“vein of galen malformation” ppt
DESCRIPTION
case presentationTRANSCRIPT
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