[Oral chemotherapy (sonigegib) of extensive basal cell carcinoma, about 4 cases]. / Traitement par chimiothérapie orale (sonidegib) de carcinomes basocellulaires étendus, à propos de 4 cas.

Basal cell carcinoma (BCC) is the most frequent cutaneous carcinoma. Its incidence is constantly increasing, primarily due to sun exposure during the two first decades of life. The lower lid and medial canthus are the most common locations. In the majority of cases, surgery with sufficient margins is the first-line treatment. In the case of inoperable tumors (extensive lesions, poor general health, high surgical morbidity, unacceptable cosmetic sequelae), oral chemotherapy can be suggested. We report the follow-up of 4 patients with advanced non-metastatic basal cell carcinoma (BCC) treated with oral chemotherapy (sonigegib). All patients were female with a mean age of 80years. The first patient had an infiltrative BCC of the right lower lid with orbital invasion, the second a nodular BCC of the right inferior lid with massive cutaneous and orbital invasion extending to the controlateral medial canthal angle, the third an advanced infiltrative BCC invading both orbits and nasal cavities, and the fourth, an infiltrative BCC with extension to the ipsilateral skin of the face. The diagnosis of BCC was confirmed by multiple biopsies in our four patients prior to starting chemotherapy. Because of the locally advanced lesions, surgery was contraindicated in all cases, chemotherapy was undertaken (sonidegib 200mg daily), and ophthalmological and dermatological examinations were performed every 3months. In all 4 patients, the outcome was very favorable, with one clear regression in tumor size and infiltration and 3 partial regressions. Many side effects were noted, including nausea, muscle cramping, dysgeusia resulting in significant weight loss, and malnutrition in the elderly patients. The treatment had to be discontinued after 11months in the patient with total macroscopic regression because of major side effects. The treatment is still underway for the other patients and will be continued as long as a clinical benefit is observed. The treatment will be discontinued if intolerable side effects develop. Oral chemotherapy (sonidegib) is an effective alternative treatment when surgery cannot be realized, especially in the case of extensive infiltrative non-metastatic BCC.

[Evaluation by quality of life questionnaires in patients undergoing strabismus surgery in Graves' disease]. / Évaluation par questionnaires de l'amélioration de la qualité de vie des patients opérés de strabisme dans le cadre d'une maladie de Basedow.

PURPOSE: To evaluate the improvement in quality of life of patients operated within 6 months for strabismus in Graves' disease using a questionnaire. MATERIALS AND METHODS: A retrospective study was based on a cohort of patients undergoing strabismus surgery for Graves' disease at the Nancy university medical center between June 2015 and January 2017. A quality-of-life questionnaire composed of the functional GO QOL questionnaire plus 10 additional questions was given to patients. The questionnaires were completed by patients in consultation before surgery and then at six months postoperatively. A subgroup analysis was conducted to identify potential risk factors such as smoking, orbital decompression, duration of disease progression, number of surgeries, previous treatments or gender influences on the progression of the postoperative score. RESULTS: Thirty-three patients (15 men/18 women) underwent surgery, with mean age 56 years; 16 (48 %) were smokers, 24 (70 %) were operated for the first time for strabismus. 8 patients (24 %) had a history of surgical orbital decompression. The mean preoperative GO QOLf was 30 (±23.8), which increased to 72 (±29.6) postoperatively (P=0.004). All the answers to the questions proposed were improved by surgery. The most improved items were difficulty in extreme gaze+6.82 points (P=0.001) and going down stairs +6.81 (P=0.002). Smokers had a lower preoperative GO QOLf than non-smokers (25±22.84 vs. 34±24.55); the same was true for their postoperative improvement (35±34.5 points vs. 50±18.8, P=0.002). History of orbital decompression did not appear to influence quality of life (21±25.39 vs. 65±33.98); the same was true for previous corticosteroid or immunosuppressant treatment (28±23.52 vs. 67±28.24), respectively P=0.42; P=0.73. Gender did not appear to influence the results P=0.17. First-time strabismus surgery patients showed a tendency which was not significant toward better improvement after 2 or 3 surgeries (respectively 45 vs. 39 vs. 33) P=0.056. There was no correlation between the duration of the disease and the change in the GO QOLf (R=0.04). CONCLUSION: This study confirms that strabismus surgery significantly improves the quality of life of patients with Graves' ophthalmopathy. The GO QOLf demonstrates its effectiveness in highlighting this progression. Our ten additional questions proved to be very good tools for assessing this condition.

