John T Lucas, Rose McGee, Catherine A Billups, Ibrahim Qaddoumi, Thomas E Merchant, Rachel C Brennan, Jiangrong Wu, Matthew W Wilson
Background/aims Non-irradiative local therapies have shown promise in delaying or supplanting external beam radiotherapy (EBRT) and enucleation in patients with retinoblastoma. We hypothesised that prior focal therapy does not compromise the efficacy of delayed episcleral plaque brachytherapy (epBRT).
Methods We performed an institutional review board-approved medical record review of patients with retinoblastoma who were treated with I-125 epBRT prior to (primary) or following chemoreduction (delayed), alone and in combination with non-irradiative focal therapy. Clinical and treatment characteristics were retrieved. Treatment failure was defined as the need for subsequent EBRT and/or enucleation. Event-free and ocular survival rates were calculated from the date of plaque placement. The cumulative incidences (CIs) of treatment failure and enucleation were compared across strata using Gray’s test.
Results We identified 50 patients with retinoblastoma (54 eyes), who received a total of 56 plaques between January 1986 and December 2010, with a median follow-up of 8.3 years (range, 0.8–21.2 years). The median time from diagnosis to plaque placement was 12.7 months (range, 0.1–128 months). The CI and 95% CI of treatment failure and enucleation following epBRT at 5 years was 37%±7.2% and42.2%±7.3%, respectively. The lack of prior diode or green laser therapy was predictive of increased risk for treatment failure (p=0.02 and 0.03). International Classification group C or D was predictive of decreased time to enucleation (p=0.004). The use of any focal therapy was not predictive of time to treatment failure (p=0.33).
Conclusions The use of non-irradiative focal therapies prior to or following epBRT does not decrease the time to enucleation or treatment failure.
Lindsay A McGrath, Sachin Madhusudan Salvi, Soupramanien Sandramouli, Rina Bhatt, Kate Cuschieri, Hardeep Singh Mudhar
Purpose To present the clinical and histological features of squamous cell carcinoma (SCC) in the anophthalmic socket in four adult patients, and to determine the presence of human papillomavirus infection (HPV).
Methods Retrospective case series of four adult patients with SCC of the anophthalmic socket. P16 immunohistochemistry and HPV testing was carried out in all cases. The authors report clinical findings, histopathology, management and outcomes for all four patients with conjunctival SCC. Previously reported cases of conjunctival SCC in anophthalmic sockets were reviewed.
Results Four adult patients presented with eyelid lumps, discharge or change in prosthesis fit. Common examination findings included papillomatous changes, eyelid masses and epithelial changes. Three out of the four cases (75%) were positive for p16 by immunohistochemistry and the same cases positive for HPV-16 DNA. All patients received cryotherapy, topical or intralesional chemotherapy. Two patients proceeded to exenteration for control of invasive disease.
Conclusions To the authors’ knowledge, this is the largest series of SCC in the anophthalmic socket with comprehensive annotation of HPV status. Although socket conjunctiva is protected from environmental radiation, there is still a risk of neoplastic transformation in this tissue, thus patient education and regular checking of sockets by ophthalmologists should be undertaken as a preventative measure. The potential role of HPV in these tumours warrants further investigation.
Sundas Ejaz Maqsood, Nikhil Cascone, Andre Grixti, Ruben Kannan, Charles Nduka, Raman Malhotra
Aim To report the functional and aesthetic outcomes of eyelid full-thickness skin grafting (FTSG) in patients with facial nerve palsy (FNP).
Methods This is a retrospective, non-comparative, single-centre review of all patients with FNP who underwent FTSG over an 8-year period. Functional outcomes were collected through case notes review: CADS (cornea, static asymmetry and dynamic and synkinesis) score facial nerve grading and lagophthalmos on blink, gentle and forced closure. Marginal reflex distance (MRD1 and MRD2) was calculated on standardised photographs. The aesthetic outcomes were assessed objectively by two blinded independent assessors who assessed standardised photographs based on a mutually agreed grading scale. Both functional and aesthetic outcomes were measured preoperatively, and at early (1–3 months), intermediate (3–6 months) and late (>9 months) postoperative periods.
