✓ The term “papilledema” indicates swelling of the optic discs secondary to increased intracranial pressure. Papilledema can be caused by an intracranial mass lesion or by other factors. Visual symptoms frequently accompany papilledema, which can lead to permanent visual loss if left untreated. Starting with an illustrative case, the authors review the pathophysiology of the visual signs and symptoms of papilledema. They also briefly review potential treatment options, focusing on the role of the neurosurgeon in the treatment of patients with papilledema.
Clemens M. Schirmer and Thomas R. Hedges III
Clemens M. Schirmer and Carl B. Heilman
Neuroendoscopic treatment of colloid cysts is limited by the reach and flexibility of the instruments that can be passed through the working channels of the rigid neuroendoscope. The authors describe a case of a third ventricular colloid cyst where a large solid colloid fragment was recovered using a nitinol stone retrieval basket as a flexible wall-guided atraumatic salvage instrument. A flexible nitinol stone retrieval basket was successfully used through an endoscopic working channel to retrieve a large portion of the colloid cyst from the occipital horn of the lateral ventricle in a 70-year-old man who presented with progressive memory loss, urinary incontinence, and slowness of gait. A flexible nitinol stone retrieval basket can be safely and effectively maneuvered in the ventricular system, using the ventricular wall for deflection, and can be used to retrieve colloid cyst fragments as a salvage technique. Remaining free-floating large colloid cyst fragments in the ventricular system do not necessarily require a second craniotomy or bur hole for access but may be retrieved using a nitinol stone retrieval basket.
Clemens M. Schirmer and Carl B. Heilman
Intracranial hemangiopericytomas are frequently located along the dural sinuses along the skull base and represent rare, aggressive CNS neoplasms that are difficult to distinguish from meningiomas based on both imaging and gross characteristics. The authors of this study describe 3 patients with these lesions and review the pertinent literature.
Two men and 1 woman, whose median age at the time of the initial presentation was 37 years (range 20–53 years), constitute this series. They underwent multimodal treatment consisting of resection, embolization, radiation therapy, and in 1 case chemotherapy.
Two of the 3 patients treated were alive after a mean follow-up of 93 months (range 4–217 months). One patient died 217 months after the initial diagnosis. The longest tumor progression–free interval after the initial or secondary resection was 43 months (range 4–84 months).
Hemangiopericytomas have been reclassified as mesenchymal nonmeningothelial tumors. They have an inevitable tendency to recur locally and metastasize distally. The mainstay of therapy remains an aggressive attempt to achieve gross-total resection at the initial surgery. Postoperative adjuvant radiotherapy should be offered to all patients, regardless of the degree of resection achieved. Diligent long-term follow-up is paramount as local recurrences and distal metastases can develop sometimes years after the initial treatment.
Ning Lin, Clemens M. Schirmer and Mark R. Proctor
Disc cysts are rare intraspinal extradural lesions that communicate with the intervertebral disc and can mimic the symptoms of acute lumbar disc herniation. Initially reported in the Japanese-language literature as a new entity (discal cyst), there are few documented cases in North America, and only 1 prior case in the pediatric population. The authors present the case of a 16-year-old girl with an intervertebral disc cyst causing lumbar radiculopathy that progressed despite conservative treatment. All medical records, imaging studies, intraoperative findings, and pertinent literature were reviewed. Serial preoperative MR imaging revealed enlargement of the intraspinal cyst at the L4–5 level, resulting in compression of the right L-5 nerve root. Enlargement of the cyst occurred over a 4-month period despite conservative treatment with physical therapy and corticosteroid injections. Microsurgical discectomy and excision of the cyst resulted in complete resolution of the preoperative radiculopathy. An intervertebral disc cyst is a rare entity in the adult population and exceedingly rare in the pediatric population but should remain in the differential diagnosis of any intraspinal extradural mass. The authors hypothesize that there exists a spectrum of this entity that may not be responsive to conservative therapy. Cyst excision alone or in conjunction with microsurgical discectomy is safe and effective in treating radiculopathy caused by disc cysts.
Clemens M. Schirmer, Daniel A. Hoit and Adel M. Malek
Distal protection devices (DPDs) have decreased the risk of embolic stroke among patients with carotid artery (CA) disease undergoing CA stent placement. The FilterWire EX is a first-generation fixed-basket DPD with a filter rigidly attached to a guidewire. Second-generation mobile-basket DPDs (RX Accunet or SpiderFX) allow movement of the filter relative to the guidewire and can thus reduce the potential for vessel irritation, vasospasm, or intimal injury during CA stent placement.
Stent angioplasty was attempted in 40 CAs (37 patients) using the fixed-basket FilterWire DPD, a second-generation mobile-basket DPD, or no protection in 12, 6, and 22 arteries, respectively. Clinical presentation, angiographic details relating to the incidence of vasospasm or dissection, and clinical outcome data were recorded and analyzed.
Vasospasm was associated with use of the fixed-basket FilterWire device (8 [67%] of 12 cases) compared with the bare unfiltered guidewire group (3 [14%] of 22) and the second-generation mobile-basket DPD group (1 [17%] of 6, p < 0.004). Secondary angioplasty was also associated with intraprocedural vasospasm. In a multivariate analysis, FilterWire use was an independent risk factor for vasospasm (p < 0.0003).
