Moyamoya vascular pattern and dural arteriovenous fistula (dAVF) are rare vascular abnormalities and both can be secondary to head trauma. The role of dural angiogenesis in the pathophysiology of vascular malformation is rather unclear. We report a unique case of moyamoya vasculopathy simultaneously associated with dAVF after heavy head trauma. It seems that both moyamoya syndrome and dAVFs are associated with dural angiogenesis induced by head trauma. The interrelationship between vascular abnormalities is complex and unclear.Vaskularna struktura moyamoya i arteriovenska fistula dure (dAVF) su rijetke krvožilne nepravilnosti koje mogu nastati kao posljedica ozljede glave. Uloga duralne angiogeneze u patofiziologiji vaskularne malformacije prilično je nejasna. Opisujemo jedinstven slučaj moyamoya vaskulopatije istodobno udružene s dAVF nakon teške traume glave. Čini se da su i sindrom moyamoya i dAVF udruženi s duralnom angiogenezom izazvanom ozljedom glave. Međuodnos vaskularnih nepravilnosti je složen i nejasan

Bojana Žvan

Janja Pretnar-Oblak

Katarina Surlan-Popović

Marjan Zaletel

English

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Acta Clin Croat,  Vol. 48,  No. 4,  2009 115Acta Clin Croat 2011; 50:115-120 Case ReportMOYAMOYA SYNDROME WITH ARTERIOVENOUS DURAL FISTULA AFTER HEAD TRAUMAMarjan Zaletel1, Katarina Surlan-Popović2, Janja Pretnar-Oblak1 and Bojana Žvan11Department of Vascular Neurology, 2Institute of Radiology, Ljubljana Medical Center, Ljubljana, SloveniaSUMMARY – Moyamoya vascular pattern and dural arteriovenous fistula (dAVF) are rare vas-cular abnormalities and both can be secondary to head trauma. The role of dural angiogenesis in the pathophysiology of vascular malformation is rather unclear. We report a unique case of moyamoya vasculopathy simultaneously associated with dAVF after heavy head trauma. It seems that both moyamoya syndrome and dAVFs are associated with dural angiogenesis induced by head trauma. The interrelationship between vascular abnormalities is complex and unclear.Key words: Moyamoya disease; Craniocerebral trauma; Intracranial arteriovenous malformations; AngiogenesisCorrespondence to: Marjan Zaletel, MD, MS, Department of Vascular Neurology, Ljubljana Medical Center, Zaloška 2, 1000 Ljubljana, SloveniaE-mail: marjan.zaletel@kclj.siReceived November 11, 2010, accepted December 29, 2010IntroductionMoyamoya syndrome is an uncommon cerebro-vascular disorder that is characterized by progressive stenosis of the terminal portion of the internal carotid artery and/or its main branches. Intracranial dural ar-teriovenous fistula (dAVF) is also a rare vascular le-sion. The etiology of both entities is unclear, although they have been tried to be connected with several conditions and diseases. Thus, both entities could ap-pear after head trauma, however, to our knowledge, simultaneous occurrence of both conditions has not yet been reported. We describe a case of moyamoya vasculopathy and AVF in a patient having sustained severe head trauma.Case ReportA 71-year-old right-handed forester was admit-ted to the Neurology Department due to burning headache, located deep in the right hemisphere, that had gradual onset and had started four days earlier. Twenty-four years before, he suffered a major head and chest injury after he was crushed under a tree. He was comatose for a couple of weeks. Upon recovery, slight left hemiparesis remained. At that time, com-puted tomography scan did not show any pathological abnormalities. He had mild arterial hypertension but did not take any medication. Over the past couple of months, he noticed that he could not empty his blad-der properly.On admission, he was sitting in a wheelchair and was unable to walk unattended.  