Clinical and Imaging Outcomes After Trigeminal Schwannoma Radiosurgery: Results From a Multicenter, International Cohort Study.

BACKGROUND AND OBJECTIVES: An international, multicenter, retrospective study was conducted to evaluate the long-term clinical outcomes and tumor control rates after stereotactic radiosurgery (SRS) for trigeminal schwannoma. METHODS: Patient data (N = 309) were collected from 14 international radiosurgery centers. The median patient age was 50 years (range 11-87 years). Sixty patients (19%) had prior resections. Abnormal facial sensation was the commonest complaint (49%). The anatomic locations were root (N = 40), ganglion (N = 141), or dumbbell type (N = 128). The median tumor volume was 4 cc (range, 0.2-30.1 cc), and median margin dose was 13 Gy (range, 10-20 Gy). Factors associated with tumor control, symptom improvement, and adverse radiation events were assessed. RESULTS: The median and mean time to last follow-up was 49 and 65 months (range 6-242 months). Greater than 5-year follow-up was available for 139 patients (45%), and 50 patients (16%) had longer than 10-year follow-up. The overall tumor control rate was 94.5%. Tumors regressed in 146 patients (47.2%), remained unchanged in 128 patients (41.4%), and stabilized after initial expansion in 20 patients (6.5%). Progression-free survival rates at 3 years, 5 years, and 10 years were 91%, 86%, and 80 %. Smaller tumor volume (less than 8 cc) was associated with significantly better progression-free survival ( P = .02). Seventeen patients with sustained growth underwent further intervention at a median of 27 months (3-144 months). Symptom improvement was noted in 140 patients (45%) at a median of 7 months. In multivariate analysis primary, SRS ( P = .003) and smaller tumor volume ( P = .01) were associated with better symptom improvement. Adverse radiation events were documented in 29 patients (9%). CONCLUSION: SRS was associated with long-term freedom (10 year) from additional management in 80% of patients. SRS proved to be a valuable salvage option after resection. When used as a primary management for smaller volume tumors, both clinical improvement and prevention of new deficits were optimized.

Clinical and Imaging Response to Trigeminal Schwannoma Radiosurgery: A Retrospective Analysis of a 28-Year Experience.

Objective The purpose of this study was to evaluate long-term clinical outcomes and tumor control after stereotactic radiosurgery (SRS) for trigeminal schwannoma (TS). Methods During a 28-year period (1989-2017), 50 patients underwent SRS for TS. The median patient age was 51 years (range: 15-87 years). A total of 17 patients had a previous tumor resection: 10 had a single procedure, 5 had two procedures, and 2 had three procedures. The median and mean times between tumor resection and SRS were 12 and 24 months (range: 1-90 months), respectively. Four patients had neurofibromatosis II (NF2). Based on location, tumors were classified as root type (7), ganglion type (22), or dumbbell type (21). The median radiosurgery target volume was 3.4 cm 3 (range: 0.10-18 cm 3 ), median target dose was 14 Gy (range: 12-20 Gy), and the median number of isocenters was 6 (range: 1-15). The median and mean times to last follow-up was 36.9 and 55.2 months (range: 4-205 months), respectively. Eighteen patients (36%) had longer than 5-year follow-up, and seven patients (14%) had longer than 10-year follow-up. Results The tumor control rate was 92% and the clinical improvement or stabilization rate was 94%. After SRS, the rates of progression free survival (PFS) at 1, 5, and 10 years were 98, 84, and 84%, respectively. Factors associated with improved PFS were female sex ( p = 0.014) and smaller tumor volume ( p = 0.022). In this series, we did not find that tumor type (root, ganglion, and dumbbell) had a statistically significant correlation to PFS. Forty-seven patients had neurological signs or symptoms at presentation. At last follow-up, neurological signs or symptoms improved in 22/47 (47%), remained unchanged in 24/50 (48%), and worsened due to tumor progression in 3/50 (6%). One patient (2%) developed temporary symptomatic adverse radiation effect (ARE) and three additional patients (6%) had transient imaging evidence of peritumoral reactive edema but no new symptoms. Conclusion As a single outpatient procedure, SRS was associated with long-term freedom from additional management in 84% of patients. Nearly half the treated patients experienced improvement in neurological symptoms or signs.

