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Mohamed Samy Elhammady, Eric C. Peterson and Mohammad Ali Aziz-Sultan

The treatment of indirect carotid cavernous fistulas (CCFs) is challenging and primarily accomplished by endovascular means utilizing a variety of embolic agents. Transvenous access to the cavernous sinus is the preferred method of embolizaiton of indirect CCFs as they are frequently associated with numerous small-caliber meningeal branches. Although the inferior petrosal sinus is the simplest, shortest, and most commonly used venous route to the cavernous sinus, the superior ophthalmic vein, superior petrosal sinus, basilar plexus, and pterygoid plexus present other endovenous options. Occasionally, however, use of these venous routes may not be possible due to vessel tortuosity or sinus thrombosis and occlusion.

The authors report a case of an indirect CCF that could not be treated endovascularly due to inability to access the cavernous sinus via a transfemoral transvenous approach. Angiography revealed a small, deeply located superior ophthalmic vein that was thought to be suboptimal for a direct cutdown. The cavernous sinus was cannulated directly via a transorbital approach using fluoroscopic guidance with a 3D skull reconstruction overlay. The fistula was subsequently obliterated using ethylene vinyl alcohol copolymer (Onyx). The technique and advantages of both 3D osseous reconstruction as well as Onyx embolization are discussed.

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Priyank Khandelwal, Nirav Patel, Alfred Pokmeng See and M. Ali Aziz-Sultan

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Ali Aziz-Sultan and Roberto C. Heros

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Mohamed Samy A. Elhammady, Hamad Farhat, Habib Ziayee and Mohammad Ali Aziz-Sultan

Carotid body tumors (CBTs) are rare highly vascular lesions that frequently require preoperative embolization to minimize surgical morbidity secondary to blood loss. Embolization has typically been performed via a transarterial route. However, this frequently results in incomplete devascularization of the tumor due to the complex angioarchitecture of the feeding arteries. Direct intralesional embolization has been used to gain easier accesses to the tumor vasculature and thus increase the likelihood of complete embolization. Cyanoacrylate glue has been the most commonly used embolic agent. The authors present a case of CBT that underwent direct intralesional embolization using Onyx (ev3; ethylene vinyl alcohol copolymer). To their knowledge, there have been no previous reports of direct percutaneous embolization of a CBT with this agent.

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Mohammad Ali Aziz-Sultan, Roham Moftakhar, Stacey Quintero Wolfe, Mohamed Samy Elhammady, Björn Herman and Hamad Farhat

Juvenile nasopharyngeal angiofibromas are vascular tumors that may make resection difficult and potentially dangerous. Preoperative embolization is frequently used to decrease surgical morbidity and blood loss. Embolization has typically been performed via a transarterial route using a variety of embolic materials. The authors present a case in which endoscopic assistance was used for direct transnasal tumor puncture and intratumoral embolization using the liquid embolic agent Onyx. In this case there was excellent infiltration of the parenchymal vasculature with complete angiographic obliteration. There were no complications related to the embolization. The tumor was resected with minimal blood loss. To the authors' knowledge, there have been no previous reports of this novel direct intratumoral embolization technique using endoscopic guidance.

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Stacey Quintero Wolfe, Hamad Farhat, Mohamed Samy Elhammady, Roham Moftakhar and Mohammad Ali Aziz-Sultan

A 2-month-old infant presented with an enlarging scalp hemangioma and consumptive coagulopathy. The patient became severely thrombocytopenic despite medical treatment. Transarterial embolization with Onyx was performed with significant reduction in the size of the tumor and complete resolution of the thrombocytopenia within 12 hours. Onyx embolization appears to be an excellent treatment option for hemangiomas presenting with Kasabach-Merritt syndrome that are unresponsive to standard medical therapy.

