Ewan G. Kemp

Improved survival rates in sebaceous carcinoma of the eyelid

AbstractPurpose To review the clinicopathological features, management, and survival rates for patients with sebaceous gland carcinoma of the eyelid, and to analyse the reasons for improved survival.Methods In the west of Scotland between 1975 and 2001, 32 cases were identified through the pathology index for sebaceous carcinoma. Pathology specimens were reviewed in all cases. The clinical data were obtained from hospital records and the database provided by the Regional Cancer Registry.Results A total of 22 female and 10 male patients were identified. Follow-up ranged from 6 months to 15 years, median of 4.8 years. Primary treatment involved excisional techniques in 27 cases, exenteration in two cases, enucleation in two cases, and two cases required adjuvant radiotherapy. Intraepithelial spread and masquerade presentations delayed the diagnosis. Poorly differentiated tumours were associated with an unfavourable outcome. Three patients developed local tumour recurrence and one metastatic disease. In all, 10 patients died from nontumour-related causes and one died from metastatic sebaceous carcinoma. The overall tumour mortality rate in the west of Scotland was 3%.Conclusions From this study, patients in the west of Scotland have a better prognosis than is indicated in the literature. Astute clinical suspicion and accurate histopathological diagnosis, together with radical and aggressive surgical approaches were key factors.

Secondary retinal changes associated with choroidal naevi and melanomas documented by optical coherence tomography

Aims: To establish the characteristics of secondary retinal changes associated with the presence of choroidal melanomas and choroidal naevi as documented by optical coherence tomography (OCT). Methods: Twenty patients with untreated choroidal melanoma and 40 patients with presumed choroidal naevi managed by regular observation were included in the study. OCT scans across the surface of the tumour and surrounding tissue were acquired for all participants. The appearance of retinal tissue on the OCT scans was considered to be abnormal if it did not display the well defined band structure characteristic of normal tissue on OCT scans. Results: Serous retinal detachments were observed in all patients with choroidal melanoma and 18 out of 20 also had abnormal retinal structure or intra-retinal splitting in the tissue overlying the tumour. Out of the 40 patients with presumed choroidal naevi, 12 had serous detachments and three had either abnormal retinal structure or intra-retinal splitting overlying the lesion. Conclusions: Secondary retinal changes associated with choroidal lesions can be documented by OCT. These changes were observed in most patients with choroidal melanoma included in the study group but were far less prevalent in the patients with presumed choroidal naevi. OCT is also able to identify the presence of small serous detachments before they become clinically visible. Follow up of these patients is required to determine whether the OCT results may be of use in the differential diagnosis of small choroidal lesions.

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%.

Impact on quality of life of botulinum toxin treatments for essential blepharospasm

purpose To determine the impact on quality of life of Botulinum toxin therapy for essential blepharospasm. methods A cross-sectional study was undertaken using the Glasgow Benefit Inventory, in the form of a postal questionnaire, to assess the subjective patient benefit of Botulinum toxin treatments for essential blepharospasm in 44 consecutive patients attending a specialist Botulinum toxin clinic. (The Glasgow Benefit Inventory generates a score from −100 (maximum harm) through 0 (no effect) to +100 (maximum benefit) for the intervention under investigation.) The Wilcoxon signed-ranks test was used to test the significance of the total scores and subdomain scores. results A high response rate of 36 out of 44 (81.8%) was achieved. The median total benefit score was +29.2 (97.1% CI = 16.7 to 38.9, p < 0.0001). The median subdomain scores were: general +43.8 (97.1% CI = 20.8 to 54.2, p < 0.0001), social 0.0 (97.1% CI = 0.0 to 16.7, p = 0.0019) and physical 0.0 (97.1% CI = 0.0 to 0.0, p = 0.4810). conclusions This study demonstrates significant quality of life benefit from Botulinum toxin therapy for essential blepharospasm, and justifies continued treatment.

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.

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.

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.

Lymphoepithelioma-like carcinoma of the eyelid: a report of two cases

Lymphoepithelioma-like carcinoma (LELC) of the skin is a rare malignant epithelial neoplasm, which resembles histologically the nasopharyngeal neoplasm of the same name.1,2 Similar tumours have been reported at a variety of sites including salivary gland, tonsil, thymus, stomach, and uterus. Those involving the skin usually present as a papulonodular lesion on the head or neck of patients above 50 years of age. Only one case originating in the eyelid has been previously described.3 We describe a further two cases and discuss the differential diagnosis. A 79 year old man presented with a fusiform swelling occupying the medial half of his right lower lid (fig 1A). This had developed 8 months previously and was gradually increasing in size. An ectropion repair had been performed on this lid 7 years before presentation. The patient underwent excision of the lesion with reconstruction of the lid using a pedicle flap. The excised lesion was submitted for histopathological examination. Figure 1  (A) Clinical photograph of case 1 showing a smooth …

Malignant melanoma of the conjunctiva with intraocular extension: a clinicopathological study of three cases

BackgroundMalignant melanoma of the conjunctiva is a rare tumour with an unpredictable behaviour, characterised by the high risk of local recurrence and metastatic spread. Intraocular extension of a conjunctival melanoma is rare. We present three cases of primary conjunctival melanoma with intraocular extension.MethodsThree patients presented with primary conjunctival melanomas, which either arose at or later involved the limbus on a background of primary acquired melanosis. Despite adequate primary local excision and adjuvant chemotherapy, all three patients experienced several recurrences, requiring further surgery. Two of the patients had excision of the tumour that required superficial keratectomy. One of these patients then required two further procedures in which the anterior chamber was breached.ResultsAll three patients ultimately required enucleation or exenteration to control local disease. All three specimens showed intraocular extension.ConclusionsThe management of conjunctival melanoma can be difficult. In addition to local recurrence and metastases, limbal melanomas may rarely show intraocular extension, particularly if surgery to excise the tumour requires the removal of Bowman’s membrane. Regular follow-up of these patients is mandatory.