[Outpatient management without day 1 clinical follow-up of patients undergoing uncomplicated filtering surgery]. / Prise en charge ambulatoire sans contrôle clinique à j1 de patients opérés d'une chirurgie filtrante non compliquée en peropératoire.

INTRODUCTION: Although the general increase in ambulatory surgery allows for a 20% reduction in costs of hospitalization, the necessity of a day 1 postoperative visit remains the main problem for elderly patients or those who reside at considerable distance. For these reasons, in 2014, we decided to only see patients with uncomplicated filtering surgery at D5 and D21 and to replace the D1 visit with a telephone call. The primary goal of our study was to analyze a population of patients undergoing uncomplicated filtering surgery who were not seen at D1 and to describe the frequency of postoperative complications, intraocular pressure results and therapeutic management at D5 and D21. PATIENTS AND METHODS: This was a retrospective monocentric study in the department of ophthalmology. All patients (naïve of surgical treatment for glaucoma) underwent surgery for primary open-angle glaucoma in an ambulatory surgery unit between May 2014 and July 2016. A nurse made a phone call to the patients at day 1 and they responded to a standardized questionnaire. Depending upon their responses, the patients were seen quickly (<24hr) or on the systematic schedule at day 5 and day 21. RESULTS: One hundred and forty-four eyes (126 patients) were included in our study. The mean preoperative IOP was 20.4±6.4mmHg. After the phone call, only five patients were examined before the first planned follow-up at day 5. For 3 of them, the examination revealed the presence of a hyphema, and their topical medication was changed. The other two patients had no treatment modifications. At day 5, the mean IOP was 10.6±5.9mmHg. Thirty-two eyes (22.2%) required a change in their medication at day 5. The mean IOP at day 21 was 12.9±4.6mmHg. Our success rate (IOP<21mmHg with no topical medication) at day 21 was 95.6%. DISCUSSION: The guidelines for filtering glaucoma surgery recommend examining the patient at day 1 (or at least before day 3), day 8, day 15 and day 30 or more frequently according to the case. We replaced the day 1 visit with a telephone call. We did not note a substantial rate of complications due to the elimination of the D1 visit. The criteria of success of filtering glaucoma surgery vary in the literature, but our success rate at D21 seems to be similar that in the literature and may suggest that the day 1 visit is not necessary if the surgery for primary open angle glaucoma is uncomplicated.

[Ultrasound biomicroscopy long-term study of filtering belbs]. / Analyse à long terme de la bulle de filtration après chirurgie de glaucome, par échographie ultrasonore.

PURPOSE: To study aspect of filtering blebs by Ultrasound Biomicroscopy (UBM) and to compare it to intraocular pressure (IOP), to type of surgery performed and to the measurement of the anterior chamber angle after glaucoma surgery. METHODS: An observational study, conducted between January and December 2010 on 46 eyes of 42 patients who underwent glaucoma surgery. 17 combined surgeries (14 non-penetrating deep sclerectomy (NPDS) with phacoemulsification and 3 trabeculectomy with phacoemulsification) and 29 filtering surgeries alone (16 NPDS and 13 trabeculectomy) were performed by the same surgeon with application of mitomycin C for 1.30 minutes during surgery. After surgery, IOP was controlled at day 5, day 30 and at UBM realisation. Filtering blebs were classified according to Yamamoto's study: functional blebs (L: Low-reflective) and non-functional blebs (H: High-reflective, E: Encapsulated and F: Flattened). RESULTS: Mean follow-up was 21 months (± 3.7). We noted a significant decrease in IOP, mean preoperative IOP 23.2mmHg (± 9.4), mean post operative IOP 14.7mmHg (± 3.3) (P<0.001). We identified 36 functional blebs (36L type) and 10 non-functional blebs (including 1 H, 7 F and 2 E). There was no significant morphological differences between filtering blebs after combined surgery and filtering surgery alone (P=0.072). We did not find any morphological difference between filtering blebs after trabeculectomy and NPSD (P=0.394). Blebs are significantly more functional with an IOP less or equal to 6mmHg after surgery (P=0.028). We didn't find any difference between efficacy of filtering blebs and depth of the anterior chamber angle. CONCLUSION: Our study reports 78% of functional blebs after glaucoma surgery (mean duration of 21 months). Type of surgery does not seem to interfere with the long-term morphological type of the bleb, however the immediate postoperative IOP less than or equal to 6mmHg is a factor of good prognosis. UBM appears to be an interesting tool for monitoring patients undergoing glaucoma surgery.

