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Fibromuscular dysplasia and aneurysm formation in the brachial artery
Eur J Vasc Surg 7, 740-743 (1993)
CASE REPORT Fibromuscular Dysplasia and Aneurysm Formation in the Brachial Artery A. R. Shipolini and J. H. N. Wolfe St Mary's Hospital, London W 2 1NY, U.K. A patient with isolated right brachial artery aneurysms is presented. As a young woman these had been misdiagnosed, leading to inappropriate surgery and a subsequent brachial artery aneurysm produced median nerve neuropraxia. Resection of the aneurysm and bypass relieved these symptoms and the aetiology was fibromuscular dysplasia. The literature on fibromuscular dysplasia and aneurysms of the brachial artery are reviewed. Key Words: Fibromuscular dysplasia; BrachiaI artery; Aneurysm.
Introduction A n e u r y s m s exclusively affecting the arteries of the u p p e r limb are rare. We p r e s e n t a case of a patient w h o d e v e l o p e d recurrent a n e u r y s m s affecting the arteries of the right arm in the presence of an otherwise normal arterial tree.
Case Report A 63-year-old w o m a n p r e s e n t e d with pain over the medial side of the right forearm for 7 months. This was associated with paraesthesia and weakness in the fingers related to exercise. Forty years previously a " g a n g l i o n " h a d b e e n r e m o v e d from the right wrist and she was told that this h a d b e e n a " v e r y difficult" operation. T w e n t y years later she d e v e l o p e d a lump in the palm of the right wrist which d r e w the middle phalanx d o w n into a flexed position. There was considerable blood loss at the operation so that a m p u tation of the middle finger a n d third metacarpal was necessary. O n examination she looked well with a regular pulse and blood pressure of 150/80. All peripheral Please address all correspondence to: J. H. N. Wolfe, St Mary's Hospital, London W2 1NY, U.K. 0950-821X/93/060740+04$08.00/0© 1993 Grune & Stratton Ltd.
pulses w e r e present and s y n c h r o n o u s with no audible bruits. There was a 4 cm expansile swelling in the u p p e r arm consistent with a brachial artery aneurysm. Some wasting of the small muscles of the right h a n d was n o t e d but p o w e r and sensation w e r e normal. All routine blood tests were normal apart from marginally raised levels of cholesterol and triglycerides. A b d o m i n a l aortic u l t r a s o u n d scanning was normal and an intravenous digital subtraction angiogram s h o w e d no abnormalities in the left arm and neck vessels. There was a right brachial artery aneury s m a n d multiple small a n e u r y s m s in an ectatic radial artery (Fig. 1). Pre-operative EMG studies s h o w e d evidence of right m e d i a n nerve compression and a mild ulnar n e r v e lesion at the elbow. The operative p r o c e d u r e involved excision of the right brachial artery a n e u r y s m . At operation the m e d i a n n e r v e was f o u n d to be stretched over the a n e u r y s m . The vessel was repaired with a r e v e r s e d long s a p h e n o u s vein graft. Histology revealed a p a t c h y thickening with focal calcification of the intima. The internal elastic lamina was intact and not fragmented. The principal lesion was considered to be focal d e g e n e r a t i o n of the media and a provisional diagnosis of arterial fibrom u s c u l a r dysplasia was made. Culture of the arterial wall was negative.
Fibromuscular Dysplasia and Aneurysm Formation
741
Fig. 1. intravenous digital subtraction angiogram showing the brachial artery aneurysm prior to surgical repair.
Fig. 2. Intravenous digital subtraction angiogram appearance of brachial artery following repair with a reversed saphenous vein graft.
The patient p r e s e n t e d again 14 m o n t h s later with ischaemic pain in the right h a n d on exercise, associated with n u m b n e s s in the hand, especially whilst driving. The n u m b n e s s was f o u n d to be in the sensory distribution of the m e d i a n nerve. O n examination both ulnar and radial pulses w e r e present but two small a n e u r y s m s in the anterior cubitat fossa w e r e palpable. There was decreased sensation a r o u n d the thumb. A selective right subclavian artery angiogram s h o w e d a n e u r y s m formation of the native brachial artery distal to the graft (Fig. 2). There was complete occlusion of the more distal brachial artery and the radial artery reconstituted above the wrist by a series of collaterals. In the forearm there was a small vessel containing multiple a n e u r y s m s w h i c h was p r e s u m e d to be the exterior interosseous artery. At operation the lower anastomosis was identified and the brachial artery exposed below the elbow. A n arm vein was h a r v e s t e d from the forearm and u s e d to bridge the segment. The brachial a n e u r y s m was ligated proximally and distally. Postoperatively the patient's s y m p t o m s imp r o v e d considerably and there were good distal pulses. Further histology s h o w e d a m a r k e d degree of
irregular intimal thickening associated with florid s m o o t h muscle obliteration. There was also a m a r k e d d e g r e e of elastin fibre production. Culture of the arterial wall was negative.
