Faculty Bibliography

OBJECTIVE:To analyze the patterns of care and survival of cutaneous angiosarcomas of the head and neck. STUDY DESIGN/METHODS:Retrospective cohort study. SETTING/METHODS:National Cancer Database. METHODS:The National Cancer Database was queried to select patients with cutaneous angiosarcoma of the head and neck between 2004 and 2015. For survival analysis, patients were included only if they received definitive treatment and complete data. Prognostic factors were analyzed by univariate and multivariable Cox regression. RESULTS:< .001) predicted worse overall survival. CONCLUSION/CONCLUSIONS:Angiosarcoma of head and neck is a rare malignancy that affects the elderly. Surgical treatment with negative margins is associated with improved survival. Even with curative-intent multimodality treatment, the survival of patients aged ≥75 years is limited.

An ideal nasal osteotomy should deliver precise, predictable, and reproducible cosmetic and functional results while minimizing soft-tissue trauma and postoperative complications. In addition to closing an open roof deformity after hump reduction, other common indications for osteotomies include the crooked nose and a wide bony vault. The literature has reported numerous and diverse osteotomy techniques as well as differences in timing of osteotomies. Each has its own merits and indications, and its proponents. In this article, we review the anatomy and nomenclature relating to osteotomies. We review the locations and paths of the osteotomies-lateral, intermediate, medial, and superior/transverse. We consider the percutaneous and endonasal approaches, as well as timing of osteotomies and other considerations. We also discuss technical considerations in the selection of instrumentation for osteotomies.

The transsphenoidal corridor for pituitary adenoma surgery was established as early as 1906 by Schloffer and was subsequently refined by Cushing throughout the early 20thcentury [1]. The use of intraoperative magnetic resonance imaging (iMRI) in endoscopic endonasal transsphenoidal resections, however, is a relatively contemporary addition to the surgical treatment of pituitary tumors. The morbidity of these cases has decreased over the years in light of advances in intraoperative navigation as well as improvements in endoscope dynamics and surgical instruments. Despite such improvements, a substantial number of patients require repeat surgeries or subsequent radiotherapy for residual and/or recurrent disease. This can be largely attributed to cavernous sinus invasion or suprasellar extension, which pose technical challenges to achieving gross total resections (GTRs). The rate of GTR for pituitary tumors cited in the literature varies from 59-88%.[2-3] The advantage of iMRI is that it provides the surgeon with immediate feedback regarding their progress and ability to safely achieve GTR which, in pituitary surgery, is critical for long term cure. Additionally, although there is concern for increased risk of postoperative endocrine dysfunction, Zhibin et al prove that this is not necessarily the case. In their series, 133 patients who underwent iMRI had higher rates of GTR and did not have a significant difference in postoperative hypopituitarism. [4] This study includes a combined retrospective and prospective comparative analysis of 238 patients who underwent transsphenoidal resection of a pituitary tumor from January 2013 until May 2019. All patients were operated on by one of four experienced neurosurgeons and one of three experienced otolaryngologists. There were 203 patients who did not undergo iMRI and 25 patients who did. A 3 tesla MRI magnet was used in all cases. All intraoperative images were read and interpreted by a senior neuroradiologist at our institution. Amongst the two groups, there was no statistically significant difference in patient age (p = 0.488), tumor size (microadenoma versus macroadenoma, p = 0.878), and primary versus recurrent tumor (p = 0.837). The use of iMRI did not yield a decrease in the length of stay (4.84 days in the no iMRI group and 5 in the iMRI group, p = 0.777). There were zero cases of a return to the OR for residual tumor in the intraoperative MRI group versus the non-MRI group. However, this did not reach statistical significance. This study did not yield a statistically significant difference in GTR (p = 0.75), near total resection (NTR, p = 0.167), or subtotal resection (p = 0.083). This is likely secondary to a low sample size and therefore power in the iMRI group. Finally, there was no significant difference in the number of patients requiring postoperative DDAVP (p = 0.099) or hydrocortisone (p = 0.873) after discharge. Preliminary results reveal a potential benefit of iMRI use to assess for residual disease which can be addressed immediately during the initial operation, thus decreasing the need for re-operations. Furthermore, the ability to correlate intraoperative findings with an intraoperative structure may lead to more precise identification and preservation of normal gland, which can possibly decrease the incidence of postoperative endocrine dysfunction

Auditory brainstem implants (ABIs) stimulate the auditory system at the cochlear nucleus, bypassing the peripheral auditory system including the auditory nerve. They are used in patients who are not cochlear implant candidates. Current criteria for use in the United States are neurofibromatosis type 2 patients 12 years or older undergoing first- or second-side vestibular schwannoma removal. However, there are other nontumor conditions in which patients may benefit from an ABI, such as bilateral cochlear nerve aplasia and severe cochlear malformation not amendable to cochlear implantation. Recent experience with ABI in the pediatric population demonstrates good safety profile and encouraging results.

