Anzalone, C. Lane

The radial forearm free flap (RFFF) has become a workhorse for soft tissue reconstruction following surgical ablation of head and neck cancer. Given the popularity of the RFFF, it is important to understand potential variants of upper extremity vascular anatomy and the effects of these findings on pre- and intraoperative planning. The purpose of this series is to synthesize the existing literature to raise awareness for potential radial artery aberrations during planned reconstruction.

Purpose: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. Methods: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. Results: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk–75 yr)] were evaluated for recurrent facial nerve paralysis at the authors’ institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2–20). The median nadir House–Brackmann score was 4, with a median recovery to House–Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1–420 d). Diagnostic evaluation confirmed Melkersson–Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. Conclusion: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

ObjectiveTo determine whether using image guidance technology with 3‐dimensional image segmentation increases the endoscopic surgeon's accuracy, efficiency, and confidence in identifying the anterior ethmoidal artery.MethodsThis is a cross‐sectional study of attending physicians and residents at an academic medical center. Because identification of the anterior ethmoidal artery during image‐guided surgery can be challenging, we studied the effect of anterior ethmoidal artery image segmentation (ie, partitioning and coloring) on surgeon test performance. A computerized test was administered to 16 surgeons who were asked to identify the anterior ethmoidal artery on multiplanar computed tomographic images and to answer multiple‐choice questions. Half the questions showed segmented images of the anterior ethmoidal artery, and half showed images without segmentation. Efficiency and accuracy of identification and subjective surgeon confidence were determined for each question. Descriptive statistics were used to compare test performance for identification of the anterior ethmoidal artery on images with or without segmentation.ResultsPercentage of correct answers (P <. 001), efficiency (P <. 001), and confidence (P <. 001) in identification of the anterior ethmoidal artery were significantly better with segmented computed tomographic images.DiscussionWe demonstrated that use of segmented images improves surgeons’ accuracy, confidence, and efficiency for identification of the anterior ethmoidal artery.Implications for PracticeWe describe how segmentation can allow surgeons to improve the surgical course by increasing their accuracy, confidence, and efficiency in identifying the anterior ethmoidal artery.

Background Age, tumor size and location, overall health, and patient preference are primary considerations driving treatment decision-making for intracranial meningiomas. However, even for the same individual patient, treatment recommendations may vary between centers and providers. Objective To study associations between geography, disease presentation, and management of intracranial meningioma in the United States. Methods The population-based Surveillance, Epidemiology, and End Results(SEER) data were queried between 2004 and 2014 for cases of intracranial meningioma. Results A total of 65,808 patients with intracranial meningioma were identified. Univariate analyses demonstrated strong associations between geographic location, age, and size of tumor at presentation. The mean age for all registries was 64.2 years, with a range from 62.0 (Utah registry) to 66.6 (Detroit registry). The greatest proportion of small tumors (<1 cm) were identified in the Utah registry (13.9% of tumors), while the greatest proportion of large tumors (> 4cm) were noted in the Hawaii registry (30.7% of tumors). Multivariable analysis demonstrated that the impact of geography on treatment selection was just as important as other established variables. For example, the distribution in tumor size between New Mexico and Greater California registries is nearly identical; however, the odds ratio for surgery was 1.5 times greater for the New Mexico population. Conclusion These data suggest that disease presentation and treatment are significantly influenced by regional referral patterns, provider or institutional treatment preferences, and regional availability of subspecialty expertise. Understanding such biases is important for patients, referring physicians, and treatment providers in an effort to provide balanced counseling and treatment.

Objective/Hypothesis The aim of the study was to determine the impact of race on disease presentation and treatment of intracranial meningioma in the United States. Study Design This study comprised of the analysis of a national population-based tumor registry. Methods Analysis of the surveillance, epidemiology, and end results (SEER) database was performed, including all patients identified with a diagnosis of intracranial meningioma. Associations between race, disease presentation, treatment strategy, and overall survival were analyzed in a univariate and multivariable model. Results A total of 65,973 patients with intracranial meningiomas were identified. Of these, 45,251 (68.6%) claimed white, 7,796 (12%) black, 7,154 (11%) Hispanic, 4,902 (7%) Asian, and 870 (1%) patients reported “other-unspecified” or “other-unknown.” The median annual incidence of disease was lowest among black (3.43 per 100,000 persons) and highest among white (9.52 per 100,000 persons) populations (p < 0.001). Overall, Hispanic patients were diagnosed at the youngest age and white patients were diagnosed at the oldest age (mean of 59 vs. 66 years, respectively; p < 0.001). Compared with white populations, black, Hispanic, and Asian populations were more likely to present with larger tumors (p < 0.001). After controlling for tumor size, age, and treatment center in a multivariable model, Hispanic patients were more likely to undergo surgery than white, black, and Asian populations. Black populations had the poorest disease specific and overall survival rates at 5 years following surgery compared with other groups. Conclusion Racial differences among patients with intracranial meningioma exist within the United States. Understanding these differences are of vital importance toward identifying potential differences in the biological basis of disease or alternatively inequalities in healthcare delivery or access Further studies are required to determine which factors drive differences in tumor size, age, annual disease incidence, and overall survival between races.

