Vitreous diagnosis in neoplastic diseases.

Vitreous body is an intraocular structure, origin of diverse pathologies, but is also the place where cells and inflammatory mediators are released coming from several pathologic processes. These inflammatory reactions can happen in any other ocular location like choroid, retina, optic nerve, or ciliary body and vitreous humor constitutes a stagnant reservoir for these resulting substances and debris. Through the recent techniques of vitreous collecting, handling, and analysis, increasingly more sophisticated and with fewer complications, cellularity and molecules in the vitreous of challenging pathologies for the ophthalmologist can now be studied. The most usefulness for vitreous diagnosis would be the masquerade syndromes, and the best exponent in this group is the primary vitreoretinal lymphoma (PVRL), in which cytology and an IL-10/IL-6 ratio more than 1 is fundamental for the diagnosis.

Evaluación de oftalmólogos no expertos en el análisis de la retinopatía del prematuro / Assessment of non-expert ophthalmologists in the analysis of retinopathy of prematurity

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Clinical Management of Orbital Rhabdomyosarcoma in a Referral Center in Spain.

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. RESULTS: The mean age at presentation was 8 years (range: 3 months to 12.5 years). In all cases, the rhabdomyosarcoma was located primarily in the orbit. Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy. All of the patients underwent surgical biopsy for diagnosis confirmation. Orbital exenteration was performed in 4 cases (28%). Twelve patients received radiotherapy. The long-term visual outcomes of the 10 patients who maintained their globe was as follows: best corrected visual acuity 20/20 to 20/40 in 6 patients (60%), 20/50 to 20/100 in 2 patients (20%), and 20/200 to no light perception in 2 patients (20%). Intraocular complications (primarily cataracts: 50%) were present in 7 patients (70%), ocular surface lesions occurred in 6 patients (60%), and orbital sequelae were found in 8 patients (80%). Local tumor recurrence was detected in 5 patients (35%) and distant metastasis occurred in 2 patients (14%). Tumor-related death occurred in 1 patient (7%). CONCLUSIONS: Orbital rhabdomyosarcoma has an excellent prognosis; nevertheless, local complications are common, including surgery-related complications. To minimize them, initial surgical planning based on individual patient characteristics and an accurate diagnosis of relapses is mandatory. The clinical presentation, management, and long-term ocular and systemic outcomes are comparable with other series published to date.

External subretinal drainage, bevacizumab, and scleral buckling for complete exudative retinal detachment after photocoagulation in retinopathy of prematurity.

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. The dense subretinal fluid was externally drained using a bent needle with an infusion placed in the anterior chamber. Additional bevacizumab and scleral buckling helped to control the plus disease and subretinal leakage. RESULTS: Retinal apposition was obtained with the described approach. CONCLUSION: Total serous retinal detachment is a rare but severe visual complication in retinopathy of prematurity. The described technique may restore the retinae immediately in a visually critical period.

Adalimumab therapy for refractory childhood uveitis.

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. RESULTS: A total of 15 patients participated in the study (12 girls; mean patient age, 12 years). Diagnoses included juvenile idiopathic arthritis in 10 patients, idiopathic uveitis in 4, and familial juvenile systemic granulomatosis or Blau syndrome in 1. Mean follow-up was 32 months (median, 36; range, 15-58 months). Improvement in inflammatory activity was initially observed in 12 (86%) of 14 children, with a mean time to achieve response of 6 weeks (median, 4; range, 1-18). Treatment was effective in 9 patients (60%), mildly effective in 2 (13%), ineffective in 2 (13%), and resulted in worsening in 2 (13%). In the juvenile idiopathic arthritis patients, response was effective in 6 cases (60%), mildly effective in 2 (20%), and ineffective in 2 (20%). Adalimumab therapy was discontinued in 4 patients. CONCLUSIONS: Adalimumab was effective in most patients in the initial control of acute inflammatory activity in children with refractory uveitis, although therapy appears to become less effective in the long term.

Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report.

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. Here, we describe a new and atypical case of medulloepithelioma of the optic nerve in a 12-year-old boy. To the best of our knowledge, he is the oldest reported patient to present with this disease and, now as an adult, has the longest documented period of disease-free survival. CASE PRESENTATION: A 12-year-old Caucasian boy with headache and unilateral amaurosis was referred for a presumed optic nerve glioma to our hospital. A computed tomography scan showed optic nerve enlargement, and fundoscopy showed a whitish mass at the optic disc. Our patient had been followed at his local hospital for four years for an 'optic disc cyst' with no change or progression. He experienced mild progressive visual impairment during that period. He was admitted for resection, and a histopathological analysis revealed a medulloepithelioma of the optic nerve. Supplemental orbital radiotherapy was performed. He remained disease-free for 25 years. CONCLUSIONS: Medulloepithelioma of the optic nerve can clinically mimic more common pediatric tumors, such as optic glioma, meningioma, or retinoblastoma. Thus, medulloepithelioma should be included in the differential diagnoses of pediatric optic nerve lesions. Fundoscopy in these patients may provide relevant information for diagnosis. Anterior optic nerve medulloepitheliomas may behave differently from and have a better prognosis than medulloepitheliomas that have a more posterior location. Our case report illustrates that long-term survival can be achieved in patients with this malignant tumor.