Distal Lenticulostriate Artery Aneurysm Presenting with Spontaneous Intracerebral and Intraventricular Hemorrhage : a Case Report and a Review of the Literature

KOR J CEREBROVASCULAR SURGERY September 2011 Vol. 13 No 3, page 129-136

Distal Lenticulostriate Artery Aneurysm Presenting With Spontaneous Intracerebral and Intraventricular Hemorrhage : A Case Report and a Review of the Literature

Department of Neurosurgery, Chungnam National University School of Medicine, Daejeon, Korea Jae-Kyung Sung, M.D.・Hyeon-Song Koh, M.D.・Chang-Woo Kang, M.D.・Hyon-Jo Kwon, M.D. Jin-Young Youm, M.D.・Seon-Hwan Kim, M.D.

ABSTRACT

The authors report here on a rare case of aneurysm involving the distal lenticulostriate artery (LSA) in a 66-year-old man who presented with intracerebral hemorrhage (ICH) in the right basal ganglia and also intraventricular hemorrhage (IVH). Three-dimensional computed tomography angiography (3D-CTA) and conventional cerebral angiography showed a 4 mm, round-shaped aneurysm in the right distal LSA and this was combined with moyamoya-like disease. We performed proximal clipping of the aneurysm using a microsurgical technique and we evacuated the hematoma. After the operation, there was recurrent bleeding around the operation site and hydrocephalus gradually developed, and we implanted a ventriculo-peritoneal (V-P) shunt. The patient did well after the final shunt surgery and rehabilitation. Presently, he has no motor weakness or significant neurologic deficit, but mild cognitive dysfunction remains. When spontaneous ICH occurs in an unusual site, a thorough investigation is important to rule out a structural vascular abnormality. (Kor J Cerebrovascular Surgery 13(3):129-136, 2011)

KEY WORDS : Aneurysm・Distal lenticulostriate artery・Hemorrhage

ports of distal LSA aneurysms in the medical literature. Introduction Due to its rarity, we report here a case of a ruptured distal LSA aneurysm that presented as spontaneous ICH and Spontaneous intracerebral hemorrhage (ICH) is reported intraventricular hemorrhage (IVH) in a healthy male pa- in about 10~20% of all strokes,4)7) and it frequently caus- tient, and we review the relevant literature. es significant neurologic deficits. Many of these hemor- rhages are related to microaneurysms that form in pa- Description of the case tients with hypertension and they are rarely discovered by cerebral angiography. Aneurysms arising from the branch- A 66-year-old man was transferred from a local clinic es of the distal lenticulostriate artery (LSA) are a rare with a continuous intractable headache that had started cause of spontaneous ICH; there are about 25 case re- three days previously. He had no history of hypertension, diabetes or taking any specific medication and he had no 논문접수일 : 2011년 5월 18일 definite neurologic deficit. A brain computed tomography 심사완료일 : 2011년 8월 8일 (CT) scan showed a right basal ganglia ICH and IVH 교신저자 : Hyeon-Song Koh, M.D., Ph.D., Department of Neurosurgery, Chungnam National University School of Medicine, 33 Munhwa-ro, (Fig. 1-A, 1-B). Three-dimensional CT angiography (3D-CTA) Jung-gu, Daejeon 301-721, Korea (Fig. 1-C) and conventional catheter cerebral angiography Tel : (042) 280-7369 ∙ Fax : (042) 280-7363 (Fig. 2-A, 2-B) showed a 4-mm, round-shaped aneurysm Email : [email protected]

129 Distal LSA Aneurysm

A B C

Fig. 1. (A)The brain computed tomography (CT) scan at admission shows intracerebral hemorrhage on the right basal ganglia and (B) intraventricular hemorrhage. (C) Three-dimensional CT angiography (3D-CTA) shows a small, round-shaped aneurysm (arrow) on the branch of the right distal lenticulostriate artery.

