OBJECTIVE Recovery of abducens nerve palsy (ANP) after endoscopic endonasal skull base surgery (ESBS) has been shown to be potentially predicted by postoperative ophthalmological examination. Triggered electromyography (t-EMG) and free-run electromyography (f-EMG) activity provide an intraoperative assessment of abducens nerve function, but associations with long-term ANP outcomes have not been explored. The objective of this study was to describe intraoperative abducens EMG characteristics and determine whether these electrophysiological profiles are associated with immediately postoperative and long-term ANP outcomes after ESBS. METHODS The authors conducted a 5-year (2011–2016) retrospective case-control study of patients who underwent ESBS in whom the abducens nerve was stimulated (t-EMG). Electrophysiological metrics were compared between patients with a new postoperative ANP (cases) and those without ANP (controls). Pathologies included chordoma, pituitary adenoma, meningioma, cholesterol granuloma, and chondrosarcoma. Electrophysiological data included the presence of abnormal f-EMG activity, t-EMG stimulation voltage, stimulation threshold, evoked compound muscle action potential (CMAP) amplitude, onset latency, peak latency, and CMAP duration at various stages of the dissection. Controls were selected such that pathologies were similarly distributed between cases and controls. RESULTS Fifty-six patients were included, 26 with new postoperative ANP and 30 controls without ANP. Abnormal f-EMG activity (28.0% vs 3.3%, p = 0.02) and lack of response to stimulation (27% vs 0%, p = 0.006) were more frequent in patients with immediately postoperative ANP than in controls. Patients with immediately postoperative ANP also had a lower median CMAP amplitude (35.0 vs 71.2 μV, p = 0.02) and longer onset latency (5.2 vs 2.8 msec, p = 0.04). Comparing patients with transient versus persistent ANP on follow-up, those with persistent ANP tended to have a lower CMAP amplitude (12.8 vs 57 μV, p = 0.07) and higher likelihood of not responding to stimulation at the end of the case (45.5% vs 7.1%, p = 0.06). Abnormal f-EMG was not associated with long-term ANP outcomes. CONCLUSIONS The presence of f-EMG activity, lack of CMAP response to stimulation, decreased CMAP amplitude, and increased CMAP onset latency were associated with immediately postoperative ANP. Long-term ANP outcomes may be associated with t-EMG parameters, including whether the nerve is able to be stimulated once identified and CMAP amplitude. Future prospective studies may be designed to standardize abducens nerve electrophysiological monitoring protocols to further refine operative and prognostic utility.

OBJECTIVES:Pediatric cranial base pathology is anatomically complex and surgical treatment is oftentimes difficult to conceptualize for patients and their families. Three-dimensional (3D) models of the sinuses and cranial base have the potential to enhance patient understanding in numerous domains. Our objective is to assess the use of 3D models in pre-operative parental and patient counseling prior to endoscopic endonasal skull base surgery in the pediatric population. METHODS:A survey was designed to assess parent and patient-perceived utility of 3D-printed models in surgical counseling prior to pediatric skull base surgery. RESULTS:A total of 10 patients were included. The median age was 9 years (range = 5 months-15 years). Pathology included juvenile nasopharyngeal angiofibroma (JNA) (N = 4), fibrous dysplasia of the maxilla and sphenoid (N = 1), juvenile ossifying fibroma (N = 1), nasal dermoid (N = 2, one with intracranial extension), encephalocele (N = 1), and parapharyngeal ectopic glial tissue (N = 1). Nearly all parents agreed or strongly agreed that 3D printed models were helpful in explaining the patient's skull base pathology (N = 10), surgical plan (N = 10), and possible complications (N = 9). All parents strongly agreed that 3D models should be used routinely in pre-operative counseling for endoscopic endonasal surgery. According to a majority of parents, patients older than 4 years old found the models helpful in understanding their pathology (75%) and surgery (88%). CONCLUSION:By allowing direct three-dimensional visualization of the target pathology, 3D models serve as a useful adjunct in enhancing patient comprehension of the pathologic entity, planned surgery, and potential complications prior to pediatric endoscopic endonasal skull base surgery.

Objectives  Sinonasal mucosal melanoma (SNMM) is an extremely rare and challenging sinonasal malignancy with a poor prognosis. Standard treatment involves complete surgical resection, but the role of adjuvant therapy remains unclear. Crucially, our understanding of its clinical presentation, course, and optimal treatment remains limited, and few advancements in improving its management have been made in the recent past. Methods  We conducted an international multicenter retrospective analysis of 505 SNMM cases from 11 institutions across the United States, United Kingdom, Ireland, and continental Europe. Data on clinical presentation, diagnosis, treatment, and clinical outcomes were assessed. Results  One-, three-, and five-year recurrence-free and overall survival were 61.4, 30.6, and 22.0%, and 77.6, 49.2, and 38.3%, respectively. Compared with disease confined to the nasal cavity, sinus involvement confers significantly worse survival; based on this, further stratifying the T3 stage was highly prognostic ( p  < 0.001) with implications for a potential modification to the current TNM staging system. There was a statistically significant survival benefit for patients who received adjuvant radiotherapy, compared with those who underwent surgery alone (hazard ratio [HR] = 0.74, 95% confidence interval [CI]: 0.57-0.96, p  = 0.021). Immune checkpoint blockade for the management of recurrent or persistent disease, with or without distant metastasis, conferred longer survival (HR = 0.50, 95% CI: 0.25-1.00, p  = 0.036). Conclusions  We present findings from the largest cohort of SNMM reported to date. We demonstrate the potential utility of further stratifying the T3 stage by sinus involvement and present promising data on the benefit of immune checkpoint inhibitors for recurrent, persistent, or metastatic disease with implications for future clinical trials in this field.

