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2023

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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SPINAL DURAL ARTERIOVENOUS FISTULA IN CHILDHOOD. CASE REPORT AND REVIEW OF LITERATURE

Amets Sagarribay1, Miguel Correia1, Mário Matos1, António Baptista1, Joana Ramalho3, Carla Conceição2, João Reis3.

1 - Serviço de Neurocirurgia, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E.;
2 - Serviço de Neurorradiologia, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E.;
3 - Serviço de Neurorradiologia, Hospital São José, Centro Hospitalar Lisboa Central, E.P.E.

- 23rd Congress of the European Society for Pediatric Neurosurgery, 1 a 5 Maio 2012, Amsterdão (poster).

Objective: Spinal vascular malformations are rare entities representing 2-4% of all spinal diseases. Spinal dural arteriovenous fistulas (SDAVF) are the most frequent vascular malformation accounting for 70% of all spinal vascular malformations. They affect aproximately 5-10/million/year in general population, with a male preponderance (5:1). It seems to be an acquired disease with a clinical manifestation in adulthood, with 1% of patients under 30 years of age and none reported under 20 years of age.The authors report a case in a 15 years old girl and review the literature.

Methods: Review of literature published in Pubmed/MEDLINE database, (key-words "spinal dural arteriovenous fistula" and "children") and reported a case of a SDAVF in a 15 years old girld describing the clinical presentation, imaging studies, surgical technique and results.

Results: The authors reported the case of a 15 years old girl, basketball player, that refere a severe neck pain suddenly after sport activity. It did not relieve after analgesic medication. She was admitted in our institution complaining of a light numbness on the right C6 dermatome. No neurological deficit was detected. Cervical CT scan showed an anterior acute extraxial hematoma from clivus to the T2-T3 level. MRI showed dilated peri- and intramedullary vessels at the postero-lateral T3 level and an anterior midline dilated vessel ascending to the C4 level. . The angiogram (DSA) showed a dural arteriovenous fistula with a single feeder by the right T4 segmental radicular artery and a superior draining vein. We performed a T3 to T6 laminotomy and obliteration of the fistula with a good recovery period. Six months after surgery she returned to normal sport activity as a basketball player. This is a rare case due to the hemorragic event at presentation and because, to our knowledge, there is no other reported case of SDAVF in patients under twenty years of age.

Conclusions: Spinal dural arteriovenous fistula are the most frequent spinal vascular malformations in adults accounting for 70-80% of cases af the cases. It seems to be an acquired disease and typically with a progressive clinical deterioration due to mielopathy in adulthood. There are no reported cases in childhood before and rarely as an acute hemorragic event. Surgery may be curative with a low morbility rate although there is increasing data of good results after endovascular treatment.

Key-words: spinal, dural, arteriovenous, fistula, childhood.