Stacey Quintero Wolfe

Supraselective intra-arterial chemotherapy: evaluation of treatment-related complications in advanced retinoblastoma.

Purpose: The purpose of this study is to report the complication profile and safety evaluation of supraselective intra-arterial melphalan chemotherapy in children undergoing treatment with advanced retinoblastoma. Methods: Twelve eyes of 10 children with advanced retinoblastoma (Reese-Ellsworth Group Vb or International Classification Group D) were treated with supraselective intra-ophthalmic artery infusion of melphalan. Eleven eyes of nine children had previously failed traditional management with systemic chemotherapy and laser ablation and underwent intra-ophthalmic artery infusion of melphalan as an alternative to enucleation. Serial ophthalmic examinations, retinal photography, and ultrasonographic imaging were used to evaluate treatment regime. Results: Ophthalmic artery cannulation was successfully performed in 12 eyes of 10 patients (total 16 times). Striking regression of tumor, subretinal and vitreous seeds were seen early in each case. No severe systemic side effects occurred. Grade III neutropenia was seen in one patient. No transfusions were required. Three patients developed a vitreous hemorrhage obscuring tumor visualization. One patient developed periocular edema associated with inferior rectus muscle inflammation per orbital MRI. This same patient had scattered intraretinal hemorrhages and peripapillary cotton wool spots consistent with a Purtscher’s-like retinopathy that resolved spontaneously. At the 6-month follow-up examination, nine eyes had no evidence of tumor progression, whereas three eyes were enucleated for tumor progression. In each enucleated case, viable tumor was identified on histopathologic examination. Conclusions: Ophthalmic intra-arterial infusion with melphalan is an excellent globe-conserving treatment option in advanced retinoblastoma cases with minimal systemic side effects. Local toxicities include microemboli to the retina and choroid (1/12, 8%), vitreous hemorrhage (3/12, 25%), and myositis (1/12, 8%). Enucleation remained a definitive treatment for tumor progression in 3 of 12 eyes in this small case series with limited follow-up. Further studies are necessary to establish the role of supraselective intra-arterial melphalan chemotherapy for children with retinoblastoma.

Safety and efficacy of vascular tumor embolization using Onyx: is angiographic devascularization sufficient?

OBJECTnThe 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.nnnMETHODSnThe 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.nnnRESULTSnForty-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.nnnCONCLUSIONSnEmbolization 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.

Onyx embolization of carotid-cavernous fistulas

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

Pituicytoma: case report.

OBJECTIVEnPituicytoma is a rare but distinct low-grade glioma of the neurohypophysis. To date, we have found only 28 cases reported in the literature and have reviewed these in addition to our own case to delineate the clinical implications of this relatively recently defined tumor.nnnCLINICAL PRESENTATIONnA 71-year-old woman presented with bitemporal hemianopsia and was found to have a large sellar tumor, resembling a pituitary adenoma. Transsphenoidal resection was attempted but was complicated by significant tumor vascularity. Pathology at that time was interpreted as a granular cell tumor. The patient was then referred to our institution for management of the residual tumor.nnnINTERVENTIONnThe patient underwent preoperative embolization to decrease tumor vascularity, and subtotal tumor resection was performed via a craniofacial approach. Postoperatively, the patient has enjoyed significantly improved visual fields despite residual tumor. No adjuvant treatment was given. There has been no regrowth of the residual tumor, as shown on magnetic resonance imaging, over a follow-up period of 1.5 years. The final pathological diagnosis was pituicytoma.nnnCONCLUSIONnPituicytomas are benign, slow-growing tumors that seem to be cured by gross total resection. The role of radiation therapy is controversial. We advocate an aggressive surgical approach with possible preoperative embolization to reduce the vascularity of the tumor.

Carotid artery sacrifice for unclippable and uncoilable aneurysms: endovascular occlusion vs common carotid artery ligation.

BACKGROUND:Optimal treatment of intracranial aneurysms involves complete occlusion of the aneurysm with preservation of the parent artery and all of its branches. Attempts to occlude the aneurysm and preserve the parent artery may be associated with a higher level of risk than parent vessel occlusion or trapping. OBJECTIVE:To evaluate our series of patients with large and giant aneurysms who underwent treatment via endovascular coiling with parent artery sacrifice or surgical ligation of the common carotid artery (CCA) and gain insight into the advantages and risks of each of these alternatives. METHODS:We retrospectively reviewed all patients with aneurysms who underwent carotid sacrifice via endovascular occlusion or surgical CCA ligation during an 8-year period at our institution. RESULTS:Twenty-seven patients with large and giant aneurysms of the internal carotid artery underwent carotid artery sacrifice via endovascular occlusion (n = 15) or CCA ligation (n = 12). Of the patients who underwent endovascular occlusion, 3 developed groin complications, 1 developed a new sixth nerve palsy, 1 died from vasospasm related to subarachnoid hemorrhage, and 1 died secondary to rupture of an associated 3-mm anterior communicating artery aneurysm 5 days postoperatively. Of the patients undergoing CCA ligation, 1 patient developed a partial hypoglossal palsy. Clinical improvement of presenting symptoms was observed in all surviving patients regardless of the method of treatment. Complete aneurysm obliteration was documented in all patients during the initial hospital stay. The mean radiographic long-term follow-up was 14.2 months, which was available in 20 of the 25 surviving patients (80%). Complete obliteration was confirmed at follow-up in all but 2 patients with large cavernous aneurysms; 1 was initially treated with endovascular occlusion and the other with carotid ligation. CONCLUSION:Parent artery sacrifice is still a viable treatment for some complex aneurysms of the internal carotid artery. CCA ligation is a reasonable alternative to endovascular arterial sacrifice.

