imagem top

2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

CHULC LOGOlogo HDElogo anuario

HEMORRHAGIC CAVERNOUS MALFORMATIONS IN CHILDREN YOUNGER THAN 3 YEARS – AN INCREASED RISK?

Pedro Branco, Lino Fonseca, Gonçalo Novais, Dalila Forte, Amets Sagarribay, Miguel Correia, Mário Matos.

Pediatric Neurosurgical Unit, Hospital Dona Estefânia. Centro Hospitalar de Lisboa Central-EPE

- 25th European Society for Pediatric Neurosurgery (ESPN), 8-11 May 2016 (poster)

Introduction – Non traumatic intracerebral hemorrhage corresponds to 50% of all strokes in children. Up to 57% of these hemorrhages are associated with vascular malformations, of which 10% are due to cavernomas. It is estimated that 0,05-0,15 hemorrhages /100.00 children every year are caused by cavernous malformations. Mixed adult and pediatric population studies estimate the hemorrhagic risk in 1.3-3,1% per year, increasing to 23% during the first year after bleeding.
Methods - Retrospective analysis of all consecutive cases of intracerebral hemorrhages associated with cavernous malformation in children younger than 3 years admitted to our center between 2009 and 2014. A revision of published literature was made.
Results – Two cases were identified. Both presented with a hemorrhagic event at 2 years old, with a second hemorrhage identified imagiologically in less than 2 month. One presented with a cavernous malformation on the right motor cortex area with mild contralateral hemiparesis. The second case had a cavernoma on the inferior right cerebellar peduncle, and presented with gait instability torticollis and vomiting. Both were operated with complete resection of the lesion
Discussion – The natural history of cavernous malformations in children is not completely understood but it appears that it has an increased hemorrhagic risk comparing to the adult population. Fetal and neonates are more at risk but the risk’s variation with increasing age has not been evaluated. The two presented cases show that the adult’s determined risk cannot be applied in pediatric population. The presence of rebleading in both cases may represent a bleeding risk similar to the one described in fetal and neonate’s cavernomas. The exact mechanism that makes this population more susceptible to a hemorrhagic event still is unknown.
Conclusion – The increase hemorrhagic risk of cavernous malformations described in nenonates may persist until latter ages, by unknown mechanisms. A better understanding of the pathology’s natural history is fundamental for a better guidance and treatment decision making in this population.

Palavras-chave: cavernous malformations,