Faculty Bibliography

Pituitary apoplexy in patients with adrenocorticotropic hormone (ACTH) producing tumors is a rare occurrence. We report four patients with Cushing's disease harboring ACTH-secreting macroadenomas who presented with pituitary apoplexy. We report the endocrinologic and visual outcomes of these patients after emergent transsphenoidal surgery. A retrospective chart review was performed in 4 patients who presented with pituitary apoplexy from hemorrhage into an ACTH-secreting pituitary adenoma. The patient charts were reviewed for clinical presentation, neuroimaging findings, intraoperative surgical findings, pathologic findings, and postoperative endocrinologic and visual outcomes. All patients presented with acute headaches, nausea, vomiting, and visual loss from optic compression. MR imaging demonstrated a hemorrhagic macroadenoma that was confirmed at surgery. All patients underwent emergent transsphenoidal decompression (within 24 h of presentation). One of these underwent an additional craniotomy to resect residual tumor. Postoperatively, all patients showed significant improvement in visual acuity and visual fields with biochemical remission confirmed on laboratory testing. Significant weight loss as well as resolution of diabetes and hypertension was noted in all cases. All four patients remained in biochemical remission at their most recent follow-up visit (mean 40 months, range: 24-72 months). Excellent endocrine and visual outcomes can be achieved after emergent transsphenoidal surgery in patients with Cushing's disease presenting with pituitary apoplexy. Although the cure rates of non-apoplectic ACTH macroadenomas are generally poor, higher rates of remission can be achieved in cases of pituitary apoplexy. This may be partly due to the effects of tumor infarction.

Cystic epithelial lesions such as Rathke's cleft cysts (RCCs) and craniopharyngiomas may be difficult to distinguish on a clinical, radiographic, and sometimes histopathological basis. We describe a case of a giant 6.5 cm suprasellar cystic lesion that was presumed to be a craniopharyngioma based on the neuroimaging findings. The lesion extended from the anterior skull base and sella turcica to the lateral ventricle and sylvian fissure resulting in obstructive hydrocephalus. Complete surgical removal of the suprasellar lesion was achieved using an extended frontotemporal transbasal skull base approach. Intraoperatively, the cyst wall was thickened and partially calcified, resembling a craniopharyngioma. However, the histopathological examination revealed findings most consistent with a RCC with additional features of extensive squamous metaplasia, metaplastic bone formation, and chronic inflammation. The case raises the issue of whether there is a pathologic continuum of parasellar ectodermal lesions which may account for the overlap of features and transitional states. In this report, we discuss the possible spectrum between RCCs and craniopharyngiomas, and also emphasize the importance of complete resection of the cyst wall in RCCs that exhibit squamous metaplasia, inflammation, or ossification to minimize the probability of recurrence.

With the relatively recent increase in the use of MRI techniques, there has been a concurrent rise in the number of vestibular schwannomas (VSs) detected as incidental findings. These incidental VSs may be prevalent in up to 0.02%-0.07% of individuals undergoing MRI and represent a significant portion of all diagnosed VSs. The management of these lesions poses a significant challenge for practitioners. Most incidental VSs tend to be small and associated with minimal symptoms, permitting them to be managed conservatively at the time of diagnosis. However, relatively few indicators consistently predict tumor growth and patient outcomes. Furthermore, growth rates have been shown to vary significantly over time with a large variety of long-term growth patterns. Thus, early MRI screening for continued tumor growth followed by repeated MRI studies and clinical assessments throughout the patient's life is an essential component in a conservative management strategy. Note that tumor growth is typically associated with a worsening of symptoms in patients who undergo conservative management, and many of these symptoms have been shown to significantly impact the patient's quality of life. Specific indications for the termination of conservative management vary across studies, but secondary intervention has been shown to be a relatively safe option in most patients with progressive disease. Patients with incidental VSs will probably qualify for a course of conservative management at diagnosis, and regular imaging combined with the expectation that the tumor and symptoms may change at any interval is crucial to ensuring positive long-term outcomes in these patients. In this report, the authors discuss the current literature pertaining to the prevalence of incidental VSs and various considerations in the management of these lesions. It is hoped that by incorporating an understanding of tumor growth, patient outcomes, and management strategies, practitioners will be able to effectively address this challenging disease entity.

