The aim of this paper is to provide a comprehensive review of clinical, imaging, and histopathological features, as well as operative and nonoperative management strategies in patients with Rathke cleft cysts (RCCs).
A literature review was performed to identify previous articles that reported surgical and nonsurgical management of RCCs. Rathke cleft cysts are often incidental lesions found in the sellar and suprasellar regions and do not require surgical intervention in the majority of cases. In symptomatic RCCs, the typical clinical presentation includes headache, visual loss, and/or endocrine dysfunction. Visual field testing and endocrine laboratory studies may reveal more subtle deficiencies associated with RCCs. When indicated, the transsphenoidal approach typically offers the least invasive and safest method for treating these lesions. Various surgical strategies including cyst wall resection, intralesional alcohol injection, and sellar floor reconstruction are discussed. Although headache and visual symptoms frequently improve after surgical drainage of RCCs, hypopituitarism and diabetes insipidus are less likely to do so. A subset of more aggressive, atypical RCCs associated with pronounced clinical symptoms and higher recurrence rates is discussed, as well as the possible relationship of these lesions to craniopharyngiomas.
Rathke cleft cysts are typically benign, asymptomatic lesions that can be monitored. In selected patients, transsphenoidal surgery provides excellent rates of improvement in clinical symptoms and long-term cyst resolution. Complete cyst wall resection, intraoperative alcohol cauterization, and sellar floor reconstruction in the absence of a CSF leak are not routinely recommended.
Alan A. Moazzam, Naveed Wagle and Gabriel Zada
Currently, few medical options exist for refractory and atypical/anaplastic meningiomas. New developments in chemotherapeutic options for meningiomas have been explored over the past decade. The authors review these recent developments, with an emphasis on emerging avenues for therapy, clinical efficacy, and adverse effects.
A review of the literature was performed to identify any studies exploring recent medical and chemotherapeutic agents that have been or are currently being tested for meningiomas. Results from included preclinical and human clinical trials were reviewed and summarized.
Current guidelines recommend only 3 drugs that can be used to treat patients with refractory and highgrade meningiomas: hydroxyurea, interferon-α 2B, and Sandostatin long-acting release. Recent developments in the medical treatment of meningiomas have been made across a variety of pharmacological classes, including cytotoxic agents, hormonal agents, immunomodulators, and targeted agents toward a variety of growth factors and their signaling cascades. Promising avenues of therapy that are being evaluated for efficacy and safety include antagonists of platelet-derived growth factor receptor, epidermal growth factor receptor, vascular endothelial growth factor receptor, and mammalian target of rapamycin. Because malignant transformation in meningiomas is likely to be mediated by numerous processes interacting via a complex matrix of signals, combination therapies affecting multiple molecular targets are currently being explored and hold significant promise as adjuvant therapy options.
Improved understanding of the molecular mechanisms driving meningioma tumorigenesis and malignant transformation has resulted in the targeted development of more specific agents for chemotherapeutic intervention in patients with nonresectable, aggressive, and malignant meningiomas.
Gabriel Zada, Patrick Pezeshkian and Steven Giannotta
✓ The presentation of spontaneous intracranial hypotension (SIH) can be associated with various clinical and neuro-imaging features that may impede a rapid diagnosis of this entity. The authors report the case of a patient who presented with bilateral third cranial nerve palsies and bilateral subdural hematomas. Intracranial pressure monitoring proved to be useful in the diagnosis and management of SIH in this patient.
Gabriel Zada, Eisha Christian, Charles Y. Liu and Steven L. Giannotta
Aneurysms of the anterior communicating artery (ACoA) can be a considerable challenge to treat surgically based on variations in the anatomy and morphological features of the ACoA complex. The fenestrated aneurysm clip can be a simple and practical tool in the operative management of ACoA aneurysms. The goal in this study was to characterize the particular surgical situations in which the use of a fenestrated clip facilitates the clip ligation of ACoA aneurysms.
The authors present their operative strategy and techniques for the use of fenestrated clips in the treatment of ACoA aneurysms.
One hundred ninety-nine patients underwent surgical clipping of an ACoA aneurysm at the authors' institution between the years 1991 and 2008. Of these patients, fenestrated aneurysm clips were used in 20 cases (10%). The following structures were enclosed in the clip aperture: ipsilateral A2 artery, 12 patients (60%); ipsilateral A1 artery, 4 patients (20%); ipsilateral A1 artery plus recurrent artery of Heubner, 1 patient (5%); ACoA, 1 patient (5%); frontopolar artery, 1 patient (5%); and no structures, 1 patient (5%). Aneurysms approached from the left side more frequently required fenestrated clips than did right-sided aneurysms (80 vs 20%, p = 0.0073). In all cases, patency of the A2 vessels was confirmed on postoperative angiography. In 2 patients, small remnant aneurysm necks were identified on postoperative angiography.
The use of fenestrated aneurysm clips can minimize tedious and potentially dangerous dissection of adherent branch vessels, while maintaining the integrity of structures placed within the clip aperture. The ACoA aneurysms pointing in a superior direction are more likely to require clip fenestration around the A2 vessel, whereas those pointing in an inferior direction are more likely to require clip fenestration around the A1 vessel. The parallel approximation of the fenestrated clip blades makes them especially useful in the treatment of large or giant aneurysms.
Gabriel Zada, J. Diaz Day and Steven L. Giannotta
The extradural temporopolar approach is used for enhanced exposure of the cavernous sinus and petroclival regions in the treatment of complex lesions not amenable to sole treatment via radiosurgical or endovascular methods. The authors' objective was to review the indications, surgical experience, and operative technique in a series of patients who underwent surgery with this approach.
The authors conducted a retrospective review to identify patients who underwent a temporopolar approach from 1992 to 2008. An orbitozygomatic craniotomy was frequently used, followed by extradural retraction of the temporal lobe. A sequential progression of bone removal at the anterior and middle skull base, followed by opening the layers of the lateral wall of the cavernous sinus was next performed to safely retract the brain and widen the exposure to the cavernous sinus, interpeduncular fossa, and upper petroclival regions.
Sixty-six patients were identified and included in the study. The mean patient age was 49 years. The main indications for surgery were as follows: meningioma (25 patients, 38%), basilar artery aneurysm (11 patients, 17%), trigeminal schwannoma (7 patients, 11%), chordoma (5 patients, 7%), hemangioma (3 patients, 5%), pituitary adenoma (3 patients, 5%), superior cerebellar artery aneurysm (3 patients, 5%), and other lesions (9 patients, 14%). Complications included hemiparesis in 4 patients (6%), infarcts in 4 patients (6%), transient aphasia in 1 patient (1.5%), and cranial nerve paresis in 20 patients (30%).
The extradural temporopolar approach offers a relatively safe and wide exposure of the sphenocavernous and petroclival regions. Mobilization of the cranial nerves and internal carotid artery allow gentle brain retraction and maximal preservation of venous outflow. This is an advantageous approach to large tumors in these regions and for complex upper basilar artery or superior cerebellar artery aneurysms.