A B

Fig. 2. (A, B) Catheter cerebral angiography in the right internal carotid artery shows a small, round-shaped aneurysm (arrow) on the right distal lenticulostriate artery.

in the right distal branch of the LSA. The right internal clipping five days after the initial attack. We identified carotid artery (ICA) angiography of this patient did not and evacuated the right basal ganglia hematoma, and then show a typical moyamoya vascular pattern, but the right we performed proximal clipping of the parent artery of anterior cerebral artery (ACA) looked stenotic, and there the aneurysm (Fig. 4-A, 4-B). We incised the aneurysm, was some collateral flow from the normal middle cerebral removed the partially thrombosed contents and then we artery (MCA) (Fig. 2-A). In addition, his left cervical wrapped the aneurysm with cotton and fibrin glue. One ICA was obstructed, and the left branch of the ICA day later, the patient suffered from postoperative bleeding showed probable moyamoya disease (Fig. 3-A, 3-B). around the operation site as well as increased IVH, but We performed a pterional craniotomy for aneurysm 3D-CTA showed good proximal clipping and the aneur-

130 Kor J Cerebrovascular Surgery 13(3):129-136, 2011 Jae-Kyung Sung・Hyeon-Song Koh・Chang-Woo Kang・Hyon-Jo Kwon・Jin-Young Youm・Seon-Hwan Kim

A B

Fig. 3. (A, B) Left cerebral angiography shows total occlusion of the left cervical internal carotid artery (arrow), and this suggested of probable moyamoya disease.

A B

Fig. 4. (A) The intraoperative findings show the round-shaped aneurysm on the right distal lenticulostriate artery (B) and proximal clipping of the aneurysm.

ysm had disappeared. The patient’s mentality was ag- was removed. After the removal of the EVD, hydro- gravated after the recurrent bleeding and we performed cephalus gradually developed, so we performed lumbar external ventricular drainage (EVD) on the first post- CSF drainage twice, and then we implanted a ven- operative day. The EVD was maintained and drainage triculo-peritoneal (V-P) shunt. The patient did well after was performed for seven days and then the drainage tube the final shunt surgery and rehabilitation. He was dis-

Kor J Cerebrovascular Surgery 13(3):129-136, 2011 131 Distal LSA Aneurysm

A B

Fig. 5. (A, B)The computed tomography scan one year after the initial attack shows another intracerebral hemorrhage (ICH) on the left basal ganglia. Also shown are an aneurysm clip on the right basal ganglia and a ventricular shunt catheter.

A B

Fig. 6. (A) Repeated cerebral angiography one year later shows the disappearance of the previous aneurysm on the right distal lenticulostriate artery. (B) There is no de novo aneurysm seen on the left cerebral angiogram.

charged about two months after admission with minor there was no newly developed aneurysm (Fig. 6-A, 6-B). cognitive dysfunction. However, a recurrent ICH was not- The recurrent ICH did not seem to be related to the pre- ed at the left basal ganglia about one year later (Fig. vious aneurysm; it might have been due to the rupture of 5-A, 5-B). Repeated cerebral angiography showed that the the previously noted left moyamoya-like vessels. The previous right distal LSA aneurysm had disappeared, and amount of recurrent ICH was small, so we administered

