Arteriovenous Malformation of the Vein of Galen presented by R2 吳佳展

VGM Definition vascular malformation of the choroid plexus within the roof of the third ventricle

VGM Clinical presentation Neonatal presentation: congestive heart failure, tachycardia, respiratory distress, cyanosis Infantile presentation: hydrocephalus Late presentation: macrocephaly, dilatation of the facial and cervical veins

VGM Treatment High surgical mortality(90%) High mortality if without treatment(90% for patients with congestive heart failure) Most mortality occurs at the first week of life(9/16 in an autopsy series) Early intervention is important for these patients

VGM Interventional Radiology *Transarterial approach: glue, microcoil, microballoon *Transvenous approach: multiple coils

Prognosis  Congestive heart failure  Brain ischemic sequel: atrophy, periventricular leucomalacia, hemorrage

Case History *GA 37+5 weeks, BW 3147 gm Apgar score: 8-9 *Brain lesion r/o VGM at GA 30 weeks but loss of follow up *Maternal history: G3P2AA1, no other associated disease

Case history *C/S due to previous C/S at LMD *On the 2 nd day, decreased activity, respiratory distress, cyanosis, skin mottling *Blood gas: bicarbonate 7.7 mmol/l *Coagulopathy: INR 4-5 *Heart echo: cardiomegaly, MR, TR, pulmonary hypertension *CT with contrast: VGM

Episode One *Initial treatment: intubation, correct acidosis, Lasix, dopamine *ETT, peripheral line, arterial line *arrived at angio room at 6pm, July 5 *vital signs: SBP mmHg, SpO2 100% HR /min *induction agents: ketamine 1.5mg/kg atracurium 0.7mg/kg

Episode One *central venous catheter placement.dilatation of jugular vein.high cerebral blood flow.low systemic blood pressure.high O2 saturation of jugular venous blood.direct pressure measurement is preferred if any doubt

Episode One *right femoral artery line placement 20G for embolization *positioning *radiologists performed TAE but guide wire could not be advanced up into aorta

Episode One: Bradycardia HR decreased to /min at 8:30 pm Atropine 0.1mg x 3, Bosmin 0.03mg but failed Left femoral artery catheterization was tried again but failed Procedure aborted because of his unstable conditions Hypothermia was noted after drape removed, less than 35 degree when he returned to NICU

Hypothermia: patient factors *newborn greater body surface area/body weight ratio immature thermoregulatory center inefficient thermogenesis *unable to cope with increased metabolic demand *more sensitive to hypothermia

Anesthesia in angio room *anesthesia machine long tube, large dead space no air source ( a drawback for a newborn or preterm) only IMV mode, may be unsuitable for newborn requiring special ventilation support( high frequency etc.)

Anesthesia in anio room *limited access to the patient

Anesthesia in angio room  Only basic monitor available NIBP, ECG, SpO2( only adult size)

Anesthesia in angio room *heat preserving equipment only heat lamp *higher environmental temperature

Treatment *peritoneal dialysis *dopamine, dobutamine, epinephrine, Lasix *high frequency(Fi02 40%)

Episode Two *portable air source ( for ventilator) *heat lamp and Bair Hugger used immediately *rapid positioning *immediate covering and draping *monitoring ABP, SpO2, ECG, BT

Episode Two *total procedure time: 9:30am to 8:30pm *BT: no less than 36.8 degree *SBP: mmHg *SpO2: %, gradually increased FiO2 requirement *desaturation to less than 90 %, increased to 97% after ambu bagging with pure O2 *endotracheal suction found blood, dry? Bosmin 0.03mg endotracheal injection

Why no Episode Three ? *desaturation, CO2 retention *increased pulmonary hypertension (PG nearly 100 mmHg) *persistent right to left shunt at PFO *braycardia *expired on July 10

Thank You for Your attention