Coil and Onyx embolization of a torcular herophili dural arteriovenous fistula in a full-term neonate with advanced heart failure using a transumbilical approach

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Neonatal dural arteriovenous fistulas (DAVFs) are rare, but if left untreated will advance to life-threatening neurological and cardiovascular compromise. Endovascular treatment is the preferred treatment modality for DAVFs. The goal of endovascular therapy is to obliterate feeding vasculature and prevent secondary complications. Endovascular access can be difficult to obtain in a neonate. The authors present the case of a full-term, normal birth weight neonate with severe congestive heart failure secondary to a congenital DAVF of the torcular herophili that was successfully treated with transumbilical arterial coil embolization and a liquid embolic agent.

ABBREVIATIONS DAVF = dural arteriovenous fistula; ECA = external carotid artery; MCA = middle cerebral artery; MMA = middle meningeal artery; MRA = MR angiography; NBCA = N-butyl-2 cyanoacrylate.

Article Information

Correspondence George W. Koutsouras: New York Institute of Technology, College of Osteopathic Medicine, Old Westbury, NY.

INCLUDE WHEN CITING Published online October 5, 2018; DOI: 10.3171/2018.6.PEDS1819.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    Time-of-flight MRA of a patient with a large torcular herophili DAVF on the day of birth. A: Sagittal view showing the partially thrombosed torcula filling with arterialized blood (solid arrow). Hypertrophied right MMA is the predominant arterial feeder (arrowhead). The right occipital artery also has a significant contribution (open arrow). B: Coronal view, once again showing the partially filling torcula (solid arrow) and the predominant MMA feeder (arrowhead). C: Axial view showing the partially thrombosed torcula (solid arrow), with feeding MMA (arrowhead) and deeper feeders originating from the internal carotid artery (star). Figure is available in color online only.

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    Transumbilical cerebral angiogram of the patient at 16 days old. A: Anteroposterior view of the early arterial phase of the right common carotid artery injection prior to treatment showing arteriovenous shunting into the torcula (arrow) from a tortuous and hypertrophied MMA (arrowhead). B: Lateral view of the same injection once again showing the MMA (arrowhead) filling the torcula (solid arrow) as well as an occipital artery feeder (open arrow). C: Anteroposterior view of the early arterial phase of right common carotid artery injection after combined coil and Onyx embolization of the MMA showing complete obliteration of flow (arrowhead). The torcular malformation shows diminished filling (arrow). D: Lateral view of the same injection showing complete obliteration of the MMA (arrowhead), diminished filling of the torcula (solid arrow), and persistent shunting through the hypertrophied occipital artery (open arrow). Figure is available in color online only.

  • View in gallery

    Transumbilical cerebral angiogram of the patient at 18 days old. A: Anteroposterior view of the early arterial phase of right common carotid injection after combined coil and Onyx embolization of the right occipital artery (solid arrow) and deep unnamed feeder (open arrow) from the ECA. The previous MMA occlusion (arrowhead) remains obliterated. B: Lateral view of the same injection showing the stable MMA occlusion (arrowhead), no filling of the torcula (solid arrow), and obliterated deep feeder (open arrow). Figure is available in color online only.

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    A: Axial time-of-flight MRA of the patient at 10 months old. There is now a left occipital artery feeder (open arrow) showing some filling of the torcular malformation. The right-sided feeders (solid arrow) remained completely obliterated. B: Coronal magnetization prepared rapid gradient echo image of the patient at 10 months showing generalized loss of cortex and ventriculomegaly on the left side (arrow), believed to be due to chronic steal phenomena. Figure is available in color online only.



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