132 Kor J Cerebrovascular Surgery 13(3):129-136, 2011 Jae-Kyung Sung・Hyeon-Song Koh・Chang-Woo Kang・Hyon-Jo Kwon・Jin-Young Youm・Seon-Hwan Kim conservative treatment. The patient presently has mild fore, the left ICA branch had developed probable moya- cognitive dysfunction, but he has no motor weakness or moya disease. Although the right ICA angiography of significant neurologic deficit. We continue to conduct reg- this patient did not show a typical moyamoya vascular ular follow-up and close neurological observation. pattern, the right ACA looked stenotic, and there was some collateral flow from the normal MCA. In addition, Discussion the aneurysm and many of the surrounding small vessels seemed fragile in the operation field, and they were sim- On our review of the literature, we found about 25 ilar to moyamoya vessels. Therefore, we think the initial cases of distal LSA aneurysms have been reported since ICH with IVH and the recurrent ICH of this case were 1980 (Table 1). In Korea, Han et al.7) reported the first due to the rupture of a moyamoya-related aneurysm and case of ruptured distal LSA aneurysm associated with ip- the fragile moyamoya-like vessels. Moyamoya-related aneur- silateral middle cerebral artery (MCA) occlusion in 2006. ysms are usually located in the basal ganglia or periven- Ahn et al.1) then documented two cases of distal LSA tricular white matter.13) Moyamoya disease usually pres- aneurysms in 2007, including a case of Han et al.7) ents with hemorrhage, which due to a rupture of the true Although the number of cases is small, these aneurysms aneurysm or pseudoaneurysm, or fragile moyamoya showed some characteristic features, such as young age of vessels. Although moyamoya-related aneurysms are often onset, no female predominance, usually non-hypertensive regarded as pseudoaneurysms and they can spontaneously with deep location and many associated vascular lesions.1) regress on the follow-up angiography, they can rerupture In the literature, the age of the patients has widely varied (there is a 20~30% incidence of this), which usually re- from 2 months to 69 years;10)18) the number of male and sults in a poor outcome.12) For this reason, surgical man- female patients was similar. Most of these aneurysms or- agement should be performed in cases with recurrent iginated from the lateral LSA territories and this results bleeding or an increasing size of the aneurysm. in basal ganglia hemorrhage. The aneurysms were usually The most ideal treatment of LSA aneurysms still re- small (< 5 mm) and in a deep location. The aneurysm in mains controversial due to the small number of cases and our case was also small, 4 mm and it was located in the the undetermined natural course. A few cases were treated deep basal ganglia. Ahn et al.1) classified five types of conservatively in the literature we reviewed,1)5)6)17)20)21) and hemorrhage in the reported cases as 1) isolated ICH, 2) two cases were treated with endovascular embolization.8)14) isolated IVH, 3) ICH and IVH, 4) ICH and SAH and 5) The other cases were managed surgically,3)4)7)9-11)15)18-20)22)23)25) a combination of all three types. ICH and IVH are the including our case. Conservative treatment and regular most common types of hemorrhage of distal LSA aneur- follow-up cerebral angiography are adequate management ysms including our case.3)7)10)14)16)17-19)22) There are many for some lesions such as pseudoaneurysms. However, di- possible underlying diseases in these types of aneurysms, rect surgery should be considered for typical saccular including moyamoya disease or moyamoya-like disease, aneurysms to prevent rebleeding. Surgical treatment is arteriovenous malformation (AVM), systemic lupus eryth- definitely indicated for a growing aneurysm and recurrent ematosus (SLE), brain tumor, vasculitis, trauma, arterio- intracranial bleeding. The surgical methods include re- sclerosis and hypertension. The associated diseases of the section of the aneurysm,4)7)10)11)15)16)19) neck clipping4)19)22)23) reported cases were moyamoya disease,2)4)5)7)8)12)13)17)21)23)24) and proximal clipping.3)4)18) However, one case underwent AVM,19) SLE11) and brain tumor.25) Moyamoya disease and only hematoma removal.20) Surgical treatments of LSA moyamoya-like disease are the most common types of aneurysms usually have a risk of damage to the eloquent underlying disease, including in this case, but a history of area of the brain due to the deep location of LSA aneur- hypertension was rarely noted.4)15)20) Moyamoya disease is ysms in the basal ganglia. Surgical treatments of LSA often accompanied by cerebral aneurysms and its in- aneurysms also carry the risk of parent artery occlusion, cidence has been reported in 3~14% of the cases.2)12) Our and this can lead to infarction of the perforating artery patient presented with occlusion of the left ICA; there- and contralateral hemiparesis. Although occlusion of the