A high incidence of vasospasm was observed following CA stent placement procedures in which the fixed-basket FilterWire EX DPD was used but not in unprotected CA stent placement or procedures in which a second-generation mobile-basket DPD was used. Although this phenomenon was self-limited in all instances, vasospasm should be considered a risk of these devices and may predispose to more serious vascular injury. Coronary artery stent placement should be performed with a second-generation mobile-basket DPD to minimize the risks of embolic complications and iatrogenic vascular injury.
Clemens M. Schirmer, Basar Atalay and Adel M. Malek
Internal carotid artery dissection (ICAD) is a common cause of stroke in young patients, which may lead to major transient or permanent disability. Internal carotid artery dissection may occur spontaneously or after trauma and may present with a rapid neurological deterioration or with hemodynamic compromise and a delayed and unstable neurological deficit. Endovascular intervention using stent angioplasty can be used as an alternative to anticoagulation and open surgical therapy in this setting to restore blood flow through the affected carotid artery.
The authors present the cases of 2 patients with flow-limiting symptomatic ICAD leading to near-complete occlusion and without sufficient collateral supply. Both patients had isolated cerebral hemispheres without significant blood flow from the anterior or posterior communicating arteries. In both cases, the patients demonstrated blood pressure–dependent subacute unstable neurological deficits as a result of the hemodynamic compromise resulting from the dissection.
Both patients underwent careful microwire-based selection of the true lumen followed by confirmatory microinjection and subsequent exchange-length microwire-based recanalization using tandem telescoping endovascular stenting. In both cases the neurological state improved, and no permanent neurological deficit ensued.
The treatment of ICAD may be difficult in patients with subacute unstable neurological deficits related to symptomatic hypoperfusion, especially in the setting of a hemodynamically isolated hemisphere. Anticoagulation alone may be insufficient in these patients. Although there is no widely accepted guideline for the treatment of ICAD, the authors recommend stent-mediated endovascular recanalization in cases of symptomatic flow-limiting hemodynamic compromise, especially in cases of an isolated hemisphere lacking sufficient communicating artery compensatory perfusion.
Clemens M. Schirmer, Steven W. Hwang, Ron I. Riesenburger, In Sup Choi and Carlos A. David
Cobb syndrome represents the concurrent findings of a metameric spinal vascular malformation and a cutaneous vascular malformation within several dermatomes of each other. This rare entity engenders many difficult decisions with respect to appropriate therapeutic management. Historically, surgical excision carried a high morbidity, and conservative management without intervention was preferred. More recently, several cases of endovascular embolization have been reported with good success.
The authors describe the case of a 17-year-old boy who presented with a right gluteal angioma and was found to have a spinal arteriovenous malformation. Multiple embolizations failed to prevent neurological deterioration, and the patient eventually became wheelchair dependent. Surgical excision of the malformation led to partial recovery of neurological function, and at the latest follow-up, 52 months postoperatively, the patient was able to ambulate independently. This case demonstrates the successful treatment of a patient with Cobb syndrome with surgical excision after multiple refractory embolizations. A multidisciplinary approach, which balances the patient's current neurological function against the risks and potential gains from any interventional and surgical procedure, is recommended.
William R. Miele, Clemens M. Schirmer, Kevin C. Yao and Carl B. Heilman
The pathogenesis of Chiari malformation Type I (CM-I) and associated syringomyelia is incompletely understood. Patients often present in middle age with incidental or minimally symptomatic CM-I, whose management is controversial. One option is clinical and radiographic observation of asymptomatic and minimally symptomatic patients. The authors here present the case of a 36-year-old woman who had been monitored for 6 years for a minimally symptomatic CM-I and cervicothoracic syrinx. After 5 years of follow-up, she suffered spontaneous rupture of a cerebral cavernous malformation when she was 27 weeks pregnant. The ruptured cavernous malformation and hematoma were operatively managed via a right frontal craniotomy. Ten months after the craniotomy for resection of the ruptured cavernous malformation, follow-up MRI demonstrated resolution of the CM-I and syrinx. Few similar cases have been reported in adults. This case argues for the presence of dynamic factors in the development and maintenance of CM-I and supports the nonoperative treatment of asymptomatic and minimally symptomatic patients.
Diana Apetauerova, Clemens M. Schirmer, Jay L. Shils, Janet Zani and Jeffrey E. Arle
The authors report the cases of 2 young male patients (aged 16 and 26 years) with dystonic cerebral palsy of unknown origin, who developed status dystonicus, an acute and persistent combination of generalized dystonia and chorea. Both patients developed status dystonicus after undergoing general anesthesia, and in 1 case, after administration of metoclopramide. In attempting to control this acute hyperkinetic movement disorder, multiple medication trials failed in both cases and patients required prolonged intubation and sedation with propofol. Bilateral deep brain stimulation of the globus pallidus internus (4 and 2 months after the onset of symptoms in the first and second case, respectively) produced immediate resolution of the hyperkinetic movement disorder in each case. Deep brain stimulation provided persistent suppression of the dystonic movement potential after a follow-up of 30 and 34 months, respectively, as demonstrated by the reemergence of severe dystonia during the end of battery life of the implantable pulse generators that was readily controlled by exchange of the generators in each case.