Fistula dural tipo adulta en un lactante: Reporte del caso y revisión de la bibliografía / Adult type dural A-V fistula in an infant: Report of a case and bibliographical review

Introduction. Dural arteriovenous malformations are rare in children. They constitute a group of entities with different origin, pathophysiology and prognosis. Case report. A 9 month old infant was brought to consultation because of cranial bruit in the left retroauricular area and opisthotonus crisis. CAT scan of the brain showed modest enlargement of ventricles and widening of the subarachnoid space. Cerebral angiography depicted an adult type dural arteriovenous fistula and thrombosis of the left transverse sinus. Angiographic cure was achieved by transarterial embolization of the fistula. The clinical condition of the patient improved significantly. Discussion. Dural arteriovenous fistulae (DAVS) constitute an heterogeneous group of disorders with an overall mortality of 38%. They have been classified by Lasjaunias in three categories: 1- Dural sinus malformations with DAVS, 2- Infantile type DAVS, and 3- Adult type DAVS. Clinical manifestations depend on the extent of the disorder and the magnitude of venous hypertension. Neonates may present with congestive heart failure (CHF). Infants and older children often show cranial bruits associated with macrocrania, seizures and developmental delay of variable severity. Conclussion. DAVS are unusual in the pediatric population. Early diagnosis and treatment are of major importance, in order to control venous hypertension and avoid the development of irreversible brain damage. Endovascular therapy has an important role in the acute treatment of CHF, and seems to be the best choice for treatment, either curative or palliative.

Diastematomielia asociada a otras malformaciones raquimedulares / Diastematomyelia associated with others rachymedullar malformations

La diastematomielia es una forma rara de disrafia espinal (menos del tres por ciento de los casos con disrafismo espinal oculto), más frecuente en el sexo femenino y en los niños. Clínicamente se manifiesta por tres grupos de sínfromes: alteraciones cutáneas, deformidades ortopédicas y síntomas o signos de disfunción neurológica, La asociación con otras malformaciónes raquimedulares está claramente documentada. Presentamos nuestra experiencia de los últimos treinta y seis meses con seis casos de diastomatomielia, de las cuales cuatro se presentaron asociadas a otra patología malformativa raquimedular. A pesar de ser una entidad poco frecuente debe tenerse presente a la hora de la resolución quirúrgica de las distintas malformaciones raquimedulares ya que tratada oportunamente la presencia de diastematomielia no empeora el pronóstico de la patología de base.