Surgical intervention for paediatric blepharoptosis: a 6-year case series.

PURPOSE: To present our experience of paediatric blepharoptosis in a tertiary referral centre and evaluate the effectiveness of surgical intervention. METHODS: A retrospective cohort study of all children receiving surgical blepharoptosis correction between 1/1/10 and 29/2/16. Children with pre-operative levator function (LF) ≥ 7 mm received levator resection, those with LF ≤ 4 mm received brow suspension, and in those children with LF of 5-6 mm, either levator resection or brow suspension was chosen depending on the degree of frontalis recruitment. RESULTS: Ninety-five children (109 eyes, 64 boys) underwent blepharoptosis surgery within the study period. Mean (range) age at surgery was 5.9 (1.2-12.5) years. Seventy-nine (83.2%) had simple levator maldevelopment. Fifteen children were excluded due to inadequate follow-up. Of the remaining 80 children, 41 (51.2%) underwent levator resection, 27 (33.8%) underwent fascia lata brow suspension, and twelve (15.0%) underwent mersilene mesh brow suspension. Margin reflex distance-1 was greatest at 6-week follow-up with a small "lid drop" by 6-month follow-up in both the levator resection (0.9 mm pre-operatively, 3.1 mm at 6-week follow-up, 2.6 mm at 6-month follow-up) and fascia lata brow suspension (0.3 mm, 2.5 mm, 2.2 mm) groups. No immediate complications, and only two serious post-operative complications, were noted. One case of residual blepharoptosis was re-operated (fascia lata brow suspension). CONCLUSIONS: Surgical correction of paediatric blepharoptosis is safe and, after an observed lid drop between 6-week and 6-month follow-up (not seen in the mersilene mesh brow suspension group), effect appears to be maintained to 6 months and beyond. Readily accessible orthoptic assessment would help identify children at risk of amblyopia, both pre-operatively and post-operatively.

The intraoperative use of polydioxanone foil to reduce the risk of sino-orbital fistula formation in orbital exenteration.

BACKGROUND: Orbital exenteration is a radical surgical procedure resulting in deformity. It involves removal of the globe, optic nerve, extra-ocular muscles, orbital fat, lacrimal gland, and peri-osteum. Sino-orbital fistula (SOF) formation is a common documented post-operative complication, usually connecting the orbit and the ethmoid sinus. SOFs can cause leaks of serous fluid, and act as an entry site for pathogens into the orbit leading to socket infection and breakdown. METHODS: This retrospective study analyzed exenterations performed over a 22-year period (1993-2015) at the National Ocular Oncology Service Centre for Scotland. PDS is a crystalline, biodegradable polyether-ester that is strong with good shape-memory and flexibility. Orbital exenterations with and without the use of PDS foil were compared in terms of SOF formation. RESULTS: A total of 30 exenterations were performed during the study period. A total of 29 were analyzed. Choroidal malignant melanoma was the most common indication for performing orbital exenteration (n = 7, 24.14%). The most common post-operative complications seen were SOF (n = 8, 27.59%). A total of 8 out 21 (38.10%) cases not using PDS developed SOFs. By contrast, none of nine patients receiving PDS plates developed SOFs (p = 0.0332). CONCLUSIONS: This is the first study to compare SOF rate in patients undergoing exenteration with and without the use of PDS foil. PDS foil is a safe material, which has effectively reduced the incidence of SOF formation.

Pleomorphic adenomas of the lower eyelid: a case series.

Pleomorphic adenomas are well-recognized tumors usually arising within the main lacrimal gland. Their occurrence, however, is not limited to the main lacrimal gland. There have been cases reported in the eyebrow, upper eyelid, lateral lower eyelid, lacrimal sac, and even intraocularly. The medial aspect of the lower eyelid is a very rare site of occurrence because it is largely devoid of accessory lacrimal glands. We describe 2 cases of pleomorphic adenomas arising in the medial aspect and 1 case arising in the middle of the lower eyelid, respectively. The likely origin of these tumors at this location is from ectopic lacrimal gland.

Visual outcomes following orbital biopsy.

