PMC5778556_01
(a) Clinical picture of patient at presentation. (b) Intraoperative photograph showing removal of the lesion with minimal bleeding. (c) Postoperative appearance of the patient
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F1
A 6-year-old girl presented to orbit clinic with a diffuse progressive soft compressible pulsatile mass lesion in the medial aspect of the right upper eyelid since birth, causing total mechanical ptosis [Fig. 1a]. Subsequently, surgical excision was performed after 72 h [Fig. 1b]. The functional and cosmetic outcome was satisfactory [Fig. 1c].
(a) Axial plain computerized tomography scan of the patient. (b) Contrast-enhanced computerized tomography shows intense enhancement in the arterial phase. (c) Rapid washout in the venous phase confirming diagnosis of arteriovenous malformation. (d) Coronal magnetic resonance imaging scan showing soft-tissue lesion in the right orbital region extending up to midline. (e) Axial magnetic resonance imaging showing multiple flow voids within the lesion
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F2
Computerized tomography (CT) scans revealed a diffuse preseptal isodense mass in the superonasal aspect of the right upper eyelid, which enhanced brightly during the arterial phase of contrast [Fig. 2a-c]. T1-weighted and mixed signal in T2-weighted images with multiple flow void signals [Fig. 2d and e]. The right superior ophthalmic vein was dilated.
(a) Right common carotid angiogram showing nidus of the right orbital arteriovenous malformation supplied by the right ophthalmic artery as well as external carotid artery branches. (b) Right external carotid angiogram showing nidus supplied by branches of the right external carotid artery, namely, superficial temporal, internal maxillary, and facial artery. (c) Direct puncture of nidus by 22-g needle and injection of glue. (d) Microcatheter in the right ophthalmic artery and embolization of nidus with glue. (e) Lateral view of the skull showing cast of glue after embolization. (f) Postembolization right external carotid angiogram showing residual nidus
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F3
Digital subtraction angiography (DSA) disclosed a compact nidus of AVM with feeder vessels from the right ophthalmic, middle meningeal, internal maxillary, superficial temporal, and bilateral facial arteries [Fig. 3a and b]. Eighty percent of the AVM was embolized using 16% glue (N-butyl 2-cyanoacrylate) by superselective cannulation of the right ophthalmic artery feeder through the right femoral artery as well as direct intralesional glue injection under fluoroscopy under general anesthesia [Fig. 3c-f].
Microphotograph of the excised lesion showing vascular malformation composed of numerous tiny and large vascular spaces lined with endothelial cells and filled with blood (H and E, x100)
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F4
The nidus was seen as cellular stroma in between the vessels, confirming the diagnosis of AVM [Fig. 4].