Introduction. Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. Methods. Case report and review of the English literature. Results. A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear. Audiometric testing confirmed profound SNHL in the left ear, while the right ear exhibited moderate-to-severe SNHL. Left-sided cochlear implantation was performed using a conventional length lateral wall electrode. Intraoperative device testing found normal impedance levels throughout the array; however, electrically evoked auditory potentials were absent on all electrodes. Upon initial activation 3 weeks after surgery, the patient reported excellent access to sound in the cochlear implant-only condition. He has made good progress at each subsequent visit; speech perception testing after seven months showed improvement from 0% to 32% on AzBio sentence and 53% on CNC phoneme testing in the cochlear implant-only condition. Conclusion. We report the third case of cochlear implantation in a patient with CMT. SNHL in CMT is hypothesized to result from disruption of synchronous activity of the cochlear nerve. In patients with CMT, cochlear implantation may reconstitute synchronous neural activity by way of supraphysiological electrical stimulation. Our results corroborate two earlier reports that cochlear implantation is a viable option for rehabilitation of SNHL in this unique subset of patients.

Objective: To describe a novel case of congenital profound bilateral sensorineural hearing loss in a patient with bilateral nodular internal auditory canal and labyrinthine enhancement and temporal bone dysplasia. Patients: A 76-day-old female was referred to the authors’ center for evaluation of congenital deafness. Behavioral observations and objective audiometric evaluation demonstrated bilateral profound sensorineural hearing loss and a comprehensive multidisciplinary evaluation identified compound heterozygous pathogenic variants in MYO7A , a gene associated with Usher Syndrome Type 1B or DFNB2. Computed tomography and contrast-enhanced magnetic resonance imaging studies demonstrated bilateral temporal bone anomalies with unique middle and inner ear malformations, as well as unique contrast enhancement in the membranous labyrinth, internal auditory canals, and cranial nerves, which have not been previously described with MYO7A variants. Interventions: Given the potential risk for progressive bilateral labyrinthitis ossificans, bilateral simultaneous cochlear implantation was performed at 4 months of age. Main Outcome Measures: Subsequent audiologic follow up after implantation shows significantly improved access to auditory information and increased vocalizations. At last testing, speech and language skills for both receptive and expressive language abilities were found to be commensurate with her chronological age. Conclusion: We report a novel presentation and imaging findings of congenital bilateral profound sensorineural hearing loss in a patient with nodular internal auditory canal and labyrinthine enhancement and coexisting inner ear dysplasia. Despite the multiple radiologic abnormalities, the patient has demonstrated good benefit from cochlear implantation. Future study of rare variants of congenital deafness, such as this, is critical toward defining new disease processes and determining optimal treatment.

Objective: To describe a unique case of an asymptomatic arteriovenous lesion of the internal auditory canal (IAC) and present the associated imaging findings. Methods: Retrospective case report and review of the literature. Results: A 55-year-old man presented for further evaluation of a left-sided sudden sensorineural hearing loss that occurred 8 years earlier. Careful review of outside serial magnetic resonance (MR) imaging revealed a contralateral, ill-defined right-sided IAC mass with low T2 signal and subtle peripheral enhancement on postcontrast T1-weighted sequencing. The patient had no history of right-sided otologic symptoms. Subsequent dedicated IAC MR imaging confirmed the presence of a space-occupying lesion adjacent to a complexity of vasculature. The presence of prominent flow voids, paucity of avid enhancement on previous studies, and marked signal within the lesion on time-of-flight MR angiography and MR venography was consistent with the diagnosis of a solitary arteriovenous lesion of the IAC. There was no radiological evidence of recent or remote parenchymal or subarachnoid hemorrhage or stroke. Conclusion: We report a novel case of an occult asymptomatic IAC arteriovenous lesion. Vascular anomalies confined to the IAC are rare. To date, there have been only four reports in the literature of IAC arteriovenous lesions and our case is the first to present asymptomatically. A high index of suspicion and dedicated imaging is required to identify and accurately diagnose these lesions to guide appropriate counseling and potential intervention.