BACKGROUND: The risks of orbital biopsy depend on the lesion's location and relationship with surrounding structures. Complications include reduced vision, although visual outcomes following orbital biopsy are not widely reported. AIMS: To determine visual outcomes following orbital biopsy in Gartnavel General Hospital's Oculoplastic and Oncology Service. METHODS: Case note review of 50 consecutive patients undergoing orbital incision or excision biopsy between January 2006 and December 2010. Data collected included preoperative clinical examination, radiological and histological features, preoperative and postoperative corrected distance visual acuity (CDVA) and surgical complications. The main outcome measure was change in CDVA. Mean follow-up duration was 1.32 years. RESULTS: Histological diagnoses following biopsy included idiopathic orbital inflammation (n = 13) and lymphoma (n = 9). Of the radiologically defined lesions, 86.7% were extraconal (13.3% intraconal). Extraconal lesions were anterior in 59.0% (41.0% posterior). Mean preoperative LogMAR CDVA was 0.10 which was maintained at day one post-biopsy, indicating the absence of immediate sight-threatening complications such as retrobulbar haemorrhage or optic nerve compression, and there was no significant reduction at one-year follow-up (p = 0.239). Further analysis of change in CDVA showed no difference between: anterior and posterior lesions; extraconal and intraconal lesions; incision and excision biopsies; anterior and lateral surgical approaches. CONCLUSIONS: CDVA is retained for one year following orbital biopsy. Significant visual loss is a very rare complication of this procedure.

Low dose orbital radiotherapy for thyroid eye disease.

PURPOSE: The use of low dose orbital radiotherapy for thyroid eye disease (TED) remains controversial. This is a review of patients with TED treated with radiotherapy in our department over the last twelve years. METHODS AND MATERIALS: Fifty-nine consecutive patients received low dose orbital radiotherapy of 20 Gray (Gy) for active TED at the Beatson Oncology Centre. Their records were retrospectively reviewed and data including duration of symptoms, clinical activity score (CAS) pre- and post-orbital radiotherapy, immunosuppression requirement, subjective assessment and range of rehabilitative surgery was collected. RESULTS: Before orbital radiotherapy, all fifty-nine patients had an initial response to glucocorticoids and therefore presumed to have active phase thyroid eye disease. They received retro-orbital radiotherapy of 20 Gy in 12 fractions over 2 weeks. After treatment, only five patients remained on steroids and only one patient had CAS ≥ 3 at last follow up. Response (change in CAS) to orbital radiotherapy was statistically significant from 3.17 ± 1.75 standard deviation (SD) to 0.73 ± 0.92 SD (P < 0.001) at mean follow up of 6.5 months. There was a reduction in CAS at each subsequent follow up for all subgroups when the patients were grouped according to disease severity i.e. mild TED(CAS 1-2), moderate (CAS 3-4), severe CAS (5-7) and optic neuropathy. CONCLUSIONS: We believe orbital radiotherapy has a definite role to play in the treatment of active thyroid eye disease. The majority of our patients experienced a reduction in their clinical activity scores after orbital radiotherapy and this was irrespective of the severity of thyroid eye disease.

Recurrent choroidal melanoma after transscleral local resection with diffuse vitreous seeding.

BACKGROUND: Choroidal melanoma usually spreads systemically via the vascular system or extraocularly through channels in the sclera. Intraocular spread through vitreous seeding is uncommon. METHODS: We present two patients in whom melanoma recurred following transscleral local resection and widespread fine patchy pigmentation of the retina was identified grossly. The eyes were submitted for histological examination. RESULTS: In one case there was recurrent melanoma in the surgical coloboma with diffuse vitreous seeding and a pre-retinal membrane composed of tumour cells. Tumour cells were also identified in the anterior chamber. In the second case there was no tumour in the coloboma although two small satellite choroidal nodules were identified. Tumour cells were also present in the vitreous and covering the surface of the retina. CONCLUSIONS: We described two cases of recurrent choroidal melanoma following transscleral local resection with vitreous seeding. Intraocular seeding following local resection is uncommon. There are several potential mechanisms for this although it seems likely that in these cases loss of the retinal barrier assisted in the intraocular spread of these tumours.

Successful medical treatment of blepharochalasis: a case series.

INTRODUCTION: Blepharochalasis describes recurrent attacks of idiopathic transient painless oedema in the adnexal tissue of the orbit with the most severe reaction in the skin and orbicularis region. This may produce permanent tissue changes associated with thinning of the eyelid skin. Previous reviews have concentrated on surgical correction of the excess skin and orbicularis created by the condition. There has, as yet, been no report of successful medical management. PATIENTS AND METHODS: We report here on 4 males and 2 females aged between 37 and 78 years. Systemic acetazolamide (initially 250 mg SR OD), was used in combination with a topical steroid (hydrocortisone cream). RESULTS: All 6 patients achieved relief of their symptoms, with decreased incidence of occurrence and duration of episode if not complete resolution. CONCLUSION: Oral Acetazolamide in combination with topical hydrocortisone provides effective relief of the periorbital oedema associated with blepharochalasis. In our short series, the cases were improved either with medical therapy alone or in combination with surgery. Relapses following surgery were diminished as a result of prior medical therapy.

Uveal melanoma-associated survival in Scotland.