Optic disc neovascularization revealing neurosyphilis.

PURPOSE: To report a case of neurosyphilis revealed by bilateral optic disc neovascularization. DESIGN: Case report. METHODS: A 39-year-old man presented with a 6-month history of progressive visual loss (20/25 RE, 20/1200 LE) and transient headaches. Fundus examination demonstrated a 1+ vitritis, mild macular edema and large optic disc neovascularization in both eyes. Serological tests for syphilis were positive. RESULTS: Specific antibiotics and systemic steroids were undertaken and complete regression of disc neovascularization was observed within 6 months. CONCLUSION: Optic disc neovascularization, which is a rare manifestation of chronic uveitis, may lead to the diagnosis of neurosyphilis and be successfully treated by specific antibiotic and steroid therapy.

[Efficacy and tolerability of highly crosslinked hyaluronic acid injections in the management of the enophthalmous orbit]. / Efficacité et tolérance des injections d'acide hyaluronique hautement réticulé dans la prise en charge des énophtalmies.

OBJECTIVE: Evaluation of the efficacy and tolerability of highly crosslinked hyaluronic acid injections in treating the enophthalmous orbit. METHODS: Retrospective study of 11 enophthalmic patients who received an intraorbital injection of Juvederm(®) Voluma between June 2007 and October 2008. The mean follow-up was 19 months (range 12-25 months). RESULTS: Twelve orbits of 11 patients were treated, including nine with post-enucleation socket syndrome (PESS). Volume loss was corrected in 66.67% of cases (eight orbits) with only one intraorbital injection. Two patients requested an additional injection, achieving a final success rate of 83.33%. A rate of 16.67% (two orbits) developed some bruising, mild swelling and hypersensivity at the injection site within 24 hours of administration of the filler, representing minor, temporary side-effects of which the patients had been forewarned. Persistent edema was noted in 16.67% of cases, likely due to an overcorrection of ptosis, and ptosis was exacerbated in 16.67% of cases (two orbits). One ptosis was corrected after several months, with no particular difficulties related to the hyaluronic acid. No additional complications were identified. CONCLUSION: Juvederm(®) Voluma appears to be an effective filler for reduction of enophthalmos with a single intraorbital injection. In our experience, this product seems to have a longer duration of action than reported by the manufacturer and appears to perform like a semi-permanent or even permanent filler.

[Recurrent unilateral optic neuropathy associated with human immunodeficiency virus (HIV)]. / Neuropathie optique unilatérale récidivante liée au VIH.

HIV-related optic neuropathy is rare compared to optic neuropathies secondary to opportunistic infections in seropositive patients. We report the case of a 39-year-old HIV-positive woman referred for unilateral visual loss leading to the diagnosis of recurrent, unilateral, inflammatory optic neuropathy directly associated with HIV. Despite initial recovery after steroid treatment, she relapsed twice. Absence of any opportunist infections or toxic causes and presence of a very high viral load due to non-compliance with treatment led to the diagnosis of HIV-related optic neuropathy. Steroids and effective anti-retroviral treatment resulted in definitive and complete recovery. Inflammatory, degenerative and/or vascular mechanisms have been hypothesized to explain the occurrence of these rare HIV-related optic neuropathies. This diagnosis remains a diagnosis of exclusion to be considered in the work-up of seropositive patients with optic neuropathies.

[Wolfram syndrome: clinical and genetic analysis in two sisters]. / Le syndrome de Wolfram : mise au point clinique et génétique à propos du cas de deux sœurs.

Wolfram syndrome is a severe genetic disorder defined by the association of diabetes mellitus, optic atrophy, deafness, and diabetes insipidus. Two sisters complained of progressive visual loss. Fundus examination evidenced optic atrophy. Their past medical history revealed diabetes mellitus and deafness since childhood. The association of these symptoms made the diagnosis of Wolfram syndrome possible. It was confirmed by molecular analysis, which evidenced composite WFS1 heterozygous mutations inherited from both their mother and father. Ophthalmologists should be aware of the possibility of Wolfram syndrome when diagnosing optic atrophy in diabetic children.