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Stacey Quintero Wolfe, Hamad Farhat, Roham Moftakhar, Mohamed Samy Elhammady and Mohammad Ali Aziz-Sultan

Endovascular obliteration of wide-necked aneurysms may be precluded by the inability to navigate across the aneurysm neck. The authors present a technique in which a Hyperform balloon is inflated within the aneurysm and used as a contact surface to “bounce” the remodeling balloon across the aneurysm neck. They have successfully used this technique in 3 patients to efficiently overcome vessel tortuosity, aneurysmal dead space, and balloon prolapse, allowing for obliteration of large, wide-necked aneurysms.

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Hamad I. Farhat, Mohamed Samy Elhammady, Habib Ziayee, Mohammad Ali Aziz-Sultan and Roberto C. Heros

Eagle syndrome is an uncommon entity but is well known in the otorhinolaryngology and oral surgery literature. This syndrome results from the compression of cranial nerves in the neck by an elongated styloid process causing unilateral cervical and facial pain. The styloid process can also cause compression of the cervical carotid arteries leading to the so-called carotid artery syndrome together with carotidynia or neurological symptoms due to flow reduction in these arteries.

The authors discuss the case of a 70-year-old man who suffered from transient ischemic attacks on turning his head to the left, with immediate remission of symptoms when his head returned to the neutral position. The patient was studied with dynamic angiography, which clearly showed focal flow restriction. Once a diagnosis was made, the styloid process was removed surgically and the patient completely recovered from his symptoms. A postoperative angiogram demonstrated complete resolution of the pathology.

Neurosurgeons might encounter patients with Eagle syndrome and should be aware of the symptoms and signs. Once the diagnosis is made, the treatment is clear and very effective.

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Eric C. Peterson, Mohamed Samy Elhammady, Stacey Quintero-Wolfe, Timothy G. Murray and Mohammad Ali Aziz-Sultan

Object

Retinoblastoma is the most common ocular neoplasm in children. Left untreated it spreads to the brain via the optic nerve. Traditional therapy is enucleation, and while this procedure is still the most common treatment worldwide, modern eye-preserving therapies can often spare the globe. However, patients with retinoblastoma often present in advanced stages of the disease when these globe-preserving strategies are often insufficient to prevent enucleation. In these challenging cases, direct infusion of chemotherapy into the ophthalmic artery has been attempted to achieve tumor control. The authors' aim in this study was to report on their initial experience with and clinical results for this approach.

Methods

The authors prospectively collected data on all cases of retinoblastoma treated with selective intraophthalmic melphalan at Bascom Palmer Eye Institute. All cases were classified as International Intraocular Retinoblastoma Classification (IIRC) Group D or Reese-Ellsworth Group Vb, had not responded to aggressive multimodal therapy consisting of chemotherapy and focal consolidating laser therapy, and were pending enucleation. Using digital subtraction angiography, a microcatheter was navigated under roadmap guidance into the ophthalmic artery, and melphalan was infused over 40 minutes. Early in the series, patients were treated with 3 or 5 mg of melphalan, but after low response rates occurred all eyes were treated with 7.5 mg of melphalan. All patients were examined with funduscopy while under anesthesia 3 weeks after treatment and every 3 months thereafter. Patients with persistent disease were retreated with repeat infusions of melphalan.

Results

Twenty-six procedures were performed to treat 17 tumors in 15 patients. Successful cannulation of the ophthalmic artery was achieved in all cases. The follow-up ranged from 3 to 12 months, with a mean of 8.6 months. Overall, 76% of the tumors responded to therapy and these cases were spared enucleation. The average number of treatments was 1.5 per tumor. Of the responders, 54% responded to a single dose of melphalan. Treatment with the higher dose of 7.5 mg up front was associated with a lower enucleation rate (0% vs 36%) as compared with the lower starting dose. Delayed vitreous hemorrhage occurred after 4 (15%) of 26 treatments, and these cases were treated with enucleation.

Conclusions

In this challenging group of advanced retinoblastomas refractory to aggressive multimodal therapy, virtually 100% of eyes are generally enucleated. In contrast, the authors' protocol of infusing melphalan directly into the ophthalmic artery led to a dramatic decrease in the enucleation rate to 23.5%. While it is now the treatment of choice for refractory retinoblastoma at their center, its role in less advanced disease remains to be elucidated.