BACKGROUND/OBJECTIVES: The Scottish Ocular Oncology Service (SOOS) manages all patients with uveal melanoma (UM) in Scotland. Our aim was to determine the long-term all-cause and cause-specific survival of patients with UM, irrespective of treatment modality. SUBJECTS/METHODS: A retrospective single-centre cohort study including all patients diagnosed with UM by the SOOS between 1/1/1998 and 31/12/2002. Data from the SOOS database were correlated with death records held by National Records of Scotland, which provided date, and all listed causes, of death for all deceased patients. RESULTS: Two hundred and eighteen patients were newly diagnosed with UM between 1/1/1998 and 31/12/2002. One hundred and fifteen (52.8%) were female. The mean (median) age at diagnosis was 63(65) years (range: 16-89). Of 179 choroidal melanomas, melanoma-specific survival was 92.3%, 87.4% and 83.8% at 5, 10 and 15 years, respectively. Cancer-specific survival was 85.8%, 71.8% and 62.3%. All-cause survival was 81.6% (146/179), 62.0% (111/179) and 46.7% (computed, fraction inexpressible). Of 26 ciliary body melanomas, melanoma-specific survival was 87.2%, 81.3% and 81.3% at 5, 10 and 15 years. Cancer-specific survival was 62.9%, 40.6% and 40.6%. All-cause survival was 61.5% (16/26), 38.5% (10/26) and 26.9% (7/26). Of 13 iris melanomas, at all three timepoints (5/10/15 years), melanoma-specific survival was 100%, cancer-specific survival was 92.3%, and all-cause survival was 76.9% (10/13). CONCLUSIONS: Correlation of SOOS and national records survival data confirms 15-year melanoma-specific survival of 83.8%, 81.3% and 100% for choroidal, ciliary body and iris melanomas, respectively. We can now provide accurate survival data to our patients in Scotland.

Use of Ruthenium-106 Brachytherapy for Iris Melanoma: The Scottish Experience.

PURPOSE: To analyse long-term outcomes of ruthenium-106 (106Ru) plaque brachytherapy for the treatment of iris melanoma. METHODS: We retrospectively reviewed medical records of 19 consecutive patients with pure iris melanoma treated with 106Ru plaque brachytherapy between 1998 and 2016 at the Scottish Ophthalmic Oncology Service, Glasgow. The iris melanoma was treated with a ruthenium plaque placed on the corneal surface to deliver a surface dose of 555 Gy. We analysed vision preservation, local tumour control, radiation-related complications, eye retention rates, symptomatic metastasis and melanoma-related mortality. RESULTS: The mean largest basal diameter of the lesions was 3.50±1.42 mm (range 1.6-6.5 mm), and the mean maximum height was 1.47±0.65 mm (range 0.7-2.8 mm). The tumour control and eye retention were 100% at a mean follow-up of 62 months (range 6-195 months). A 62% reduction in tumour height was observed on ultrasonography. Complications included cataract (68%), dry eye (47%), uveitis (37%) and scleral thinning (5%). At the final follow-up visit, the mean loss of Snellen visual acuity was 1.11±2.90 lines and vision of 6/9 or better was maintained in 53% of patients. None of the patients had evidence of symptomatic metastasis (non-imaged) or melanoma-related mortality. CONCLUSIONS: 106Ru plaque treatment for iris melanoma was highly effective a high tumour control, no tumour recurrences and a relatively a low complication rate.

The T1799A BRAF mutation is present in iris melanoma.

PURPOSE: An activating mutation in exon 15 of the BRAF gene has been found in a high proportion of cutaneous pigmented lesions, but only in one case of uveal melanoma. Iris melanoma is the least common uveal melanoma and displays a less aggressive clinical course compared with posterior uveal melanoma. To date, no study has been conducted to investigate the T1799A mutation in iris melanoma. The purpose of this study was to determine whether the T1799A BRAF mutation is present in iris melanoma. METHODS: DNA was extracted from 19 archival, paraffin-embedded tissue sections of iris melanomas. Nested PCR was used to amplify exon 15 of the BRAF gene, and the product was purified, cloned into a sequencing vector, and sequenced. The sequences obtained were compared with the wild-type sequence of the BRAF gene. The presence or absence of the BRAF mutation was also compared with the clinicopathological features. RESULTS: The T1799A mutation was identified by sequencing in 9 of 19 iris melanomas. Six of the 9 cases with the BRAF mutation were recurrent tumors. All other tumors were resections for primary tumors. There was a statistically significant association between the BRAF mutation and recurrent tumor (P = 0.003). There was no association between the presence of the BRAF mutation and other clinicopathological characteristics. CONCLUSIONS: In this small study, the T1799A BRAF mutation was identified in almost half of the iris melanoma tissues samples examined. This finding suggests that there may be genetic as well as clinical differences between iris and posterior uveal melanomas.