Discussion
A review of the literature reveals that isolated cases of brachial a r t e r y a n e u r y s m s are a very rare p h e n o m enon. A m o n g s t t h e r e p o r t e d cases, the majority are associated with physical injuries, 1-4"x6 or have an infective aetiology, 5' 6 both of which were excluded in o u r patient: A n u m b e r of congenital brachial artery a n e u r y s m s have also been r e p o r t e d 7'8 and in these cases the diagnosis has usually b e e n arrived at by the exclusion of other possible causes. Connective tissue disorders have the potential to inflict arterial wall d a m a g e and give rise to s u b s e q u e n t a n e u r y s m formation, but i n v o l v e m e n t of the m e d i u m sized arteries has only rarely b e e n r e p o r t e d in association with neurofibromatosis, 9 Kawasaki disease, ~° giant cell arteritis, 11'12 periarteritis nodosa, 13'14 Behcet's disease ~5 a n d Takayasu's disease. Eur J Vasc Surg Vol 7, November 1993
742
A.R. Shipolini and d. H. N. Wolfe
A brachial artery aneurysm due to fibromuscular dysplasia and exclusively affecting the upper limb has been described only once to our knowledge. 17 Fibromuscular dysplasia was originally described in the renal artery by Leadbetter is and until 196419 it was thought to be confined to that vessel. Reports have shown the involvement of fibromuscular dysplasia in the intra-cranial, 2°-24 carotid, 26-31 renal, 32-4° coronary, 41'42 visceral, 43-46 posterior auricular, 47 spinal, 48 femoral, 49 cervical sl"s2 and external iliac 52 arteries. The aetiology of fibromuscular dysplasia remains uncertain but a number of theories have been proposed and the pathogenesis would appear to be multifactoral. Physical stresses to the arterial wall either by direct trauma or mediated through stretching forces on the vessel have been implicated as a possible cause of dysplastic changes, but we consider this to be unlikely. Damage to the vasa vasorum resulting in localised ischaemic injury may also be implicated, s3 The propensity of fibromuscular dysplasia for involvement of the renal, carotid and iliac arteries may relate to their more sparse vasa vasorum and add further support to the theory of mural ischaemia, s4 Further evidence is added by the findings of dysplasia frequently involving the outer media which is more dependent for its nourishment upon the arterial wall's blood supply. 5s The greater incidence of fibromuscular dysplasia found in females, 22 especially in their fourth and fifth decades, has given rise to speculation that endocrine factors have an important bearing on the development of the condition, s6 Experiments have shown that oestrogens are capable of stimulating secretions from fibroblastic and smooth muscle cells with the formation of abnormal fibrous bands. Infective agents, such as the Rubella and Herpes viruses, have also received consideration as potential aetiological factors but their significance remains speculative. Increased evidence of fibromuscular dysplasia amongst siblings 21 implicates a genetic factor in the pathogenesis of the condition. Clinical presentation of fibromuscular dysplasia can take the form of an aneurysmal dilatation or distal arterial insufficiency resulting from stenoses, dissection or multi-embolic events. The precise nature of
the arteriopathy can be determined by angiographtc studies. One of the typical appearances is the "string of beads" sign due to multifocal stenoses and aneurysms. The histology is classified according to the site of the lesion in the arterial wall. Intimal fibroplasia is rare but more common in childrenY The media and Eur J Vasc Surg Vol 7, November 1993
adventitia are usually unaffected. By far the commonest site of involvement is the media (94-97% of cases). Adventitial fibroplasia is very rare and usually results in a stenotic segment due to the formation of fibrous bands. Surgical reconstruction remains the mainstay treatment for aneurysmal lesions and long stenotic segments. The use of a reversed vein graft with endto-end anastomosis is the best conduit.
