FIGURE 10.

Orbital Doppler ultrasonography in the Sturge-Weber syndrome. Scans confirm the slowed and occasionally reversed venous drainage within affected orbits both in patients with isolated facial port-wine stains and in those with the Sturge-Weber syndrome. The color red is assigned to blood flowing away from the L 12-5 transducer (assumed by the device to be arterial), and blue is assigned to blood flowing toward the transducer (assumed by the device to be venous). Top left, blood flow is measured by the cursor of the Doppler ultrasound device (Phillips 5000, Bothell, Washington) placed over the superior ophthalmic vein of the affected right eye and is found to be −3.6 cm/sec, compared to −6.5 cm/sec for the unaffected left eye (top right). The flat waveform for the right eye, moreover, indicates elevated intraluminal pressure preventing a normal pulsatility as noted in the left eye (top right) produced by variations in orbital pressure caused by increased arterial blood flow during systole. Similar findings are noted in other patients (middle left) with nonpulsatile superior ophthalmic vein flow of −6.31 cm/sec in the affected right orbit, but highly pulsatile with peak flow velocities up to −21.6 cm/sec in the unaffected left orbit (middle right). Bottom (patient 20, Table, also seen in Figures 2 and 16), blood flow within the left superior ophthalmic vein could not initially be detected. Once the patient performed a Valsalva maneuver, however, the blood flow within the vessel suddenly became apparent (bottom left), but with blood flowing away from the cavernous sinus toward the face, with a velocity of +7.5 cm/sec and erroneously encoded as arterial (red) by the device (anterograde venous blood flow). After the patient released her breath (bottom right), venous blood flow quickly ceased and could no longer be visualized, with neither red nor blue coding assigned, indicating venous stasis. (Captured images courtesy of M. Robert De Jong, RDCS, RDMS, RVT, The Johns Hopkins Hospital, Baltimore, MD.)