BACKGROUND:Noninvoluting congenital hemangiomas (NICH) are rare and poorly understood vascular tumors that are present at birth, characterized by lack of growth after birth and lack of involution. We report uncharacteristic cases of NICH hypertrophy occurring later in life.METHODS:This is a case series describing the clinical presentation, management, and histologic characteristics of two cases of NICH hypertrophy.RESULTS:Two patients with a NICH of the scalp experienced lesion hypertrophy in teenage or early adult life. Case 1 is a 14-year-old female who presented with a flat left parietal scalp lesion that at first grew slowly with the patient; however, over the span of months grew substantially resulting in an exophytic lesion. The patient had the lesion surgically excised. Case 2 is a 26-year-old female with NICH of left occipital scalp and posterior neck who noted new nodules on the inferior border of the lesion. MRA/MRI showed extension into the occipital calvarium, level V of the neck, and paraspinal musculature. The patient elected to observe given the extent of the lesion and her minimal symptoms.CONCLUSION:Although postnatal growth of NICH have been described, cases usually occur during the pre-adolescent period where growth is usually proportional to overall growth of the patient. This study describes two cases of rapid onset NICH hypertrophy occurring later in life. Knowledge of the potential for delayed hypertrophy may lead families to seek earlier intervention or opt for more definitive interventions. Additionally, recognition of these variable distinctions will contribute to a better understanding of CH and its various subtypes.

Objectives: To determine which surgical factors are associated with quality-of-life (QOL) outcomes in oral cavity cancer survivors after free flap reconstruction of the oral cavity. Patients and Methods: A cross-sectional study was conducted from a multidisciplinary head and neck cancer (HNC) survivorship clinic. Oral cavity cancer survivors with at least 6-months of postoperative follow-up from ablation and free flap reconstruction were included. Primary outcome measures were validated patient-reported outcome measures (PROMs) including the Eating Assessment Tool-10 (EAT-10) measure of swallowing-specific QOL, University of Washington Quality of Life (UW-QOL) physical and social-emotional subscale scores and feeding tube dependence. Results: Extent of tongue resection was associated with EAT-10 and the UW-QOL Physical subscale scores. Pa-tients with oral tongue defects reported worse scores than with composite defects in the EAT-10 and UW-QOL physical domain (p = 0.0004, 0.0025, respectively). This association no longer applies when controlling for differences in extent of tongue resection. Patients with anterior composite resections reported worse EAT-10 scores than lateral resections (p = 0.024). This association no longer applies when controlling for extent tongue resection (p = 0.46). Gastric tube dependence demonstrates similar trends to PROMs. Conclusion: Extent of tongue resection was strongly associated with poor QOL outcomes after free tissue reconstruction of the oral cavity and mediates the associations between other defect characteristics and QOL. These findings demonstrate the need for emphasis on expected oral tongue defects when counseling patients and highlight the need to address QOL in a multidisciplinary fashion post-operatively.

OBJECTIVES:Recent cochlear implant (CI) electrodes are designed to be atraumatic to inner ear structures. Studies in adults have demonstrated improved hearing preservation rates with the CI532/632 electrode, but none have examined this in children. Our objective is to describe the hearing preservation rate with CI532/632 in pediatric patients and determine factors that influence hearing preservation.METHODS:We conducted a retrospective cohort study of children undergoing CI over a three-year period. Inclusion criteria were: CI with the 532/632 electrode, presence of pre-operative low frequency hearing defined by pure tone average (PTA) of ≤80 dB at 250 Hz or at the average of 250 and 500 Hz, and post-operative unaided audiometry. Other data collected included demographics, otologic history, imaging, and surgical details.RESULTS:A total of 13 patients and 15 ears were included. Hearing was preserved in 10/15 (66%) ears at an average follow-up of 6 months, similar to that reported in the adult literature. Patients with preserved hearing post-operatively were more likely to have a positive family history of hearing loss. There was a trend towards patients with anatomic inner ear abnormalities being more likely to lose hearing after CI, but this was not statistically significant. Pre-operative thresholds were not predictive of hearing preservation. Patients with preserved hearing had a significantly smaller shift in thresholds after cochlear implantation. Therefore, hearing preserved and non-preserved groups differed more by the magnitude of change in threshold, rather than their preoperative threshold. Other factors such as age, sex, surgeon, and surgery duration were not associated with hearing preservation.CONCLUSION:This study describes low frequency hearing preservation after pediatric CI532/632 implantation. The hearing preservation rate in our cohort was consistent with that reported in the adult literature. Our data suggest that preoperative thresholds do not solely determine which patients will go on to experience hearing preservation. We believe this will aid surgeons with patient-specific device selection and counseling potential pediatric CI recipients with preserved hearing.