Osborn and Anderson 1977). (ii) The second and less common angiographic pattern is unifocal o r multifocal tubular stenosis which may be associated with any of the three histological types. (iii) Localised wall involvement with formation of a diverticulum-like outpouching which may later form a saccular aneurysm is described as atypical FMD (Houser and Baker 1968, Houser er al 1971). Web-like or septa1 lesions are uncommon (Rainer and Cramer 1968, Houser er a1 1971, Osbom and Anderson 1977, So et a1 1981). Septa1 FMDs most likely correspond to the point of constrictions along the string of beads, but are non-circumferential dysplastic lesions that proliferate to an exagerrated degree (So et al 1979). Fusiform aneurysmal dilatation is also less frequently seen. Both these types of lesions are usually adjacent to a string of beads abnormality ( O h m and Anderson 1977, So et a1 1981).
These different morphologic patterns may represent different stages of development or progression of this arteriopathy. Bragin and Cherkasov (1979) suggest that fibroblastic transformation of smooth muscle cells plays the primary role in the morphogenesis of different patterns of FMD lesions. Hata et al(1976) consider that an elastic tissue defect underlying the disease process results in a reparative response by smooth muscle and fibrous tissue proliferation. Atherosclerosis and post-traumatic aneurysm may be considered in the differential diagnosis of fusiform aneurysm of left common carotid artery. The age of the patient and a past history of trauma are relevant clues to diagnosis. False aneurysm due to penetrating trauma, intravascular injury during angiography or post-operative injury are rarely found. Mycotic aneuryms of the great vessels are usually related to contiguous infections of the soft tissue or lung apex. Syphilitic aneurysms of the aortic arch branches are quite rare though lesions of the innominate and subclavian arteries have been reported (Abrams 1983). Congenital aneurysms (Hammon et al 1972) and those associated with coarctation of aorta (Stem er al 1970) are also described. The web-like lesion adjacent to the aneurysmal dilatation in our young female patient strongly indicates the diagnosis of FMD and resembles a similar observation by others (Osbom and Anderson 1977, So et a1 1981). The fusiform dilatation with undulating margins in our case probably represents an extreme form of or progression of a string of beads lesion.
REFERENCES Abrams HL (ed). Abrams Angiography. Vascular and Interventional Radiology; Boston: Little, Brown and Company, 3rd Edition 1983: 434435.
Bradac GB. Considerations Concerning a Case of Fibromuscular Hyperplasia of the Carotid Arteries. Neuroradiology 1970 1 : 217-219.
Bragin M A and Cherkasov AP. Morphogenesis of Fibromuscular Dysplasia of Renal Arteries, An Ultrastructural Study. Arch Pathol Lab Med 1979; 41 : 46-52. Hammon JW. Silver D and Young WG. Congenital Aneurysms of the Extracranial Carotid Arteries. Ann Surg 1972: 176 : 777-781. Harrison EG and McCormack LJ. Pathologic Classification of Renal Artery Disease in Renovascular Hypertension. Mayo Clin Proc 1971: 46:161-167. Hata J, Hosoda Y and Shimizu K . Flbromuscular Dysplasia: a Histopathological Study. Exp Clin Med 1976; 1 : 67-78. Houser OW and Baker HL Jr. Fibromusculal Dysplasia and Other Uncommon Diseases of Cervical Carotid Artery : Angiographic Aspects. Amer J Roentgenology 1968: 104 : 201-212. Houser OW. Baker HL Jr. Sandok BA and Holley KE. Cephalic Artenal Fibrornuscular Dysplasia. Radiology 1971: 101 : 605-611. Kincaid OW. Davis GD. Hatterman FJ and Hunt JC. Fibromuscular Dysplasia of the Renal Arteries: Arteriographic Features. Classificanon and Observation on Natural History of the Disease. Amer J Roentgenology 1968: 104 : 271-282. Leadbetter WF and Burkland CE. Hypertension in Unilateral Renal Disease. J Urol 1938: 34 : 61 1-626. FIGURE 2 - Lateral view showing the distal web-like lesion and good distal tilling of internal and external carotid arteries. Australasian Radiology. Val. XXXN. No. 4. Nowmber. I990
McComack U,Pouttasse EF, Meaney TF. Noto TJ Jr and Dustan HP. A Pathologic-Arteriographic Correlation of Renal Arterial Direase. Am Heart J 1966: 72 : IXX-19X.
35 1
R.V. PHADKE ef a1 McCormack LJ, Dustan Kp and Meaney W. Selected Pathology of the Renal Altery. Semin. Roentnenol 1967: 2 : 126. Osborn AG-and Anderson Angiographic Spectrum of Cervical and Intracmial Fibromuscular Dysplasia. Stroke 1977; 8 :617-626. Rainer WG, ‘&mer GC and Newby Jp. Fibromuscula HYmlasia of the Carotid Artery Causing Positional Cerebral Ischemia. Ann Surg 1968: 167 :4.44-445. So E, Toole JF, Dalal P and Moody DM. Cephalic Fibromuscular Dysplasia in 32 Patients - Clinical Findings and Radiologic Features. Arch Neurol 1981; 38 :619-622.
&.
352
So EL, Toole JF,Moody DM and Challa VR. Cerebral Embolism from Septa1 Fibromuscular Dysplasia of the Common Carotid Artery. Ann . . Neurol i979; :75-78. Stanley JC, Gewertz EL, Bove EL, Sottiura V and Fry WJ. Arterial Fibromuscular Dysplasia: Histopathologic Character and Currenr Etiologic concepts. Arch sue 1975: 110 :561-566. Stern W Z , Richardson JO Jr and Wolfe R. Multiple Calcified Aneurysms in Coarctation of Aorta, Radiology 1970: 96 : 331-334.
Australasian Radiology, Vol. XXXN, No. 4, November, 1990