Leksell Radiosurgery for Movement Disorders.

Tremor is the most prevalent movement disorder in adults. Patients who are refractory to medical management can explore surgical intervention. Deep-brain stimulation (DBS) and radiofrequency thalamotomy (RFT) are surgical procedures for intractable tremor that target the ventralis intermedius (VIM) nucleus to relieve contralateral tremor. For patients who are not candidates for surgical procedures, stereotactic radiosurgery (SRS) is a minimally invasive management option for tremor relief. SRS has been used for the elderly, those considered high surgical risk for other surgical procedures, those who are unresponsive to either DBS or RFT, and those who prefer a less invasive option. Radiosurgical thalamotomy is performed using a central dose of 130-140 Gy delivered to the VIM nucleus of the thalamus. The critical aspect of the dose planning procedure is the selection of the target, which requires a neurosurgeon experienced with movement disorder surgery. More than 90% of patients experience improvements in their tremor and quality of life after radiosurgical thalamotomy. Adverse radiation effects rates are low (4%). In selected cases, staged bilateral procedure can also be performed to relieve bilateral refractory tremor. In patients with tremor-predominant Parkinson's disease, VIM thalamotomy is also effective.

Is staged bilateral thalamic radiosurgery an option for otherwise surgically ineligible patients with medically refractory bilateral tremor?

OBJECTIVE Unilateral Gamma Knife thalamotomy (GKT) is a well-established treatment for patients with medically refractory tremor who are not eligible for invasive procedures due to increased risk of compications. The purpose of this study was to evaluate whether staged bilateral GKT provides benefit with acceptable risk to patients suffering from disabling medically refractory bilateral tremor. METHODS Eleven patients underwent staged bilateral GKT during a 17-year period (1999-2016). Eight patients had essential tremor (ET), 2 had Parkinson's disease (PD)-related tremor, and 1 had multiple-sclerosis (MS)-related tremor. For the first GKT, a median maximum dose of 140 Gy was delivered to the posterior-inferior region of the nucleus ventralis intermedius (VIM) through a single isocenter with 4-mm collimators. Patients who benefitted from unilateral GKT were eligible for a contralateral GKT 1-2 years later (median 22 months). For the second GKT, a median maximum dose of 130 Gy was delivered to the opposite VIM nucleus to a single 4-mm isocenter. The Fahn-Tolosa-Marin (FTM) clinical tremor rating scale was used to score tremor, drawing, and drinking before and after each GKT. The FTM writing score was assessed only for the dominant hand before and after the first GKT. The Karnofsky Performance Status (KPS) was used to assess quality of life and activities of daily living before and after the first and second GKT. RESULTS The median time to last follow-up after the first GKT was 35 months (range 11-70 months). All patients had improvement in at least 1 FTM score after the first GKT. Three patients (27.3%) had tremor arrest and complete restoration of function (noted via FTM tremor, writing, drawing, and drinking scores equaling zero). No patient had tremor recurrence or diminished tremor relief after the first GKT. One patient experienced new temporary neurological deficit (contralateral lower-extremity hemiparesis) from the first GKT. The median time to last follow-up after the second GKT was 12 months (range 2-70 months). Nine patients had improvement in at least 1 FTM score after the second GKT. Two patients had tremor arrest and complete restoration of function. No patient experienced tremor recurrence or diminished tremor relief after the second GKT. No patient experienced new neurological or radiological adverse effect from the second GKT. Statistically significant improvements were noted in the KPS score following the first and second GKT. CONCLUSIONS Staged bilateral GKT provided effective relief for medically refractory, disabling, bilateral tremor without increased risk of neurological complications. It is an appropriate strategy for carefully selected patients with medically refractory bilateral tremor who are not eligible for deep brain stimulation.