References 1 RAO PL, RAO KM, WARRIERPK. Traumatic pseudo-aneurysms of the brachial artery in children. Indian ] Paediatr 1983; 50(402): 85-88. 2 MILLER DR, MALKI A. Large saccular aneurysms and arteriovenous fistula of the brachial vessels. J Cardiovasc Sug Torino 1985; 26(6): 605-607. 3 REY C, MARACHE P, WATEL A, FRANCARTC. Iatrogenic false aneurysm of the brachial artery in an infant. Eur J Paediatr 1987; 146(4): 438-439. 4 LOUGHLINV, BENIWALJS. Post-traumatic brachial artery aneurysm and arterio-venous fistulae. J Cardiovasc Surg Torino 1988; 29(5): 570-571. 5 CABELLON S JR, CAINES TG, MONSIVAIS JJ, APGAR RG, CAVANAUGH DG. Brachial artery mycotic aneurysms: complications of cardiac catheterisation. Milit Med 1986; 151(1): 54-56. 6 Ho PK, YAREMCHUKMJ, DELLONAL. Mycotic aneurysms of the upper extremity, report of two cases. J Hand Surg Pr 1896; 11(2): 271-273. 7 JONESTR, FRUSHAJD, STROMEYERFW. Brachial artery aneurysm in an infant: case report and review of the literature. J Vasc Surg 1988; 7(3): 439-442. 8 LIE JT, HAYES CW, FEINTUCH TA. Congenital brachial artery aneurysm in an infant--a case report. Angiologv 1988; 39(1881): 40-44. 9 TAMERISA, TURECHID, LAZARL, WARNOCKR. Congenital subclavian arterial aneurysm in a four year old male. Angiology 1974; 25: 769-772. 10 SASAGURIY, KATO H. Regression of aneurysms in Kawasaki disease; a pathological study. J Paediatr 1982; 100: 225-231. 11 HOLLEMANJH JR, MARTIN BF, PARKERJH. Giant cell arteritis causing brachial artery aneurysm in an eight year old child. J Miss State Assoc 1983; 24: 327-328. 12 WAGENVOORTGA, HARRIS LE, BROWNAL JR, VEENEKLASASG. Giant cell arteritis with aneurysm formation in children. Paediatrics 1963; 32: 861-867. 13 CHAMBERLAINJL III, PERRY LW. Infantile polyarteritis nodosa with coronary and brachial aneurysms: a case diagnosed during life. J Paediatr 1971; 78: 1039-104t. 14 ALMGREN B, ERICKSSONR, FOUCARDT, LORELIUSL, OLSEN L. Multiple aneurysms of visceral arteritis in a child with polyarteritis nodosa. J Paediatr Surg 1980; 15: 347-348. 15 KRISTENR, SCHMITZ-RIxENT,HUBERP, ERASHIH. Aneurysms of the brachial artery--a rare complication of Behcet's disease. 16 Ho PK, WEILANDAl, McCLINTON MA, SHAWWILGISEF. Aneurysrns of tim upper extremity. ] Hand Surg 1987; lgA: 39-46.
17 OLSANLA, FAJl~llDB, LtMARJV, ROLrrMANBN. Fibromuscular hyperplasia of the brachial artery--failure of calcium channel therapy. Angiology 1984; 790-796. 18 LEADBETTERWF,BURKLANDCE.Hypertension in unilateralrenal disease. ! Urol 1938; 39: 611-626. 19 PALUBINSKASAJ/ RIPLEYHR. Fibromuscular dysplasia in extrarenal arteries. Radiology 1964; 82: 451-455. 20 VLESJS, HENDRIKSJJ, LODDERJ, JANEVSKIB. Multiple vertebro-
Fibromuscular Dysplasia and Aneurysm Formation
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22
23
24
25 26 27 28
29 30
31 32 33 34 35 36 37 38
basilar infarctions from fibromuscular dysplasia related dissecting aneurysm of the vertebral artery in a child. Neuropediatrics 1990; 21(2): 104-105. OUCHI Y, TAGAWAH, YAMAKADOM, TAKANASHIR, TANAKAS. Clinical significance of cerebral aneurysm in renovascular hypertension due to fibromuscular dysplasia: two cases in siblings. Angiology 1989; 40(6): 581-588. GEORGEB, ZERAH M, MOURIERKL, GELBERTF, REIZlNED. Ruptured intracranial aneurysms. The influence of sex and fibromuscular dysplasia upon prognosis. Acta Neurochir Wien 1989; 97(1-2): 26-30. ITOYAMAY, FUKUMURAA, NONAKA N, ITOH Y, TAKAMURAS, MATSUKADOY. Fibromuscular dysplasia accompanied by giant intracranial fusiform aneurysm--report of two cases. Neurol Med Chit Tokyo 1988; 28(6): 579-583. PEREZ-HIGUERASA, ALVAREZ-RuIz F, MARTINEZ-BERMEJOA, FRUTOSR, VILLARO, DIEZ-TEJEDORE. Cerebellar infarction from fibromuscular dysplasia and dissecting aneurysm of the cerebral artery. Report of a child. Stroke 1988; 19(4): 521-524. IMAYAH, KOBAYASHIS, OKADAT, MURAYAMAK, NAKAZAWAS. IA case of fibromuscular dysplasia with middle cerebral aneurysm. No Shinkei Geka 1985; 13(6): 653-685. PHADKERV, RAORIKB, DIVEKARVK, BHUTADAVR. Fibromuscular dysplasia of common carotid artery. A case report. Australas Radiol 1990; 34(4): 350-352. POZZATI E, GIULIANI G, ACCIARRI N, Nuzzo G. Long term follow-up of occlusive cervical carotid dissection. Stroke 1990; 21(4): 526-531. ROSSILLONR, SIX C, DARDENNEG- Dysplasia and spontaneous bilateral dissection of the internal carotid arteries: Apropos of a case. Value of surgical treatment. Acta Chir Belg 1990; 90(3): 97101. D'ANGLEJAN-CHATILLONJ, RIBEIRO V, MAS JL, BOUSSERMG, LEPLANED. Dissection of the extracranial internal carotid artery. 