[Central retinal artery occlusion following coronary angiography: a case report]. / Occlusion de l'artère centrale de la rétine après une coronarographie : à propos d'une observation.

We report the case of a 74-year-old man referred in ophthalmology for acute central visual loss in his right eye 2 hours after a coronary angiography. Visual acuity was limited to light perception RE and 20/20 LE. Fundus examination revealed a central retinal artery occlusion with retinal edema and a cherry-red spot in the right eye. Fluorescein angiography confirmed that the central retinal artery was not filling. Despite selective ophthalmic artery fibrinolysis, visual acuity remained very low. Embolic occlusion is the most probable etiology in this complication. Central retinal artery occlusion is a serious but very rare complication of coronary angiography.

[Facial necrotizing fasciitis after mild trauma of the eyelid: role of nonsteroidal anti-inflammatory treatment]. / Fasciite nécrosante de la face après un traumatisme palpébral mineur: rôle des anti-inflammatoires.

Facial necrotizing fasciitis is a rare but potentially serious and fatal infection, especially in children. We report the case of a five-year-old girl who developed rapidly progressive necrotizing fasciitis of the face secondary to a mild trauma, with a small wound in the upper eyelid. She had received nonsteroidal anti-inflammatory treatment before she was referred to our hospital. Despite surgical debridement of necrotic tissues and intravenous antibiotic therapy, recurrence occurred. Final progression was satisfactory without sequelae. We discuss here the role of nonsteroidal anti-inflammatory drugs in the development of necrotizing fasciitis in cutaneous lesions, whether traumatic or not.

[Limbal-conjunctival autograft in pterygium surgery]. / L'autogreffe limbo-conjonctivale dans la prise en charge chirurgicale des ptérygions.

INTRODUCTION: The aim of this study was to estimate the efficacy of limbal-conjunctival autograft in the treatment of pterygium. MATERIAL AND METHODS: We conducted a retrospective study including 15 patients (16 eyes) operated on for pterygium by limbal-conjunctival autograft. The examination included the search for risk factors and visual acuity, as well as corneal astigmatism and pterygium measurement. The follow-up included an examination at day 8, 1 month, and 3 months and assessed the integration of the transplant on the eye's surface, the aesthetic aspect, and recurrence. We describe the patients' characteristics, visual acuity, and astigmatism before and after surgery. RESULTS: The mean age of the patients was 56 years (range, 31-81 years). Before surgery, irritative signs and astigmatism, were found in 37% and impaired visual acuity in 81%. The mean duration of the follow-up after surgery was 19 months. Visual acuity and astigmatism improved in 56% of our patients. All transplants and harvest sites had a satisfactory macroscopic aspect. However, one patient suffered from a corneal invasion by fibrovascular tissue, 7 months postoperatively, which remained stable at the end of follow-up. DISCUSSION/CONCLUSION: In our study, the limbal-conjunctival autograft was a safe and effective surgical treatment with a very low recurrence rate at long-term follow-up.

[External dacryocystorhinostomy under local anesthesia and sedation]. / Les dacryocystorhinostomies par voie externe sous anesthésie locale et sédation.

INTRODUCTION: External dacryocystorhinostomy (DCR) surgery is highly advantageous in that it can be performed under local anesthesia associated with sedation. We aimed at verifying the efficiency of the anesthesia, studying the general behavior of the patient and the quality of the surgery. PATIENTS AND METHODS: A prospective study of a local anesthetic protocol associated with sedation was conducted in our Oculoplastic Department on 34 patients (71.5+/-8.3 years of age) between may 2007 and march 2008. The anesthetic protocol consisted of blocking four nerves based on the analysis of the anatomy of facial innervation. The including criteria were patient antecedents such as arterial hypertension, cardiac or lung problems, diabetes, and no contraindications for local anesthesia. The hemodynamic constants, variability of the Ramsay score, and complications such as bleeding or pain were studied. The progress of the surgery was simultaneously evaluated by the patients, anesthesiologists, and surgeons. RESULTS: Both the hemodynamic constants and the Ramsay score remained stable intra- and postoperatively. In addition, the visual analog scale (VAS) scores remained low. Both surgeon and patient satisfaction was excellent (88.4 % of the patients declared that they were ready to choose the same anesthetic protocol if new surgery were to be performed). The surgery's success rate was 79.3 % (no watering at 3 months) versus 82 % for the patients operated under general anesthesia. CONCLUSION: The protocol of local anesthesia associated with sedation for external DCR is therefore safe and efficient.

