Retinoblastoma is the most common malignant intraocular tumor in children under 5 years old. Survival rates, ocular and visual preservation are major challenges in this disease. This study aims to determine the epidemio-clinical, therapeutic and prognostic profile of patients with retinoblastoma who have been treated in the pediatric hemato-oncology department of the university Hospital Hassan II of Fez. It is a retrospective and descriptive study of all children followed for retinoblastoma at the pediatric hemato-oncology department, over a period of 11 years and 10 months from January 1st, 2012 to October 31, 2023. During this period, we collected 65 cases. The mean age at diagnosis is 25 months and the ratio is 0.9. The mean time to diagnosis is 5 months. Symptoms are dominated by leukocoria (84.2% of cases), followed by exophthalmia (15.8% of cases) and sarbismus (13,2% of cases). The retinoblastoma is unilateral in 70.7% of cases and bilateral in 29,3% of cases. Enucleation was indicated in 97% of cases. The majority of patients were treated by the combination of chemotherapy and surgery (69.2% of cases). Surgery only was indicated in 27,7% of cases and conservative treatment in 26,1% of cases. Radiotherapy was indicated in 9,5% of cases. The overall survival rate at the end of treatment is 75.4% and the five-year survival rate is 60%. The prognosis of retinoblastoma is generally good. In our context, most cases are still diagnosed at advanced stages of the disease, which considerably reduce the overall survival rate and the preservation of the eye and the vision.

Infantile neuroaxonal dystrophy is a rare neurodegenerative disorder, with onset in the first or second year of life, after an interval of normal psychomotor development. It is characterized by rapid motor and cognitive regression and hypotonia evolving into spasticity. This is a retrospective and descriptive study realized over a period of 10 years [January 20-October 2023], including all patients diagnosed with Infantile neuroaxonal dystrophy in the neurology unit of the Paediatric Department of the Hassan II University Hospital of Fez. We report 7 patients with Infantile neuroaxonal dystrophy, three girls and four boys. Earliest symptoms presented between 10 and 18 months of age. The first manifestations were a psychomotor regression in all cases and pshymotor developmental delay in 5 cases. Fast rhythms on electroencephalogram (EEG) were observed in all patients. Brain imaging studies showed cerebellar atrophy, with signal hyper- intensity in the cerebellar cortex on T2-weighted images in six patients. Mutations in the PLA2G6 gene were identified in the four patients studied. Infantile neuroaxonal dystrophy is a rare and serious disease in children with a risk of generally fatal complications, but it remains a preventable disease thanks to genetic counselling.