Trans-cranial placement of an amplatzer device to control intractable cardiac failure in an infant with a vein of galen anomaly. A case report.

Neonates presenting with intractable cardiac failure due to vein of Galen aneurysmal malformations (VGAMs) rapidly progress to multisystem organ failure and death if left untreated. Currently the only viable treatment option is endovascular embolization. Although intracranial embolization of a neonate is a high-risk procedure, successful treatment can reverse cardiac failure and prevent neurological complications associated with VGAMs. Embolization via the arterial route is thought to have a better outcome than embolization via the venous system. However, multiple transarterial embolizations in different sessions may well be contraindicated in neonates, because repeat access via the femoral artery, carries a risk of arterial trauma which, in turn, can jeopardize lower limbs. With this case study we show that after repeat failure of arterial embolization, the transcranial placement of an Amplatzer PFO occluder (AGA Medical, Plymouth, USA) in the aneurysm can effectively reduce intrafistular pressure and venous outflow velocity. We also propose a mathematical model that can be used to calculate flow velocity through the aneurysm, which, in turn, could be used to aid clinical decision-making. Unlike some conventional techniques, the placement of an Amplatzer occluder does not pose the risk of completely obstructing venous drainage and therefore does not increase the risk of venous breakthrough hemorrhage. We propose this endovascular technique as a treatment option for high risk neonates in need of emergency embolization of VGAMs, where multiple arterial embolizations failed to control the condition sufficiently.

Primary amyloidosis of urethra.

Primary localized amyloidosis of the urethra is rare; only 17 cases reported to date. Its clinical importance is significant, however, in that its presentation mimics carcinoma and treatment should be conservative. We describe an additional case and briefly review the literature.