His blood pressure was 187/97 mm Hg, heart rate 69 beats per minute and body temperature 37.5 oC. He was abulic, disori-ented and unwilling to cooperate. On the mini mental test, he scored 14/30. He had mild central paresis of the left facial nerve and spastic hemiparesis with brisk reflexes and extensor plantar response on the left side. In addition, paratonia of the right limbs and poor dex-terity was present. The gait was slow, widely based and hemiparetic. CT showed subarachnoid hemorrhage in both frontal and parietal lobes (Fig. 1). Magnetic res-onance imaging showed bilateral subcortical ischemic lesions distributed mainly in the territory supplied by the internal carotid artery (Fig 3). Cerebral angiogra-phy revealed severe narrowing of both internal carotid 116 Acta Clin Croat,  Vol. 50,   No. 1,  2011M. Zaletel et al. Moyamoya syndrome and dural AV fistulaarteries and occlusion of the M1 segment of both middle cerebral arteries. Collateral perforating lenti-form arteries were prominent (Fig. 3). Angiography of the right common carotid artery showed collateral cerebral circulation from external carotid artery and a dAVF that was fed by branches of occipital artery. The AVF drained into the right transverse sinus. Angiog-raphy of the left vertebral and basilar arteries revealed prominent and tortuous perforate and posterior chor-oidal arteries presenting a typical moyamoya pattern. Apart from arterial hypertension, he had no risk fac-tors for cerebrovascular diseases. Color duplex sonog-raphy of the neck arteries showed small, partially calcified, and hemodynamically insignificant plaques in both carotid bifurcations. The patient was treated conservatively using calcium channel antagonist, an-algesics and antihypertensive therapy. Subarachnoid hemorrhage spontaneously reabsorbed. The cognitive status remained the same. We did not decide for op-erative therapy due to the preexisting cognitive im-pairment. The patient was discharged in stable clinical condition.DiscussionWe describe an unusual case of bilateral moyamoya syndrome associated with dural arteriovenous fistula in an elderly Caucasian patient admitted to neurology department due to subarachnoid hemorrhage. Exten-sive review of the literature revealed only a few cases of simultaneous presentation of moyamoya vasculopa-thy and arteriovenous fistula. There is only one report of unilateral moyamoya syndrome and dural arterio-venous fistula and only a few reports of moyamoya disease in combination with different arteriovenous malformations1,2. The main question about the men-tioned entities is the pathophysiological mechanism that remains unexplained. Our case report, in which moyamoya syndrome and dural arteriovenous fistula occurred simultaneously years after head trauma, may suggest some new speculations concerning the pathophysiological mechanisms that partially or com-pletely underlie both entities. In our patient, clinical presentation was by no means uncommon for both entities. The subarachnoid Fig 1. Computed tomography in axial plane shows bleeding into the subarachnoid space of the frontal and parietal lobes on both sides (subarachnoid hemorrhage).Acta Clin Croat,  Vol. 50,  No. 1,  2011 117M. Zaletel et al. Moyamoya syndrome and dural AV fistulahemorrhage could be attributed either to the dural arteriovenous fistula or to the moyamoya syndrome. The rupture of the fragile collateral vessels associated with stenosis of the internal carotid artery resulting in intracranial hemorrhage is common in patients with moyamoya syndrome3. The cognitive decline could be explained by the widespread subcortical ischemic le-sions, which were probably also part of the pathologi-cal process occurring in moyamoya syndrome. Although moyamoya disease is commonly found in East Asia, the moyamoya vasculopathy has been observed throughout the world4. It has been shown that it has two peaks of occurrence: one at 5 years of age and another