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ORIGINAL ARTICLE
Year : 2014  |  Volume : 62  |  Issue : 2  |  Page : 144-148

Primary intraventricular hemorrhage: Clinical features, risk factors, etiology, and yield of diagnostic cerebral angiography


1 Department of Neurology, Sawai Man Singh Medical College Hospital, Jaipur, Rajasthan, India
2 Department of Radiology, Sawai Man Singh Medical College Hospital, Jaipur, Rajasthan, India

Date of Submission06-Oct-2013
Date of Decision18-Nov-2013
Date of Acceptance31-Mar-2014
Date of Web Publication14-May-2014

Correspondence Address:
Raghavendra Bakki Sannegowda
Plot No. 256/C, Sector 3, Chitrakoot, Vaishalinagar, Jaipur - 302 021, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.132333

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 » Abstract 

Background: Primary intraventricular hemorrhage (PIVH) is a rare neurological disorder, with bleeding confined to the ventricles only, without recognizable parenchymal or subarachnoid component. Aim: The purpose of this retrospective study was to identify clinical features, predisposing risk factors, etiology, radiological features and yield of diagnostic cerebral angiography in identifying the etiological causes. Settings and Design: Records of patients admitted in neurology division were analyzed in a tertiary care teaching hospital. Materials and Methods: We analyzed the records of 27 patients with PIVH evaluated and treated at our institute from August 2010 to April 2013. PIVH was diagnosed as hemorrhage in the ventricles only, detected by computed tomography scan without evidence of intraparenchymal, subarachnoid hemorrhage or intraventricular hemorrhage associated with trauma. CT angiography (CTA) alone was done in 10 patients (37.03%), digital subtraction angiography (DSA) in 2 patients (7.4%) and both CTA as well as DSA was done in 15 patients (55.5%). Statistical Analysis Used: Categorical and continuous data were analyzed using SPSS version 17. Results: 17 (62.96%) patients were females and 10 (37.03%) were males with ratio of F:M= 1.7:1. Headache was the commonest mode of presentation (85.18%). Hypertension was most common predisposing factor (29.62%) followed by arterio-venous malformations (AVMs) (25.92%), moyamoya disease (MMD) (11.11%), lenticuostriate artery aneurysm (LSA) (11.11%), arterial dissections (7.4%) and dural arteriovenous fistula (dAVF) (3.7%). Conclusions: PIVH is rare and hypertension is important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiology. AVMs and other rare etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with a high index of suspicion and warrants cerebral angiography in them, as some of the causes are potentially treatable.


Keywords: Arterial dissection, arteriovenous malformations, cerebral angiography, moyamoya disease, primary intraventricular hemorrhage


How to cite this article:
Srivastava T, Sannegowda RB, Satija V, Jain RS, Tejwani S, Mathur T. Primary intraventricular hemorrhage: Clinical features, risk factors, etiology, and yield of diagnostic cerebral angiography. Neurol India 2014;62:144-8

How to cite this URL:
Srivastava T, Sannegowda RB, Satija V, Jain RS, Tejwani S, Mathur T. Primary intraventricular hemorrhage: Clinical features, risk factors, etiology, and yield of diagnostic cerebral angiography. Neurol India [serial online] 2014 [cited 2021 Feb 28];62:144-8. Available from: https://www.neurologyindia.com/text.asp?2014/62/2/144/132333



 » Introduction Top


Primary intraventricular hemorrhage (PIVH) was first defined by Sanders [1] and is defined as bleeding in the ventricular system without a discernable parenchymal component or arising within 15 mm from the ventricular wall. [1],[2],[3],[4],[5] PIVH is rare and accounts for 3% of all the spontaneous intracranial hemorrhage. [1],[5],[6] Hypertension is common associated risk factor, [4],[5] but it has been described with arteriovenous malformations (AVMs), aneurysms, moyamoya disease (MMD), coagulopathy, and arteriovenous fistula. [3],[5],[7] In this report, we discuss our experience with PIVH.


 » Materials and Methods Top


This study is a retrospective review of case records of patients with PIVH admitted to our hospital between August 2010 and April 2013. PIVH was defined as hemorrhage in the ventricles only, detected by computed tomography (CT) scan. All the patients with intraparenchymal hemorrhage, subarachnoid hemorrhage, and intraventricular hemorrhage associated with trauma were excluded. All the PIVH patients underwent cerebral angiography, CT angiography (CTA), or digital subtraction angiography (DSA) to establish the possible etiological diagnosis. CTA alone was done in 10 (37.03%) patients, DSA in two (7.4%), and both CTA and DSA in 15 patients (55.5%) [Table 1].
Table 1: Depicting salient features and etiological causes for PIVH