Stereotactic radiosurgery for medically refractory multiple sclerosis-related tremor.

OBJECTIVE Multiple sclerosis (MS) is a neurodegenerative disease that can lead to severe intention tremor in some patients. In several case reports, conventional radiotherapy has been reported to possibly exacerbate MS. Radiosurgery dramatically limits normal tissue irradiation to potentially avoid such a problem. Gamma Knife thalamotomy (GKT) has been established as a minimally invasive technique that is effective in treating essential tremor and Parkinson's disease-related tremor. The goal in this study was to analyze the outcomes of GKT in patients suffering from medically refractory MS-related tremor. METHODS The authors retrospectively studied the outcomes of 15 patients (mean age 46.5 years) who had undergone GKT over a 15-year period (1998-2012). Fourteen patients underwent GKT at a median maximum dose of 140 Gy (range 130-150 Gy) using a single 4-mm isocenter. One patient underwent GKT at a dose of 140 Gy delivered via two 4-mm isocenters (3 mm apart). The posteroinferior region of the nucleus ventralis intermedius (VIM) was the target for all GKTs. The Fahn-Tolosa-Marin clinical tremor rating scale was used to evaluate tremor, handwriting, drawing, and drinking. The median time to the last follow-up was 39 months. RESULTS After GKT, 13 patients experienced tremor improvement on the side contralateral to surgery. Four patients noted tremor arrest at a median of 4.5 months post-GKT. Seven patients had excellent tremor improvement and 6 had good tremor improvement. Four patients noted excellent functional improvement, 8 noted good functional improvement, and 1 noted satisfactory functional improvement. Three patients experienced diminished tremor relief at a median of 18 months after radiosurgery. Two patients experienced temporary adverse radiation effects. Another patient developed a large thalamic cyst 60 months after GKT, which was successfully managed with Ommaya reservoir placement. CONCLUSIONS Gamma Knife thalamotomy was found to be a minimally invasive and beneficial procedure for medically refractory MS tremor.

Stereotactic Radiosurgery for Intractable Tremor-Dominant Parkinson Disease: A Retrospective Analysis.

OBJECTIVE: The purpose of this study was to retrospectively analyze the outcomes of stereotactic radiosurgery for patients suffering from medically refractory Parkinson disease (PD) tremor. METHODS: We retrospectively studied the outcomes of 33 patients who were treated with gamma knife thalamotomy (GKT) over a 19-year period. Twelve patients were ≥80 years. A median dose of 140 Gy (range, 130-150 Gy) was delivered to the nucleus ventralis intermedius through a single 4-mm isocenter. We used the Fahn-Tolosa-Marin clinical tremor rating scale to score tremor, handwriting, drawing, and ability to drink fluids. The median time to the last follow-up was 23 months (range, 9-144 months). RESULTS: After GKT, 31 patients (93.9%) experienced improvement in tremor. Twenty-three patients (70.0%) had complete or nearly complete tremor arrest. Nine patients (27.2%) noted tremor arrest and resolution of impairment in writing, drawing, and ability to drink fluids. One patient (3%) improved in bradykinesia, 3 patients (9%) improved in rigidity, and 3 patients (9%) decreased their dosage of dopa after GKT. Tremor relief was fully maintained in the last follow-up for 96.8% of responding patients. Two patients (6%) experienced temporary adverse radiation effects. CONCLUSIONS: GKT is a safe and effective treatment for medically refractory PD tremor, especially for the elderly or those not suitable for deep brain stimulation or thermal thalamotomy.

Stereotactic radiosurgery for essential tremor: Retrospective analysis of a 19-year experience.