Results A total of 28 eyelid FTSGs were performed on 21 patients (11 female, 10 male) between 2008 and 2016. The mean age was 68 (range, 16–89) years and the mean follow-up was 20.8 (range, 12–30) months. The CADS score (cornea (p<0.001), static asymmetry (p<0.001), dynamic function (p<0.001)), MRD2 (p=0.002) and lagophthalmos (blink (p=0.003), gentle (p<0.001), forced (p=0.003)) improved through early and late postoperative periods. Aesthetically, the grafts appeared to look natural in terms of colour, surface contour and graft edge from the intermediate postoperative period and continued to improve significantly by the late postoperative period.
Conclusion The use of periocular FTSG is effective in improving lagophthalmos and periorbital symmetry in patients with FNP where skin contraction exists. They should be considered as an adjunct to other oculoplastic procedures for both functional and aesthetic rehabilitation.
Saraniya Sathiamoorthi, Ryan D Frank, Brian G Mohney
Background/aims The literature on the prevalence and demographics of congenital nasolacrimal duct obstruction (CNLDO) is 30–70 years old and largely comprises small sample sizes. This study provides epidemiological findings of this common disorder from the largest cohort reported to date.
Methods The medical records of all children (<5 years of age) residing in Olmsted County, Minnesota, when diagnosed with CNLDO from 1 January 1995 through 31 December 2004, were reviewed.
Results Of 17 713 newborns born during the 10-year study period, 1998 were diagnosed with CNLDO, yielding a birth prevalence of one in nine live births. The diagnosis was made in approximately 90% by a primary care physician, at a median age of 5 weeks, with no gender predilection. Compared with the reference population, CNLDO was associated with premature birth (p=0.005) and was more prevalent among Caucasians (p<0.001). Two-thirds of patients initially presented with discharge alone, 18% with tearing alone and 15% with both discharge and tearing.
Conclusions In this large population-based cohort, CNLDO occurred in one in nine live births with no gender predilection. Prematurity and Caucasian race were associated with the development of CNLDO. Mucopurulent discharge was a much more common feature than tearing at initial presentation.
Yun Su, Qin Shen, Xiaoping Bi, Ming Lin, Xianqun Fan
Background Trapdoor fracture is a special type of orbital blowout fracture. Although early surgery is recommended, there still remain some patients delayed by various reasons. In this study, we analysed the clinical characteristics of delayed paediatric patients, especially those with different levels of ocular motility restriction before surgery.
Methods Thirty patients (3 to 14 years old) who underwent delayed surgery for trapdoor fractures between January 2008 and September 2016 were enrolled. Their demographics, causes of injury and delay, clinical features, imaging data and follow-up information were collected.
Results Muscular entrapment was found in 17 patients (group A) and soft-tissue entrapment in 13 patients (group B). 12 (7 in group A) presented with severe motility restriction and 18 (10 in group A) with mild restriction before surgery. 41.7% with severe restriction recovered after surgery, compared with 83.3% with mild restriction. Four (23.7%) in group A (all with severe restriction) and six (46.2%) in group B (half with severe restriction) presented with persistent diplopia.
Conclusions Long recovery time and a high percentage of persistent diplopia are the main problems of delayed trapdoor fracture in children. A prompt surgery within 48 hours is strongly recommended in patients with muscular entrapment even if an urgent treatment is hard to achieve. So are patients with soft-tissue entrapment and significant motility restriction. In the other patients without such indications, even though some recovery might be possible in the long term, a prompt surgery right after diagnosis is still preferable regardless of the entrapped contents.
Niels J Brouwer, Marina Marinkovic, Gregorius P M Luyten, Carol L Shields, Martine J Jager
Aim To investigate whether differences in iris colour, skin colour and tumour pigmentation are related to clinical outcome in conjunctival melanoma.
