Vismodegib for periocular basal cell carcinoma: an international multicentre case series.

INTRODUCTION: Vismodegib (Erivedge, Genentech) is a first-in-class inhibitor of the hedgehog (Hh) pathway, which is licensed for use in locally advanced basal cell carcinoma (BCC) and metastatic BCC. The National Institute for Health and Care Excellence withdrew recommendation for use of vismodegib secondary to a lack of data comparing vismodegib to standard supportive care. The purpose of this multicentre, international case series is to report outcomes of patients with locally advanced periocular BCC who have been treated with vismodegib. METHODS: The medical records of all patients treated with vismodegib were retrospectively reviewed across seven institutions in the United Kingdom, Australia, and New Zealand. RESULTS: Thirteen patients were identified. Seven (54%) patients were male. All BCCs were ill-defined, with seven (58%) having orbital involvement at presentation. Median treatment time was 7 months (range 2-36 months). Eleven out of 13 patients developed side effects, the most common being fatigue in six patients (46%). Median follow-up was 24 months (range 12-48 months). Complete response was found in 5/13 patients (38%) and a partial response in 8/13 patients (62%). Six patients had further surgery after vismodegib, with three classed as globe-sparing operations. Three patients developed recurrence (23%). Three patients (23%) ultimately underwent exenteration. DISCUSSION: This study demonstrates vismodegib to be a well-tolerated treatment which may, in some cases, facilitate globe-sparing surgery and hence avoid disfiguring operations such as exenteration. Uncertainty does remain regarding the long-term outcomes of patients treated with vismodegib.

Upper lid ptosis surgery: what is the optimal interval for the postoperative review? A retrospective review of 300 cases.

PurposeCorrection of upper eyelid ptosis is one of the most commonly performed oculoplastic procedures on the NHS but there is currently no data in the literature informing the surgeon of the optimal time for the first postoperative review. Our aim was to investigate how often a complication that warranted intervention occurred in the first 6 weeks after surgery and whether such a complication could have been predicted preoperatively.Patients and methodsA retrospective review was performed of 300 operations in 239 patients over a 9-month period at Moorfields Eye Hospital, London. Electronic medical record software was used to extract data regarding the timing of first postoperative review, complications, any return to theatre, and any underlying risk factors or co-morbidities.ResultsAt 1 week 44 % (133) cases were reviewed, 30% (89) at 2 weeks, 17% (50) at 3 weeks, and 9% (28) at 4 or more weeks. The overall complication rate at any time during the 6-week follow-up interval was 8%. The majority of these complications were minor (24 eyes, 8%) and 1 was major (0.3%). Of the 25 complications, an underlying risk factor was identified in 14 cases.ConclusionsThese data indicate that postoperative complications are very low in the absence of preoperative risk factors. In our institution, as the risk of overcorrection is low, most patients without risk factors for exposure (51% in this series) can safely be reviewed later than 1 week after surgery, but for those with risk factors earlier follow-up is warranted.

Long-term results of 405 refractory trigeminal neuralgia surgeries in 256 patients.

BACKGROUND AND OBJECTIVES: The initial response of trigeminal neuralgia to medication is about 69%. However drug therapy is ineffective in 25% of patients and about 8% become drug-intolerant. These patients proceed to surgical interventions, which require constant appraisal to determine their efficacy and acceptability. The purpose of this study was to evaluate the long-term outcome of surgical interventions for trigeminal neuralgia to offer a guide to patients and surgeons when choosing the right procedure for the appropriate patient and to investigate the effects of patients' and surgeons' preferences on the outcome. PATIENTS AND METHODS: The study design was consecutive case review. Participants were 256 consecutive patients with refractory trigeminal neuralgia, who underwent 405 surgical procedures to control trigeminal neuralgia. The main outcome measures were: the response rate, time to pain recurrence and surgical complications. 172 were fit for microvascular decompression (MVD), glycerol injection (GI) or radiofrequency thermocoagulation (RF) and were offered the choice between the three procedures; 95 went for MVD and 77 underwent either GI or RF. The choice between GI and RF was a surgical decision. RESULTS: The 3-year success rate was 54.8% in patients who underwent GI and 70.7% in patients who underwent percutaneous RF. In contrast 85.6% of patients who underwent MVD remained pain-free at 3 years. The complication rate following these surgical interventions was relatively low with no deaths. CONCLUSIONS: Surgical intervention for the treatment of refractory trigeminal neuralgia is effective and safe and should be considered in patients after failed medical therapy. Whilst MVD offered the best long-term outcome in this series, percutaneous GI or RF offered a safe and reliable alternative for those who chose to undergo these procedures.

