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Cardiac Failure and PPHN due to extra cardiac cause in newborn
(Vein of Galen malformation)

Dr. Dinakar Prithviraj, Dr.ManjunathCB, Dr.Vivek Chail, Dr. Swagata M,Dr Aravind N
Neonatal ICU, Dept of Pediatrics, Dept of Radiology. Vydehi institute of medical Sciences  and research centre.Whitefield, Bengaluru Phone no : 080-28413385-89, Ext-273.
Email-  drdinakar.nishanth@gmail.com, vchail@gmail.com

A 6 hours old male child born to primi mother out of non-consanguinous marriage. Baby cried immediately after birth and APGAR score was good. Birth weight 3kgs. Noted mild respiratory distress at birth, admitted in the NICU and given supplemental Oxygen and IVFs. Gradually respiratrory distress increased and noted grunting, increasing precordial pulsations, SPO2 fluctuation and a cardiac murmur. Chest X-ray was taken and it revealed Cardiomegaly. Referred to us for further management.

On admission noted grunting, tahypnoea, sternal, subcostal and intercostal retractions, mild precordial and epigastric pulsations, HR:188/min., RR 110/min., SPO2 80-85%fluctuating,BP:40/30/38mmHg,CFT:4 secs., all peripheral pulses felt, but feeble, colour appears to be pale.With nasal prong Oxygen, SPO2 improved to 92%. On systemic examination: CVS: S1 S2 heard, loud P2 present and  systolic murmur of grade 4/6 was present. Respiratory system: respiratory distress present and bilateral air entry was equal. Abdominal examination: Right hypochondriac fullness present, liver enlarged 4 cm below the RCM. CNS examination; normal AF and irritability present, tone and reflexes were normal.

Taken chest X-ray and it revealed cardiomegaly. Echocardiography revealed severe pulmonary artery hypertension, tricuspid regurgitation, enlargement of right atrium and right ventricle,dilatation of great vessels and no structural cardiac abnormalities. Taken blood for all septic work-up. At this present clinical scenario with normal hematological findings (Hb:17.9g% ,TC:17,000/mm3, DC:P-78%,L-19%,E-3%, platlets:260000/mm3), we started nasal prong Oxygen., Digoxin, furosemide, fluid restriction and inotrope (Dopamine). Gradually over 6 hours baby started passing urine, SPO2 increased  to 96% and heart rate came down to 170/min. On 2nd day, the calculated urine output was < 1ml/kg/hr and also noted mild haematuria, jaundice (serum bilirubin Total:10mg/dl and direct: 1mg/dl), liver 5 cm below the RCM, mild  abdominal distension,HR:160-170/min, RR:80-90/min with increase indrawing,CFT:>4secs,BP:45/35/42mmHgSPO2 89-90% fluctuating,increase in precordial pulsations and colour appears to be pale. Taken second opinion for the cardiac status and the reports were same (ECHO). Placed the baby on CPAP 5cm with 80% Oxygenation and SPO2 stabilized to >95% .Continued the same treatment along with phototherapy for 2days.On day 4, the urine output was 0.9ml/kg/hr, urea:68mg/dl, creatinine:1.8mg/dl. Serum bilirubin:14mg/dl, baby’s irritability increased, perspiration noted, indrawing increased, murmur was of same intensity, liver:5.5cm below the RCM,all peripheral pulses were bounding  , neck pulsations were prominent and mild bleeding from the nasogastric tube noted . Repeat chest X-ray was taken and it revealed cardiomegaly with mild increase in pulmonary markings. With all these features we suspected extra cardiac  problem for the cardiac failure. with high index of suspicion of. A-V malformation/ aneurysm the cranium examintion revealed Bruit. Done the bedside cranial ultrasound which showed huge vein of Galen aneurysm with shifting of the right lateral ventricles without hydrocephalus. At the same time, ultrasound abdomen revealed hepatospleenomegaly, ascites and gall bladder congestion which were suggestive of congestive heart failure. For more confirmation requested MRI brain scan with Venogram which revealed huge vein of Galen aneurysm / malformation with multiple feeders (Choroidal in type). Later taken Neurosurgeon’s opinion. After collective decision about baby’s poor prognosis and financial burden for parents so decided to withdraw the treatment.

Discussion:  Vein of Galen malformation is not a true aneurysm. It is only a  artrio- venous fistula (AVF) between deep choroidal arteries and the median prosencephalic vein (MPV) of Markowski. Because of this fistula, vein of Galen is not able to form an aneurysm.It is only a pooling site(Pouch).

Abnormal connection of choroidal arteries to median prosencephalic vein (MPV) occurs at 6-11 weeks of gestation. Flow through this fistula prevents normal regression of MPV, so multiple abnormal connections will develop. It is sporadic in occurance and there is no increased risk to siblings. Male: female ratio is 2:1.It is most commonly diagnosed in the newborn period and rarely after 3 years. If diagnosed in older children or adults, it is usually compensated. It comprises less than 1% of cerebral vascular malformations at any age, but in the paediatric vascular malformation group it might account upto 30%. It is the most common extra cardiac cause of high output cardiac failure in newborns. Sometimes it may be associated with ASD(may excerbate CHF) and aortic coarctation.
Probable causes includes (assumption): a) Venous occlusion/ stenosis leading to increased flow and pressure. b) Cerebral ischemia/atrophy leading to arterial steal and /or chronic venous hypertension. c) Hydrocephalus due to decreased CSF  resorption or  cerebral aqueductal obstruction.