Kor J Cerebrovascular Surgery 13(3):129-136, 2011 133 Distal LSA Aneurysm

Table 1. Summary of the reported distal lenticulostriate artery aneurysms in our literature review

Angio- Pathology Age / Pre-existing Imaging Associated Size Treatment Author (Year) graphic of the Outcome Gender hypertension findings disease (mm) modalities location aneurysm Okuma21) 29/F - NA* Moyamoya - - Conservative - GR (1980) Ohta19) 26/F - ICH, IVH AVM - - Surgical excision True VS (1980) Murakami17) 33/M - ICH, IVH Moyamoya-like 5 Lateral LSA Conservative True Death (1984) Kidoguchi11) 24/F - ICH, SAH SLE 3 - Surgical excision True Death (1987) Gupta6) 36/F No ICH - Multiple Lateral LSA Conservative - GR (1989) Grabel5) 60/M No ICH Moyamoya-like 5 Lateral LSA Conservative - MD (1989) Oka20) 44/F Yes ICH, SAH - 4 Lateral LSA Removal of ICH - GR (1991) 32/M - SAH Moyamoya - Medial LSA Conservative - GR Petrela22) 27/M - ICH, IVH - - - Neck clipping - MD (1992) Endo3) 12/F - ICH, IVH - - - Proximal clipping - GR (1996) Kaptain10) 2 mo/F - ICH, IVH - 15 - Surgical excision True MD (2001) Larrazabal14) Endovascular 57/F - ICH, IVH Moyamoya-like 4 Lateral LSA -MD (2001) embolization Vates26) 35/M No IVH Neurocytoma 7 Lateral LSA Surgical excision True MD (2001) Maeda15) ICH, IVH, 62/F Yes - 5 Lateral LSA Surgical excision True MD (2001) SAH Narayan18) 69/F No ICH, IVH - 5 Lateral LSA Proximal clipping - GR (2004) Horn9) Neck clipping 44/F No ICH - 2 Lateral LSA -GR (2004) and coating Sakai23) 61/M No ICH Moyamoya-like 4 Lateral LSA Neck clipping - MD (2005) Han7) Ahn1) 49/M No ICH, IVH Moyamoya-like 3 Lateral LSA Surgical excision True GR (2006) Ahn1) ICH, IVH, 24/M No - 4 Lateral LSA Conservative - Death (2007) SAH Matushita16) 5/M No ICH, IVH - - Medial LSA Surgical excision True GR (2007) Gandhi4) 59/M No SAH, IVH Moyamoya 4 Lateral LSA Proximal clipping - GR (2008) 41/M No ICH Cocaine use 4 Medial LSA Surgical excision - GR 44/F Yes SAH - 3 Lateral LSA Neck clipping - GR Harreld8) Unruptured Endovascular 35/F No Moyamoya 3-4 Lateral LSA -GR (2011) (growing) embolization Present case 66/M No ICH, IVH Moyamoya-like 4 Lateral LSA Proximal clipping - GR

NA=not available; mo=months; M=male; F=female; ICH=intracerebral hemorrhage; IVH=intraventricular hemorrhage; SAH=subarachnoid hemorrhage; LSA= lenticulostriate artery; GR=good recovery; MD=moderate disability; VS=vegetative state