BRAF mutations in conjunctival melanoma.

PURPOSE: An activating mutation in exon 15 of the BRAF gene has been found in a high proportion of cutaneous melanomas and cutaneous nevi but not in uveal melanoma. Conjunctival melanoma shows greater clinical similarity to cutaneous melanoma than does uveal melanoma. The purpose of this study was to determine whether the T1799A BRAF mutation found in cutaneous melanoma is also present in conjunctival melanoma. METHODS: DNA was extracted from paraffin sections obtained from glutaraldehyde or formalin-fixed, paraffin-embedded conjunctival melanomas. Forty-two specimens were identified from 25 patients. Seminested PCR was used to amplify exon 15 of the BRAF gene, and the resultant PCR product was purified and directly sequenced. Sequences from conjunctival melanomas were compared with the wild-type sequence of the BRAF gene. The presence or absence of the BRAF mutation was compared with the clinicopathological features. RESULTS: The T1799A (V600E) mutation was detected by sequencing in melanomas from 5 of 22 patients as well as in the positive control, a cutaneous melanoma cell line. In this small series, no statistically significant associations between the presence of the BRAF mutation and clinicopathological characteristics were detected, although tumors with this mutation tended to have a larger diameter and greater depth of invasion and to contain epithelioid cells. CONCLUSIONS: Others have demonstrated a BRAF T1799A-activating mutation in cutaneous but not uveal melanoma. In this study, this BRAF mutation was demonstrated in some conjunctival melanoma tissue samples, suggesting that some conjunctival melanomas may share biological features in common with cutaneous melanoma.

Lymphomatoid papulosis of the eyelid.

Lymphomatoid papulosis (LyP) is a chronic papulonecrotic or papulonodular skin disease, with histological features suggestive of malignant lymphoma. It was originally described by Macauley (1968) as a "self-healing paradoxical eruption, histologically malignant but clinically benign." Unless accompanied by systemic lymphoma, most patients have no constitutional symptoms, and physical findings are limited to the skin. The distribution of lesions is characteristically on the trunk or extremities, but the face, scalp, or oral mucosa may be involved. We report an unusual case of lymphomatoid papulosis of the eyelid. To the best of our knowledge, this is the first report of eyelid involvement by lymphomatous papulosis.

Proton beam therapy for the treatment of uveal melanoma in Scotland.

BACKGROUND AND AIMS: In Scotland, uveal melanomas are primarily treated with eye-preserving modalities with a low primary enucleation rate. Proton beam therapy (PBT) is reserved for those not suitable for brachytherapy. The authors' aim was to review the outcome of Scottish patients undergoing treatment for uveal melanoma with PBT. METHOD: All ciliary body or choroidal melanomas treated initially with PBT between 1 January 1993 and 30 March 2008 were identified. Data were gathered from retrospective review of case notes, database information, PBT planning data, ultrasound and pathology reports. RESULTS: 147 patients were identified. The mean age was 62.3 years, 94.6% were choroidal melanomas. 97.8% of all treated melanomas were of medium or large size. Enucleation during follow-up occurred in 22.4%. Mean time to this was 23.8 months and main reasons were suspected recurrence (48%) and neovascular glaucoma (42%). Metastatic death occurred in 9.5%, the mean time to this was 28.9 months. The actuarial 5-year eye retention rate was 71.3% and disease-specific survival rate was 87.7%. CONCLUSION: PBT is reserved for the treatment of mainly medium-sized and large-sized uveal melanomas in Scotland. The eye retention and disease-specific survival rates confirm its suitability as an eye-preserving primary treatment in these patients. It is evident that PBT is a conservative treatment option for uveal melanoma, including those of larger size.

Treatment review of sight threatening circumscribed choroidal haemangioma.

AIM: To describe our clinical experience in treating circumscribed choroidal haemangioma (CCH) in a tertiary referral centre over a fifteen year period prior to photodynamic therapy. METHODS: The departmental database and photographic records of a tertiary referral center were used to identify patients who were treated for CCH between 1992 and 2007. Their case records were reviewed. RESULTS: Visual acuity improved (>2 Snellen lines) in eleven patients (69%) remained stable in one patient (6%) and deteriorated in four patients (25%). Six of the seven treated with brachytherapy and three of the four treated with transpupillary thermotherapy achieved better visual acuity after treatment. 86% of patients treated within six months of onset of symptoms and 50% of patients treated after six months of onset of symptoms noted an improvement in visual acuity. Only one patient in our series had a final VA of 6/60 or worse. Mean follow-up was thirty-five months. CONCLUSION: Visual outcome is better when treatment is performed within 6 months of symptoms. The majority of patients achieved an improvement in visual acuity without any adverse effect following treatment.