Methods Data of 70 patients with conjunctival melanoma from the Leiden University Medical Center (Leiden, The Netherlands) and 374 patients from the Wills Eye Hospital (Philadephia, USA) were reviewed. The relation between iris colour, skin colour and tumour pigmentation versus clinical parameters and outcome was investigated using univariate and multivariate regression analyses.
Results A light iris colour (blue, grey, green) was present in 261 (59%) patients and a dark colour (hazel, brown) in 183 (41%). A low tumour pigmentation was detected in 130 (40%) and a high pigmentation in 197 (60%) patients. Low tumour pigmentation was associated with light iris colour (p=0.021) but not related to skin colour (p=0.92). In univariate analysis, neither iris nor skin colour was related to clinical outcome, while a low tumour pigmentation was related to metastasis formation (HR 2.37, p=0.004) and death (HR 2.42, p=0.020). In multivariate analysis, low tumour pigmentation was related to the development of recurrences (HR 1.63, p=0.043), metastasis formation (HR 2.48, p=0.004) and death (HR 2.60, p=0.014).
Conclusion Lightly pigmented tumours occurred especially in individuals with lightly coloured irises. While iris colour or skin colour was not significantly related to clinical outcome, a low tumour pigmentation was related to a worse outcome in patients with conjunctival melanoma. The amount and type of melanin in conjunctival melanocytes may be involved in the pathogenesis and behaviour of selected conjunctival melanoma.
Stephanie Ming Young, Yoon-Duck Kim, Hyun Jin Shin, Yukihiro Imagawa, Stephanie S Lang, Kyung In Woo
Aims To investigate the clinical and imaging features of primary and recurrent lacrimal gland pleomorphic adenoma (LGPA), as well as lacrimal gland malignant epithelial tumours (LGMET).
Methods Retrospective comparative case series from September 2000 to September 2016 in a single tertiary institution. Medical records of cases with histopathologically proven primary LGPA (PLGPA), recurrent LGPA (RLGPA) and LGMET were reviewed.
Results Sixty-four patients with LGPA (55 primary and nine recurrent) and 36 patients with LGMET underwent surgical excision in the study period. There was no significant difference in terms of age, gender or laterality. In terms of symptom duration, that of LGMET (7.1 months) was significantly shorter than PLGPA (23.9 months), which in turn was significantly shorter than RLGPA (127.1 months). Proptosis was the most common presenting symptom among all three groups. On CT, LGMET and RLGPA were significantly more likely to have ill-defined margins (p<0.001) and be heterogeneous (p<0.001) than PLGPA. RLGPAs (56%) were significantly more likely to have calcification than LGMET (34%), which in turn was more likely to have calcification than PLGPA (13%); LGMET (40%) and RLGPA (33%) were significantly more likely to have bony invasion than PLGPA (2.2%). On MRI, LGMETs (55%) were significantly more likely to have a tail or wedge sign indicating infiltration into the posterior orbit than PLGPA (0%) or RLGPA (0%).
Conclusion Our study adds important information regarding differentiating clinical and radiological features between malignant and benign epithelial lacrimal gland tumours that would aid in their management.
Ga-In Lee, Yoon-Duck Kim, Stephanie Ming Young, Seonae Shin, Kyung In Woo
Aims To evaluate the clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma (NKTL) involving the ocular adnexa.
Methods Retrospective, comparative, observational case series. A total of 350 patients with NKTL, including 27 patients with NKTL involving the ocular adnexa from 1999 to 2016. The patients were grouped into two groups: group 1 comprised patients presenting with ophthalmic symptoms, and group 2 comprised patients presenting with symptoms from other organs but subsequently developed ophthalmic involvement.
Results Group 1 comprised 12 patients (44.4%) and group 2 comprised 15 (55.6%). Mean duration of symptoms in group 1 was 1.8±1.2 months, while the time from diagnosis of NKTL to development of ophthalmic symptoms in group 2 was 45.3±65.6 months. Periorbital swelling was the most common presenting symptom in both groups (83.3% in group 1 and 73.3% in group 2). Symptoms mimicking cellulitis and pseudotumor were present in 50.0% and 16.7% of cases, respectively. The 5-year overall survival rate was 18.5% in group 1 and 26.4% in group 2, while the 5-year progression-free survival rate was 0% and 13.3%, respectively.
