Incidence and indications for pars plana vitrectomy following the treatment of posterior uveal melanomas in Scotland.

BACKGROUND: Globe-sparing treatments such as plaque brachytherapy, local or endoresection, and proton beam therapy (PBT) are the treatments of choice for posterior uveal melanoma. However, both early and late complications can arise from these techniques, including vitreous haemorrhage (VH) and retinal detachment (RD). Choroidal melanomas in Scotland are managed by a single unit, the Scottish Ocular Oncology Service (SOOS). METHODS: Indications and outcomes from surgery were analysed for patients undergoing vitrectomy following treatment for uveal melanoma in the SOOS between 1998 and 2013. RESULTS: Seventeen from 715 cases (2.4%) required vitrectomy, of which 8/445 (1.8%) followed plaque brachytherapy, 7/43 (16.3%) combined local resection and brachytherapy, and 2/227 (0.9%) PBT. Case notes were reviewed for 16/17 cases, with surgery indicated for VH in 10 (63%), RD in 5 (31%), and combined VH/RD in 1 (6%). The median interval from initial tumour treatment to vitrectomy was 5.8 months (range 10 days to 8.8 years). Ten (63%) required early vitrectomy (within 6 months), of which the majority (70%) followed combined resection/brachytherapy. Six (37%) required late vitrectomy (after 6 months), of which all were non-clearing VH following plaque brachytherapy, with proliferative retinopathy in 4/6 (67%), and tumour recurrence in 2/6 (33%). Overall vision improved in 8 eyes (50%), remained the same in 2 (12.5%), and deteriorated in 6 (37.5%). CONCLUSIONS: Early vitrectomy was most commonly indicated for RD following local resection, and late vitrectomy for VH due to radiation retinopathy. The majority of patients undergoing vitrectomy gained or maintained vision.

Topical mitomycin C chemotherapy in the management of ocular surface neoplasia: a 10-year review of treatment outcomes and complications.

INTRODUCTION: The use of topical mitomycin C (MMC) has gained popularity in the management of ocular surface neoplasia. The aim of this study is to determine outcomes and complications following such treatment. METHODS: This study is a retrospective review of patients treated with topical MMC for ocular surface neoplasia, including primary acquired melanosis (PAM), melanoma, corneal-conjunctival intraepithelial neoplasia (CCIN), squamous cell carcinoma (SCC) and sebaceous gland carcinoma (SGC). Data regarding diagnosis, short- and long-term outcomes, and short- and long-term complications, were recorded. RESULTS: 58 patients were identified, with a mean age of 63 years and mean follow-up of 36 months. 21 received MMC as primary therapy and 37 as surgical adjuvant. The regimen was 0.04% MMC four times a day for 3 weeks on, 3 weeks off, 3 weeks on, with topical steroid and lubricants throughout. Initial clinical response was either partial or complete in 93%. Overall, 26% developed recurrent disease at a mean of 13 months post treatment. Recurrence rates by pathology were 20% PAM, 25% melanoma, 0% CCIN, 67% SCC and 57% SGC. Short-term complications occurred in 52%, but only 7% required treatment cessation. Long-term complications such as persisting keratoconjunctivitis, epiphora and corneal problems, occurred in 31%. CONCLUSION: The results confirm the effectiveness of topical MMC chemotherapy in the management of ocular surface neoplasia. Self-limiting short-term complications were common; however, limbal stem cell deficiency appears to be a significant long-term complication of treatment, occurring in 12%.

The T1799A point mutation is present in posterior uveal melanoma.

An activating mutation in exon 15 of the BRAF gene is present in a high proportion of cutaneous pigmented lesions. Until recently this mutation had however only been identified in one case of posterior uveal melanoma. Despite this apparent lack of the BRAF mutation, inappropriate downstream activation of the Ras/Raf/MAPK pathway has been described in posterior uveal melanoma. Based on the already recognised morphological and cytogenetic heterogeneity in uveal melanoma, we hypothesised that the BRAF mutation may be present in uveal melanoma but only in some of the tumour cells. In this study, we analysed 20 ciliary body and 30 choroidal melanomas using a nested PCR-based technique resulting in the amplification of a nested product only if the mutation was present. This sensitive technique can identify mutated DNA in the presence of wild-type DNA. The mutation was identified in 4 of 20 (20%) ciliary body and 11 of 30 (40%) choroidal melanomas. Further analysis of separate areas within the same choroidal melanoma demonstrated that the mutation was not present in the entire tumour. In conclusion, the T1799A BRAF mutation is present in a proportion of posterior uveal melanomas but within these tumours the distribution of the mutation is heterogeneous.

Oral acetazolamide: A treatment option for blepharochalasis?

This is a report of a middle-aged male with blepharochalasis, who was successfully treated with oral acetazolamide. To our knowledge, this is the first case in the literature reporting the benefits of the use of acetazolamide in the treatment of blepharochalasis.

Orbital exenteration--a retrospective study over an 11 year period analyzing all cases from a single unit.