BACKGROUND: The advances in endoscopic skull base surgery have led to the resection of increasingly larger cranial base lesions and the creation of larger skull base defects with the potential for increased postoperative high-flow cerebrospinal fluid (CSF) leaks. These concerns led to the development of the vascularized pedicled nasoseptal flap (PNSF), which is now used as the mainstay for repair of large skull base defects in many academic centers. In this report, we review the incidence of postoperative CSF leaks in our institution in patients undergoing endoscopic skull base repair of high-flow CSF leaks with a vascularized PNSF without concurrent CSF diversion. METHODS: We performed a retrospective analysis at our tertiary care medical center on patients who underwent endoscopic repair of high-flow CSF leaks using a PNSF without CSF diversion between July 2008 and August 2011. Repair materials, incidence of postoperative CSF leaks, and demographic data were collected. RESULTS: Fifty-nine high-flow CSF leaks were repaired with a PNSF and other repair materials, without the use of lumbar catheter drainage. No postoperative CSF leak occurred in this cohort of patients. The overall postoperative CSF leak rate was 0%. CONCLUSION: Meticulous multilayer-closure of skull base defects is critical to prevent postoperative CSF leaks. Although lumbar drainage may be useful in select scenarios, it carries inherent risks of intracranial hypotension and pneumocephalus, and may not be necessary for routine management of high-flow CSF leaks in conjunction with a robust PNSF. Further prospective randomized controlled studies may be warranted to evaluate the efficacy of postoperative lumbar drainage.

OBJECTIVES/HYPOTHESIS: Numerous professional societies, clinical practices, and hospitals provide Internet-based patient education materials (PEMs) to the general public, but not all of this information is written at a reading level appropriate for the average patient. The National Institutes of Health and the US Department of Health and Human Services recommend that PEMs be written at or below the sixth-grade level. Our purpose was to assess the readability of endoscopic sinus surgery (ESS)-related PEMs available on the Internet and compare readability levels of PEMs provided by three sources: professional societies, clinical practices, and hospitals. STUDY DESIGN: A descriptive and correlational design was used for this study. METHODS: The readability of 31 ESS-related PEMs was assessed with four different readability indices: Flesch-Kincaid Grade Level (FKGL), Flesch Reading Ease Score (FRES), Simple Measure of Gobbledygook (SMOG), and Gunning Frequency of Gobbledygook (Gunning FOG). Averages were evaluated against national recommendations and between each source using analysis of variance and t tests. RESULTS: The majority of PEMs (96.8%) were written above the recommended sixth-grade reading level, based on FKGL (P < .001). Only one article (3.2%) had an FKGL at or below the sixth-grade level. The mean readability values were: FRES 47.1 +/- 13.4, FKGL 10.7 +/- 2.4, SMOG 13.7 +/- 1.6, and Gunning FOG 12.4 +/- 2.7. CONCLUSIONS: Current Internet-based PEMs related to ESS, regardless of source type, were written well above the recommended sixth-grade level. Materials from the hospitals/university-affiliated websites had lower readability scores, but were still above recommended levels. Web-based PEMs pertaining to ESS should be written with the average patient in mind.

OBJECTIVE: Fractures of the hyoid bone are rare occurrences. They are mainly caused by strangulation/asphyxiation injuries, trauma to the neck, and motor vehicle accidents (MVAs). As a result of their rarity, proper treatment guidelines are not in place for dealing with these injuries. In this study, a systematic literature review was conducted with the goal of identifying optimal management for patients with fracture of the hyoid bone. DATA SOURCES: MEDLINE and PubMed databases. REVIEW METHODS: The MEDLINE and PubMed databases were searched for patients diagnosed with hyoid bone fracture. Further cases were obtained from the bibliographies of relevant articles. Full-text articles were obtained. Patient presentation, method of diagnosis, treatment regimen, and outcomes are discussed. RESULTS: Forty-six cases were collected from 36 articles. No randomized controlled trials regarding treatment of hyoid fractures were found. The most common etiologies were MVA, assault, and neck trauma during athletic activities. Most common presenting symptoms included dysphagia, odynophagia, and pain upon neck rotation. Most frequent presenting signs included anterior neck tenderness and swelling. Five cases out of 46 had surgical repair of the fractured hyoid bone. In the remaining 41 cases, 26 were treated with conservative management, which included rest/observation, diet changes, and analgesia, while the other 15 cases required tracheotomy or surgical treatment for related injuries. All patients survived and had excellent outcomes with resolution of symptoms. CONCLUSION: This review shows that direct surgical treatment of hyoid fractures was performed in only 10.9% of cases. Both conservative and surgical management yielded positive outcomes.

