This document discusses a case of an 8-hour-old male infant presenting with breathlessness, abdominal distension, and swelling in the lower limbs and sacral area, who was diagnosed with a vein of Galen malformation. Vein of Galen malformations are congenital abnormalities that develop during weeks 6-11 of fetal development. They can cause congestive heart failure, hydrocephalus, and developmental delays. Investigation with cranial ultrasound, MRI/CT, and angiography are used for diagnosis. Treatment involves a multidisciplinary team and endovascular embolization of the abnormal vessels, though care must be taken with neonates. Prognosis has improved with advances in minimally invasive treatments.

1. Vein of Galen
malformation
DR MANDAR HAVAL
MBBS DCH DNB
FELLOWSHIP IN NEONATOLOGY(NNF)

2. Case
• 8hr day old male child
• 2nd issue of non consanguineous marriage
• Referred from periphery

3. Complains
• Breathlessness
• Abdominal distension
• Swelling over sacral area and lower limb

4. ANC
• Mother is registered case.
• No h/o fever, rash, lymphadenopathy.
• Blood group AB positive
• Polyhydrominios in 3rd trimester

5. Birth History
• Full term normal delivery of male child ,
hospital delivery baby cried immediately
• Breast feeding was attempted within an hour
but refusal to feed (No Retracted or flat
nipple)

6. ON ADMISSION
• Pale
• Vitals - HR - 173/min
RR - 54/min
SPo2 – 94% without O2
BP – 46/ 24 mm of Hg rt arm supine
position
BP of all the four limb appers to be below 3rd
percentile
cranial bruit, and marked carotid pulses was
present

7. System examination
• Cardiovascular – S1 S2 herd
gallop +
systolic murmur +
• Respiratory – Tacypnea
B/l crepts

8. • Abdominal – Liver palpable 4cm below the
RCM , firm
Spleen just palpable
• CNS – Irritable , neonatal reflex ABSENT

9. We started investigating
• CBC

10. ABG
• ABG ON FIO2 21%
suggestive of
METABOLIC METABOLIC
ACIDOSIS

12. Note - potassium is 8mmol/L
treatment started
ECG – No changes

13. So carried out RFT on DAY 2

14. Considering all these report with
clinical examination
• Our conclusion was congestive cardiac failure
with pre renal ARF

15. Causes of CCF on DAY 1

16. 2D echo
• Normal with ejection fraction of 50 %

17. HEAD IMAGING

21. Diagnosis
Vein of Galen
malformation

22. Cause..
• Although any vessel may be affected, the vein
of Galen is the most frequently affected.
Congenital malformation develops during
weeks 6-11 of fetal development as a
persistent embryonic prosencephalic vein of
Markowski.

23. Clinical presentation
• Congestive heart failureNeonates may present
with tachypnea, respiratory distress, and
cyanosis.
• They often require ventilatory support and
institution of aggressive management of heart
failure.

24. Cont…
• Hydrocephalus - Hydrocephalus may be the
presenting feature in older infants.
• A cause should be sought in neonates with
macrocephaly.
• Infants may have hydrocephalus, in which case
prominent scalp veins or "sunset" eye findings
are noted.

25. Cont..
• Developmental delay: Signs of hydrocephalus
and congestive heart failure should be looked
for in infants with developmental delay.
• In early childhood, symptoms include
headache, convulsive seizures, hydrocephalus,
and cardiac failure.

26. D/D
• Abnormal Neonatal EEG
• Arteriovenous Malformations
• Cavernous Sinus Syndromes
• Cerebral Palsy
• Cerebral Venous Thrombosis
• Epilepsy in Children with Mental Retardation
• Hydrocephalus
• Intracranial Hemorrhage
• Mental Retardation
• Neonatal Seizures
• Pseudotumor Cerebri

27. Investigation
• Cranial ultrasound
This will help to localize or identify the lesion.
Doppler studies can help further to
understand the hemodynamics of the lesion.

28. Cranial MRI and/or CT scan with and
without contrast administration

29. MR angiography

30. Cranial angiography
• In patients being considered for surgery or for
occlusive therapy, cranial angiography is
required to define the extent of aneurysmal
dilatation and details for arterial feeders

31. Yasargil’s classification of vein of Galen
malformations
•Pure cisternal fistula between pericallosal arteries (anterior or
posterior), posterior cerebral artery (P4 and its branches) and the
vein of Galen
•Fistulous connections between the thalamoperforators ( basilar
and
P1 segment) and the vein of Galen.
•Mixed form with characteristics of both Type 1 and Type 2 lesions
•Plexiform AVM with one or more intrinsic niduses within the
mesencephalon or thalamus with draining veins emptying into the
vein of Galen
a.Pure plexiform nidus in the parenchyma of mesencephalon
or thalamus
b.Nidus within the parenchyma combined with fistulous
cisternal nidus (Type 1)

32. Consideration for Treatment
• If the child can be managed medically, it is best to wait until
aged 5 or 6months old.
• Embolisation of a neonate is a high risk procedure. There are
some limitation of the procedure (amount of contrast
medium, flush solution can be given to baby).
• Surgical attempts at closure of the shunt have high mortality
or severe morbidity. Embolisation is the only way to treat
VOGM at this stage.
• Large shunt with many feeding vessels will need several
embolisation sessions.

33. Fraser’s score
1 (cardiac) + 5 (cerebral) + 2 (respiratory) + 2 (hepatic) + 1 (renal) = 11
For emergency treatment 8/ 21 < score> 12 / 21
Score less than 8 = Not for treatment
Score more than 12 = Medical management until age over 5 months old.

34. Treatment
• Recently, prognosis of
patients with “Vein of
Galen” has improved,
largely due to
improvements in
endovascular treatments
and techniques.
• These technique involve the
use of the catheter that is
inserted in to a feeding
artery to block off the
supply by using coils and
glue like substances.

35. Team Management
• Team approach is critical to
successful management
Fetal medicine
Neonatology
Pediatric cardiology
Intensive care
Neurologist
Neurosurgeon
Interventional Neuroradiology
Fetal Medicine
Interventional
Neuroradiology
Peadiatric
Cardiology
Intensive care
Neonatology
Patient
Neurosurgery Neurologist

36. THANK YOU