Objective: To describe cochlear implantation (CI) outcomes for rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment.
Method(s): Retrospective review between 1995 and 2019 from a single tertiary care center of all patients with retrocochlear pathology who underwent CI. Demographics, clinical history, and audiometric data were reviewed. Study endpoints include (1) logged device use, (2) ability to achieve auditory perception, and (3) word recognition score (WRS) in the CI-only condition.
Result(s): Thirty-two patients (63% of females) with retrocochlear pathology were implanted at our center. The average age at implantation was 46.9 years (SD: 19, range: 13-80). Mean duration of deafness was 4.5 years (SD: 5.0, range: 0.4-19.0). Etiology of hearing loss included VS in 24 (75%), CNS malignancy treated with radiation in 4 (13%), intralabyrinthine schwannoma in 2 (6%), head and neck malignancy treated with radiation in 1 (3%), and superficial siderosis in 1 (3%). The mean preoperative PTA was 95.8 dBHL (SD 24.7) and WRS was 7.2% (SD 13.1). Of the 24 VSs, 21 were NF2-associated and 3 were sporadic. The mean tumor size was 1.64 cm (SD: 0.6, range: 0.5-2.6 cm). At the time of CI, 11 patients had prior microsurgery, 6 patients had prior radiation to the ipsilateral tumor, and 7 patients had stable tumors without prior surgery or radiation. Device use was classified as regular (>7 hours/day) in 15 (47%), limited (<7 hours/day) in 12 (38%), and nonuse is 5 (16%). The audiometric outcomes of 26 patients are reported, as the other 6 patients have been implanted too recently for review. Auditory perception was achieved in 24/26 patients. The two patients who failed to achieve auditory perception underwent prior surgery. Open-set speech recognition (WRS > 20%) was achieved in 18 patients. Meaningful sound perception but without significant open-set speech (WRS < 20%) was seen in six patients. Altogether, the mean WRS at most recent follow-up (mean: 3.4 years, SD: 1.8) for the observation, microsurgery, and radiation cohorts was 51% (SD: 15), 36% (SD: 28), and 39% (SD: 26), respectively. Over long-term follow-up, two patients experienced decline in CI performance associated with tumor regrowth and necessitated additional surgery; both underwent explantation of the CI and successful auditory brainstem implantation. The remaining patients have demonstrated durable benefit. A multivariate analysis is presented to evaluate the effects of the following variables: duration of deafness, time interval between treatment and CI, diagnosis of NF2, treatment cohort, pathology, and status of hearing in the contralateral ear.
Conclusion(s): In appropriately selected patients, cochlear implantation is feasible for the rehabilitation of hearing loss due to retrocochlear pathology and/or its treatment. Given the heterogeneity inherent to this population, outcomes are variable. In most cases, auditory percept was achieved and over half of the patients obtained open-set speech perception, irrespective of prior management and treatment

This study aims to document the historical conceptualization of the inner ear as the anatomical location for the appreciation of sound at a continuum of frequencies and to examine the evolution of concepts of tonotopic organization to our current understanding. Primary sources used are from the sixth century BCE through the twentieth century CE. Each work/reference was analyzed from two points of view: to understand the conception of hearing and the role of the inner ear and to define the main evidential method. The dependence on theory alone in the ancient world led to inaccurate conceptualization of the mechanism of hearing. In the sixteenth century, Galileo described the physical and mathematical basis of resonance. The first theory of tonotopic organization, advanced in the seventeenth century, was that high-frequency sound is mediated at the apex of the cochlea and low-frequency at the base of the cochlea. In the eighteenth and nineteenth centuries, more accurate anatomical information was developed which led to what we now know is the accurate view of tonotopic organization: the high-frequency sound is mediated at the base and low-frequency sound at the apex. The electrical responses of the ear discovered in 1930 allowed for physiological studies that were consistent with the concept of a high to low tone sensitivity continuum from base to apex. In the mid-twentieth century, physical observations of models and anatomical specimens confirmed the findings of greater sensitivity to high tones at the base and low tones at the apex and, further, demonstrated that for high-intensity sound, there was a spread of effect through the entire cochlea, more so for low-frequency tones than for high tones. Animal and human behavioral studies provided empirical proof that sound is mediated at a continuum of frequencies from high tones at the base through low tones at the apex of the cochlea. Current understanding of the tonotopic organization of the inner ear with regard to pure tones is the result of the acquisition over time of knowledge of acoustics and the anatomy, physical properties, and physiology of the inner ear, with the ultimate verification being behavioral studies. Examination of this complex evolution leads to understanding of the way each approach and evidential method through time draws upon previously developed knowledge, with behavioral studies providing empirical verification.

