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.
Gabriel Zada, Ning Lin, and Edward R. Laws Jr.
Growth patterns of pituitary adenomas have been observed to vary by histopathological subtype. The authors aimed to analyze variations in the patterns of extrasellar extension of nonfunctional macroadenomas (NFMAs) and growth hormone (GH)–secreting macroadenomas.
A retrospective review was conducted of data obtained in 75 patients who underwent transsphenoidal operations for histologically confirmed NFMAs (50 patients) and GH-secreting macroadenomas (25 patients) at the Brigham and Women's Hospital over an 18-month period. Patients with microadenomas and prior operations were excluded from the analysis. Preoperative MR images were reviewed to assess patterns of extrasellar extension in the varying tumor subtypes.
The mean maximal tumor diameter in NFMAs and GH-secreting macroadenomas was 26 and 16 mm, respectively (p < 0.0001). Extension of the NFMAs occurred into the following regions: infrasellar, 23 patients (46%); suprasellar, 41 patients (82%); and cavernous sinus, 20 patients (40%). Extension of GH-macroadenomas occurred into the following regions: infrasellar, 18 patients (72%); suprasellar, 4 patients (16%); and cavernous sinus, 4 patients (16%). Compared with GH-adenomas, NFMAs were more likely to develop suprasellar extension (82% vs 16%, p < 0.0001), cavernous sinus extension (40% vs 16%, p = 0.04), and isolated suprasellar extension (30% vs 4%, p = 0.0145). GH-macroadenomas had higher overall rates of infrasellar extension (72% vs 46%, p < 0.05), and isolated infrasellar extension (52% vs 6%, p < 0.0001). Of the 13 GH-macroadenomas with isolated infrasellar extension, 5 (42%) met WHO criteria for atypical adenomas.
Substantial differences in extrasellar growth patterns were observed among varying histological subtypes of pituitary macroadenomas. Despite smaller tumor diameters, GH-macroadenomas demonstrated a proclivity for infrasellar extension, whereas NFMAs exhibited preferential extension into the suprasellar region.
Gabriel Zada, Thomas C. Solomon, and Steven L. Giannotta
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.
The authors conducted a review of the literature to date to identify all studies of patients with ICH and ocular manifestations.
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.
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.
Grace Lam, Vivek Mehta, and Gabriel Zada
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.
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.
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.
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.
Gabriel Zada, Rose Du, and Edward R. Laws Jr.
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.”
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.
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.
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.