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Intracranial arteriovenous malformation in an infant-vein of Galen malformation

Intracranial arteriovenous malformation in an infant—vein of Galen malformation

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Page 1: Intracranial arteriovenous malformation in an infant—vein of Galen malformation

Intracranial arteriovenous malformation in an infant-vein of Galen malformation

Page 2: Intracranial arteriovenous malformation in an infant—vein of Galen malformation

Apollo Medicine 2011 DecemberCase Report

Volume 8, Number 4; pp. 307–309

© 2011, Indraprastha Medical Corporation Ltd

Intracranial arteriovenous malformation in an infant—vein of Galen malformation

Vineet B Gupta*, Hemant Choudhary**, Harsh Rastogi†, VP Singh‡

*Senior Consultant, Paediatric Neurology, **DNB Fellow, †Senior Consultant, Neuroradiology, ‡Senior Consultant, Neurosurgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi – 110076, India.

ABSTRACT

The vein of Galen malformation is a rare cerebrovascular disorder which is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. In neonates, it usually causes con-gestive heart failure. Infants, older children, and adults usually present with mass effect, seizures, or intracranial hemorrhage. Here, we report a 6-month-old infant diagnosed with the vein of Galen malformation antenatally and presented to us with acute hydrocephalus. She underwent emergency embolization, developed in right femoral artery thrombosis as a complication requiring emergency thrombectomy and end-to-end repair. After embolization, she stabilized but hydrocephalus and raised intracranial tension persisted for which she required endoscopic third ventriculostomy.

Keywords: Hydrocephalus, noninvasive treatment, vein of Galen malformation

Correspondence: Dr. Vineet B Gupta, E-mail: [email protected]: 10.1016/S0976-0016(11)60014-8

INTRODUCTION

The vein of Galen malformation is a rare cerebrovascular disorder, which is characterized by an abnormal direct com-munication between one or several cerebral arteries and the vein of Galen. Posterior choroidal artery, anterior cerebral artery, and transmesencephalic artery are the most common arteries that join the malformation.1 It may appear in the neonatal period or afterwards.2

CASE REPORT

A 6-month-old female child was brought to our hospital by her parents with complaints of lethargies, vomiting, drowsi-ness, decreased activity, not responding to the mother, and no eye contact with the mother for the past 3 days.

There was no history of fever, cough, loose stools, breathlessness, or seizures. The child was diagnosed as ven-triculomegaly on antenatal ultrasonogram. Birth history and postnatal histories were uneventful. The child had attained developmental milestones appropriately for her age.

Physical examination showed her weight 6.5 Kg (15th percentile), length 65 cm (15–50th percentile) and head

circumference 46 cm (> 97th percentile). Her anterior fonta-nel was full and sutural diastasis was noted. She had a down-ward gaze. Pupils were bilaterally equally reacting. Her limb movements were normal. Vital signs were within normal range. Rest of the systemic examination was unremarkable.

A diagnosis of the acute hydrocephalus was made. She was started on intracranial hypertension management pro-tocol. An urgent MRI brain performed showed the vein of Galen arteriovenous fistula compressing on the posterior third ventricle and cerebral aqueduct with obstructive hydrocepha-lus and periventricular ooze. Echocardiogram was normal.

The child was immediately taken for digital subtraction angiography (DSA) and glue embolization. The DSA showed a large vein of Galen aneurism with a single feeding arterial channel from a common trunk from the left posterior choroi-dal and anterior cerebral artery. There was negligible drainage. The single arterial feeder was super selectively embolized suc-cessfully using 80% glue. No residual filling of aneurysmal sac was seen on the postembolization check angiography.

However, at the end of the procedure, there were weak distal pulses in the right lower limb. Intra-arterial nitroglyc-erin and papaverine and heparin were given to overcome the arterial spasm but not much of improvement was seen. As further thrombolysis was contraindicated, the vascular surgeon was requested to see the case.

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308 Apollo Medicine 2011 December; Vol. 8, No. 4 Gupta et al

© 2011, Indraprastha Medical Corporation Ltd

On exploration, it was found that she had a thrombus in the right femoral artery and that had resulted in acute ischemia of the limb. Vascular surgeon did end-to-end repair of a seg-ment of the right femoral artery. Postvascular repair, there were good pulses in the right lower limb (Figures 1 and 2).

