Clinical features, diagnosis, treatment, and course of ocular sarcoidosis with or without uveitis: A retrospective, comparative study.

OBJECTIVE: To evaluate and compare characteristics, diagnosis, treatment, visual prognosis, and course between ocular sarcoidosis with or without uveitis in a population in Southern France. METHODS: We retrospectively analyzed data from patients with ocular sarcoidosis in a tertiary eye care center in Nice from January 2003 to December 2021. The inclusion criterion was biopsy-proven ocular sarcoidosis according to IWOS criteria as the first clinical manifestation of sarcoidosis. RESULTS: A total of 25 patients were included. Twenty patients had uveitis (70% panuveitis, 20% intermediate uveitis, and 10% anterior uveitis) and five patients had non-uveitic ocular sarcoidosis (one patient with dacryoadenitis, one patient with orbital granuloma, two patients with palpebral granuloma, and one patient with episcleritis). Only the cases with uveitis had bilateral involvement (85% of cases). There was no significant difference in ethnicity, biopsy diagnosis, systemic manifestations, or treatment between the two groups. Final visual outcomes remained good for both groups, with 96% of patients with BCVA>20/50, with no significant difference. Patients with non-uveitic sarcoidosis experienced less recurrence on treatment (P=0.042) and more remission (P=0.038) than patients with uveitis. Eighty percent of patients with uveitis had at least three suggestive clinical intraocular signs meeting IWOS criteria. CONCLUSION: In this population in Southern France, uveitis was the most common presentation of ocular sarcoidosis. The type of ocular sarcoidosis does not appear to be correlated with the type of systemic manifestations, use of systemic therapy, or visual prognosis, but patients with non-uveitic ocular sarcoidosis appear to have a better course with fewer recurrences on treatment and more remission than patients with uveitic ocular sarcoidosis.

[Local, regional and systemic dissemination of choroidal melanoma : Clinical and pathological correlation from 2 cases]. / Dissémination locorégionale et systémique du mélanome choroïdien : corrélation anatomoclinique à partir de 2 cas.

PURPOSE: To describe didactically the local, regional and systemic spread of choroidal melanoma. PATIENTS AND METHODS: Two patients who had undergone primary enucleation for the management of choroidal melanoma in 2018 at the University Hospital of Nice were included. Extrascleral extension and invasion of the vortex veins were evaluated, as well as synchronous and metachronous metastases, based on our database. RESULTS: Patient 1 was diagnosed with large choroidal melanoma with partial scleral invasion and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3, and a gain of chromosome 8q. Systemic work-up was unremarkable. Patient 2 was diagnosed with a large choroidal melanoma with extrascleral extension and vortex vein involvement. Cytogenetic analysis demonstrated a loss of chromosome 3 and a gain of chromosome 8q. Systemic work-up revealed several liver metastases. A total of 1762 patients were included in our database. Eighty-five patients (4.8 %) and 46 patients (2.6 %) experienced vortex vein invasion and extrascleral extension respectively. Patients with vortex vein invasion were diagnosed with synchronous and metachronous liver metastases in 1.2 % and 18.8 % respectively. Patients with extrascleral extension had synchronous and metachronous liver metastases in 6.5 % and 30.4 % respectively. The mean follow-up was 49.4 months (1-180). CONCLUSION: Extrascleral extension and vortex vein invasion illustrate the local, regional and systemic spread of choroidal melanoma. The latter are often associated with genetically aggressive tumours associated with high metastatic risk.

Pain after tonsillectomy: effectiveness of current guidelines?

