Arteriovenous Malformation of the Vein of Galen presented by R2 吳吳吳
Feb 20, 2016
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 failureBrain 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 2nd 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 50-60 mmHg, SpO2 100% HR 150-160/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 110-120/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: 50-70 mmHg*SpO2: 90-95 %, 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