Conclusions Our series is to our knowledge the largest cohort study on NKTL reported to date and demonstrates that ocular adnexal NKTL is a rare but seriously fatal disease. It is characterised by acute inflammatory signs as present in as many as two-thirds of our patients in this series. It should be considered as a differential diagnosis in patients presenting with rapidly progressing proptosis and diagnosed promptly for optimal management.
Stacey C Lam, Emmy Y M Li, Hunter K L Yuen
Aims Sebaceous gland carcinoma (SGC) of the eyelid is a rare but potentially deadly cancer. The purpose of this study was to determine whether the American Joint Committee on Cancer (AJCC) 7th Edition T category for SGC correlated with metastasis and survival in the Chinese population.
Methods This was a retrospective, single-centre cohort study. Patients with surgically resected eyelid SGC between January 2001 and May 2015 at the Hong Kong Eye Hospital were reviewed. Tumours were staged using the AJCC criteria. The main outcome measures included local recurrence, metastasis and death. Disease-free survival (DFS) was measured from the completion of treatment; overall survival was measured from the date of initial diagnosis.
Results The study included 22 Chinese patients with a mean age of 65.4 years. The majority presented as a nodular lesion (91%) with 12 eyes (54.5%) initially misdiagnosed and a mean presentation time of 1 year. It was found that those with AJCC stage T2b or higher were significantly associated with lymph node metastasis (P=0.002) when compared with those with stage T2a. Older age at diagnosis (P=0.035) and no misdiagnosis (P=0.025) were associated with shorter DFS. Those with stage 3a or higher were associated with shorter DFS (P=0.007) and overall survival (P=0.024).
Conclusion Similar to previous reports, in this Chinese cohort, AJCC staging for SGC correlated with lymph node metastasis, DFS and overall survival. Those with stage 2b or higher on presentation will need closer surveillance for lymph node metastasis and may benefit from sentinel lymph node biopsy.
Aneta Klimova, Jarmila Heissigerova, Eva Rihova, Michaela Brichova, Robert Pytlik, Ivan Spicka, Katerina Mrazova, Jana Karolova, Petra Svozilkova
Background Vitreoretinal lymphomas belong to the family of central nervous system (CNS) lymphomas. The optimal approach for the treatment of isolated primary vitreoretinal lymphoma is unclear because of the lack of large comparative clinical series. Combination of intravitreal and systemic chemotherapy is recommended in many reports. The aim of our retrospective study was to compare the survival rate and prognosis of patients with vitreoretinal lymphoma with and without CNS involvement.
Methods Twenty patients with vitreoretinal lymphomas were observed between the years 2004and2016, 10 patients with primary vitreoretinal lymphoma and 10 with primary CNS lymphoma. To compare survival rates, we included 53 patients diagnosed with primary CNS lymphoma without vitreoretinal involvement between the years 2002and2011 from our haemato-oncology department.
Results The 5-year survival rate was estimated 71% in patients with vitreoretinal lymphoma in our observation. Significantly longer 5-year overall survival (P˂0.01) was observed in patients with vitreoretinal lymphoma compared with patients with primary CNS lymphoma without vitreoretinal involvement. Progression-free survival was almost equal in both groups of patients with primary vitreoretinal lymphoma and primary CNS lymphoma (P=0.363). The relapse of lymphoma was frequent (50%–60%) with the median time to first relapse of 31 months. Combined treatment (local and systemic) in patients without CNS involvement significantly prolonged progression-free survival in our study (P˂0.05).
Conclusion Combined treatment of primary vitreoretinal lymphoma significantly delays the relapse of lymphoma compared with local therapy alone. Intraocular involvement brings significant positive prognostic value when overall survival is compared.