A Case of Congenital Supratentorial Dural Arteriovenous Fistulas with Disseminated Cutaneous Hemangiomatosis and Skull Hemangiomas

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A case of congenital dural arteriovenous fistulas with disseminated cutaneous hemangiomatosis and four skull hemangiomas is presented.<BR>This boy was admitted to the hospital because of cutaneous hemangiomas and enlarged head at the age of 3 days. The course of pregnancy and delivery was uneventful. There was no family history of any specific diseases. The head circumference was 38.0cm. Cutaneous hemangiomas sized 2-5 mm were noted on his body and extremities.<BR>Skull x-ray examination disclosed four bony tumors at the bilateral parietal, the occipital and the frontal bones. The bony masses were partially radiolucent and partially sclerotic with parallel bone spicula. A retrograde venous angiogram revealed the tortuous vascular network at the bony tumors, and the radioactive technetium brain scan showed a large amount of uptake. The right brachial angiogram at the age of 2 months disclosed the enlarged middle meningeal and the occipital arteries with abnormal venous circula tion suggesting dural arteriovenous fistulas which was confirmed afterwards at the age of six. No abnormality was found by scintigram of visceral organs, ophthalmoscopy and blood examination.<BR>He was well with no severe disorder of mental and somatic development, after ventriculo-peritoneal shunt was performed because of obstructed hydrocephalus at the age of 7 months.<BR>The bilateral carotid angiogram at the age of 6 revealed the direct communications between the bilateral middle meningeal and the right occipital arteries and the superior sagittal sinus enlarged spherically superior to the confluence. The superior sagittal sinus was not opacified posterior to the shunting site. The contrast medium flowed anteriorly in the superior sagittal sinus. The enlarged tortuous superior cerebral veins drained inferiorly toward the superficial middle cerebral veins. The insular veins were dilated and drained to the basal sinuses.<BR>Dural arteriovenous fistulas are well recognized lesions now, but the cases of purely supratentorial involvement are not so common. The combination of arteriovenous fistulas and obstruction of the superior sagittal sinus is of great interest. Several kinds of association of cutaneous angiomatosis with central nervous lesions are well known, but the reports similar to the present case can not be found in the literature. There was no special family history suggesting the heredity. The early onset and the combination with other vascular lesions suggest one causative factor at a given embryonic stage. This is noteworthy to consider the etiology of the dural arteriovenous fistulas.
一般社団法人日本小児神経学会の論文

一般社団法人日本小児神経学会 | 論文

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