Faculty Bibliography

Congenital absence of portal vein is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypass the liver and may drain directly into inferior vena cava or the left renal vein or the left hepatic vein. This rare anomaly is commonly associated with other congenital malformations and generally limited to females. We describe a rare case of aberrant portal vein development with congenital portocaval shunt (end-to-side) in a 3.5-year male child associated with cardiac defects (atrial and ventricular septal defects), skeletal deformities (flexion deformity and clinodactyly of digits and toes), and lichen planus with cafe au lait macules of skin.

A saccular aneurysm at the basilar artery bifurcation associated with a persistent primitive hypoglossal artery (PPHA) was successfully treated by endovascular occlusion with Guglielmi detachable coils. As both vertebral arteries were aplasitc, a microcatheter was advanced via PPHA. To the best of our knowledge, this is the first case report describing the treatment of a basilar top aneurysm through the PPHA.