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  • Author or Editor: M. Aziz-Sultan x
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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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Seth B. Hayes, Ronald J. Benveniste, Jacques J. Morcos, Mohammad A. Aziz-Sultan and Mohamed Samy Elhammady

Surgical evacuation of nontraumatic, supratentorial intracerebral hemorrhage (SICH) is uncommonly performed, and outcomes are generally poor. On the basis of published experimental data and the authors' anecdotal observations, a retrospective chart review study was performed to test the hypothesis that large decompressive craniectomies (DCs), compared with craniotomies, would improve clinical outcomes after surgical evacuation of SICH. For patients with putaminal SICH, DC was associated with a statistically significant improvement in midline shift, compared with craniotomy. Decompressive craniectomies also resulted in a strong trend toward decreased likelihood of poor neurological outcome (modified Rankin Scale score > 3). For patients with lobar SICH, no associations were found between DC or craniotomy and clinical outcomes. For patients selected to undergo surgical evacuation of putaminal SICH, a DC in addition to surgical evacuation of the hematoma might improve outcome.

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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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Mohamed Samy Elhammady, Hamad Farhat, Mohammad Ali Aziz-Sultan and Jacques J. Morcos

Juxtafacet cysts of the atlantooccipital joint that present with isolated hypoglossal nerve palsy are rare and may mimic more common pathological entities. The authors report on the third such case in the literature and discuss the differential diagnosis, imaging hallmarks, preoperative recognition, and surgical management of this lesion, and provide a review of the literature.

The authors discuss their experience with the treatment of a 67-year-old woman who presented with an isolated hypoglossal nerve palsy caused by a nonenhancing cystic septated lesion abutting the lateral medulla just medial to the left hypoglossal canal. The lesion was presumed to be a necrotic hypoglossal schwannoma or epidermoid tumor. Intradural surgical exploration failed to demonstrate an intradural lesion, but confirmed the presence of an extradural mass caudal to the hypoglossal nerve. Extradural exploration revealed a synovial cyst of the atlantooccipital joint, which was then resected. Postoperatively, the patient developed worsening dysphagia and hoarseness. Failure to recognize this rare entity preoperatively resulted in unnecessary intradural exploration and cranial nerve morbidity. In retrospect, the preoperative diagnosis of this lesion was suggested by lack of central enhancement, absence of dumbbell formation and the presence of erosive synovial changes. Regardless, the extreme rarity of this lesion at this location will always make its recognition challenging.

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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.

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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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Brandon G. Gaynor, Mohamed Samy Elhammady, Daniel Jethanamest, Simon I. Angeli and Mohammad A. Aziz-Sultan

Object

The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.).

Methods

The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded.

Results

Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction).

Conclusions

Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.

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

Object

The authors conducted a study to determine the safety and efficacy of embolization of carotid-cavernous fistulas (CCFs) with the ethylene vinyl alcohol copolymer, Onyx.

Methods

They prospectively collected data in all patients with CCFs who underwent Onyx-based embolization at their institution over a 3-year period. The type of fistula, route of embolization, viscosity of Onyx, additional use of coils, extent of embolization, procedural complications, and clinical follow-up were recorded.

Results

A total of 12 patients (5 men and 7 women who were age 24–88 years) underwent embolization in which Onyx was used. There were 1 Barrow Type A, 1 Type B, 3 Type C, and 7 Type D fistulas. Embolization was performed via a transvenous route in 8 cases and a transarterial route in 4 cases. Onyx 34 was used in all but 2 cases: a direct Type A fistula embolized with Onyx 500 and an indirect Type C fistula embolized with Onyx 18. Adjuvant embolization with framing coils was performed in 7 cases. All procedures were completed in a single session. Immediate fistula obliteration was achieved in all cases. Clinical resolution of presenting symptoms occurred in 100% of the patients by 2 months. Neurological complications occurred in 3 patients. One patient developed a complete cranial nerve (CN) VII palsy that has not resolved. Two patients developed transient neuropathies—1 a Horner syndrome and partial CN VI palsy, and 1 a complete CN III and partial CN V palsy. Radiographic follow-up (mean 16 months, range 4–35 months) was available in 6 patients with complete resolution of the lesion in all.

Conclusions

Onyx is a liquid embolic agent that is effective in the treatment of CCFs but not without hazards. Postembolization cavernous sinus thrombosis and swelling may result in transient compressive cranial neuropathies. The inherent gradual polymerization properties of Onyx allow for casting of the cavernous sinus but may potentially result in deep penetration within arterial collaterals that can cause CN ischemia/infarction. Although not proven, the angiotoxic effects of dimethyl sulfoxide may also play a role in postembolization CN deficits.