62 cases. PresseMed 1990; 19(14): 661-667. MAIURIF, GALLICCHIOB, GANGEMIM, BRIGANTIF, IACONETTAG, CORRIERO G. Fibromuscular dysplasia of the carotid arteries. Clinical and radiological considerations. Clin Neuro Neurosurg 1988; 90(1): 57-60. WESENCA, ELLIOTTBM. Fibromuscular dysplasia of the carotid arteries. Am J Surg 1986; 151(4): 448-451. HARRIS JP, WALKER PJ, WHtATE GH, MAY J. Bench repair of complex renal arterial lesions. Ann Vasc Surg 1991; 5(2): 138142. NovlcK AC. Management of renovascular disease. A surgical perspective. Circulation 1991; 83 (2 Suppl): 1167-1171. PARKSH, CHI JG, CHOI Y. Primary intimal fibroplasia with multiple aneurysms of renal artery in childhood. Child Nephrol Urol 1990; 10(1): 51-55. SAVASTANOS, FELTRIN GP, MIOTTO D, CHIESURA-CORONAM. Renal aneurysm and arteriovenous fistula. Management with transcatheter embolisation. Acta Radiol 1990; 31(1): 73-76. SIEGELBAUMMH, WEISSJP. Renal infarction secondary to fibrous dysplasia and aneurysm formation of renal artery. Urology 1990; 35(1): 73-75. BARTELM, RICHTERS, WAGNERW, CLAUSNERG. Surgical intervention in stenoses and aneurysms of the renal artery. Zentralbl Chit 1989; 114(19): 1259-1268. HAU T. Kidney autotransplantation in therapy of complex reno-
39 40
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50
51 52
53 54 55 56 57
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vascular diseases. Indications, technique and results. Zentralbl Chir 1988; 113(16): 1024-1034. MERCIERC, PIQUETP, PILIGIANF, FERDANIM. Aneurysms of the renal artery and its branches. Ann Vasc Surg 1986; 1(3): 321-327. LORELIUSLE, HEMMINGSSONA, HAGG A, MORLIN C, ABERGH. Progressive fibromuscular dysplasia of the renal artery. Angiographic and clinical follow-up. Acta Radiol Diagn Stockh 1985; 26(6): 705-708. SCHNEIDERJ, COESTERC, MEYERW, OECHSLINE. Unusual cases of coronary death. Schweiz Med Wochenschr 1987; 10, 117(41): 1577-1582. LIE JT, BERGKK. Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction. Hum Pathol 1987; 18(6): 654-656. DETROUXM, FIEVEZM, MASSIN H, BLAMPAINJP. Rupture of a superior mesenteric artery aneurysm in a child. Ann Vasc Surg 1990; 4(5): 505-509. UNOVARE, PISKIN B. Hemobilia from a ruptured hepatic artery aneurysm in a 16 year old girl. Turk J Pediatr 1989; 31(1): 63-70. ASCHWANDENM, SCHMIDP. Ruptured aneurysm of the greater omentum in fibromuscular displasia. Vasa 1989; 18(2): 157-161. SMITH JA, MACLEISHDG, COLLIERNA. Aneurysms of the visceral arteries. Aust NZ J Surg 1989; 59(4): 329-334. CtNA C, WILLIAMSONC, AMELIFM. Fibromuscular dysplasia of the posterior auricular artery: an unusual aneurysmal lesion. J Cardiovasc Surg Turino 1988; 29(1): 56-59. SAUNDERsFW,BIRCHARDD,WlLLMERJ. Spinal artery aneurysm. Surg Neurol 1987; 27(3): 269-272. HERPELSV, VAN DEVOORDEW, WILMSG, VERBEKENE, BAERTA, LAUWERYNSJ, NEVELSTEENA. Recurrent aneusysms of the upper arteries of the lower limb: an atypical manifestation of fibromuscular dysplasia--a case report. Angiology 1987; 38(5): 411416. FUKAZAWAS, IMAI S, SAITOA, YAMADAH. Fibromuscular dysplasia of the cervical arteries associated with a distal vertebral trunk aneurysm. Case report. NeurolMed Chir Tokyo 1990; 30 (11 Spec No): 899-903. GEORGEB, MOURIERKL, GELBERTF, REIZINED, RAGGUENEAUJL. Vascular abnormalities in the neck associated with intracranial aneurysms. Neurosurgery 1989; 24(4): 499-508. PATELKS, WOLFEJH, MATHIASC. Left external iliac artery dissection and bilateral renal artery aneurysms secondary to fibromuscular dysplasia: a case report. Neth J Surg 1990; 42(4): 118120. STANLEYJC, GEWERTZBL, BOVEEL, SOTTIURAIV, FRY WJ. Arterial fibrodysplasia--histopathological character and current aetiological concepts. Arch Surg 1975; 110: 561-566. KELLY TF JR, MORRIS GC JR. Arterial fibromuscular disease. Observations on pathogenesis and surgical management. Ann J Surg 1982; 143: 232-236. NAKATAY. An experimental study on vascular lesions caused by obstruction of the vasa vasorum. Jap CircJ 1987; 31: 275-287. SATTIURAIUS, FRY WJ, STANLEYJC. Ultrastructure of medial smooth muscle and myofibroblasts in human arterial dysplasia. Arch Surg 1978; 113: 1280-1288. PERRYMD. Fibromuscular dysplasia. Surg Gynaecol Obstet 1974; 139: 97-104b.
