X-linked retinoschisis (XLRS) is an inherited retinal disorder due to mutations in retinoschisin 1, characterized by impaired central vision secondary to parafoveal cystic cavities and visual field loss by splitting through the retinal nerve fibre layer in the peripheral retina. It is the leading cause of juvenile macular degeneration in males, and currently there is no approved treatment but carbonic anhydrase inhibitors can be used. A retrospective review of the medical records of 17 children with confirmed XLRS seen in the Paediatric Ophthalmology Department of La Paz University Hospital from the 1st of January 2009 to the 1st of June of 2023 was conducted.
View Article and Find Full Text PDFJ Pediatr Ophthalmol Strabismus
September 2020
Purpose: To analyze clinical characteristics, treatment, and long-term outcomes of pediatric patients with glaucoma after congenital cataract surgery at a single tertiary care hospital.
Methods: Medical records of pediatric patients diagnosed as having glaucoma secondary to congenital cataract surgery between 1996 and 2016 were reviewed retrospectively.
Results: A total of 58 eyes of 42 patients were included with a median follow-up time of 55 months (interquartile range [IQR]: 27 to 128) after glaucoma diagnosis.
Purpose: To evaluate the outcomes of primary Ahmed glaucoma valve in refractory secondary glaucoma following congenital cataract surgery, particularly in persistent foetal vasculature.
Method: Retrospective review of paediatric patients after Ahmed glaucoma valve implantation for refractory post-lensectomy glaucoma in a tertiary referral centre in Spain. Surgical complications, additional and/or replacements of Ahmed glaucoma valve, intraocular pressure, cup-to-disc ratio, glaucoma medications and final visual acuity were studied.
J Pediatr Ophthalmol Strabismus
December 2016
Purpose: To review the systemic and ocular outcomes and long-term status of ocular rhabdomyosarcoma in pediatric patients in a tertiary center in Spain.
Methods: All patients younger than 18 years who were diagnosed as having ocular rhabdomyosarcoma and treated between 1982 and 2011 at La Paz University Hospital, Madrid, Spain, were included. Clinical presentation, management, complications, and ocular and systemic outcomes were reviewed.
Background: Total serous retinal detachment after laser photocoagulation for retinopathy of prematurity is an infrequent type of retinal detachment in preterm babies.
Purpose: To describe the successful outcome for treatment by scleral drainage, bevacizumab, and scleral buckling for complete serous exudative retinal detachment in a patient with retinopathy of prematurity.
Methods: A preterm baby with primary pulmonary hypertension under treatment with sildenafil developed a total (retrolental) serous retinal detachment after photocoagulation for threshold retinopathy.
Purpose: To report the results of adalimumab therapy in a cohort of children with refractory noninfectious uveitis.
Methods: The medical records of patients diagnosed with uveitis and treated with biweekly adalimumab injections for a period of at least 3 months at the University Hospital of La Paz from 2007-2012 were retrospectively reviewed. Improvement in inflammatory activity was graded according to grading schema of the Standardization of Uveitis Nomenclature Working Group.
Objectives: The aim of this study is to report a case of conjunctival lymphangiectasia simulating a pediatric pterygium.
Methods: A 10-year-old girl with Turner syndrome and familial history of pterygia presented because of a conjunctival growing lesion clinically consistent with pterygium in her left eye. Visual acuity (VA) was 20/20 in the right eye and 20/40 in the left eye.
Introduction: Medulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and is usually misdiagnosed clinically as a different type of optic nerve tumor.
View Article and Find Full Text PDFA 6-year-old boy presented with neovascular glaucoma secondary to total retinal detachment. Magnetic resonance imaging findings suggested diffuse infiltrating retinoblastoma. Enucleation was subsequently performed and led to a final histopathological diagnosis of advanced Coats' disease.
View Article and Find Full Text PDFPurpose: To describe epidemiologic and clinical findings of open-globe trauma (OGT) in the southeast area of Spain.
Methods: A retrospective descriptive study of 94 eyes with OGT evaluated between 1999 and 2007, in a primary referral hospital.
Results: The incidence of OGT in our area was 6.