Citations

Objective: To evaluate quality-of-life (QOL) in patients with sporadic vestibular schwannoma (VS) with particular focus on those recently diagnosed. Study Design: Cross-sectional survey, Penn Acoustic Neuroma Quality of Life (PANQOL) instrument. Setting: Acoustic Neuroma Association and a single tertiary referral center. Patients: Patients with sporadic VS. Main Outcome Measures: Domain-specific and total PANQOL scores comparing treatment modalities after adjusting for baseline covariates of interest. Results: Among all 1,288 respondents there were 229 (18%) who were recently diagnosed and had not yet selected a treatment modality, 303 (24%) who were observed, 185 (14%) who underwent radiosurgery alone, 507 (39%) who underwent microsurgery alone, and 64 (5%) who underwent radiosurgery and microsurgery. After adjusting for covariates of interest, total PANQOL scores were highest for the observation cohort (65; 95% CI 62–68), lowest for patients treated with multimodality therapy (56; 51–61) and those recently diagnosed (58; 55–62), and intermediate for those who received microsurgery alone (60; 58–62) and radiosurgery alone (61; 57–64) (global comparison, p = 0.001). When comparing groups that received single-modality therapy, there were no statistically significant differences in total PANQOL scores at short (0–5 yr), intermediate (6–10 yr), or long-term (≥11 yr) follow-up after adjusting for baseline covariates (all, p > 0.05). Conclusion: A new diagnosis of VS frequently imparts significant anxiety on the patient and leads to a temporary reduction in QOL. Notably, anxiety domain QOL scores among patients with newly diagnosed VS are poorer than patients who have observed their tumor for 6 months or longer. When feasible, an initial period of observation may afford patients valuable time to reconcile with the new diagnosis, to learn more about treatment options, and to discover whether there is tumor growth. In turn, this valuable information can be used to inform sound decision-making regarding tumor management.

Introduction Large and even moderate sized, extra-axial cerebellopontine angle (CPA) tumors may fill this restricted space and distort the regional anatomy. It may be difficult to determine even with high resolution magnetic resonance imaging (MRI) if the tumor is dural-based, or what the nerve of origin is if a schwannoma. While clinical history and exam are helpful, they are not unequivocal, particularly since many patients present with a myriad of symptoms, or conversely an incidental finding. We present an atypical appearing, asymptomatic CPA tumor, ultimately identified at surgery to be a trigeminal schwannoma. Case History A 40-year-old man presented with new-onset seizure. MRI identified an incidental heterogeneously contrast-enhancing CPA lesion (Fig. 1A–D). The tumor was centered on the internal auditory canal (IAC) with no tumor extension into Meckel's cave, IAC or jugular foramen. Audiometry demonstrated 10db of relative left-sided hearing loss with 100% word recognition. Physical examination was negative for focal neurologic deficits. A retrosigmoid craniotomy was performed and an extra-axial, yellow-hued mass was encountered and resected, which was ultimately confirmed to originate from the trigeminal nerve (Video 1). Gross total resection was achieved, and the patient recovered from surgery with partial ipsilateral trigeminal sensory loss and no other new neurologic deficits. Conclusion Pure CPA trigeminal schwannomas are rare, but should be considered in the differential for enhancing CPA lesions. Although, Meckel's cave involvement is frequently observed, it is not universal, and pure CPA schwannomas of all cranial nerves IV–XII have been reported in the literature.The link to the video can be found at: https://youtu.be/AlodYCu70F8.

Abstract BACKGROUND The diagnosis of vestibular schwannomas (VS) is associated with reduced patient quality of life (QOL). Minimal clinically important difference (MCID) was introduced as the lowest improvement in a patient-reported outcome (PRO) score discerned as significant by the patient. We formerly presented an MCID for the Penn Acoustic Neuroma QOL (PANQOL) battery based on cross-sectional data from 2 tertiary referral centers. OBJECTIVE To validate the PANQOL MCID values using prospective data. METHODS A prospective registry capturing QOL was queried, comprising patients treated at the authors’ institution and Acoustic Neuroma Association members. Anchor- and distribution-based techniques were utilized to determine the MCID for domain and total scores. We only included anchors with Spearman's correlation coefficient larger than 0.3 in the MCID threshold calculations. Most domains had multiple anchors with which to estimate the MCID. RESULTS A total of 1254 patients (mean age: 57.4 yr, 65% females) were analyzed. Anchor-based methods produced a span of MCID values (median, 25th-75th percentile) for each PANQOL domain and the total score: hearing (13.1, 13-16 points), balance (14, 14-19 points), pain (21, 20-28 points), face (25, 16-36 points), energy (16, 15-18 points), anxiety (16 [1 estimate]), general (13 [1 estimate]), and total (12.5, 10-15 points). CONCLUSION Current findings corroborate our formerly shared experience using multi-institutional, cross-sectional information. These MCID thresholds can serve as a pertinent outcome when deciphering the clinical magnitude of VS QOL endpoints in cross-sectional and longitudinal studies.

OBJECTIVEGeniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN).METHODSA retrospective review of a prospective neurosurgical database at our institution was performed, 2000–2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem.RESULTSEleven patients were surgically treated for GN—9 primary, 2 reoperations. The median age was 48, 7 patients were female, and the median follow-up was 11 months (range 3–143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)—all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients.CONCLUSIONSGN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.

Abstract A 52-yr-old woman was referred to a tertiary medical center for evaluation of 2 yr of progressive visual symptoms and 1 yr of retro-orbital pressure. Her ophthalmologic exam was unrevealing except for mild asymmetrical impairment in color perception. A gadolinium contrast-enhanced magnetic resonance image of the head showed a left extraconal orbital apex lesion consistent with cavernous hemangioma. Computed tomography reveled bony remodeling of the medial-inferior orbital walls with superior orbital fissure expansion. The lesion was endoscopically resected and confirmed by pathological analysis. The endoscopic approach is demonstrated in detail, including correlation with cadaveric anatomic specimens ‡ . During the approach, a rescue nasoseptal flap was raised in case the orbit required further support after tumor resection, but was replaced as this was not needed. This patient had a brief period of postoperative diplopia, which resolved 1 wk after surgery. Her subjective visual deficits and pressure have also resolved. Advantages of the endoscopic approach include improved direct visualization of the lesion, lack of external skin incisions, avoidance of significant neurovascular retraction, and shorter hospital stays than alternative orbitotomy or craniotomy approaches. This 4-handed approach demands endoscopic expertise of 2 surgeons, and is often performed by rhinology-neurosurgery or rhinology-ophthalmology surgical teams. The risk of postoperative diplopia should be discussed with the patient during informed consent. ‡ Anatomic specimen photography courtesy Dr Peris-Celda.