Cerebral Arteriovenous Malformations in Children

Toru Kurokawa, MD, Akinobu Matsuzaki, MD, Kanehiro Hasuo, MD,

Masashi Fukui, MD, Shigeru Tomita, MD, Makoto Matsuo, MD,

Yung-Jung Chen, MD and Charnchai Kasemkosolsri, MD

Differences in clinical features of arteriovenous malformations (A VM) in six children with rup­tured A VM (RAM) and three with unruptured A VM (URAM) are herein reported. The age at onset ranged from the neonatal period to 8 yrs in cases of URAM and 10 to 12 yrs in those with RAM. The children with RAM had a sudden onset of intracranial hemorrhage, except for one with prodromal symptoms such as vertigo or headache. The clinical features of URAM were chronic mild symptoms such as a certain degree of delayed development, focal neurologic signs or in­creased intracranial pressure in addition to macrocephalus and cranial bruit. CT scan revealed an evenly enhanced A VM and dilatation of ventricles in cases of URAM. Th e RAMs were angiograph­ically relatively small while the URAMs were large.

Kurokawa T, Matsuzaki A, Hasuo K, Fuku i M, Tomita S, Matsuo M, Chen Y-J, Kasemkosolsri C. Cerebral arteriovenous malformations in children.

Arteriovenous malformations (A VM) are one cause of intracranial hemorrhage [1]. The un­ruptured A VM has seldom been mentioned as a cause of chronic neurologic disorders in chil­dren and frequently there is a delay in the diagnosis. We classified A VM into ruptured AVM (RAM) and unruptured AVM (URAM), in an attempt to clarify differences in the clinical picture.

Subjects

The subjects were six cases of RAM and three of URAM. The ages at onset were 10 to 12 yrs in cases of RAM and the time of birth, 7 yrs

From the Departments of Pediatrics (TK, AM, ST, MM, YJC, CK), Radiology (KH) and Neurosurgery (MF), Faculty of Medicine, Kyushu University, Fukuoka.

Received for pUblication: May 9, 1984. Accepted for pUblication: April 3, 1985.

Key words: Arteriovenous malformation, vein of Galen, mental retardation, intracranial hemorrhage, anomaly. Correspondence address: Toru Kurokawa, MD, De­partment of Pediatrics, Faculty of Medicine, Kyushu University, Maidashi, Higashi-ku, Fukuoka 812, Japan.

Brain Dev 1985; 7:408-13

and 8 yrs in URAM (Table I). Two boys and four girls were in the RAM group and two boys and one girl were in the URAM group.

Results

Initial symptoms were acute intracranial hemor­rhage in five with RAM and an ep isode of headache , vomiting and vertigo in the other one with RAM . Macrocephalus, headache and hand tremor occurred in one child each with URAM. The intervals between the onset of the disease and the first visit were 5 days to 3 mos in RAM. One of the patients with RAM was initially treated for periodic vomiting and was transferred after the acute stage had passed. The intervals were 6 mos in the baby with aneurysm of the great vein of Galen and in whom a large head had been present since birth , about 4 yrs in the 7-yr-old boy who complained of headache when a sweater was pulled over the head, and 5 yrs in the 13-yr-old girl whose behavior was slow and who had tremor of the right hand.

Presenting features are summarized in Table 2. Intracranial hemorrhage in RAM was con­firmed by lumbar puncture in all cases. Bruit was heard in none with RAM . The eye symp­toms included ocular pain, papilledema, retinal

Table 1 Ages at onset

Ages Ruptured Unruptured AVM AVM (yrs) (cases) (cases)

0 1 - 6 0

7 8 9 0

10 3 11 1 12 2

Total 6 3

hemorrhage and posthemorrhagic hemianopsia. A lO-yr-old girl with RAM had an episode of headache, nausea and vomiting. She there­after experienced vertigo several times after a Japanese style hot bath. Three mos later, intracranial hemorrhage occurred and she was brought to the hospital.

Concerning URAM, all three patients had mental retardation or slow behavior. They also had the setting sun phenomenon and vertigo in one each. Cranial bruit was audible in all with URAM . Associated dilated veins were observed on the periocular region or head skin in two. Papilledema and venous dilatation of retinae were present. The past history disclosed pre- or peri-natal abnonnalities in two with URAM , one a case of threatened abortion and the other a small-for-date baby.

Electroencephalography was done for all these patients. In six with RAM, four had focal slow waves, one focal spike-and-wave com­plexes and one a borderline abnormality with slight focal slow waves. In URAM , focal slow wave bursts and generalized slow wave bursts were demonstrated in one each. In the child with aneurysm of the great vein of Galen a normal sleep recording was obtained.

Skull radiographs in URAM showed suture separation with enlargement of the Turkish sella, dilatation of the venous groove with bone thickening and normal findings in one, respectively .

CT scans were done in one with RAM and two with URAM. The one with RAM seemed nonnal although a contrast enhancement was not done. The scan in those with URAM demonstrated mass lesions in the thalamus and in the region of the great vein of Galen (Fig 1)

Table 2 Symptoms and signs of ruptured and unruptured A VM

Symptoms and signs Ruptured Unruptured

AVM AVM (cases) (cases)

Intracranial hemorrhage 6 Vertigo 1 2 Episodic headache 2 Enlarged head 3 Macewen's sign 1 Bruit 3 eu taneous dilated veins 2 Heart murmur Ocular or neck pain Papilledema 1 Retinal hemorrhage Retinal venous dilatation Hemianopsia 4 Anisocoria Setting sun phenomenon Mental retardation 1 Borderline retardation 1 Slow behaviour 1 Tremor (chronic) Posthemorrhagic sequelae 4 Short stature 1

Total 6 3

Fig 1 6-mo-old boy. Aneurysm of great vein of Galen. CT scan shows a round mass with enhancement in the posterior midline and symmetrical dilatation of lateral and third ventricles.

