Pediatric Neurointervention: Vein of Galen Malformations

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1 Pediatric Neurointervention: Vein of Galen Malformations Johanna T. Fifi, M.D. Assistant Professor of Neurology, Neurosurgery, and Radiology Icahn School of Medicine at Mount Sinai November 9 th, 2014

2 Consultant - Microvention Disclosures

3 Intracranial Arteriovenous Shunts in Pediatric Population Vein of Galen Malformations Pial AVMs and fistulas Dural Sinus AV Shunts in Children Dural Sinus Malformations

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5 Onset of Pediatric Intracranial Arteriovenous Shunts (Lasjaunias)

6 Intrauterine Diagnosis VGAM or Dural Sinus Malformation (DSM) Imaging with Fetal ultrasound and MRI Encephalomalacia Poor Prognosis Macrocephaly VGAM: Unrelated to prognosis DSM: Poor prognosis Cardiomegaly Fetal echo

7 Prenatal Ultrasound

8 Fetal MRI

9 Fetal Echocardiogram M-mode and doppler Structural defects and flow Cardiomegaly with enlarged right side Prenatal Digoxin treatment

10 VGAM Median vein of prosencephalon Embryonic precursor of vein of Galen VGAM Subarachnoid Choroidal Quadrigeminal Connect to embryonic falcine sinus Raybaud et al. Neuroradiology (1989) 31:

11 VGAM vs. VGAD Connection to cerebral veins VGAM (Mural) VGAM (Choroidal) VGAD (Pial AVM) Lasjaunias

12 Angiographic Differentiation bet. VGAM & VGAD Connection between dilated central vein and cerebral vein is the sign of VGAD ICV Reflux to cortical vein Cerebral drainage to the central vein

13 Hemodynamic Changes at Birth Removal of low resistance placenta Relative increase in left atrial pressure leading to closure of foramen ovale Muscular contraction of ductus arteriosus Decrease in pulmonary vascular resistance Low cardiac reserve Undeveloped sympathetic compensation

14 Pretherapeutic Evaluation History and Physical (e.g. Sz-brain damage) Weight gain and Head Circumference Renal and liver function Cardiac echo Cardiac tolerance PDA, patent Foramen Ovale, associated anomaly Brain imaging: CT, MRI, Ultrasound encephalomalacia, calcification, ventriculomegaly Coagulation profile Angiography is not indicated unless embolization is planned

15 No Indication for Treatment of VGAM Multi-organ failure Encephalomalacia, Calcification Small head circumference (brain atrophy) Intrauterine cardiomegaly?

16 Medical Tx for High Output Failure Diuretics: Decrease preload Cathecolamine: Increase cardiac contractility Dopamine and Dobutamine Digoxin effect is controversial Prenatal Digoxin

17 F/U Newborns not Rx CT in 4 weeks CT 3 months of age Cross section at 6 months Embolization at 6-9 months

18 Early Intervention Calcifications/ Encephalomalacia Hydrocephalus/ Macrocephaly Failure to thrive Milestone regression Developmental delay?seizures

19 Ventriculo-peritoneal shunt for VGAM and other pediatric AVMs Enlargement of VGAM Seizure, hemorrhage Subdural hematoma Slit ventricle Infection

20 Treatment for VGAM Transarterial embolization with n butyl-cyanoacrylate (NBCA): First choice Transvenous approach is secondary option May create subependymal / intraventricular hemorrhage Surgery Radiosurgery

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23 Endovascular Treatment Anesthesia / Nursing Fluid management BP management Ultrasound for access 4F introducer sheath Contrast sparing Liquid embolic agents N-Butyl Cyanoacrylate 50-90% NBCA/Ethiodal/Tantalum Hypotension (systolic mmhg)

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25 Day 3 of life

26 Hypotension

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29 Neonatal VGAM Case Patient required another embolization Weaned off IV cardiac medications Discharged home after several weeks of hospitalization on PO medications Monitored clinically and with imaging

30 6 months old Brought back for further embolization

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33 VPS shunt placed

34 Avoid Ventriculo-peritoneal shunt for VGAM and other pediatric AVMs Enlargement of VGAM Induce calcifications Seizure, hemorrhage Slit ventricle Infection

35 1 year of age

36 16 months

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38 Potential Complications

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40 Outcomes Vein of Galen malformation: diagnosis and management. (1987) Johnston IH, Whittle IR, Besser M, Morgan MK. 245 patients 91 patients (37.1%) were treated by direct operation and 29 patients (11.3%) were treated by predominantly shunting or remote vessel ligation. 46 patients (18.8%) were treated by medical means. In 79 patients (22.2%), there was no treatment or no details of treatment. There was an overall series mortality of 55.6% (no details were available in 33 cases) and a 37.4% mortality for surgically treated cases. After operation, there was a 46.3% incidence of significant morbidity in survivors. Neonatal patients fared worst, 91% overall mortality of 64 of 70 cases. Over the age of 1 year, the surgically treated patients had a 25.6% mortality and a 42.3% major morbidity in survivors.

41 Conclusion Consideration is given to some of the ways in which these figures may be improved, in particular a staged approach during the neonatal period, with the use of selective embolization or occlusion of vessels to reduce the volume of the arteriovenous shunt until the patient is older and better able to tolerate major operation.

42 Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up. Jones BV 1, Ball WS, Tomsick TA, Millard J, Crone KR All endovascular treatment 8/13 neonatal 2 normal/near normal, 1 impaired, 5 deceased 5/13 older all 5 normal or near normal

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44 Neonatal Vein of Galen Patients 46 VGAM patients treated 16 neonates babies (excluding VGAD patients) required neonatal intervention 1 death in premature baby 6 patients normal 1 patient with mild language delay, 1 mild hemiparesis 89% of patients with good functional outcome with closure of lesion

45 TEAM Neonatal Intensivist Pediatric Intensivist Pediatric Cardiologist Pediatric Neurosurgery Pediatric Neurology Pediatric Anesthesiologist Pediatric Neuroendovascular Surgery Nurses Technologists

46 Unusual procedures when they become routine, they become safe!!!

47 Conclusions Pediatric intracranial vascular diseases are dynamic conditions and mainly ruled by venous drainage pattern. Careful clinical observation and timely intervention is important for good results. Trans arterial embolization with a liquid embolic agent is the first choice of treatment.

48 Neuroendovascular Surgery Mount Sinai Health System Thank you!