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Sometimes it might cause malformation of adjacent brain structures like Penial gland and 3rd ventricle. Microscopically wall of the venous pouch is thickened. The classification (based on angio-architecture):
1.Choroidal type has multiple feeders from pericallosal, choroidal and thalamo perforating arteries.
2
.Mural type: has single or few feeders from collycular or posterior choroidal arteries.

Clinically most common signs/symptoms
:
a) in Neonate: high-output cardiac failure and cranial bruit..
b)In infants and older children: Macrocrania, prominant superficial cranial veins, developmental delay, failure to thrive, hydrocephalus, seizures, headache and hepatic failure.
Imaging findings: Ultrasound:
a
)Prenatal studies can identify malformation in 2nd and 3rd trimester.Cardiac dilatation and hydrops foetalis carries poor prognosis.
b)Postnatally it is  useful for early bedside diagnosis ,provided there is large midline varix located in quadrigerminal plate cisterna. Size can vary upto several centimeters. Morphologically it might look spherical / tubular varix.

Echocardiography
: Shows dilatation of right heart, SVC and ascending aorta, severe pulmonary artery hypertension and PDA with significant R-L shunt (poor prognosis).

MRI findings
: Fetal MR can identify malformation along with absence of brain or other end organ injury (significant antenatal end organ injury is a contraindication to aggressive  treatment). MRA (magnetic resonance angiography) study is the key for pre treatment assessment of feeders to malformation (MRA is unaffected by coils or acrylic embolic material). MRV (magnetic resonance venogram) is essential in initial and follow-up  evaluation( presents and degree of venous  stenosis can have major influence on prognosis). Varix contents are hypointense and homogenous (T1 signal), sharp delineation of malformation  and prominent flow voids from feeding arteries around varix (T2 signal).

Prognosis is related to volume of shunt and timing/ success of treatment. High volume shunts requiring treatment in newborn period have worst prognosis. Delay in treatment until 4-6 months is associated with better outcome. Without treatment, progression of CHF or  brain damage  results in death. Up to 60% will be neurologically normal after treatment.

Treatment:
Intractable CHF, multi organ failure and brain damage at presentation are contraindication to treatment.

Medical therapy for CHF until 4-6 months( failure of therapy warrants earlier neuro- intervention.).

Trans
catheter embolisation (TCE) at 4-6 months is the best available treatment  at present. Permenent occlusion of fistula point from arterial side is done..May require several stages of embolisation (upto 5-7 stages).. Filling of venous pouch is less effective Frequent neurological and MRI assessment after TCE is needed. If deterioration is noted, further embolisations are needed. Treatement for hydrocephalus is controversial and is reserved for refractory hydrocephacus after all TCEs performed. Because shunt placemnent is associated  with exacerbation of venous ischaemia and there is risk of haemorrhage from enogorged subependymal veins. Over all these procedures are cost effective (3-5 lakhs).

Conclusion:
In our case, baby had high volume shunt and progressive multi organ failure, poor prognostic indicators were present, and also In view of financial aspects parents were not willing to continue the treatment and baby was discharged at request.

References:

  1. Rennie JM, Roberton NRC. CNS malformations, Textbook of Neonatology 3rd edn ; 1309.
  2. Kliegman RM, Behrman RE, Jenson HB. Diseaes of the Peripheral vascular System. Nelson Textbook of Pediatrics 18th edn; 1988-1995
  3. Abernethy LJ: Classification and imaging of vascular malformations in children. Eur Radiol 2003;13:2483-2497
  4. Friedman DM, Verma R, Madrid M et al: Recent improvement in outcome using transcatheter embolization techniques for neonatal aneurismal malformations of the vein of Galen. Pediatrics 1993;91:583-586
  5. Fong LV, Lee SH, Salmon AP: Diagnosis of cerebral arteriovenous malformations by colour Doppler examination. Eur Heart J 1992;111113:415-417.
  6. Ford EG, Stanley, Tolo V, et al: Peripheral congenital arteriovenous fistulae:Observe, operate, or obdurate? J Pediatr Surg 1992;27:714-719
  7. Lylyk P, Vineula F, Dion J E et al 1993 Therapeutic alternatives for vein of Galen vascular malformations. Journal of Neurosurgery 78: 438-445.

PRECORDIAL BULGE &

CARDIOMEGALY

TALL & WIDE P WAVES

TRICUSPID REGURGITATION


D SHAPED LEFT VENTRICLE EDEMATOUS GALL BLADDER & ASCITES


DOPPLER STUDY OF VEIN OF GALEN & MARKINGS IN U/S BRAIN

M R I PICTURE OF THE SAME


M R I VENOGRAM SHOWING MALFORMATION AND FEEDING ARTERIES

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