A Rathke cleft cyst to craniopharyngioma: is there a spectrum?
Stacey Quintero Wolfe and Roberto C. Heros
Stacey Quintero Wolfe and Roberto C. Heros
Stacey Quintero Wolfe, Sanjiv Bhatia, Barth Green and John Ragheb
✓The authors report on a 17-year-old boy with cervical myelopathy from dilated epidural veins due to cerebrospinal fluid (CSF) overdrainage. The patient had a long-standing subdural–peritoneal shunt and presented with incapacitating spastic tetraparesis. Magnetic resonance imaging revealed significant cervical spinal cord compression from a markedly dilated epidural venous plexus. The shunt was externalized so that CSF flow dynamics could be assessed, and the patient was found to have low intracranial pressure (ICP). The patient was gradually acclimated to higher ICPs, and a new shunt was placed with an antisiphon device and a programmable valve set at the higher pressure. Postoperatively the child experienced significant clinical improvement, and reduction of spinal cord compression was evident on images. Compensatory engorgement of the epidural venous plexus due to long-term shunt usage should be considered in the differential diagnosis when cervical myelopathy due to a dilated epidural venous plexus is present.
Report of three cases
Stacey Quintero Wolfe, Luisa Cervantes, Greg Olavarria, Carole Brathwaite, John Ragheb and Glenn Morrison
✓Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely in that of children. Although desmoplastic fibromas of the pediatric skull are uncommon, their similarity to benign skull lesions and their locally aggressive nature make them an important part of the differential diagnosis. Local recurrence is common after curettage alone but complete resection appears to be curative. Close follow up of incompletely resected lesions is essential. The authors detail three cases of pediatric desmoplastic fibromas of the skull and discuss diagnosis and treatment.
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.
Mohamed Samy Elhammady, Stacey Quintero Wolfe, Ramsey Ashour, Hamad Farhat, Roham Moftakhar, Baruch B. Lieber and Mohammad Ali Aziz-Sultan
The authors assessed the safety and efficacy of embolization of head, neck, and spinal tumors with Onyx and determined the correlation between tumor embolization and intraoperative blood loss.
The authors prospectively collected all head, neck, and spinal tumors embolized with Onyx at their institution over a 28-month period. Information on tumor type, location, extent of tumor devascularization, endovascular and surgical complications, and intraoperative estimated blood loss (EBL) was evaluated.
Forty-three patients with various head, neck, and spinal lesions underwent vascular tumor embolization with Onyx. Indications for embolization included uncontrolled tumor bleeding in 8 cases, elective preoperative devascularization in 34, and tumor-induced consumptive thrombocytopenia in 1 case. Embolization was performed via direct tumoral puncture in 14 cases and through the traditional transarterial route in the remaining lesions. Embolization was successful in ending uncontrolled tumor bleeding in all 8 cases and in reversing the consumptive coagulopathy in 1 case. Intraparenchymal penetration of embolic material was possible in all percutaneously embolized tumors and in 4 of the 20 tumors embolized preoperatively via the transarterial route. The mean percentage of devascularization in tumors with intraparenchymal penetration of Onyx was 90.3% compared with 83.7% in tumors without intraparenchymal penetration. The mean EBL with intraparenchymal penetration of Onyx was significantly lower than when there was no intraparenchymal penetration (459 vs 2698 ml; p = 0.0067). There were no neurological complications related to the embolization procedures.
Embolization of vascular tumors with Onyx can be performed safely but may not reach optimal effectiveness in reducing intraoperative EBL if the embolic material does not penetrate the tumor vasculature. In the authors' experience, the best method of intraparenchymal penetration is achieved with direct tumor puncture. Transarterial embolization may not result in tumor penetration, particularly when injected from a long distance through small caliber or slow flow vessels.
Mohamed Samy Elhammady, Stacey Quintero Wolfe, Hamad Farhat, Roham Moftakhar and Mohammad Ali Aziz-Sultan
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.
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.
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.
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.
Eric C. Peterson, Mohamed Samy Elhammady, Stacey Quintero-Wolfe, Timothy G. Murray and Mohammad Ali Aziz-Sultan
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.
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.
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.
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.