Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications.

BACKGROUND: Up to now, only little is known about hydrocephalus (HC) in vein of Galen malformation (VGM). We want to present the different etiologies and our long-term experience (1992-2015) in the management of HC. METHODS: Out of 44 treated children with VGM, we retrospectively reviewed all cases with HC. We analyzed the etiologies, our treatment results and complications. RESULTS: Twenty-one children (48 %) presented either with HC or developed it over time. In 21 % of those cases, high venous pressure was presumably the sole cause. Until 2009, seven of them received ventriculoperitoneal (VP) shunting; six of those resulted in severe postoperative complications. The remaining children have been treated successfully by endovascular embolization. Five out of the 44 children (11 %) developed HC after intraventricular hemorrhage. In four cases, those children were treated with positive results by using transient external ventricular drainages. In one case a VP shunt with highest valve pressure was inserted. Another four children (9 %) presented with aqueductal stenosis-related HC caused by either dilated venous outflow or space-occupying coil masses after embolization. The latter case was successfully treated by ventriculocisternostomy, whereas endovascular treatment decreased the venous outflow in size and thus resolved the HC in the other cases. In the remaining cases (7 %), atrophy due to melting brain syndrome led to HC ex vacuo. CONCLUSIONS: HC in VGM is a common phenomenon with several etiologies requiring different treatments. In most cases, embolization of the VGM as sole treatment is completely sufficient in order to decrease high venous pressure. However, certain other causes of HC should be treated in an interdisciplinary setting by specialized neurosurgeons.

The transmaxillary endoscopic approach to the orbit.

OBJECTIVE: In this surgical-anatomical cadaveric study we investigate the feasibility of the transmaxillary endoscopic approach to the intraorbital space. Anatomical landmarks are defined, the endoscopic view in the orbital space is studied and complications that can occur are discussed. METHODS: Nine formalin-fixed heads were used to study the transmaxillary endoscopic approach to the orbit. The approach was used twice on each head (once for each maxilla). Therefore, we report our results on 18 transmaxillary intraorbital approaches. For better differentiation of anatomical structures, the veins and arteries were injected with blue and red plastic respectively in six cadaveric heads. RESULTS: The transmaxillary approach enables viewing the inferior intraconal structures without endangering the infraorbital nerve and its artery and without diversion of the inferior rectus muscle. The optic nerve was visualised more easily through the approach medial to the inferior rectus muscle instead of lateral to the muscle since the ciliary nerves are in the way in the lateral approach. The combination of the approaches medial and lateral to the inferior rectus muscle allows very good identification of all important anatomical structures in the inferior intraconal space. CONCLUSION: The transmaxillary endoscopic approach to the orbit is a useful new approach in the surgical armamentarium for orbital lesions. The overview of the inferior part of the orbit is excellent, and the lateral part of the optic nerve can be visualised. Careful anatomical dissection allows visualisation of important anatomical structures in the orbit without damaging nerves or arteries.