Kurokawa et al: Arteriovenous malformations 409

in one each along with dilatation of ventricular systems in both. The mass lesions were evenly enhanced .

Cerebral angiography was performed in all nine patients. The locations are listed in Table 3. The feeder arteries were anterior cerebral artery in one and middle cerebral artery in five

410 Brain & Development, Vol 7, No 4, 1985

with RAM (Fig 2) while they were middle cerebral artery in two and middle meningeal artery in one with URAM (Fig 3).

Total excision was carried out in five of six of RAM. Ligation of the feeder artery and ventriculo-peritoneal shunt were performed in the case of aneurysm of the great vein of Galen

and ventriculo-peritoneal shunt in thalamic A VM. Surgical was not done in the child with a huge para sagittal URAM.

Fig 3 Arteriovenous malformations (Unruptured). a: 9-yr-old girl. Internal carotid angiography shows arteriovenous malformation in the thalamus mainly fed by the anterior thalamo-perforating artery and medial posterior choroidal artery. b: 7-yr-3-mo-old boy. Dural arteriovenous malformation in the parieto­occipital region.

Table 3 Location of arteriovenous malformations

Ruptured Unruptured Location AVM AVM

(cases) (cases)

Frontal 1 Parietal 2 Parieto-occipital 1 Temporal Thalamus 1 Paracallosal 1 Vein of Galen 1

Total 6 3

Discussion

Intracranial hemorrhage was the initial symp­toms in five of the nine children. The most frequent clinical manifestation is hemorrhage [2-5] and is followed by seizures and head­ache [4-6], in adults. However, psychomotor retardation [7,8], progressive neurological def­icit [9] or congestive heart failure [9] do occur in children. The present series confirmed that longstanding mild neurologic symptoms or mild developmental retardation in association with macrocephaly are observed characteristically in cases of URAM.

Kelly 11 et al [4] reported a delay between the onset of the disease and diagnosis in the cases of URAM. The delay was 6 mos to 5 yrs in our study. This may be because many pediatricians and obstetricians are unfamiliar with URAM and do not routinely apply their sthetoscope to the scalp of children. Although perinatal problems have been given little attention in the literature, there was an ab­normal history including threatened abortion, intrauterine growth retardation or poor sucking in two of our children with URAM. There was a silent period between the perinatal and the first symptom in infancy and childhood.

All three with URAM presented with de­velopmental problems including slow reactions, awkwardness, retardation or the lowest achiever in a class, or chronic mild motor impairment. The clinical manifestations differed from those seen in cases of RAM. Delayed psychomotor development has been reported in an infant with URAM [7]. Onuma T et al [8] reported that among six with an onset at under 6 yrs of age, three had hemorrhage, two mental retarda­tion and one headache as the initial symptoms. Focal neurologic deficits such as unilateral tremor may be caused by congestion or by arterial steal [10]. Visual obscuration, dimness in vision and papilledema are signs probably related to an increased intracranial pressure [11] and were present in our patients. Dilated veins on the scalp [7, 10, 12], forehead [7], oral venous angiomata [13] or cutaneous angio­mata [5] all aid in the diagnosis. Venous dilatations at the periocular region as well as at ocular fundi were present in our patients. Cranial bruit was heard exclusively in all three with URAM but not in those of RAM. Gold AP et al [14] classified aneurysm of the vein of

Kurokawa et al: Arteriovenous malformations 411

Galen into three categories; I) neonatal period, 2) infancy and 3) older child and adult. Our case belonged to the infancy group because of a hydrocephalus without cardiac failure. Cardiomegaly [10] or heart failure [5,7,10] is common, especially in the newborn with aneurysm of the vein of Galen [14-18]. There are concomitant patent ductus arteriosus or atrial septal defects in case of aneurysm of the vein of Galen [18]. Heart disease was absent in our patients.

CT scan without contrast enhancement may not be diagnostically contributory , although it sometimes will reveal an ischemic lesion , cal· cification, hemorrhage, low density cystic areas, ventricular dilatation or displacement [15, 19]. CT scan with enhancement was useful for clarifying A VM in our study, as also found by other workers [16,20] .

The size of A VMs of URAM was large in our patients. Large A VMs are characterized by seizures [21] or progressive neurologic deficit [4] willie small A VMs are silent until rupture when they bleed massively [2, 4] . Hydrocepha­lus is caused by obstruction of the aqueduct of Sylvius by the engorged vein or markedly increased pressure in dural sinuses [7] . Progno­sis is less favorable in children than in adults [21] . The younger the patient, the greater the chance of recurrent hemorrhage from A VM [6] . There has been a recent great advance in the therapy of AVM. Total removal is the most desirable treatment [22,23], but it is not necessarily attained in a huge A VM or A VM occupying the thalamus, basal ganglia or brain stem. As the supplementary procedure , intra­vascular artificial embolization has become to play an important role for preoperative treat­ment or for management of a unremovable AVM [24-27]. Gamma-ray irradiation is re­ported to be effective for a small A VM in the non-resectable areas [28] . These procedures are effective even for deep-seated A VM or dural AVM [10,29]. The recovery from the neuro­logical deficits caused by surgical operation is more easily attained in children than in adults. To avoid a possible rupture , efforts should be made to establish an early diagnosis.

Acknowledgments

We are grateful to M Ohara for critical reading of the manuscript. This study was supported by Grant No 83-05-14 from the National Center for Nervous,

412 Brain & Development, Vol 7, No 4,1985

Mental and Muscular Disorders (NCNMMD) of the Ministry of Health and Welfare, Japan.

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