Clinicopathologic review of enucleated eyes after intra-arterial chemotherapy with melphalan for advanced retinoblastoma.

change if the air had direct access to the venous circulatory system. The clinical situation is further complicated by a patent foramen ovale (which is present in 23%-45% of adults, based on autopsy studies). A patent foramen ovale allows trapped air to access the arterial circulatory system through the opening between the right and left atrium. This can result in air emboli throughout the systemic arterial circulatory system, causing cerebral, cardiac, and visceral infarction, as occurred in the second case. Treatment of VAE consists of immediately flooding the operative field with fluid to stop entry of the air. Additional measures would be at the discretion of the anesthesiologist and include increasing inspired oxygen, discontinuing nitrous oxide, if it is being used, inotropic support, and aspiration of air if a right atrial catheter has been placed. Although this complication is rare, ophthalmologists should be aware that it can occur during an air/fluid exchange, especially in the setting of significant trauma. During retinal cases, the anesthesiologist may not be fully aware of maneuvers happening within the eye, and the operating surgeon may be the first to recognize this problem if a patient becomes hemodynamically unstable after an air/fluid exchange is initiated. Promptly terminating the air infusion and returning the eye to a fluid filled state may help avoid systemic morbidity.

Supraselective injection of intraarterial melphalan as the primary treatment for late presentation unilateral multifocal stage VB retinoblastoma

Purpose: The purpose of this study was to report a case of a 7-year-old girl with unilateral, multifocal Reese Ellsworth Stage Vb retinoblastoma who was successfully treated using intraarterial chemotherapy infusion as the primary therapy. Methods: This is an interventional case report. A 7-year-old girl presented with advanced unilateral retinoblastoma. The patient received intraarterial melphalan infusion therapy as the primary treatment. Results: Complete tumor resolution was seen at 1 month after intraarterial melphalan infusion. Conclusion: This case of advanced retinoblastoma in a 7-year-old girl was successfully treated with intraarterial melphalan infusion alone. Treatment resulted in complete resolution of the tumor 1 month after treatment. In comparison with systemic chemotherapy, intraarterial melphalan infusion therapy may be a less toxic and more effective primary treatment option in the future management of advanced retinoblastoma.

Primary central nervous system lymphoma mimicking pituitary apoplexy: case report

Lymphoma involving the pituitary gland is very rare and usually results from metastatic spread of systemic lymphoma. We present a case of primary central nervous system (CNS) large B cell lymphoma that manifested as pituitary apoplexy. A 45-year-old woman presented with headache, and then rapidly developed a third nerve palsy and bitemporal hemianopsia. Imaging suggested a pituitary macroadenoma, with spontaneous necrosis, extending into the suprasellar region, compressing the optic chiasm and invading the right cavernous sinus. The patient underwent transsphenoidal resection which revealed a vascular, firm tumor. An aggressive decompression of the optic chiasm was performed with complete resolution of both visual fields and third nerve palsy. Final pathology showed B cell lymphoma. Systemic work-up including bone marrow aspiration and CSF studies showed no other foci of lymphoma, and the patient was HIV-negative. Chemotherapy with methotrexate, vincristine, procarbazine, and dexamethasone was administered for primary CNS lymphoma. This is an uncommon diagnosis of which the clinician should be aware in order to tailor surgical intervention and provide early institution of proper therapy.

Optimizing the transplant dose of a human neuronal cell line graft to treat SCI pain in the rat

Neuropathic pain is a prevalent and difficult problem in the setting of spinal cord injury (SCI). The use of cellular transplant therapy to treat this pain has been successful with the use of a human neuronal cell line, hNT2.17 [M.J. Eaton, S.Q. Wolfe, M.A. Martinez, M. Hernandez, C. Furst, J. Huang, B.R. Frydel, O. Gomez-Marin, Subarachnoid transplant of a human neuronal cell line attenuates chronic allodynia and hyperalgesia after excitotoxic SCI in the rat, J. Pain 8 (2007) 33-50]. Intrathecal transplant of these cells potently reverses behavioral hypersensitivity after excitotoxic spinal cord injury in the rat model. This study focuses on delineating the optimal dose of these cell grafts in the same model. Two weeks after intraspinal injection of quisqualic acid (QUIS) with subsequent behavioral hypersensitivity, terminally differentiated hNT2.17 cells were transplanted into 300 g Wistar-Furth rats in a logarithmic variation of doses: 10(6), 10(5) and 10(3) cells. Behavioral hypersensitivity testing was performed weekly for 6 weeks following transplant. The dose of 10(6) cells (or approximately 3 million/kg) potently and permanently reversed both cutaneous allodynia (CA) and thermal hyperalgesia (TH). Reduced transplant doses of the hNT2.17 cell line did not permanently reverse behavioral hypersensitivity, suggesting that there is an optimal dose that can be used as a clinical tool to treat SCI-associated neuropathic pain.

Transarterial embolization of a scalp hemangioma presenting with Kasabach-Merritt syndrome: Case report

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.