[CINCA syndrome: a rare cause of papilledema. The case of homozygous twins]. / Une cause rare d'oedème papillaire: le CINCA syndrome. Les cas de jumeaux homozygotes.

CINCA syndrome is an autoinflammatory disease in childhood characterized by multisystemic manifestations: cutaneous, articular, and neurological including sensory organs. We report the case of homozygous twins affected by CINCA syndrome. The diagnosis was evoked on the basis of multiple systemic symptoms (multiple episodes of fever of unknown origin, mental retardation, short stature, meningitis, hearing loss, bilateral papilledema) and confirmed by the presence of a CIAS1 mutation on genetic analysis. After few months of treatment by anakinra (an interleukin-1 receptor antagonist) the children began to grow again and we noted regression of the biological inflammatory syndrome.

[Sentinel lymph node biopsy for lymphophilic conjunctival and eyelid tumors: report of 8 cases]. / Biopsie du ganglion sentinelle dans les tumeurs lymphophiles conjonctivales et palpébrales: à propos de 8 cas.

OBJECTIVE: To report our experience in sentinel lymph node biopsy for lymphophilic conjunctival and eyelid tumours. METHODS AND RESULTS: Preliminary study (2005-2007) on 8 patients with conjunctival and or eyelid tumours (melanomas, epidermoid carcinoma, Merkel cell carcinoma). All patients underwent preoperative lymphoscintigraphy 18 FdG pet CT Surgery was performed with complete neck dissection. Sentinel nodes and other nodes harvested were processed separately for histopathologic study; the sentinel were confirmed as histologicaly positive for five patients. Additional positive nodes were found in two out of those five patients. No positive lymph node was found in patients with negative lymphoscintigraphy. After a follow up ranging from 12 to 43 months: two patients died, and 6 are free of disease. CONCLUSIONS: These results demonstrate a very hight level of invaded NO lymph node and confirm the interest of sentinel node technique for optimisation of the therapeutic strategy in lymphophilic conjunctival and eyelid tumours.

[Surgical treatment of benign intracranial hypertension: optic nerve sheath fenestration]. / Traitement chirurgical de l'hypertension intracrânienne bénigne: fenestration des gaines du nerf optique.

Optic nerve sheath fenestration (ONSF) is rarely used in France. The aim of surgery is to release pressure around the optic nerve. Although debated, ONSF can be used in selected cases of benign intracranial hypertension. The authors describe the approach of the anterior part of the optic nerve and the technique itself. The indications, results and potential complications of this technique are developed in this paper.

Mid-term rhinosinusal consequences of bony orbital decompression in Graves' disease: a retrospective study.

OBJECTIVES: The aim of this work was to detect and study mid-term rhinosinusal complications arising after bony orbital decompression via the inferior palpebral approach in patients with Graves' ophthalmopathy. MATERIALS AND METHODS: This retrospective study considered 48 patients treated for proptosis from 1999 to 2001 who underwent bony orbital decompression via the inferior palpebral approach. Nineteen (19) of these patients agreed to participate further by consenting to follow-up examinations performed specifically for the purpose of this study, which consisted of ophthalmologic (visual acuity, proptosis) and ENT (nasal dysfunction, rhinoscopy) examinations. RESULTS: Mean mid-term postoperative follow-up of the 19 patients was 43.5 months (+/-12 months). Predominant rhinosinusal signs (rhinorrhea, altered sense of smell, nasal obstruction) were observed in 20% of this group (4 patients) without any unfavorable effect on daily life. Fiber-optic rhinoscopy disclosed mucosal secretions and edema in 5% (one patient). CONCLUSION: Mid-term complications of bony orbital decompression via an inferomedial approach are relatively rare. Late postoperative sinusitis has not been reported. Signs of minor nasal dysfunction may occur but would have little impact on quality of life.