Accepted 23 January 1992
Eur J Vasc Surg Vol 7, November 1993
CASE REPORT Fibromuscular Dysplasia and Aneurysm Formation in the Brachial Artery A. R. Shipolini and J. H. N. Wolfe St Mary's Hospital, London W 2 1NY, U.K. A patient with isolated right brachial artery aneurysms is presented. As a young woman these had been misdiagnosed, leading to inappropriate surgery and a subsequent brachial artery aneurysm produced median nerve neuropraxia. Resection of the aneurysm and bypass relieved these symptoms and the aetiology was fibromuscular dysplasia. The literature on fibromuscular dysplasia and aneurysms of the brachial artery are reviewed. Key Words: Fibromuscular dysplasia; BrachiaI artery; Aneurysm.
Introduction A n e u r y s m s exclusively affecting the arteries of the u p p e r limb are rare. We p r e s e n t a case of a patient w h o d e v e l o p e d recurrent a n e u r y s m s affecting the arteries of the right arm in the presence of an otherwise normal arterial tree.
Case Report A 63-year-old w o m a n p r e s e n t e d with pain over the medial side of the right forearm for 7 months. This was associated with paraesthesia and weakness in the fingers related to exercise. Forty years previously a " g a n g l i o n " h a d b e e n r e m o v e d from the right wrist and she was told that this h a d b e e n a " v e r y difficult" operation. T w e n t y years later she d e v e l o p e d a lump in the palm of the right wrist which d r e w the middle phalanx d o w n into a flexed position. There was considerable blood loss at the operation so that a m p u tation of the middle finger a n d third metacarpal was necessary. O n examination she looked well with a regular pulse and blood pressure of 150/80. All peripheral Please address all correspondence to: J. H. N. Wolfe, St Mary's Hospital, London W2 1NY, U.K. 0950-821X/93/060740+04$08.00/0© 1993 Grune & Stratton Ltd.
pulses w e r e present and s y n c h r o n o u s with no audible bruits. There was a 4 cm expansile swelling in the u p p e r arm consistent with a brachial artery aneurysm. Some wasting of the small muscles of the right h a n d was n o t e d but p o w e r and sensation w e r e normal. All routine blood tests were normal apart from marginally raised levels of cholesterol and triglycerides. A b d o m i n a l aortic u l t r a s o u n d scanning was normal and an intravenous digital subtraction angiogram s h o w e d no abnormalities in the left arm and neck vessels. There was a right brachial artery aneury s m a n d multiple small a n e u r y s m s in an ectatic radial artery (Fig. 1). Pre-operative EMG studies s h o w e d evidence of right m e d i a n nerve compression and a mild ulnar n e r v e lesion at the elbow. The operative p r o c e d u r e involved excision of the right brachial artery a n e u r y s m . At operation the m e d i a n n e r v e was f o u n d to be stretched over the a n e u r y s m . The vessel was repaired with a r e v e r s e d long s a p h e n o u s vein graft. Histology revealed a p a t c h y thickening with focal calcification of the intima. The internal elastic lamina was intact and not fragmented. The principal lesion was considered to be focal d e g e n e r a t i o n of the media and a provisional diagnosis of arterial fibrom u s c u l a r dysplasia was made. Culture of the arterial wall was negative.
Fibromuscular Dysplasia and Aneurysm Formation
741
Fig. 1. intravenous digital subtraction angiogram showing the brachial artery aneurysm prior to surgical repair.
Fig. 2. Intravenous digital subtraction angiogram appearance of brachial artery following repair with a reversed saphenous vein graft.
The patient p r e s e n t e d again 14 m o n t h s later with ischaemic pain in the right h a n d on exercise, associated with n u m b n e s s in the hand, especially whilst driving. The n u m b n e s s was f o u n d to be in the sensory distribution of the m e d i a n nerve. O n examination both ulnar and radial pulses w e r e present but two small a n e u r y s m s in the anterior cubitat fossa w e r e palpable. There was decreased sensation a r o u n d the thumb. A selective right subclavian artery angiogram s h o w e d a n e u r y s m formation of the native brachial artery distal to the graft (Fig. 2). There was complete occlusion of the more distal brachial artery and the radial artery reconstituted above the wrist by a series of collaterals. In the forearm there was a small vessel containing multiple a n e u r y s m s w h i c h was p r e s u m e d to be the exterior interosseous artery. At operation the lower anastomosis was identified and the brachial artery exposed below the elbow. A n arm vein was h a r v e s t e d from the forearm and u s e d to bridge the segment. The brachial a n e u r y s m was ligated proximally and distally. Postoperatively the patient's s y m p t o m s imp r o v e d considerably and there were good distal pulses. Further histology s h o w e d a m a r k e d degree of
irregular intimal thickening associated with florid s m o o t h muscle obliteration. There was also a m a r k e d d e g r e e of elastin fibre production. Culture of the arterial wall was negative.