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 » Results Top


Of the 27 patients, 17 (62.96%) were females and 10 (37.03%) were males (F:M: 1.7:1). Patients were aged between 14 to 60 years (median age, 37.38%). Four (14.81%) were in pediatric (0-18 years) age and 23 (85.18%) were adults [Table 1]. Clinical features are summarized in [Table 2]: Headache at the time of presentation in 23 (85.18%), loss of consciousness (LOC) in nine (33.33%), and focal deficits in seven (25.92%) patients. Etiology and predisposing factors have been summarized in [Table 1]: Hypertension with normal cerebral angiography in eight (29.62%), AVMs in seven (25.92%) [Figure 1], MMD in three (11.11%) [Figure 2], lenticulostriate artery (LSA) aneurysm in three (11.11%), two (7.4%) had arterial dissection (basilar artery (BA) dissection in one and internal carotid artery (ICA) dissection in one), and dural arteriovenous fistula (dAVF) in one (3.7%) patient. Etiology remained undetermined in three (11.53%) patients (eg., [Figure 3]).
Figure 1: (a) NCCT head shows intraventricular clots in right temporal horn and fourth ventricle along with hyperdensities adjacent to temporal horn suggests partially calcified AVM, (b) intraventricular clot in bodies of bilateral lateral ventricles, (c) T2-weighted MRI coronal images reveals bunch of abnormal vessels in right medial temporal lobe suggestive of AVM, (d) selective catheter angiography of left vertebral artery injection confirms the presence of AVM, supplied from branches of right PCA. NCCT = Noncontrast computed tomography, AVM = Arteriovenous malformation, MRI = Magnetic resonance imaging

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Figure 2: (a) NCCT head reveals intraventricular clots in left temporal horn and fourth ventricle with mild ventriculomegaly, DSA (selective left ICA catheterization) (b) anteroposterior and (c) lateral view reveals short segment significant narrowing is seen at the origin of left MCA. Irregular outline of A1segment of left ACA seen. Multiple collaterals are seen in suprasellar cistern, interhemispheric fissure cistern, producing puff of smoke appearance, suggestive of moyamoya disease. DSA = Digital subtraction angiography, ICA = Internal carotid artery, MCA = Middle cerebral artery, ACA = Anterior cerebral artery

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Figure 3: (a) NCCT head reveals intraventricular clots in right frontal and right temporal horns, CT angiography images -(b and c) anteroposterior view and (d) lateral view reveals normal vascular anatomy and branching patterns. No evidence of AVM or aneurysm arising from visualized vessels

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Table 2: Summary of clinical features

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Follow-up and outcome was not available in all the patients with PIVH. This was the major limitation of our study. Most of the patients were managed conservatively. Of the eight patients with PIVH in the hypertension group, three had external ventricular drainage (EVD). Of the nine patients with LOC, seven regained consciousness during hospital stay, and two patients' condition worsened and died. Forty percent of the patients developed hydrocephalus.

All the three patients with LSA aneurysm and PIVH were managed conservatively with excellent recovery and were symptom free at the time of discharge. Seven patients with AVMs were referred for surgical management after initial stabilization. Of the three patients of MMD, two had hemiparesis and all the three patients were managed conservatively. Patient with dAVF was given endovascular treatment and had good functional outcome. One with BA dissection presented with headache only and another patient with ICA dissection had presented with LOC. The patient with BA dissection recovered completely, however, the patient with ICA dissection worsened and was referred for surgical intervention. Mortality rate could not be assessed in our patients as most were referred to higher centers and follow-up study was not available.


 » Discussion Top


PIVH is defined as bleeding within the ventricles of the brain without associated parenchymal or subarachnoid hemorrhage as demonstrated on noncontrast head CT. [2] It accounts for 2-4% of all intracranial hemorrhages. [6] Passero et al., [8] published large series of 26 patients with PIVH followed by Giray et al., [5] consistingof 24 patients. Our cohort included 27 patients. Male predominance was reported in some series, [5],[9] whereas, in some series it was female predominance. [3],[10] In our study too, there was a female preponderance. Our study included all age groups including four children. In many studies headache was the commonest presenting feature, followed by loss of consciousness [3],[5],[6],[10] In our study too, headache was the commonest manifestation followed by loss of consciousness.

Though hypertension is a common predisposing factor for PIVH, its association varied in different studies, 38.4-80%. [3],[5],[8],[9],[10] In our series, hypertension was the main predisposing factor and accounted for 29.62%. Cerebral angiography, both CTA and DSA, has high yield. [5],[6],[9],[11],[12] In the study by Zhu et al., [11] the yield was 67% in the elderly and 63% in the young. The angiography yield included: Aneurysms, AVMs, and fibromuscular dysplasia, [3] In our study, the cerebral angiography revealed LSA aneurysms, AVMs, MMD, dAVF, and arterial dissections. From our study it is difficult to determine which modality, CTA or DSA is superior in determining the possible etiology for PIVH. However, two patients, one patient with dAVF and one patient with LSA aneurysm were diagnosed of their etiology only after DSA study.