BACKGROUND: Essential Tremor (ET) is a common movement disorder that can be disabling. Initial treatment is in the form of medical therapies. Patients with medically refractory ET seek surgical intervention which include radiofrequency thalamotomy, deep brain stimulation, and radiosurgical thalamotomy. Radiosurgical thalamotomy is a minimally invasive surgical option which is especially valuable for elderly and high surgical risk patients. OBJECTIVE: The purpose of this study was to retrospectively analyze the outcomes of stereotactic radiosurgery for patients suffering from medically refractory essential tremor. METHODS: During a 19-year period (1996-2015), 73 patients underwent gamma knife thalamotomy for intractable essential tremor. A median central dose of 140 Gy (range, 130-150) was delivered to the nucleus ventralis intermedius through a single 4-mm isocenter. We used the Fahn-Tolosa-Marin clinical tremor rating scale to score tremor, handwriting, drawing, and ability to drink fluids. The median time to last follow-up was 28 months (range, 6-152). RESULTS: After gamma knife thalamotomy, 93.2% improved in tremor. Forty-four patients (60.3%) experienced tremor arrest or barely perceptible tremor. Eighteen patients (24.7%) noted tremor arrest and complete restoration of motor function. Tremor improvement was sustained at last follow-up in 96% of patients who experience tremor relief. Mean tremor score improved from 3.19 before to 1.27 after gamma knife thalamotomy (P < 0.0001). Mean handwriting score improved from 2.97 to 1.25 (P < 0.0001). Mean drawing score improved from 3.16 to 1.26 (P < 0.0001). Mean drinking score improved from 3.14 to 1.56 (P < 0.0001). Imaging follow-up showed three types of lesions: enhancing lesion, streaking along internal capsule on fluid-attenuated inversion recovery, and significant reactive changes. Three patients (4%) experienced temporary adverse radiation effects. CONCLUSION: Radiosurgery is a safe and valuable treatment option for medically refractory essential tremor, especially for the elderly or those with high surgical risk for DBS or radiofrequency thalamotomy. © 2017 International Parkinson and Movement Disorder Society.

Psoriasis and lasting implications.

Psoriasis is a chronic immunological disorder that affects the joints and skin. The disease is strongly correlated with genetic predisposition and environmental factors that can act as triggers of the innate and adaptive immune response. Once this immune response has begun many different signaling factors lead to inflammation and plaques which are visible throughout the body. As psoriasis progresses, it strongly affects the mental and physical health of the individual. This article reviews the different types of psoriasis, genetic and environmental triggers of psoriasis, the molecular progression of psoriasis, and current and future therapies.

Surgical management of ocular surface squamous neoplasms: the experience from a cornea center.

PURPOSE: To report the surgical outcomes of treatment for ocular surface squamous neoplasms. METHODS: Retrospective review of squamous neoplasms of the ocular surface managed at a cornea center over a 15-year period. Surgical treatment was divided into two methods. The procedure was identical within each group. One group of patients was treated with simple excision of the lesion. The second group of patients was treated with excision followed by focal cryotherapy to the involved limbus and/or conjunctival margin. Follow-up is reported. RESULTS: A total of 28 lesions were reviewed. Included were 20 primary tumors and 8 recurrent tumors referred following initial treatment at other centers. Histopathologic diagnoses included 1 actinic keratosis, 7 dysplasias, 18 carcinomas in situ, and 2 invasive squamous cell carcinomas. The rate of recurrence for primary tumors was 28.5% with simple excision and 7.7% for excision combined with cryotherapy (p = 0.27). The rate of recurrence for recurrent tumors was higher: 16.6% for tumors treated with excision and cryotherapy and 50% (one in two) for lesions treated with simple excision (p = 0.46). CONCLUSION: Simple excision of ocular surface squamous neoplasms appears to result in a higher recurrence rate when compared to excision with adjunctive cryotherapy. Judicious cryotherapy to the involved limbus and conjunctival margins results in recurrence rates comparable with other, more extensive cryotherapy procedures. This technique of cryotherapy has very few adverse effects on the eye.

Spectrum of fungal keratitis at Wills Eye Hospital, Philadelphia, Pennsylvania.