Polyurethanes as potential substrates for sub-retinal retinal pigment epithelial cell transplantation.

Transplantation of cultured retinal pigment epithelial (RPE) cells under the failing macular is a potential treatment for age related macular degeneration. An important step in the development of this procedure is the identification of a suitable membrane on which to grow and transplant the cells. This paper evaluates the potential of using polyurethanes in this application since they possess several of the required properties, such as, flexibility, robustness, biostability and good biocompatiblilty although their hydrophobicity can limit cell adhesion. Three commercially available polyether urethanes (Pellethane, Tecoflex and Zytar) were evaluated in terms of their wettability using dynamic contact angle analysis and their ability to support a monolayer of functioning RPE cells (ARPE-19) . Furthermore Pellethane and Tecoflex were treated with a simple air plasma treatment and analysed as above. In the "as received condition" only a few RPE cells attached to the Pellethane and Tecoflex and remained clumped. RPE cells grew to confluence on the Zytar substrate by 7 days without further surface modification. Air gas plasma treatment of both Pellethane and Tecoflex increased the wettability of the surfaces and this resulted in the growth of a monolayer of well-spread RPE cells on both materials. Morphologically these cells grew with a normal 'cobblestone' phenotype. These results demonstrate the potential of these polyurethanes for this application.

Adjustable versus non-adjustable sutures for strabismus.

BACKGROUND: Strabismus, or squint, can be defined as a deviation from perfect ocular alignment and can be classified in many ways according to its aetiology and presentation. Treatment can be broadly divided into medical and surgical options, with a variety of surgical techniques being available, including the use of adjustable or non-adjustable sutures for the extra ocular muscles. There exists an uncertainty as to which of these techniques produces a better surgical outcome, and also an opinion that the adjustable suture technique may be of greater benefit in certain situations. OBJECTIVES: The objectives of this review were to examine whether adjustable or non-adjustable sutures are associated with a more accurate long-term ocular alignment following strabismus surgery and to identify any specific situations in which it would be of benefit to use a particular method. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials - CENTRAL on The Cochrane Library (which includes the Cochrane Eyes and Vision Group Trials Register) (Issue 3 2004), MEDLINE (1966 to July 2004), EMBASE (1980 to August 2004), LILACS (Latin American and Caribbean Literature on Health Sciences) (July 2004). We contacted experts in the field for further information. There were no language restrictions in the electronic searches. SELECTION CRITERIA: We planned to include only randomised controlled trials comparing adjustable to non-adjustable sutures for strabismus surgery. DATA COLLECTION AND ANALYSIS: No studies were found that met the inclusion criteria for this review. MAIN RESULTS: No studies were found that met the inclusion criteria for this review, therefore none were included for analysis. Results of non-randomised studies that compared these techniques are reported. AUTHORS' CONCLUSIONS: No reliable conclusions could be reached regarding which technique (adjustable or non-adjustable sutures) produces a more accurate long-term ocular alignment following strabismus surgery or in which specific situations one technique is of greater benefit than the other. High quality randomised controlled trials are needed to obtain clinically valid results and to clarify these issues.

Chordoma presenting as pseudoprolactinoma.

Chordomas are relatively rare neoplasms that appear to originate from benign remnants of primitive notochord. They account for about 0.15% of all intracranial neoplasm and are the most common of extradural neoplasms that involve the clivus. Although they present mostly with signs and symptoms of other intracranial lesions they rarely present with high prolactin levels mimicking prolactinomas. We report a case of chordoma presenting as a pseudoprolactinoma. We discuss the presentation, radiological features and treatment of this rare presentation.