Vein of Galen malformation

DR MANDAR HAVAL

MBBS DCH DNB

FELLOWSHIP IN NEONATOLOGY(NNF)

Case

• 8hr day old male child

• 2nd issue of non consanguineous marriage

• Referred from periphery

Complains

• Breathlessness

• Abdominal distension

• Swelling over sacral area and lower limb

ANC

• Mother is registered case.

• No h/o fever, rash, lymphadenopathy.

• Blood group AB positive

• Polyhydrominios in 3rd trimester

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)

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

System examination

• Cardiovascular – S1 S2 herd

gallop +

systolic murmur +

• Respiratory – Tacypnea

B/l crepts

• Abdominal – Liver palpable 4cm below the RCM , firm

Spleen just palpable

• CNS – Irritable , neonatal reflex ABSENT

We started investigating

• CBC

ABG

• ABG ON FIO2 21% suggestive of

METABOLIC METABOLICACIDOSIS

Note - potassium is 8mmol/Ltreatment started ECG – No changes

So carried out RFT on DAY 2

Considering all these report with clinical examination

• Our conclusion was congestive cardiac failure with pre renal ARF

Causes of CCF on DAY 1

2D echo

• Normal with ejection fraction of 50 %

HEAD IMAGING

Diagnosis

Vein of Galen malformation

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.

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.

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.

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.

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

Investigation

• Cranial ultrasound

This will help to localize or identify the lesion. Doppler studies can help further to understand the hemodynamics of the lesion.

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

MR angiography

Cranial angiography

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

Yasargil’s classification of vein of Galen malformations•Pure cisternal fistula between pericallosal arteries (anterior orposterior), posterior cerebral artery (P4 and its branches) and thevein of Galen

•Fistulous connections between the thalamoperforators ( basilar andP1 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 themesencephalon or thalamus with draining veins emptying into thevein of Galen

a.Pure plexiform nidus in the parenchyma of mesencephalonor thalamus

b.Nidus within the parenchyma combined with fistulous cisternal nidus (Type 1)

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.

Fraser’s score 1 (cardiac) + 5 (cerebral) + 2 (respiratory) + 2 (hepatic) + 1 (renal) = 11

For emergency treatment 8/ 21 < score> 12 / 21Score less than 8 = Not for treatment

Score more than 12 = Medical management until age over 5 months old.

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.

Team Management

• Team approach is critical to successful management

Fetal medicine

Neonatology

Pediatric cardiology

Intensive care

Neurologist

Neurosurgeon

Interventional Neuroradiology

Fetal Medicine

Interventional

Neuroradiology

Neurosurgery Neurologist

Intensive care

Peadiatric

Cardiology

Neonatology

Patient

THANK YOU