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2018

ANUÁRIO DO HOSPITAL DONA ESTEFÂNIA
REPOSITÓRIO MÉDICO CIENTÍFICO

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PAEDIATRIC NEUROVASCULAR CONSULTATION IN SICKLE CELL DISEASE

Rita Lopes Silva1, Manuel Manita2, Carla Conceição3, Raquel Maia4, Paula Kjollerstrom4

1- Pediatric Neurology Unit, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisboa;
2- Neurosonology Unit, Hospital S José, Centro Hospitalar Lisboa Central, Lisboa;
3- Neuroradiology Unit, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisboa;
4- Haematology Unit, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, Lisboa

- XXVI Congresso Brasileiro de Neurologia, 9-12 Nov 2014, Curitiba (comunicação oral)

Introduction: Cerebrovascular disease strongly contributes to mortality and morbidity in Sickle Cell Disease (SCD) patients. Since the STOP Study (published in 2008) systematic transcranial doppler (TCD) is recommended in order to stratify stroke risk and implement regular blood transfusions if elevated blood flow velocities are present. Our Hospital is a SCD reference center and a Pediatric Neurovascular Consultation was created in articulation with the Hematology and the Neurosonology Units to improve the neurological care of these children and adolescents.
Objectives: To evaluate the neurological surveillance and stroke prevention programme of the paediatric SCD patients followed in our Hospital.
Materials and methods: Data were collected from SCD children/adolescents followed in the Haematology Unit with at least one appointment since January 1st 2013. Variables analysed were age, gender, TCD and MRI findings, formal neurological evaluation and the occurrence of stroke, learning disability and headache in this population.
Results: The Paediatric Haematology Unit currently follows 96 patients with SCD (91% HbSS), 55% male, 64% between 7 and 18 years and 29% between 2 and 7 years old. Positive stroke history was present in 4 (4%) patients, occurred from 3,5 to 12 years of age, mainly in the middle cerebral artery territory and with recurrence in one patient. Of the 91 patients older than 2 years of age, 88% had performed TCD according to the International Guidelines, 94% with normal blood flow velocities and 2,5% with pathologically elevated values. MRI was performed in only 25 (26%) patients and 52% of them had pathological findings, namely 2 with intracranial arterial stenosis, 5 with isolated silent infarcts and 4 with stroke (2 with silent infarcts and 2 also with moyamoya pattern). A formal Paediatric Neurology evaluation was obtained in 55 (57%) children/adolescents with SCD. Headaches were present in 23 (41%) and learning disability in 19 (41%).
Conclusions: Our SCD patients are followed according to the International Guidelines, 88% of them had performed TCD regularly and 4% have a positive history of stroke. The Paediatric Neurovascular Consultation had an important role, promoting the communication between the Haematology and Neurosonology Units, preventing cerebrovascular disease.

Palavras Chave: Doença de Células Falciformes, neurovascular, AVC