BACKGROUND: Tonsillectomy is one of the most common major surgical procedures performed in children. In 2013, the use of codeine in children was severely restricted. French guidelines for treating tonsillectomy's postoperative pain at home have been reconsidered OBJECTIVE: The aim of our study was to measure effectiveness and safety of two schedules: acetaminophen + ibuprofen (A + I) and acetaminophen + tramadol (A + T) in children who underwent tonsillectomy. SETTING AND PATIENTS: We undertook a 1 year prospective and observational single-center study. All children who underwent tonsillectomy were eligible. The choice of the regimen, A + I group or A + T group, was left for the anesthesiologist in charge, done during the pre-anesthetic assessment. After hospital discharge, parents had to give systematically A + I or A + T, 4 times a day during 5 days and then acetaminophen alone for the next 5 days The primary endpoint was the home pain assessed using Parents' Postoperative Pain Measurement Short Form (PPPM-SF) scale. Secondary endpoints were the rate of further hospitalization and/or surgery due to tonsillectomy-related adverse events. RESULTS: Over the study period, 342 tonsillectomies were performed. The return rate of PPPM-SF scales was 58%. Two hundred patients were analyzed. The median age was 4 [3; 5.2] years and was lower in group A + I (4 [3; 5]; 5 [4; 7]; p < 0.0001). PPPM-SF scores were greater than or equal to 3 in both groups during the first 6 postoperative days. The mean decrease of PPPM-SF score over time was higher in group A + I than in group A + T (p = 0.007). Readmission rate was significantly higher in group A + T (A + I: 0; A + T: 7; p = 0.002) as the rate of reoperation for bleeding (A + I: 0; A + T: 3; p = 0.049). CONCLUSION: Home pain management after tonsillectomy should be improved. In clinical practice, A + I seems at least as effective as the combination A + T, without increasing readmission and/or additional surgery for bleeding.

Tumour Response in Uveal Melanomas Treated with Proton Beam Therapy.

AIMS: Post-proton therapy surveillance of uveal melanomas relies on decreased thickness on repeat ultrasound B every 6 months for 2 years and yearly thereafter. Earlier pseudoprogression, a phenomenon described in other tumour types within the first months of irradiation, can also be observed in uveal melanomas and may lead to inappropriate enucleation. The Collaborative Ocular Melanoma Study (COMS) has defined ultrasound criteria to identify tumour progression after brachytherapy. We aimed to determine the reliability of ultrasound as a means to measure tumour height after proton therapy and predict local relapse. MATERIALS AND METHODS: All 1992-2012 consecutive patients with at least three ultrasound B measurements during follow-up were included. RESULTS: There were 55 local relapses of 886 patients (6.2%). Ultrasound B reliability was highest at 24 months, with specificity higher than 95% starting at 18 months. CONCLUSION: Before 18 months post-proton therapy, the risk of falsely concluding in favour of a relapse exceeds 5% and should prompt repeat measurements 3 and 6 months later but should not prompt enucleation without further clinical assessment.

[Malignant tumours of the eye: Epidemiology, diagnostic methods and radiotherapy]. / Tumeurs malignes ophtalmologiques : indications de la radiothérapie et techniques.

Malignant tumours of the eye are not common, barely representing 1 % of all cancers. This article aims to summarise, for each of the main eye malignant diseases, aspects of epidemiology, diagnostic methods and treatments, with a focus on radiation therapy techniques. The studied tumours are: eye metastasis, intraocular and ocular adnexal lymphomas, uveal melanomas, malignant tumours of the conjunctive, of the lids, and retinoblastomas. The last chapter outlines ocular complications of radiation therapy and their management.

Management of choroidal metastases.

BACKGROUND: Choroidal metastases (CM) are the most common malignant intraocular lesion observed in up to 4-12% of necropsy series of patients with solid cancer. The spectrum of presentations varies from prevalent CM in disseminated cancer to isolated CM. CM are responsible for visual deterioration. Depending on the primary cancer, estimated life expectancy, overall cancer presentation and ocular symptoms, the management of CM varies widely. We address the multidisciplinary management of CM and technical aspects of radiotherapy. MATERIAL AND METHODS: A systematic review of literature was performed from 1974 to 2014. RESULTS: Choroidal metastases occur preferentially in breast and lung carcinomas but are reported in all cancer types. The standard treatment remains external beam radiotherapy, applying 30Gy in 10 fractions or 40Gy in 20 fractions. The reported complete response and improved visual acuity rates are 80% and 57% to 89%, respectively. Some chemotherapy or new targeted therapy regimens yield promising CM response rates. DISCUSSION: Radiation therapy consistently shows rapid symptom alleviation, yield excellent local control and functional outcomes. However, there are only few reports on late toxicities after 6months given the unfavorable prognostic of CM patients. Selected patients may live more than two years, underlying the need to better assess mean and long term outcomes. Some authors have favored exclusive systemic strategies with omission of irradiation. The current literature suffers from the scarcity of prospective trials. Duration of tumor response following systemic therapy is rarely reported but appears less favorable as compared to radiotherapy. Systemic treatments may be proposed for pauci-symptomatic CM in a polymetastatic context while radiation therapy remains necessary in symptomatic CM either upfront or as an alternating treatment. Focalized radiation like brachytherapy and proton therapy may be proposed for isolated CM with long disease-free interval between primary and CM, as these techniques have the potential to yield better tumor and functional outcomes in patients with long life expectancy.