PURPOSE: To report the spectrum of fungal keratitis at Wills Eye Hospital, Philadelphia. METHODS: We reviewed the records of 24 cases of culture-positive fungal keratitis treated from January 1991 to March 1999 at Wills Eye Hospital. Risk factors, fungal identification, antifungal treatment, and outcomes were evaluated. RESULTS: The study included 24 eyes (24 patients). Fourteen patients (58.3%) were female. The mean age was 59 years (range, 19-86 years). Predisposing factors included chronic ocular surface disease (41.7%), contact lens wear (29.2%), atopic disease (16.7%), topical steroid use (16.7%), and ocular trauma (8.3%). Early identification of fungal elements was achieved by staining of corneal scrapings in 18 cases (75%). Half of the cases (12 eyes) had corneal infections caused by yeast, and the other half by filamentous fungi. Candida albicans was the most commonly isolated organism (45.8%), followed by Fusarium sp (25%). Natamycin and amphotericin B were the topical antifungals most frequently used, while systemic treatment commonly used included fluconazole, ketoconazole, or itraconazole. Six patients (25%) had penetrating keratoplasty during the acute stage of infection. After a mean follow-up of nine months, 13 eyes (54.1%) had the best corrected visual acuity 20/100 or better. CONCLUSIONS: In contrast to other studies from the northern United States, we found Fusarium sp the most commonly isolated filamentous fungus. In our series, C. albicans was the most frequent cause of fungal keratitis, and a past history of ocular trauma was uncommon.

The negative psychosocial impact of strabismus in adults.

INTRODUCTION: The goal in the treatment of strabismus is to realign the visual axes to eliminate diplopia, produce or restore binocular vision, expand the binocular visual field, and/or provide a normal appearance. The treatment of strabismus in adults who do not experience diplopia or who do not have binocular potential has sometimes been regarded as "cosmetic." Many adults with strabismus have stated that it has had a negative effect on their lives. This study was designed to ascertain whether there is a negative social bias against adult patients with strabismus that could affect interpersonal relationships and limit employment opportunities. METHODS: An orthotropic person was photographed in primary, right, and left gaze. These photographs were then digitally altered to produce an esotropia and exotropia of similar magnitude. Subjects were then asked to evaluate a single photograph with reference to personality characteristics that are important for social interaction and employment capability. RESULTS: Overall, the strabismic faces were judged significantly more negatively, across 11 descriptive characteristics, than the nonstrabismic face. The effect of esotropia was worse than exotropia. CONCLUSION: Strabismus creates a significant negative social prejudice. These biases can have a detrimental impact on socialization and employability. Treatment of strabismus may positively alter perceived characteristics of individuals and improve their ability to socialize normally and obtain employment. Therefore, the treatment of strabismus should not be considered cosmetic even when there is no hope of improving binocular vision.

The impact of the herpetic eye disease studies on the management of herpes simplex virus ocular infections.

Herpes simplex virus (HSV) is a leading cause of chronic infectious ocular disease in the United States. The morbidity from recurrent herpetic episodes is high, and the resultant corneal scarring may require penetrating keratoplasty for visual rehabilitation. Effective treatments for acute episodes of HSV have been verified by early Herpetic Eye Disease Study (HEDS) trials. The recent HEDS trial on the efficacy of oral acyclovir as prophylaxis against recurrent stromal keratitis represents the first report of a treatment likely to reduce long-term scarring from herpetic disease. This article reviews all the HEDS trials and the implications of their findings for the management of patients with ocular HSV.

The association between the oculocardiac reflex and post-operative vomiting in children undergoing strabismus surgery.