Discussion
A review of the literature reveals that isolated cases of brachial a r t e r y a n e u r y s m s are a very rare p h e n o m enon. A m o n g s t t h e r e p o r t e d cases, the majority are associated with physical injuries, 1-4"x6 or have an infective aetiology, 5' 6 both of which were excluded in o u r patient: A n u m b e r of congenital brachial artery a n e u r y s m s have also been r e p o r t e d 7'8 and in these cases the diagnosis has usually b e e n arrived at by the exclusion of other possible causes. Connective tissue disorders have the potential to inflict arterial wall d a m a g e and give rise to s u b s e q u e n t a n e u r y s m formation, but i n v o l v e m e n t of the m e d i u m sized arteries has only rarely b e e n r e p o r t e d in association with neurofibromatosis, 9 Kawasaki disease, ~° giant cell arteritis, 11'12 periarteritis nodosa, 13'14 Behcet's disease ~5 a n d Takayasu's disease. Eur J Vasc Surg Vol 7, November 1993
742
A.R. Shipolini and d. H. N. Wolfe
A brachial artery aneurysm due to fibromuscular dysplasia and exclusively affecting the upper limb has been described only once to our knowledge. 17 Fibromuscular dysplasia was originally described in the renal artery by Leadbetter is and until 196419 it was thought to be confined to that vessel. Reports have shown the involvement of fibromuscular dysplasia in the intra-cranial, 2°-24 carotid, 26-31 renal, 32-4° coronary, 41'42 visceral, 43-46 posterior auricular, 47 spinal, 48 femoral, 49 cervical sl"s2 and external iliac 52 arteries. The aetiology of fibromuscular dysplasia remains uncertain but a number of theories have been proposed and the pathogenesis would appear to be multifactoral. Physical stresses to the arterial wall either by direct trauma or mediated through stretching forces on the vessel have been implicated as a possible cause of dysplastic changes, but we consider this to be unlikely. Damage to the vasa vasorum resulting in localised ischaemic injury may also be implicated, s3 The propensity of fibromuscular dysplasia for involvement of the renal, carotid and iliac arteries may relate to their more sparse vasa vasorum and add further support to the theory of mural ischaemia, s4 Further evidence is added by the findings of dysplasia frequently involving the outer media which is more dependent for its nourishment upon the arterial wall's blood supply. 5s The greater incidence of fibromuscular dysplasia found in females, 22 especially in their fourth and fifth decades, has given rise to speculation that endocrine factors have an important bearing on the development of the condition, s6 Experiments have shown that oestrogens are capable of stimulating secretions from fibroblastic and smooth muscle cells with the formation of abnormal fibrous bands. Infective agents, such as the Rubella and Herpes viruses, have also received consideration as potential aetiological factors but their significance remains speculative. Increased evidence of fibromuscular dysplasia amongst siblings 21 implicates a genetic factor in the pathogenesis of the condition. Clinical presentation of fibromuscular dysplasia can take the form of an aneurysmal dilatation or distal arterial insufficiency resulting from stenoses, dissection or multi-embolic events. The precise nature of
the arteriopathy can be determined by angiographtc studies. One of the typical appearances is the "string of beads" sign due to multifocal stenoses and aneurysms. The histology is classified according to the site of the lesion in the arterial wall. Intimal fibroplasia is rare but more common in childrenY The media and Eur J Vasc Surg Vol 7, November 1993
adventitia are usually unaffected. By far the commonest site of involvement is the media (94-97% of cases). Adventitial fibroplasia is very rare and usually results in a stenotic segment due to the formation of fibrous bands. Surgical reconstruction remains the mainstay treatment for aneurysmal lesions and long stenotic segments. The use of a reversed vein graft with endto-end anastomosis is the best conduit.
References 1 RAO PL, RAO KM, WARRIERPK. Traumatic pseudo-aneurysms of the brachial artery in children. Indian ] Paediatr 1983; 50(402): 85-88. 2 MILLER DR, MALKI A. Large saccular aneurysms and arteriovenous fistula of the brachial vessels. J Cardiovasc Sug Torino 1985; 26(6): 605-607. 3 REY C, MARACHE P, WATEL A, FRANCARTC. Iatrogenic false aneurysm of the brachial artery in an infant. Eur J Paediatr 1987; 146(4): 438-439. 4 LOUGHLINV, BENIWALJS. Post-traumatic brachial artery aneurysm and arterio-venous fistulae. J Cardiovasc Surg Torino 1988; 29(5): 570-571. 5 CABELLON S JR, CAINES TG, MONSIVAIS JJ, APGAR RG, CAVANAUGH DG. Brachial artery mycotic aneurysms: complications of cardiac catheterisation. Milit Med 1986; 151(1): 54-56. 6 Ho PK, YAREMCHUKMJ, DELLONAL. Mycotic aneurysms of the upper extremity, report of two cases. J Hand Surg Pr 1896; 11(2): 271-273. 7 JONESTR, FRUSHAJD, STROMEYERFW. Brachial artery aneurysm in an infant: case report and review of the literature. J Vasc Surg 1988; 7(3): 439-442. 8 LIE JT, HAYES CW, FEINTUCH TA. Congenital brachial artery aneurysm in an infant--a case report. Angiologv 1988; 39(1881): 40-44. 9 TAMERISA, TURECHID, LAZARL, WARNOCKR. Congenital subclavian arterial aneurysm in a four year old male. Angiology 1974; 25: 769-772. 10 SASAGURIY, KATO H. Regression of aneurysms in Kawasaki disease; a pathological study. J Paediatr 1982; 100: 225-231. 11 HOLLEMANJH JR, MARTIN BF, PARKERJH. Giant cell arteritis causing brachial artery aneurysm in an eight year old child. J Miss State Assoc 1983; 24: 327-328. 12 WAGENVOORTGA, HARRIS LE, BROWNAL JR, VEENEKLASASG. Giant cell arteritis with aneurysm formation in children. Paediatrics 1963; 32: 861-867. 13 CHAMBERLAINJL III, PERRY LW. Infantile polyarteritis nodosa with coronary and brachial aneurysms: a case diagnosed during life. J Paediatr 1971; 78: 1039-104t. 14 ALMGREN B, ERICKSSONR, FOUCARDT, LORELIUSL, OLSEN L. Multiple aneurysms of visceral arteritis in a child with polyarteritis nodosa. J Paediatr Surg 1980; 15: 347-348. 15 KRISTENR, SCHMITZ-RIxENT,HUBERP, ERASHIH. Aneurysms of the brachial artery--a rare complication of Behcet's disease. 16 Ho PK, WEILANDAl, McCLINTON MA, SHAWWILGISEF. Aneurysrns of tim upper extremity. ] Hand Surg 1987; lgA: 39-46.
