Arteriovenous Malformation of the Vein of Galen

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Arteriovenous Malformation of the Vein of Galen

• presented by R2 ???

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VGM

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

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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

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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

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VGM Interventional Radiology

• Transarterial approach glue, microcoil,
  microballoon
• Transvenous approach multiple coils

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Prognosis

• Congestive heart failure
• Brain ischemic sequel atrophy, periventricular
  leucomalacia, hemorrage

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Case History

• GA 375 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

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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

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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

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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

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Episode One

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

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Episode One Bradycardia

• HR decreased to 110-120/min at 830 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

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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

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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.)

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Anesthesia in anio room

• limited access to the patient

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Anesthesia in angio room

• Only basic monitor available
• NIBP, ECG, SpO2( only adult size)

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Anesthesia in angio room

• heat preserving equipment
• only heat lamp
• higher environmental temperature

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Treatment

• peritoneal dialysis
• dopamine, dobutamine, epinephrine, Lasix
• high frequency(Fi02 40)

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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

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Episode Two

• total procedure time 930am to 830pm
• 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

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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

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Thank You for Your attention