Direct access through the sinuses and nasopharyngeal mucosa in the endoscopic endonasal approach (EEA) raises concern for a contaminated operative environment and subsequent infection. The reported rate of meningitis in endoscopic endonasal skull base surgery in the literature ranges from 0.7 to 3.0% [1, 2]. The only factor identified as being independently associated with meningitis in a statistically significant manner is cerebrospinal fluid (CSF) leak [1-5]. However, many centers performing high volume of EEAs use postoperative antibiotic coverage independent of the presence intraoperative or postoperative CSF leak. Furthermore, while meningitis remains a severe concern, most centers use postoperative gram-positive coverage to prevent toxic shock syndrome caused by Staphylococcus aureus infection in the setting of prolonged nasal packing. There are currently a multitude of approaches regarding perioperative antibiotic coverage in EEAs [1-4]. Given the lack of consensus in the literature and our experience regarding the benefit of discontinuation of prolonged prophylactic antibiotics throughout the breadth of neurosurgical procedures, we sought to analyze the need for postoperative antibiotics in EEAs further. As such, we performed a prospective analysis compared with a retrospective cohort to delineate whether discontinuation of postoperative antibiotics leads to a change in the rate of postoperative infections. The retrospective cohort consisted of patients who underwent an EEA from January 1, 2013 to May 31, 2019. These patients all received postoperative antibiotics while nasal packing was in place (median 7 days). Starting on April 1, 2019 until August 1, 2019, we discontinued postoperative antibiotic use. Patients from this group made up the prospective cohort. The retrospective cohort had 315 patients (66% pituitary macroadenomas vs. 7% microadenomas, 4% meningiomas, 4% craniopharyngiomas, 4% chordomas, and 15% others) while the prospective group had 23 patients (57% pituitary macroadenomas, 30% craniopharyngiomas, 8% meningiomas/chordomas, and 5% others). The primary endpoint was rate of postoperative infections and specifically, meningitis and multidrug resistant organism (MDRO) infections. There was no statistically significant difference in the use of nasal packing (p = 0.085), intraoperative CSF leak (p = 0.133), and postoperative CSF leak (p = 0.507) between the two groups. There was also no significant difference in the number of patients with positive preoperative MSSA and MRSA nasal swabs (p = 0.622). There was a significant decrease in the number of patients discharged with antibiotics (55.1% in the retrospective and 4.5% in the prospective group, p = 0.000). The number of patients with positive blood cultures (p = 0.701) and positive urine cultures (p = 0.691) did not differ significantly between the two groups. Finally, there was no statistically significant difference in postoperative CSF infections (p = 0.34) or MDRO infections (0.786) between the two groups. We describe promising preliminary results that demonstrate that discontinuation of postoperative antibiotics in EEAs do not lead to a statistically significant increase in the rate of postoperative CSF or MDRO infections. The previous algorithm for postoperative antibiotic coverage in our center, like many centers, called for gram-positive coverage, which may have contributed to the overall preponderance of gram-negative meningitis cases in this cohort

Tumors of the pineal region comprise several different entities with distinct clinical and histopathological features. Whereas some entities predominantly affect adults, pineoblastoma (PB) constitutes a highly aggressive malignancy of childhood with a poor outcome. PBs mainly arise sporadically, but may also occur in the context of cancer predisposition syndromes including DICER1 and RB1 germline mutation. With this study, we investigate clinico-pathological subgroups of pineal tumors and further characterize their biological features. We performed genome-wide DNA methylation analysis in 195 tumors of the pineal region and 20 normal pineal gland controls. Copy-number profiles were obtained from DNA methylation data; gene panel sequencing was added for 93 tumors and analysis was further complemented by miRNA sequencing for 22 tumor samples. Unsupervised clustering based on DNA methylation profiling separated known subgroups, like pineocytoma, pineal parenchymal tumor of intermediate differentiation, papillary tumor of the pineal region and PB, and further distinct subtypes within these groups, including three subtypes within the core PB subgroup. The novel molecular subgroup Pin-RB includes cases of trilateral retinoblastoma as well as sporadic pineal tumors with RB1 alterations, and displays similarities with retinoblastoma. Distinct clinical associations discriminate the second novel molecular subgroup PB-MYC from other PB cases. Alterations within the miRNA processing pathway (affecting DROSHA, DGCR8 or DICER1) are found in about two thirds of cases in the three core PB subtypes. Methylation profiling revealed biologically distinct groups of pineal tumors with specific clinical and molecular features. Our findings provide a foundation for further clinical as well as molecular and functional characterization of PB and other pineal tumors, including the role of miRNA processing defects in oncogenesis.