one, lower peak at about 40 years of age5. Importantly, our patient was significantly older. Therefore, we could convincingly assume that moy-amoya vasculopathy and dural arteriovenous fistula in our patient were acquired rather than congenital.The precipitating factor for both entities in our pa-tient could have been previous severe head trauma. It has already been reported that head trauma is associ-ated with dural arteriovenous fistula5. Our patient had a dural arteriovenous fistula located on the side of the previous head trauma. On the other hand, head trau-ma could have been a secondary cause of moyamoya vasculopathy as well. We could assume with certainty that the altered angiogenesis occurred within dura after the trauma. Altered dural angiogenesis can be accompanied by sinus venous thrombosis7, facilitating the occurrence of dural arteriovenous fistula. Further-more, altered angiogenesis could induce the occur-rence of moyamoya vasculopathy. The relationship between moyamoya disease and dural arteriovenous fistula is not clear. It seems that dural arteriovenous fistula could cause the moyamoya pattern and vice versa. According to Mawad et al.8, the predilection of vascular occlusion for the internal carotid may be related to the increase in blood tur-bulence proximal to the dural arteriovenous fistula. Namely, the increased turbulent flow related to the hemodynamics of the arteriovenous malformation could lead to intimal hyperplasia, endothelial thicken-ing, and progressive occlusion of the feeding vessels. Nevertheless, the fistula in our patient was supplied entirely from the external carotid artery, thereby re-ducing blood flow through the internal carotid artery. The idea that vascular occlusive disease leads to du-ral arteriovenous fistula seems to be more appealing. Fig 2. MRI flair sequence in axial plane shows subarachnoid hemorrhage and ischemic lesions bilaterally.118 Acta Clin Croat,  Vol. 50,   No. 1,  2011M. Zaletel et al. Moyamoya syndrome and dural AV fistulaFig. 3 Angiography: (A) angiographic injection of the left internal carotid artery showing severe narrowing of the left internal carotid artery and occlusion of the left M1 segment; (B) angiographic injection of the right internal carotid artery showing severe narrowing of the right internal carotid artery, occlusion of the right M1 segment and collateral cerebral circulation from external carotid artery. Collateral perforating lentiform arteries are prominent. dAVF is fed by branches of occipital artery and drained into the right transverse sinus; (C) and (D) angiographic injection of the left vertebral and basilar artery showing prominent and tortuous perforating arteries in posterior choroidal arteries presenting typical moyamoya pattern.3 A 3 B3 C 3 DActa Clin Croat,  Vol. 50,  No. 1,  2011 119M. Zaletel et al. Moyamoya syndrome and dural AV fistulaSeveral previous studies have described an association between moyamoya disease and arteriovenous mal-formations indicating causal relationship1,2. Previous studies have also shown that the levels of several fac-tors including basic fibroblast growth factor (bFGF)9, transforming growth factor β-1, hepatocyte growth factor, vascular endothelial growth factor10, matrix metalloproteinases, intracellular adhesion molecules, and hypoxia-inducing factor 1α are elevated in the dura of patients with moyamoya disease11,12. It could be speculated that such ischemic environment may have contributed to the formation of dural arterio-venous fistula in our patient. Recent investigations have shown separately that both moyamoya syndrome and dural arteriovenous fistula could be associated with dural angiogenesis promoted by head trauma13,14. Thus, angiogenesis in response to the release of angio-genic factors induced by trauma may simultaneously lead to the development of moyamoya