After hypertension, AVM is an important cause of PIVH and varied between 14.2 and 32% [3],[5],[6],[9],[10] and in our series it accounted for 25.92% of the etiology. Coagulopathy and hematological disorders were the etiology in some series of PIVH. [5],[8],[9],[10] In our series, three (11.1%) LSA aneurysms was the cause for PIVH. LSA aneurysm rupture more often results in parenchymal hemorrhage, [13] PIVH secondary to LSA rupture is extremely rare. [14] LSA aneurysm was not the cause of PIVH in other series, however, few case reports have been documented. [15],[16] These aneurysms are relatively in an inaccessible location for direct surgical or endovascular occlusion and spontaneous resolution has been be observed. [14] PIVH can be the presenting feature of MMD. [2],[5],[17],[18] In our study 3 patients had MMD as etiology of PIVH. Also, two (7.4%) patients had arterial dissection, which has not been reported earlier in any of the PIVH studies; one had BA dissection and another had ICA dissection. ICA dissection usually presents with ischemia and rarely BA and ICA dissections are associated with SAH. [19],[20] PIVH is a rare presentation of a dAVF. [7],[21] dAVF consists of an AV shunt in the wall of a dural sinus without an intervening nidus. [7],[22] Selected series suggest that they occur only in one-tenth as frequently as intraparenchymal AVM. [23] The venous outflow restriction causing retrograde diversion of the venous outflow to the cortical veins, then to the deep medullary veins with resultant subependymal venous congestion and rupture of the congested fragile subependymal venous network has been attributed to the intraventricular hemorrhage. [7],[21] In our study, one patient had dAVF with PIVH, who presented with left-sided hemiparesis, ataxia, and lower motor facial palsy. Hameed et al., [9] too described one case of dAVF in their series. Even after extensive investigations, no cause could be established in 16.7-26.92% of patients. [5],[8] In our series, cause was not found in three patients (11.11%) even after cerebral angiography.

To conclude, PIVH is rare and hypertension is the most important predisposing factor. Yield of cerebral angiography is high in diagnosing the etiological causes of PIVH. Apart from hypertension and AVMs other etiological causes like MMD, LSA aneurysm, arterial dissection, and dAVF should be kept in mind with high index of suspicion and warrants cerebral angiography in all these patients with PIVH as some of the causes are potentially treatable. The major limitation of this study is lack of follow-up in the majority of patients.



 
 » References Top

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7.Padmanabhan R, Stacey R, Wimalaratna S, Kuker W. Dural arteriovenous fistula causing primary intraventricular haemorrhage. Br J Radiol 2008;81:e44-7.  Back to cited text no. 7
    
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11.Zhu XL, Chan MS, Poon WS. Spontaneous intracranial hemorrhage: Which patients need diagnostic cerebral angiography? A prospective study of 206 cases and review of the literature. Stroke 1997;28:1406-9.  Back to cited text no. 11
    
12.Mart′ý-F`abregas J, Piles S, Guardia E, Martí-Vilalta JL. Spontaneous primary intraventricular hemorrhage: Clinical data, etiology and outcome. J Neurol 1999;246:287-91.  Back to cited text no. 12
    
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16.Srivastava T, Sannegowda RB, Sharma B, Tejwani S. Lenticulostriate artery aneurysm presenting as primary intraventricular haemorrhage. BMJ Case Rep 2013;2013.  Back to cited text no. 16
    
17.Garg AK, Suri A, Sharma BS. Ten-year experience of 44 patients with moyamoya disease from a single institution. J Clin Neurosci 2010;17:460-3.  Back to cited text no. 17
    
18.Saeki N, Nakazak S, Kubota M, Yamaura A, Hoshi S, Sunada S, et al. Hemorrhagic type Moyamoya disease. Clin Neurol Neurosurg 1997;99:192-7.  Back to cited text no. 18
    
19.Chaves C, Estol C, Esnaola MM, Gorson K, O′Donoghue M, De Witt LD, et al. Spontaneous intracranial internal carotid artery dissection: Report of 10 patients. Arch Neurol 2002;59:977-81.  Back to cited text no. 19
    
20.Kim BM, Suh SH, Park SI, Shin YS, Chung EC, Lee MH, et al. Management and clinical outcome of acute basilar artery dissection. AJNR Am J Neuroradiol 2008;29:1937-41.  Back to cited text no. 20
    
21.Irie F, Fujimoto S, Uda K, Toyoda K, Hagiwara N, Inoue T, et al. Primary intraventricular haemorrhage from dural arteriovenous fistula. J Neurol Sci 2003;215:115-8.  Back to cited text no. 21
    
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]

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