[Benign conjunctival tumors]. / Tumeurs conjonctivales bénignes.

Benign conjunctival tumors include congenital, melanocytic, epithelial and stromal lesions. Clinical observation often fails to make the diagnosis, and complete excisional biopsy must be performed as soon as growth is suspected. Histology remains the best way to exclude malignancy or lesions with a risk of transformation, because malignant lesions carry a poor prognosis and require specific therapy and follow-up. Onco-ophthalmologists must have a perfect knowledge of benign conjunctival tumors, since during adulthood, nevi can be confused with melanomas, and epithelial or stromal lesions with carcinomas. For children and teenagers, surgery is only recommended when growth or functional problems are observed.

Melanotic neuroectodermal tumor of infancy: case report and review of the literature.

Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare but distinct neoplastic entity in infancy. Diagnosis is usually made before the age of 12 months. The common clinical presentation is a rapidly growing mass of the pre-maxillary area. Its surface is unevenly pigmented. To affirm the diagnosis a biopsy is necessary. Few cases of malignancy have been described (5% of cases). Adequate surgical excision is the treatment of choice. Recurrence rate is about 10 to 15% within 5 years. We report in this article the case of a newborn with MNTI illustrating that an R0 surgical excision can be correlated to a favourable prognosis. In this case the 5 years follow up didn't show any local or distant recurrence.

[Surgery and additional protontherapy for treatment of invasive and recurrent squamous cell carcinomas: technique and preliminary results]. / Association chirurgie-protonthérapie dans le traitement des carcinomes invasifs et récidivants de la conjonctive: technique et résultats préliminaires.

INTRODUCTION: Invasive squamous cell carcinomas are uncommon neoplasias with high recurrence and mortality rates. The improvement of tumoral control requires additional treatments such as cryotherapy, topical chemotherapy, and radiotherapy. We present the technique and preliminary results of associating treatment with surgery and proton beam therapy for recurrent and invasive squamous cell carcinomas. MATERIALS AND METHODS: From June 2001 to September 2008, 15 patients were treated in our ocular oncologic center for squamous cell carcinomas either with recurrences or with invaded resection margins. The treatment combined new surgical resection with protontherapy. Specific improvements in proton beam therapy have been made at the Nice Cyclotron to adapt the treatment to conjunctival tumors. Proton beam carving consists in using a specific device to treat the thickness of the whole lesion site and the adjacent conjunctiva and to spare the surrounding healthy structures. RESULTS: Patients were staged according to the TNM classification of malignant tumors in T2: 3; T3: 5; T4: 7. Mean follow-up was 39.1 months (range, 6-90 months). Our 15 patients included 12 males and three females. Left eyes were involved in eight cases. The mean age at first consultation was 63.7 years (range, 46-80 years). In 13 cases (86.8%), the bulbar and limbic conjunctiva was involved, in five of these cases the cornea was invaded, and the anterior chamber was involved in one case. In one case, the tumor was located on bulbar conjunctiva near the caruncle (6.6%) and in one case in the fornix (6.6%). One patient died of another cancer after 48 months of follow-up. We obtained local tumor control for 13 patients (86.8%) and recurrences for two patients (13.2%). One of them has presented with cervical node metastases. These two patients who presented recurring and extensive tumors had had previous repeated surgeries in other centers. Moreover, proton beam therapy was performed more than 6 months after the initial treatment. Exenteration and enucleation had to be performed to treat these recurrences 6 and 24 months after proton beam therapy. The exentered patient has been lost to follow-up. No patients developed recurrences with additional proton beam therapy performed within 6 months after initial surgical resection. As for side effects, seven patients suffered from sicca syndrome, six needed cataract surgeries, three unesthetic dilatations of episclera vessels, two conjunctival postradiation dysplasias, two experienced eyelash loss, one stenosis of the lacrimal duct, and one glaucoma controlled by monotherapy. Conjunctiva and amniotic grafts had to be performed on one of the patients presenting with dysplasia. Due to the rarity and diversity of these cases, it is nearly impossible to carry out prospective and comparative studies. CONCLUSION: Traditional adjuvant treatments often failed to control recurring and invasive squamous cell carcinomas. We often ended up performing exenteration to control local recurrences. The preliminary results of the present study suggest that proton beam therapy may be considered as a good alternative to traditional treatments with acceptable side effects.