ABSTRACT
Objective To explore prenatal MRI findings of fetal midline dural sinus malformation(DSM).Methods Eleven fetuses of midline DSM were collected.Abnormalities in the fetal occipital or cerebellar area were found from 21 to 27 weeks of gestation.Then MR scanning in the following week was performed.Of all 11 fetuses,9 were pathologically proved to be midline DSM,and the other 2 were followed up and proved after birth.The imaging findings and pathological features were analyzed.Results MRI of 11 fetuses showed cystic dilation of the occipital cranium of torcular herophili area,with the size of 10 mm× 15 mm to 35 mm× 55 mm,and the adjacent sinuses were also dilated.On axial or coronal images,the lesions were cuneiform,while on sagittal images,the lesions were spindle-or crescent-shaped.The lesions displayed isointensity or slightly hyperintensity on T1WI,while slightly hypointensity,isointensity or slightly hyperintensity on T2WI.In 9 of the 11 fetuses,class round or bar thrombosis on the side wall of the cystic mass were observed,which were hyperintensity on T1WI and isointensity and hypointensity,isointensity or hyperintensity on T2WI.Hemosiderin deposition was observed around the thrombi in 6 fetuses.Lesion diameters in 2 fetuses were more than 30 mm,and the adjacent brain tissue was significantly dislocated.Nine fetuses of postmortem pathology after induced labor showed deformed and dilated venous sinuses,in which eccentric thrombi were seen in 7 fetuses with side branches around them.Normal brain development was observed in 2 live birth newborns.Conclusion Prenatal MRI can accurately diagnose fetal midline dural sinus malformation,and estimate the development of fetal brain,which may be helpful to prognosis prediction.
ABSTRACT
Background: Arteriovenous malformations (AVM) are rare disease in pediatric age group and dural sinus malformation (DSM) has even a lower incidence rate. DSMs are associated with a mild male dominance and onset symptoms appear around 5 months of age. The most common clinical presentation is macrocrania, seizures, psychomotor delay, intracranial hemorrhage, congestive heart failure and brain ischemia. Early recognition of these lesions is essential to prevent brain injury for ischemia and intracranial hypertension. Case description: We discuss the case of a 4 month-old boy presenting with macrocrania and signs of intracranial hypertension secondary to a transverse sinus dural arteriovenous malformation. This case was successfully treated by endovascular procedure reaching the goal of the treatment that is to obliterate the arterial portion of the fistula while preserving cerebral venous drainage to reduce the pial reflux in order to prevent venous hypertension and ischemic complications.
Contexto: As malformações arteriovenosas (MAVs) são raras na faixa etária pediátrica, e as malformações de seio dural (MSD) possuem uma taxa de incidência ainda menor. As MSDs estão associadas a uma pequena predominância no sexo masculino e os sintomas aparecem por volta dos 5 meses de idade. As apresentações clínicas mais comuns são: macrocrania, crises convulsivas, atraso no desenvolvimento neuropsicomotor, hemorragia intracraniana, insuficiência cardíaca congestiva e isquemia cerebral. O reconhecimento precoce dessas lesões é essencial para prevenir o dano cerebral por isquemia e hipertensão intracraniana. Relato do caso: Discutimos o caso de um garoto de 4 meses de idade apresentando macrocrania e sinais de hipertensão intracraniana secundários a uma malformação arteriovenosa de seio dural transverso. Este caso foi tratado com sucesso por procedimento endovascular, alcançando o objetivo do tratamento, que é ocluir a porção arterial da fístula e preservar a drenagem venosa cerebral, para reduzir o refluxo pial e assim prevenir a hipertensão venosa e possíveis complicações isquêmicas.
Subject(s)
Humans , Male , Infant , Arteriovenous Fistula , Arteriovenous Malformations , Transverse SinusesABSTRACT
Dural sinus malformation (DSM) with arteriovenous fistula (AVF) is very rare in pediatric group. We experienced one case of DSM and treated AVF by endovascular embolization. Herein we report this rare case occurred in infant.