The oculocardiac reflex (OCR) is a potentially serious complication of ophthalmic surgery which is most commonly elicited during paediatric strabismus surgery. Post-operative vomiting (POV) is also extremely common after such procedures and may result in admission following planned day-case surgery. Although many factors play a part in the occurrence of POV, stimulation of the trigemino-vagal reflex arc is thought to explain the particularly high rate of vomiting after strabismus surgery. The OCR and the vaso-vagal response share this neuronal pathway, the bradycardia of the OCR often being the only objective feature of the vaso-vagal response while the patient is anaesthetised. The aim of this study was to investigate the possible association between the occurrence of the OCR and subsequent POV in children undergoing strabismus surgery. We have studied this relationship in 79 children, aged between 1 and 13 years, undergoing strabismus surgery under standardised anaesthetic conditions. A positive OCR was regarded as a drop in heart rate of 10% or more, or the onset of a dysrhythmia. An intraoperative OCR was elicited in 51 (64.6%) of the 79 children, whilst 29 (36.7%) developed POV in the subsequent 24 h period. There was a significant association between a positive intraoperative OCR and POV (p = 0.01): children with a positive OCR were 2.6 times more likely to vomit than those without the reflex. We conclude that there is an association between the occurrence of the OCR and POV and discuss possible preventive strategies.

Clinical features of bleb disorder of the cornea.

BACKGROUND: Bleb disorder of the cornea is a rare corneal epithelial disorder that has previously been described in asymptomatic patients or those with recurrent nontraumatic corneal erosions. METHODS: We report two cases of bleb disorder, each presenting with blurred vision from irregular astigmatism secondary to the bleb changes. We also report on the detection of bleb disorder in siblings. RESULTS: Both patients underwent surgical debridement of the epithelium with resolution of symptoms on epithelial resurfacing. CONCLUSION: The diagnostic clues, inheritance pattern, differential diagnosis, and treatment options of bleb disorder of the cornea are discussed.

Infection of the conjunctiva by Rhinosporidium seeberi.

Rhinosporidiosis is an unusual fungal disease that rarely affects the eye. It was first described as a pathogen in humans at the turn of the century. The etiologic agent, Rhinosporidium seeberi, commonly produces granulomatous inflammation of the upper respiratory mucosa. Most reported ocular infections have occurred in hot, dry climatic regions. We present a case of conjunctival rhinosporidiosis in an 11-year-old boy from western New York state, the first such report from the temperate zones of North America. The clinical characteristics, histopathologic features, and recommended therapeutic measures are discussed.

Audit of patients' awareness of ophthalmic diagnoses.

Providing information to patients about their medical condition and treatment options is important in medical management. To assess patients' knowledge of their ocular disease, prognosis, and treatment a questionnaire based survey was performed. 219 patients selected by random systematic sampling during six months from patients attending general ophthalmic clinics in Selly Oak Hospital, Birmingham, were questioned and 217 questionnaires were analysed. The findings showed that patients' knowledge of their diagnosis depended on their condition: patients with common conditions such as glaucoma and cataract had a better understanding of their condition, its treatment, and prognosis compared with patients with rarer conditions such as retinal detachment or patients with multiple diagnoses. In all, 152 patients (70%) wanted more information about their condition; 49 (23%) did not (although 12 (25%) had attempted to obtain information); and 16 (7%) were undecided. In view of the few patients with a precise understanding of their ophthalmic diagnosis and prognosis and the majority's wish for access to further information, that access needs improvement and different modes of disseminating the information should be implemented.

Accuracy of Goldmann tonometry in clinical practice.

The intra- and inter-observer variability of Goldmann applanation tonometry was studied in 8 hospital tonometrists, 4 of whom received brief revision training in tonometer use. Intraocular pressure (IOP) measurements were obtained from 16 glaucoma patients. Over a series of four readings taken from individual eyes, intraocular pressure did not significantly vary. However, a decline in IOP was observed when a second tonometrist obtained a further series of four readings from a previously measured eye. Mean pressures recorded by tonometrists who had received revision training were greater than those obtained by the untrained observers though this could be accounted for by two highly experienced individuals in the untrained group who tended to record low pressures. Comparisons of average readings obtained by two observers on the same eye showed that approximately one-quarter of measurements differed by 2 or more mmHg and approximately one-fifth by 3 or more mmHg.