PURPOSE: A retrospective analysis of all cases of orbital exenteration performed at the National Scottish Ocular Oncology Center over an 11 year period. METHODS: We sought all exenteration cases (1993-2003) from ocular oncology and oculoplastic databases. Fifteen exenterations were performed. One case record was irretrievable. Clinicopathological features and outcome of 14 patients were analyzed. All operations were performed by the same consultant. RESULTS: The male to female ratio was 1:1 and the average age at surgery was 66 years. There was a left sided predominance of pathology demonstrated (right to left orbit ratio was 1:1.3. The average follow up period postsurgery was 35 months. The most common underlying diagnosis was malignant melanoma (9/14). Ocular/orbital discomfort was the most frequent presenting symptom (7/14); a mass lesion (10/14) was the most common presenting sign. The average duration of symptoms/signs prior to tertiary institution referral was 10.9 months (range: 0.5-60 months). Exenteration was performed on average 39.7 months (range: 0.13-204) after initial tertiary center presentation. This included patients managed from the outset who failed treatment(s) and then required exenteration. The eyelids were sacrificed in ten cases (10/14). Sockets were lined with eyelid skin (4/10), split skin (thigh) (3/10) or healed secondarily (7/10). Thirteen cases (13/14) had clear histological margins. The average post operative stay was 8.3 days. The most common complication was socket fistula formation (7/14). Ethmoid sinuses were always involved. The average time to fistula development was 4.9 months (sockets lined by skin = 2.2 months compared with sockets left to heal secondarily = 6.9 months). The majority of patients wore occlusive shields long term. CONCLUSION: The most common pathology necessitating exenteration was malignant melanoma. Most patients presented with a mass lesion. Histological tumor free margins were obtained in thirteen of fourteen cases. The most common complication was socket fistula formation.

Screening for ophthalmic involvement in asymptomatic patients with metastatic breast carcinoma.

BACKGROUND: Breast carcinoma metastasises to the eye more frequently than is clinically recognised. The incidence is perhaps not appreciated, either because of the more common involvement and consequences of spread to major organs (such as lung, liver, or bone) or because a number of eye lesions are small and asymptomatic. Over a 6-month period, all patients with locally advanced or metastatic breast cancer were screened for ocular involvement and as a result management recommendations made. MATERIALS AND METHODS: Between January 2001 and June 2001, 68 patients with known locally advanced or metastatic breast carcinoma were referred for a screening ophthalmic examination. The aim of the study was to assess the frequency of asymptomatic ocular metastases by breast carcinoma in visually asymptomatic patients. The recognition and early treatment of both ocular metastases and ocular manifestations of metastatic breast carcinoma are important in maximising the quality of life in this group of palliative patients. These patients were all referred and recruited from the Beatson Oncology Centre and Breast Unit at the Western Infirmary, Glasgow by the oncologist (ANH). Examination included visual acuity assessment, slit-lamp examination, tonometry, and indirect ophthalmoscopy. RESULTS: The median time from diagnosis of breast carcinoma to ophthalmic screening was 5 years (range 6 months-23 years). No patient had any evidence of choroidal metastases on ophthalmic examination. Four patients (5.8%) had ophthalmic manifestations of metastatic breast carcinoma and a further two had ocular complications of treatment. One patient had a restrictive motility problem from a metastatic deposit to her lateral rectus muscle and another had corneal punctate epitheliopathy secondary to a seventh nerve palsy. A further patient had coarse nystagmus from cerebellar metastases and the final patient of the four had a Horner's syndrome from metastases in the neck. In addition, two patients had symptomatic dry eyes whose onset coincided with commencement of chemotherapy. CONCLUSION: Ophthalmic manifestations of metastatic breast carcinoma occurred in 5.8% of asymptomatic patients. Orbital metastases were documented in one patient. No case of choroidal metastases was observed in this group with advanced or metastatic disease. Therefore, patients do not need to be routinely screened particularly for choroidal metastases.

Preoperative topical and intraoperative local mitomycin C adjuvant therapy in the management of ocular surface neoplasias.

AIMS: To demonstrate the efficacy of mitomycin C as adjuvant therapy preoperatively and intraoperatively in the management of recurrent or diffuse ocular surface neoplasias. METHODS: The case notes of 11 patients receiving mitomycin C adjuvant therapy as 0.04% eye drops four times a day in two weekly courses preoperatively and/or a single intraoperative application of 0.4 mg/ml of mitomycin C were reviewed. The histopathology included conjunctival primary acquired melanosis, conjunctival melanomas, sebaceous cell carcinomas with conjunctival intraepithelial spread, and conjunctival intraepithelial squamous neoplasias. Seven patients had additional limited local excision of the residual tumour mass and one had cryotherapy. RESULTS: All cases showed a favourable response to mitomycin C adjuvant therapy with regression in size or retardation of a rapid growth pattern and no serious sequelae. Postoperative follow up of 6-36 months following excision of the lesion with or without intraoperative mitomycin C showed no clinical recurrence in any of the cases. CONCLUSION: In this series, mitomycin C adjuvant therapy of recurrent or diffuse ocular surface neoplasias was well tolerated and showed favourable clinical results.

