The Holistic Nursing Care of A Child With Vein of Galen Aneurysm

Francesca White

Practice Facilitator for Neuroscience

Aims and Objectives

To discuss the presentation of Vein of Galen Malformation

To discuss the pathway and nursing care currently delivered at Great Ormond Street Children’s Hospital

Discuss Trust specific statistics and outcomes

Discuss recent research at GOSH

What is a Vein of Galen Malformation?

• A congenital vascular malformation

• Two Types- Choroidal & Mural

Hoang et al (2009)

Anatomy & Physiology • The congenital malformation develops during weeks

6-11 of foetal development

• In the median prosencephalic vein of Markowski

(embryonic structure that precedes the Vein of Galen)

Geibprasert et al 2010

Presentation

Neonate

• High cardiac output failure

• Abnormal flow CSF

Late presentation/infancy

• Intracranial haemorrhage

• Focal neurological deficits

• Psychomotor delay

• Increase head circumference

Cardiac PresentationVenous return to the right atrium

Pulmonary blood flow & Blood pressure= Congestive heart failure

Blood flow in arteries = Myocardial ischaemic& Renal hypoperfusion

Does not produce cardiac failure until after the child is born

Hoang et al (2009)

Hydrocephalus Presentation

Venous pressure & lack of maturation of the arachnoid granulation=

Impaired CSF reabsorption

Obstructive Hydrocephalus- compression of the aqueduct

Hoang et al (2009)

Angiogram Embolisation

Case study 1

Neonatal Presentation Presentation-

Antenatal diagnosis on ultrasoundSinus venous defect ASD with severe RHVO

Treatment-4 embolisation- Successful treatment

Cardiac surgical at 2 1/2 years- Successful Complications

Had recurrent chest infection prior to cardiac surgical/not gaining weight

Long term outcomeNo active neurological issues

Case study 23 year old-Late Presentation

Presentation-R sided weakness, Vomiting & seizures

Treatment- 3xEmbolisation Complications:

Thrombus formation in R Sigmoid sinus-Ischaemic damage & Intra-ventricular

haemorrhages Long term outcome

Behavioural problems

2005-current

• Total 122

• Intact 58

• Impaired 30

• Dead 34

47%

25%

28%

2005-2016 Outcomes from treatment of Vein of Galen malformation

Intact

Impaired

Dead

Comparison

Lasjunias et al 2006- 233 patients

• Mortality rate- 10.6% (12/23 deaths in neonates) Most deaths were due to not treating.

• 74% Neurologically normal

Geibprasert et al 2010- 25 patients

• Mortality rate - 36%

• 36% Neurologically normal (Intact)

• 28% neurological deficits

Outcomes

Research project

• Prediction for Outcomes for 5, 10, 20 years.

• Cognitive development- using pictures and Puzzles

• Recruitment 40/54 (Only presentation at 28 weeks)

• Links with Autism (treatment versus abnormal brain)

RASA gene link

• We have two cases

• Boston has cases

• We test for all patients

Conclusion

• Present with cardiac issues/ seizures/ hydrocephalus

• Treatment- embolisation

• High risk of morbidity and mortality

• Similar outcomes to other centres

• Possible link to autism

• Possible genetic link

Thank you

Any Questions?

References

• Bhattacharya JJ & Thammaroj J (2003) Vein of Galen Malformation Journal of Neural Neurosurgical psychiatry 74 pp42-44

• Geibprasert, S., Krings, T. Armstrong, D., Karel, G., Raybaud, C. (2010) Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations Childs Nervous System 26 pp35-46

• Ahmad, M., Mir S., Wahab, S., Rizvi, I. (2012) Antenatal diagnosis of vein of Galen aneurysm - case report BMJ case reports doi:10.1136/bcr-2012-006822

• Hoang, S., Choudhri, O, M.D., Edwards M., & Guzmand, M.D. (2009) Vein of Galen MalformationNeurosurgical focus 27 (5)

• Lasjuanias, PL., Chng, SM., Sachet, M., Alvarez, H., Rodesch G., Gracia-Monaco, R. (2006) The management of vein of Galen aneurysmal malformation Neurosurgical 59 pp 84-94.

• Jea A1, Bradshaw TJ, Whitehead WE, Curry DJ, Dauser RC, Luerssen TG (2010) The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatric Neurosurgical 46(2):141-5. doi: 10.1159/000319399.

• GOSH neurovascular team- Claire Toolis, Fergus Robertson, Adam Rennie, Sanjay Bhate