A little over a century ago, in 1907, at the University of Innsbruck, Hermann Schloffer performed the first transsphenoidal surgery on a living patient harboring a pituitary adenoma. Schloffer used a superior nasal route via a transfacial lateral rhinotomy incision. This was perhaps his greatest academic contribution to neurosurgery. Despite the technological limitations of that time, Schloffer's operation was groundbreaking in that it laid the foundation for future development and refinement of transsphenoidal pituitary surgery, influencing prominent surgeons such as Oskar Hirsch and Harvey Cushing. Even after undergoing multiple modifications and a brief fall into obscurity, the transsphenoidal approach has endured through generations of surgeons and remains the preferred approach for lesions of the sella turcica to this day. Although Schloffer performed primarily abdominal surgery in his practice, his contributions to the transsphenoidal approach have had a lasting impact in the field of pituitary and skull base surgery. The authors review the life and career of Hermann Schloffer, the surgical details of his transsphenoidal operation, and the legacy that it has left on the field of pituitary surgery.

OBJECTIVE: We compared the incidence of postoperative cerebrospinal fluid (CSF) leaks in patients undergoing endoscopic skull base repair with a pedicled nasoseptal flap (PNSF) with or without the addition of a dural sealant. STUDY DESIGN AND SETTING: Retrospective analysis at a tertiary care medical center. METHODS: A retrospective analysis was performed at our tertiary care medical center on patients who underwent endoscopic repair of high-flow CSF leaks using a PNSF between December 2008 and August 2011. Repair materials, incidence of postoperative CSF leaks, and demographic data were collected. RESULTS: Thirty-two high-flow CSF leaks were repaired with a PNSF alone without dural sealant (group A), and 42 were repaired with a PNSF with the addition of a dural sealant (group B). In group A, there were no postoperative CSF leaks (0%), whereas in group B, there was 1 delayed postoperative CSF leak, resulting in a 2.4% leak rate. The incidence of postoperative CSF leakage was not significantly different between the 2 groups (P = .38). The overall postoperative CSF leak rate was 1.4%. CONCLUSIONS: The use of dural sealants when performing endoscopic PNSF repair of high-flow CSF leaks is not supported by our data. In addition, this practice may significantly increase surgical cost. We encountered no postoperative CSF leaks in patients with high-flow CSF leaks treated with PNSF alone without dural sealants. Meticulous surgical technique and proper positioning of the PNSF seem to obviate the need for dural sealants during endoscopic skull base reconstruction of high-flow CSF leaks.

OBJECTIVES/HYPOTHESIS: Onodi cells are the posterior-most ethmoid air cells that lie superior to the sphenoid sinus. Identification of these cells is essential prior to endoscopic sinus and skull base surgery due to their intricate relationship with the optic nerves and carotid arteries, which may lead to deleterious complications. In this study, high-resolution computed tomography (HRCT) scans from 170 adult-patients were analyzed by two independent observers for the presence of Onodi cells. STUDY DESIGN: Radiographic analysis at a tertiary care medical center. METHODS: A retrospective analysis was performed on patients undergoing HRCT between July 2008 and September 2010. Incidence of Onodi cells and demographic data were collected. RESULTS: The overall prevalence of Onodi cells in this cohort was 65.3%. Subgroup analysis based on ethnicity showed a rate of Onodi cells of 83.3% in Asians, 73.1% in whites, 57.0% in African Americans, and 62.7% in Hispanics. The prevalence of Onodi cells was not significantly different among the different ethnicities (P > .05). However, this was limited by a small sample size in some ethnic groups. Onodi cell prevalence was equivalent among males and females: 62.2% and 63.5% respectively (P > .05). Overall, our results show a greater prevalence of Onodi cells than previously reported. CONCLUSIONS: We found a higher prevalence of Onodi cells in our cohort than previously reported in the literature. Therefore, it is important for surgeons to anticipate the presence of these cells during endoscopic sinus and skull base procedures to prevent potential complications.