[Prognostic factors of neurofibromatosis type 1-associated optic pathway gliomas in children]. / Facteurs pronostiques des gliomes des voies optiques associés à la neurofibromatose de type 1 chez les enfants.

INTRODUCTION: Optic pathway glioma (OPG) is a classic complication of neurofibromatosis type 1 (NF1) and can impair visual function in children with this condition. The objective of this study is to describe clinical, paraclinical and prognostic characteristics of OPG associated with NF1. MATERIALS AND METHODS: In this retrospective observational study of children followed for OPG associated with NF1 in a University Hospital, we analyzed the ophthalmological examination, brain and orbital imaging, management and the presence of associated endocrinopathy. RESULTS: We examined 114 children with NF1, of which 26 (22.81%) presented with OPG. Mean ages at diagnosis of NF1 and OPG were 3.83 years and 6.23 years, respectively. Mean visual acuity was 20/24.4 for the worse eye and 20/23.1 for the better eye. The RNFL (retinal nerve fiber layer) was thinner in subjects than in age-matched controls (p <0.0001). Retrochiasmal location of the OPG (DodgeC) was associated with lower binocular visual acuity than other locations and <20/32 (p=0.028); 28.03% of OPG (5 girls and 1 boy) were treated with chemotherapy, and the others were monitored; 19.23% had an associated endocrinopathy. CONCLUSIONS: OPG complicates 22.81% of NF1 cases in our series. Our study shows that retrochiasmal location of the glioma and female sex are poor prognostic factors. It also highlights the important role of OCT, since a decrease in RNFL is statistically associated with the presence of an OPG.

[Congenital abnormalities of the optic disc]. / Anomalies congénitales de la papille.

Congenital abnormalities of the optic disc are not uncommon in clinical practice and should be recognized. Size abnormalities of the optic disc include optic disc aplasia, hypoplasia, megalopapilla, and optic disc cupping in prematurity. Among congenital excavations of the optic disc head, morning glory disc anomaly and optic disc pit can be complicated by serous retinal detachment; the papillorenal disc is an association of bilateral optic disc cupping and renal hypoplasia which should be ruled out; optic disc coloboma is caused by an abnormal closure of the embryonic fissure and can be complicated by choroidal neovascularization and retinal detachment. Other abnormalities that will be discussed are congenital tilted disc syndrome, duplicity of the optic disc head, congenital pigmentation of the optic disc head and myelinated retinal nerve fibers. All of these abnormalities can be associated with syndromes and neurological diseases, as well as other potentially blinding ophthalmological defects which can be secondarily complicated by amblyopia, strabismus and nystagmus. Thus, they should be recognized in order to plan for appropriate follow-up.

[Brown's syndrome]. / Syndrome de Brown.

Brown's syndrome is related to an abnormality of the superior oblique muscle: it is manifested by an oculomotor disorder with active and passive limitation of elevation in adduction, the field of action of the inferior oblique muscle. The origin is congenital or acquired secondary to multiple causes - inflammatory-infectious, traumatic or iatrogenic. The clinical and paraclinical signs are suggestive. Cerebral and orbital imaging including CT and magnetic resonance imaging (MRI) is essential for the diagnosis and management of congenital or acquired Brown's syndrome. The course may yield improvement (spontaneous or after etiological treatment) or a steady state. Treatment is rarely surgical. The indication for surgery requires specific functional and clinical signs: torticollis, hypotropia in primary position, associated strabismus and impaired binocular vision; the long-term results of the various surgical techniques are variable.

[The Boston keratoprosthesis in the management of corneal blindness: Indications and limitations]. / La kératoprothèse de Boston dans la prise en charge de la cécité cornéenne : intérêts et limites.

INTRODUCTION: Keratoprostheses offer a therapeutic alternative to patients with bilateral corneal blindness who cannot undergo corneal allograft. The goal of this work was to evaluate the indications and limitations of Boston keratoprosthesis. MATERIALS AND METHODS: Seven patients underwent unilateral implantation of a Boston type I keratoprosthesis between December 2012 and November 2016. The following data were collected: surgical indication, preoperative visual acuity, postoperative visual acuity at D1, D7, D30, 6 months and 12 months, complications and postoperative treatment. RESULTS: The mean age of implantation was 58.7±23.4 years. The surgical indications included 1 case of congenital bilateral aniridia, 2 cases of chemical burn and 4 graft decompensations after multiple keratoplasties. Preoperative visual acuity was limited to "light perception" in 6 cases and "hand motion" in one case. A gain in visual acuity was observed in 4 patients, which corresponded to a mean gain of 1.53 logMAR at last follow-up. Postoperative visual acuity averaged 2.33 logMAR. All patients experienced an improvement in their quality of life and independence. DISCUSSION: Patients should be selected carefully, favoring patients with a remaining functional potential and able to engage in close postoperative follow-up, in order to ensure the best possible treatment success. CONCLUSION: Boston keratoprosthesis provides improved vision and improved quality of life for patients suffering from bilateral corneal blindness.

[Autologous serum tears: Long-term treatment in dry eye syndrome]. / Collyres de sérum autologue : traitement à long-terme dans le syndrome sec oculaire.

INTRODUCTION: Dry eye disease is a multifactorial pathology of the ocular surface. The high incidence of this pathology, as well as its significant impact on quality of life and vision and its financial cost, makes it a real public health problem. While the treatment of mild cases is generally simple and effective, treatment of severe forms is often disappointing. The use of autologous serum tears (AST) represents a therapeutic alternative for the most severe cases. The purpose of our study is to evaluate the efficacy of long-term AST treatment in patients with severe dry eye disease refractory to conventional treatment or secondary to systemic diseases such as Sjögren's syndrome or Graft versus Host disease (GVH), or ocular pathologies such as neurotrophic keratitis, chemical burns and ocular cicatricial pemphigoid. PATIENTS AND METHODS: This is a monocentric retrospective observational study conducted on 47 patients, with 83 eyes treated with autologous serum eye drops for isolated or secondary dry eye disease at the Marseille Public Hospitals between April 2014 and April 2017. The patients' subjective symptoms (ocular surface disease index [OSDI] score), their degree of satisfaction and the side effects were collected using questionnaires. Tear Break Up Time (BUT) and Schirmer scores were noted. A clinical evaluation based on fluorescein staining (Oxford score) was carried out prior to treatment with AST at P0 followed by 5 periods: P1 (between 1 and 3 months), P2 (3 to 9 months), P3 (9 to 15 months), P4 (15 months to 24 months), and P5 (>24 months). RESULTS: Out of the 83 eyes treated, the mean age was 54.39±21.56. There were 20 males (42.55 %) and 27 females (57.44 %); treatment indications consisted mainly of 25.53 % GVH, 21.27 % severe dry eye disease and 19.14 % Sjögren syndrome. The mean duration of follow-up was 9.82 months±15.50. The OSDI score decreased by 19.32 points±29.37 (P<0.05) between P0 and P1 and by 23.06 points±18.41 (P<0.05) between P0 and P4. The Oxford clinical score showed a significant decrease by the third month of treatment, between P0 and P2, by 1.32 points±1.76 (P<0.05). The Schirmer test and the BUT also showed an improvement in dry eye symptoms over time with AST, significantly at P1 (P<0.05). DISCUSSION: Complementary biological analyzes on the composition of AST are under way in order to identify predictive factors of effectiveness; patients not responding to AST treatment might respond to allogeneic serum from healthy donor cord blood. CONCLUSION: On this first series of 83 eyes treated with ASD, clinical efficacy was noted in most of the patients. No infectious complications were reported, and the satisfaction rate was very high.