Following surgery, the child was regularly monitored for the perfusion in the right lower limb as well as for signs of raised intracranial pressure. She was put on low-molecular-weight heparin.

The child remained asymptomatic for a week, but then developed vomiting and increased irritability. In view of the increased intracranial tension, a computerized tomography scan of the brain was done which showed increasing hydro-cephalus.

The child was then taken for endoscopic third ventricu-lostomy, which she tolerated well.

The child was discharged after 3 weeks of hospitaliza-tion in a stable condition.

DISCUSSION

Intracranial arteriovenous malformations (AVM) are com-plex medical emergencies in infants. It is reported that 30% of AVM in childhood is the vein of Galen malformation.3

Systemic manifestations and congestive heart failure are the most common presentations encountered in the neo-natal period and infancy whereas neurological signs and symptoms and hemorrhage belong mostly to the adult symptomatology.4

The malformation develops between the 6th and 11th weeks of gestation after the development of the circle of Willis. It is thought to result from the development of an arteriovenous connection between the primitive choroidal vessels and the median prosencephalic vein of Markowski. The abnormal flow through the connection retards the nor-mal involution of this embryonic vein and thus prevents the development of the vein of Galen.5

Figure 1 (A) Pre-embolization angiogram, and (B) postembolization angiogram.

A B

A B

Figure 2 (A) Postembolization computerized tomography, and (B) postembolization magnetic imaging source.

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Intracranial arteriovenous malformation in an infant Case Report 309

© 2011, Indraprastha Medical Corporation Ltd

Neonatal Rating Score6 (Table 1)

Lasjaunias and Ter brugge score is derived from the measures of cardiac, cerebral, hepatic, renal and respiratory function.• < 8 results in a decision not to treat,• 8–12 prompts emergency endovascular intervention,• 12 recommends medical treatment alone and delayed

embolization at 5 months.

CONCLUSION

With more and more technological advances in the develop-ments in the field of interventional neuroradiology and availability of better postprocedure intensive care, these once nontreatable conditions with a very high mortality rate are now potentially curable using interventional neuroradi-ological techniques with excellent clinical results, low com-plication rate and very low morbidity and mortality. In the future with more centers having advanced neuroradiologi-cal facilities, the treatment of this condition will be well within the reach of many patients.

Table 1 Neonatal rating score.

Score Cardiac Cerebral Respiratory Renal Hepatic

5 Normal Normal Normal – –4 Nontreated Infraclinical EEG Tachypnea bottle – – overload anomalies finished3 CCF stable with Nonconvulsive Tachypnea bottle Normal Normal treatment CNS signs not finished2 CCF unstable with Isolated convulsion Assisted ventilation Transient anuria Hepatomegaly treatment normal function1 Need ventilation Seizure permanent Assisted ventilation Unstable diuresis Moderate hepatic CNS signs FiO2 > 25% with treatment impairment0 Resistant to Rx Coma Assisted ventilation Anuria Coagulation disorder

CCF: congestive cardiac failure; EEG: electroencephalogram; CNS: central nervous system.

REFERENCES

1. Smilari P, Incorpora G, Sciacca P, et al. Vein of Galen aneu-

rysmal malformation. Different clinical expressiveness. Three

case reports. Minerva Pediatr 2000;52:55–62.

2. Lasiaunias P, Rodesch G, Terbrugge K, et al. Vein of

Galen aneurysmal malformations. Report of 36 cases

managed between 1982–88. Acta Neurchir (Wien) 1989;99:

26–37.

3. Wiggins CW, Loisel D, Budock AM. Intracranial arterio-

venous malformation in a neonate: aneurysm of the Great

Vein of Galen. Neonatal Netw 1991;9:7–17.

4. Meyers PM, Halbach VV, Phaturos CP, et al. Hemorrhagic

complications in vein of Galen malformations. Ann Neural

2000;47:748–55.

5. Raybaud CA, Strother CM, Hald JK. Aneurysms of the vein of

Galen: embryonic considerations and anatomical features relat-

ing to the pathogenesis of the malformation. Neuroradiology

1989;31:109–28.

6. Lasjaunias P, Alvarez H, Rodesh G, et al. Aneurysmal

malformations of VOG. Intervent Neuroradiol 1996;2:

15–26.

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