vasculopathy and dural arteriovenous fistula as well. Although somehow speculative, our explanation(s) might help explain or at least bring a new view on the unknown etiology of these entities. However, our case could also represent the extremely rare coinci-dence of two rare phenomena. In conclusion, based on our particular case, we suggest that both moyamoya syndrome and dural arteriovenous fistula might be as-sociated with dural angiogenesis due to severe head trauma.References1. CHEN Z, ZHU G, FENG H, LIN J, WU N. Giant arte-riovenous malformation associated with unilateral moyamoya disease in a child: case report. Surg Neurol 2007;67:89-92. 2. FUSE T, TAKAGI T, FUKUSHIMA T, HASHIMOTO N, YAMADA K. Arteriovenous malformation associated with moyamoya disease. Child Nerv Syst 1996;12:404-8.  3. SCOTT RM, SMITH JL, ROBERTSON RL, MADSEN JR, SORIANO SG, ROCKOFF MA. Long-term outcome in children with moyamoya syndrome after cranial revascu-larization by pial synangiosis. J Neurosurg 2004;100:142-9.  4. CALDARELLI M, Di ROCCO C, GAGLINI P. Surgical treatment of moyamoya disease in pediatric age. J Neurosurg Sci 2001;45:83-91.w  5. KURODA S, HOUKIN K. Moyamoya disease: current con-cepts and future perspectives. Lancet Neurol 2008;7:1056-66.  6. CORDONNIER C, Al-SHAHI SALMAN R, BHAT-TACHARYA JJ, COUNSELL CE, PAPANASTASSIOU V, RITCHIE V, ROBERTS RC, SELLAR RJ, WARLOW C; SIVMS collaborators. Differences between intracranial vascular malformation types in the characteristics of their presenting haemorrhages: prospective, population-based study. J Neurol Neurosurg Psychiatry 2008;79:47-51.  7. CHALOUPKA JC, MARX WF, KALLMES DF. Dural arteriovenous fistulas. J Neurosurg 2001;94:858-61.  8. MAWAD ME, HILAL SK, MICHELSEN WJ, STEIN B, GANTI SR. Occlusive vascular disease associated with cere-bral arteriovenous malformations. Radiology 1984;153:401-8.   9. HALATSCH ME, RUSTENBECK HH, JANSEN J. Pro-gression of arteriovenous malformation in moyamoya syn-drome. Acta Neurochir (Wien) 1997;139:82-5.10. HOSHIMARU M, TAKAHASHI JA, KIKUCHI H, NA-GATA I, HATANAKA M. Possible roles of basic fibroblast growth factor in the pathogenesis of moyamoya disease: an immunohistochemical study. J Neurosurg 1991;75:267-70.11. SAKAMOTO S, KIURA Y, YAMASAKI F, SHIBU-KAWA M, OHBA S, SHRESTHA P, SUGIYAMA K, KURISU K. Expression of vascular endothelial growth factor in dura mater of patients with moyamoya disease. Neurosurg Rev 2008;31:77-81.12. YOSHIMOTO T, HOUKIN K, TAKAHASHI A, ABE H. Angiogenic factors in moyamoya disease. Stroke 1996;27:2160-5.13. SCOTT RM, SMITH ER. Moyamoya disease and moy-amoya syndrome. N Engl J Med 2009;360:1226-37.14. KIM GH, HAHN DK, KELLNER CP, HICKMAN ZL, KOMOTAR RJ, STARKE RM, MACK WJ, MOCCO J, SOLOMON RA, CONNOLLY ES Jr. Plasma levels of vas-cular endothelial growth factor after treatment for cerebral arteriovenous malformations. Stroke 2008;39:2274-9.120 Acta Clin Croat,  Vol. 50,   No. 1,  2011M. Zaletel et al. Moyamoya syndrome and dural AV fistulaSažetakMOYAMOYA SINDROM S ARTERIOVENSKOM FISTULOM DURE NAKON OZLJEDE GLAVEM. Zaletel, K. Surlan-Popović, J. Pretnar-Oblak i B. ŽvanVaskularna struktura moyamoya i arteriovenska fistula dure (dAVF) su rijetke krvožilne nepravilnosti koje mogu na-stati kao posljedica ozljede glave. Uloga duralne angiogeneze u patofiziologiji vaskularne malformacije prilično je nejasna. Opisujemo jedinstven slučaj moyamoya vaskulopatije istodobno udružene s dAVF nakon teške traume glave. Čini se da su i sindrom moyamoya i dAVF udruženi s duralnom angiogenezom izazvanom ozljedom glave. Međuodnos vaskularnih nepravilnosti je složen i nejasan.Ključne riječi: Bolest moyamoya; Kraniocerebralna ozljeda; Intrakranijske arteriovenske malformacije; Angiogeneza

Moyamoya sindrom s arteriovenskom fistulom dure nakon ozljede glave

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