134 Kor J Cerebrovascular Surgery 13(3):129-136, 2011 Jae-Kyung Sung・Hyeon-Song Koh・Chang-Woo Kang・Hyon-Jo Kwon・Jin-Young Youm・Seon-Hwan Kim parent artery may result in neurological deterioration, this isting hypertension in thalamic, putaminal or posterior procedure may be tolerated when performed in the distal fossa hemorrhage. Therefore, aggressive investigation in segment because of the rich anastomotic network. Many patients who present with spontaneous intracranial hemor- authors have trapped the parent artery and resected these rhage should include 3D-CTA, magnetic resonance imag- aneurysms without further neurological damage; however, ing (MRI) with magnetic resonance angiography (MRA) the parent artery should be preserved, if possible. Gandhi and conventional cerebral angiography, and especially in et al.4) reviewed their surgical cases and they insisted that those patients who are young or who have no known risk surgical treatment of ruptured LSA aneurysms can be an factors such as hypertension, old age or coagulopathy. appropriate, effective and safe therapy. They categorized the morphology of LSA aneurysms into two types based Conclusions on whether the LSA was not incorporated into the aneurysm dome (Type I) or it was incorporated into the dome Although spontaneous ICH is relatively common, an (Type II). They were able to spare the parent LSA by underlying vascular lesion is rarely found. It might be performing only aneurysm clipping in three cases of Type that most spontaneous ICHs are related to hypertensive I aneurysms. Our case was a Type II aneurysm, so we ICH, moyamoya disease, AVM, amyloid angiopathy or performed a proximal clipping of the parent artery. It is other vascular anomalies. We think the initial ICH with also important that the underlying structural vascular ab- IVH and the recurrent ICH of this case were due to the normalities such as AVMs and aneurysms should be ap- rupture of a moyamoya-related aneurysm and the fragile propriately treated to prevent rebleeding. It is difficult to moyamoya-like vessels. When spontaneous ICH occurs in find the parent artery and small aneurysms because of an unusual site, a thorough clinical workup is important their deep ICH location, so the use of a navigation sys- to rule out the presence of a structural vascular abnormality. tem or frameless stereotactic guidance may be very useful In addition, selection of an appropriate treatment method for localizing and detecting these aneurysms. is mandatory. Pathologic examinations of these aneurysms were performed in eight of the previous surgical cases, and all of 6)9)10)14)15)16)18)23) REFERENCES the lesions were revealed to be true aneurysms. Our case looked like a true aneurysm, but we could not 1) Ahn JY, Cho JH, Lee JW. Distal lenticulostriate artery aneurysm perform a pathologic exam as it was difficult to excise in deep intracerebral haemorrhage. J Neurol Neurosurg Psychiatry the lesion due to the many fragile surrounding small 78:1401-3, 2007 2) Borota L, Marinkovic S, Bajic R, Kovacevic M. Intracranial vessels. aneurysms associated with moyamoya disease. Neurol Med Chir The clinical outcomes of the reported cases have var- (Tokyo) 36:860-4, 1996 ied, and they mainly depended on the initial neurological 3) Endo M, Ochiai C, Watanabe K, Yoshimoto Y, Wakai S. status. The outcomes were a good recovery in about half the Ruptured peripheral lenticulostriate artery aneurysm in a child: reported cases,1)3)4)6)7)8)9)16)18)20)21) including this one, moder- case report. No Shinkei Geka 24:961-4, 1996 4) Gandhi CD, Gilad R, Patel AB, Haridas A, Bederson JB. ate to severe disability in some cases,5)10)14)15)19)22)23)25) and Treatment of ruptured lenticulostriate artery aneurysms. J Neurosurg death in three cases.1)11)17) The reported cases of distal 109:28-37, 2008 lenticulostriate artery aneurysms in our literature review 5) Grabel JC, Levine M, Hollis P, Ragland R. Moyamoya-like dis- are summarized in Table 1. ease associated with a lenticulostriate region aneurysm. Case It has been reported in the literature that spontaneous report. J Neurosurg 70:802-3, 1989 ICH is associated with a higher incidence of vascular 6) Gupta AK, Rao VR, Mandalam KR, Kumar S, Joseph S, Unni M et al. Thrombosis of multiple aneurysms of a lateral lentic- anomalies in young patients who are without preexisting ulostriate artery. An angiographic follow-up. Neuroradiology 31:193-5, 26) hypertension. Zhu et al. suggested that diagnostic cere- 1989 bral angiography should be considered for all spontaneous 7) Han IB, Ahn JY, Chung YS. Ruptured distal lenticulostriate ar- ICH patients, except those over 45 years old, with preex- tery aneurysm associated with ipsilateral middle cerebral artery

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