17 OLSANLA, FAJl~llDB, LtMARJV, ROLrrMANBN. Fibromuscular hyperplasia of the brachial artery--failure of calcium channel therapy. Angiology 1984; 790-796. 18 LEADBETTERWF,BURKLANDCE.Hypertension in unilateralrenal disease. ! Urol 1938; 39: 611-626. 19 PALUBINSKASAJ/ RIPLEYHR. Fibromuscular dysplasia in extrarenal arteries. Radiology 1964; 82: 451-455. 20 VLESJS, HENDRIKSJJ, LODDERJ, JANEVSKIB. Multiple vertebro-
Fibromuscular Dysplasia and Aneurysm Formation
21
22
23
24
25 26 27 28
29 30
31 32 33 34 35 36 37 38
basilar infarctions from fibromuscular dysplasia related dissecting aneurysm of the vertebral artery in a child. Neuropediatrics 1990; 21(2): 104-105. OUCHI Y, TAGAWAH, YAMAKADOM, TAKANASHIR, TANAKAS. Clinical significance of cerebral aneurysm in renovascular hypertension due to fibromuscular dysplasia: two cases in siblings. Angiology 1989; 40(6): 581-588. GEORGEB, ZERAH M, MOURIERKL, GELBERTF, REIZlNED. Ruptured intracranial aneurysms. The influence of sex and fibromuscular dysplasia upon prognosis. Acta Neurochir Wien 1989; 97(1-2): 26-30. ITOYAMAY, FUKUMURAA, NONAKA N, ITOH Y, TAKAMURAS, MATSUKADOY. Fibromuscular dysplasia accompanied by giant intracranial fusiform aneurysm--report of two cases. Neurol Med Chit Tokyo 1988; 28(6): 579-583. PEREZ-HIGUERASA, ALVAREZ-RuIz F, MARTINEZ-BERMEJOA, FRUTOSR, VILLARO, DIEZ-TEJEDORE. Cerebellar infarction from fibromuscular dysplasia and dissecting aneurysm of the cerebral artery. Report of a child. Stroke 1988; 19(4): 521-524. IMAYAH, KOBAYASHIS, OKADAT, MURAYAMAK, NAKAZAWAS. IA case of fibromuscular dysplasia with middle cerebral aneurysm. No Shinkei Geka 1985; 13(6): 653-685. PHADKERV, RAORIKB, DIVEKARVK, BHUTADAVR. Fibromuscular dysplasia of common carotid artery. A case report. Australas Radiol 1990; 34(4): 350-352. POZZATI E, GIULIANI G, ACCIARRI N, Nuzzo G. Long term follow-up of occlusive cervical carotid dissection. Stroke 1990; 21(4): 526-531. ROSSILLONR, SIX C, DARDENNEG- Dysplasia and spontaneous bilateral dissection of the internal carotid arteries: Apropos of a case. Value of surgical treatment. Acta Chir Belg 1990; 90(3): 97101. D'ANGLEJAN-CHATILLONJ, RIBEIRO V, MAS JL, BOUSSERMG, LEPLANED. Dissection of the extracranial internal carotid artery. 62 cases. PresseMed 1990; 19(14): 661-667. MAIURIF, GALLICCHIOB, GANGEMIM, BRIGANTIF, IACONETTAG, CORRIERO G. Fibromuscular dysplasia of the carotid arteries. Clinical and radiological considerations. Clin Neuro Neurosurg 1988; 90(1): 57-60. WESENCA, ELLIOTTBM. Fibromuscular dysplasia of the carotid arteries. Am J Surg 1986; 151(4): 448-451. HARRIS JP, WALKER PJ, WHtATE GH, MAY J. Bench repair of complex renal arterial lesions. Ann Vasc Surg 1991; 5(2): 138142. NovlcK AC. Management of renovascular disease. A surgical perspective. Circulation 1991; 83 (2 Suppl): 1167-1171. PARKSH, CHI JG, CHOI Y. Primary intimal fibroplasia with multiple aneurysms of renal artery in childhood. Child Nephrol Urol 1990; 10(1): 51-55. SAVASTANOS, FELTRIN GP, MIOTTO D, CHIESURA-CORONAM. Renal aneurysm and arteriovenous fistula. Management with transcatheter embolisation. Acta Radiol 1990; 31(1): 73-76. SIEGELBAUMMH, WEISSJP. Renal infarction secondary to fibrous dysplasia and aneurysm formation of renal artery. Urology 1990; 35(1): 73-75. BARTELM, RICHTERS, WAGNERW, CLAUSNERG. Surgical intervention in stenoses and aneurysms of the renal artery. Zentralbl Chit 1989; 114(19): 1259-1268. HAU T. Kidney autotransplantation in therapy of complex reno-