A review of the outcome of upper lid lowering for eyelid retraction and complications of spacers at a single unit over five years.

AIM: To review the outcome of upper lid lowering for eyelid retraction at a single unit over five years and to report the high complication rate with mersilene mesh as a spacer material. METHODS: We retrospectively reviewed the case records of 48 patients who underwent upper lid lowering for lid retraction between January 1995 and January 2000. RESULTS: The indication for surgery was eyelid retraction secondary to dysthyroid disease in 41 patients, seventh nerve palsy in 3 cases, overcorrection of ptosis elsewhere in 3 cases and previously treated orbital lymphoma in one patient. A good result was achieved in 80% with a single procedure. Undercorrection occurred in 10% and overcorrection occurred in 8%. Spacer material, either mersilene mesh or more recently vicryl, was used in 42% of lids. Late extrusion of mersilene mesh occurred in 20% of lids that had a spacer inserted an average of 18 months postoperatively. CONCLUSIONS: The high extrusion rate with mersilene was unacceptable and the alternative spacer material vicryl is now used. The possibility of revisional surgery because of under- or overcorrection should be explained to patients.

Mersilene mesh as an alternative to autogenous fascia lata in brow suspension.

PURPOSE: To evaluate the outcome and complications of brow suspension surgery for ptosis repair performed with Mersilene mesh as the suspensory material. METHODS: A retrospective review was undertaken of all patients undergoing Mersilene mesh brow suspension surgery during a 9-year period. All patients had undergone the same surgical procedure performed by the same surgeon. Preoperative and postoperative palpebral aperture heights were recorded, and the results were analyzed to provide a mean change in palpebral aperture height. The case records of patients with a poor response to surgery were examined carefully, and possible risk factors for a poor outcome were identified. RESULTS: The mean (95% confidence interval) increase in palpebral aperture height was 1.8 mm (1.2-2.4 mm). Factors associated with a poorer outcome were previous ptosis surgery and Marcus Gunn jaw-winking syndrome. CONCLUSIONS: Mersilene mesh has advantages over other materials for use as a suspensory material in brow suspension surgery, and is associated with a good outcome and low complication rate.

Metastatic breast cancer presenting as Tolosa-Hunt syndrome.

Choroidal involvement is a well-recognized manifestation of metastatic disease, particularly from breast and lung primaries. However, breast cancer can involve other ocular structures. The two patients presented illustrate diffuse orbital involvement of the extraocular muscles, simulating Tolosa-Hunt syndrome. This association has not been reported previously. Both patients responded well to local radiotherapy.

Abnormal extracellular material in the levator palpebrae superioris complex in congenital ptosis.

OBJECTIVE: To determine the pathologic abnormalities of the levator palpebrae superioris in congenital ptosis. METHODS: By means of light and electron microscopy and immunohistochemistry, anterior levator tissue specimen from 15 patients with congenital ptosis excised during routine levator resections were examined. RESULTS: All specimens showed lack of muscle fibers with endomysial and perimysial fibrosis and thickening of the aponeurosis. In addition, in four of the 15 patients, an abnormal extracellular material was present. By light microscopy this appeared as an unusual amorphous material arranged in clumps and bands, and electron microscopy showed it to consist of parallel coarse bundles of fibrillogranular material. Collagen type III and fibronectin were identified within this material by immunohistochemistry. There was no detectable collagen types I, II, IV, V, VI, or VII or laminin, and the material did not stain for actin, myosin, myoglobin, amyloid P component, or amyloid A. CONCLUSIONS: In four of 15 samples of levator palpebrae superioris from patients with congenital ptosis, we identified an unusual amorphous extracellular material that stained positively for collagen type III and fibronectin on immunohistochemistry. This novel material, which we call "amorphocollagenoid," may represent a product of dysgenesis of the levator tissues. The source and full composition of this material merit further study.

The routine use of adjustable sutures in adult strabismus surgery.

A review is given of the adjustable suture technique in adult strabismus surgery, and our experience with its use over a 4-year period. Twenty patients underwent adjustable surgery during that time, both for cosmetic reasons and for alleviation of diplopia. Postoperative adjustment in the alert patient enabled the eyes to be aligned in the most favourable position. The stability of alignment was good, and a success rate of 85% was achieved over a mean follow-up period of 17 months. Our results demonstrate the value of the technique, even when undertaken in a non-specialist ophthalmological department of a district general hospital. We now offer adjustable sutures routinely to all adults able to cooperate, to safeguard against the unpredictable nature of standard strabismus surgery.

Surgical management of upper lid entropion.

One hundred and eighty-three surgical procedures were conducted on 107 patients over seven years. 91% of the cases of upper lid entropion were corrected satisfactorily with only one operation. It is postulated that this level of success is achieved by grading the degree of surgical intervention according to the clinical established on systematic examination of upper lid entropion.