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Gabriel Zada

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.

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Gabriel Zada, Thomas C. Solomon and Steven L. Giannotta

Object

Intracranial hypotension (ICH) can present with a wide variety of visual symptoms and findings. Deficits in visual acuity and visual fields as well as ophthalmoplegia due to cranial nerve dysfunction have been frequently described. The aim of this review was to identify the most commonly reported ocular manifestations associated with ICH.

Methods

The authors conducted a review of the literature to date to identify all studies of patients with ICH and ocular manifestations.

Results

The most commonly encountered cranial nerve deficit resulting from ICH (> 80% of reported cases) is an abducens nerve paresis, which may occur unilaterally or bilaterally. Although less common, oculomotor and trochlear nerve palsies have been reported as well. The optic nerve complex is frequently involved in ICH and may manifest as deficits in visual acuity and field cuts. Visual deficits and ophthalmoplegia improved following appropriate management in 97% of reported cases.

Conclusions

Intracranial hypotension can present with a wide spectrum of visual deficits, the causes of which are multifactorial. Cranial nerve paresis, especially of the abducens nerve, is frequently reported. The majority of symptoms and cranial nerve deficits reviewed respond favorably to conservative management, epidural blood patch administration, or in a minority of cases, surgical intervention.

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Grace Lam, Vivek Mehta and Gabriel Zada

Object

Spontaneous and medically induced CSF leaks are uncommonly associated with pituitary adenomas. The aim of this study was to further characterize the clinical scenarios most closely associated with this phenomenon.

Methods

A review of the literature was conducted to identify all cases of nonsurgical CSF leaks associated with pituitary adenomas. The authors aimed to identify associated risk factors and management strategies used to treat these tumors and repair the CSF leak site.

Results

Fifty-two patients with spontaneous or medically induced CSF leaks in the setting of a pituitary adenoma were identified from 29 articles published from 1980 through 2011. In 38 patients (73%), CSF rhinorrhea developed following initiation of medical therapy, whereas spontaneous CSF leakage developed as the presenting symptom in 14 patients (27%). The patients' mean age was 42.8 years (range 23–68 years). There were 35 males and 17 females. Forty-two patients (81%) had a prolactinoma, with the remaining patients having the following tumors: nonfunctioning pituitary adenoma (6 patients), growth hormone–secreting adenoma (2 patients), mammosomatotroph cell adenoma (1 patient), and ACTH-secreting adenoma (1 patient). Infrasellar tumor invasion into the paranasal sinuses was specifically reported in 56% of patients. The medical agents associated with CSF leakage were dopamine agonists (97%) and somatostatin analogs (3%). The average time from initialization of medical treatment to onset of rhinorrhea was 3.3 months (range 3 days–17 months). Nonsurgical management was successful in 4 patients, including 1 who had successful resolution with a temporary lumbar drain. Forty-six patients (88%) underwent surgical intervention to treat the CSF leak and/or resect the tumor. In 2 patients, surgery was not performed due to medical contraindications or patient preference.

Conclusions

Nonsurgical development of CSF rhinorrhea may occur in the setting of pituitary adenomas, especially following favorable response of invasive prolactinomas to initiation of dopamine agonist therapy. Additional cases have been reported as the presenting symptom of a pituitary adenoma and are likely to be related to decreased tumor volume due to intrinsic infarction or hemorrhage, ongoing invasion, and/or increases in intracranial pressure. Surgical repair, preferentially via a transsphenoidal approach, is the recommended initial treatment for definitive repair of the CSF leak and achievement of maximal tumor resection.

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Alan A. Moazzam, Naveed Wagle and Gabriel Zada

Object

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.

Methods

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.

Results

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.

Conclusions

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.

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Gabriel Zada, Rose Du and Edward R. Laws Jr.

Object

Endonasal approaches have become the gold standard intervention for many anterior and middle skull base tumors. The authors aimed to define some of the existing limitations of these approaches by reviewing their experience with complex sellar region tumors that were initially considered for both transsphenoidal and open skull base approaches and were thus deemed tumors at “the edge of the envelope.”

Methods

Between April 2008 and April 2010, 250 transsphenoidal operations were performed at Brigham and Women's Hospital. All cases were retrospectively reviewed to identify patients with complex sellar region tumors that were initially considered for, or soon thereafter required, an open craniotomy as the definitive treatment. The anatomical tumor characteristics that posed limitations to performing safe and effective endonasal skull base operations were reviewed.

Results

Thirteen cases exemplifying some of the existing limitations to achieving optimal surgical outcomes via transsphenoidal-based approaches are presented. The following 8 factors are separately discussed that repeatedly limited the extent of resection, increased the risk of the operation, and contributed to perioperative complications: significant suprasellar extension, lateral extension, retrosellar extension, brain invasion with edema, firm tumor consistency, involvement or vasospasm of the arteries of the circle of Willis, and encasement of the optic apparatus or invasion of the optic foramina.

Conclusions

Although the ability to approach and resect complex tumors using endonasal skull base techniques has evolved dramatically in recent years, several inherent tumor characteristics mandate extensive preoperative consideration. In selected cases these characteristics may lend support to selecting an open craniotomy as the initial operation.

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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.