39 40
41 42 43 44 45 46 47 48 49
50
51 52
53 54 55 56 57
743
vascular diseases. Indications, technique and results. Zentralbl Chir 1988; 113(16): 1024-1034. MERCIERC, PIQUETP, PILIGIANF, FERDANIM. Aneurysms of the renal artery and its branches. Ann Vasc Surg 1986; 1(3): 321-327. LORELIUSLE, HEMMINGSSONA, HAGG A, MORLIN C, ABERGH. Progressive fibromuscular dysplasia of the renal artery. Angiographic and clinical follow-up. Acta Radiol Diagn Stockh 1985; 26(6): 705-708. SCHNEIDERJ, COESTERC, MEYERW, OECHSLINE. Unusual cases of coronary death. Schweiz Med Wochenschr 1987; 10, 117(41): 1577-1582. LIE JT, BERGKK. Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction. Hum Pathol 1987; 18(6): 654-656. DETROUXM, FIEVEZM, MASSIN H, BLAMPAINJP. Rupture of a superior mesenteric artery aneurysm in a child. Ann Vasc Surg 1990; 4(5): 505-509. UNOVARE, PISKIN B. Hemobilia from a ruptured hepatic artery aneurysm in a 16 year old girl. Turk J Pediatr 1989; 31(1): 63-70. ASCHWANDENM, SCHMIDP. Ruptured aneurysm of the greater omentum in fibromuscular displasia. Vasa 1989; 18(2): 157-161. SMITH JA, MACLEISHDG, COLLIERNA. Aneurysms of the visceral arteries. Aust NZ J Surg 1989; 59(4): 329-334. CtNA C, WILLIAMSONC, AMELIFM. Fibromuscular dysplasia of the posterior auricular artery: an unusual aneurysmal lesion. J Cardiovasc Surg Turino 1988; 29(1): 56-59. SAUNDERsFW,BIRCHARDD,WlLLMERJ. Spinal artery aneurysm. Surg Neurol 1987; 27(3): 269-272. HERPELSV, VAN DEVOORDEW, WILMSG, VERBEKENE, BAERTA, LAUWERYNSJ, NEVELSTEENA. Recurrent aneusysms of the upper arteries of the lower limb: an atypical manifestation of fibromuscular dysplasia--a case report. Angiology 1987; 38(5): 411416. FUKAZAWAS, IMAI S, SAITOA, YAMADAH. Fibromuscular dysplasia of the cervical arteries associated with a distal vertebral trunk aneurysm. Case report. NeurolMed Chir Tokyo 1990; 30 (11 Spec No): 899-903. GEORGEB, MOURIERKL, GELBERTF, REIZINED, RAGGUENEAUJL. Vascular abnormalities in the neck associated with intracranial aneurysms. Neurosurgery 1989; 24(4): 499-508. PATELKS, WOLFEJH, MATHIASC. Left external iliac artery dissection and bilateral renal artery aneurysms secondary to fibromuscular dysplasia: a case report. Neth J Surg 1990; 42(4): 118120. STANLEYJC, GEWERTZBL, BOVEEL, SOTTIURAIV, FRY WJ. Arterial fibrodysplasia--histopathological character and current aetiological concepts. Arch Surg 1975; 110: 561-566. KELLY TF JR, MORRIS GC JR. Arterial fibromuscular disease. Observations on pathogenesis and surgical management. Ann J Surg 1982; 143: 232-236. NAKATAY. An experimental study on vascular lesions caused by obstruction of the vasa vasorum. Jap CircJ 1987; 31: 275-287. SATTIURAIUS, FRY WJ, STANLEYJC. Ultrastructure of medial smooth muscle and myofibroblasts in human arterial dysplasia. Arch Surg 1978; 113: 1280-1288. PERRYMD. Fibromuscular dysplasia. Surg Gynaecol Obstet 1974; 139: 97-104b.
Accepted 23 January 1992
Eur J Vasc Surg Vol 7, November 1993