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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Outcomes of Ahmed glaucoma valve in paediatric glaucoma following congenital cataract surgery in persistent foetal vasculature.

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. RESULTS: A total of 29 eyes, 41% with persistent foetal vasculature and 59% with non-persistent foetal vasculature were included with mean follow-up of 105 ± 67 and 74 ± 45 months, respectively, after first Ahmed glaucoma valve implant. Median survival time for the first Ahmed glaucoma valve was significantly lower in persistent foetal vasculature (7.0 ± 3.2 months) compared to non-persistent foetal vasculature (over 129 months), p = 0.001. The cumulative probability of success in persistent foetal vasculature and non-persistent foetal vasculature eyes with Ahmed glaucoma valve were, respectively, 37.5% and 88.2% at year 1 and 28.1% and 71.9% at year 5. Cox regression model suggested persistent foetal vasculature as predictive risk factor of time to Ahmed glaucoma valve failure (hazard ratio: 5.77, p = 0.004). Four eyes developed phthisis bulbi. Mean intraocular pressure prior glaucoma surgery was 32.66 ± 6.73 mmHg and decreased to 16.54 ± 2.75 mmHg (p < 0.001) at final visit. The most frequent early postoperative complication was severe hypotony (32.6%) which tended to be self-limiting. Vitreous haemorrhage was associated with persistent foetal vasculature (p = 0.024). Ahmed glaucoma valve replacements after complications and additional Ahmed glaucoma valve implantations due to unsatisfactory intraocular pressure were more common in the persistent foetal vasculature group. CONCLUSION: Eyes with persistent foetal vasculature and secondary glaucoma after congenital cataract surgery followed by AGV implantation had a higher number of complications and a decreased probability of success compared to the non-persistent foetal vasculature group. Both groups achieved a significant decrease in intraocular pressure; thus, Ahmed glaucoma valve may be considered as first-line treatment in refractory glaucoma following congenital cataract surgery.

Clinical Characteristics and Treatment of Secondary Glaucoma After Pediatric Congenital Cataract Surgery in a Tertiary Referral Hospital in Spain.

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. Mean time of glaucoma onset after cataract surgery was 35 months (IQR: 5 to 96). At diagnosis, 81% of the eyes were aphakic and the majority presented with an open angle (86%). Multivariate analysis demonstrated that glaucoma diagnosis was made earlier in eyes with persistent fetal vasculature (ß = -0.334, P = .006) and aphakic eyes (ß = 0.404, P = .001). Two-thirds of eyes required surgical treatment for glaucoma. Seventy percent had an Ahmed glaucoma valve (New World Medical, Inc) implantation as their primary procedure, followed by trabeculectomy (24%) and synechiolysis with peripheral iridotomy (6%). All medically treated eyes and 78% of the surgically treated eyes achieved intraocular pressure (IOP) control at the final visit. CONCLUSIONS: Diagnosis of glaucoma after congenital cataract surgery seems to follow a bimodal distribution (years 1 and 5 after cataract surgery). Two-thirds of the eyes required surgical hypotensive treatment to achieve IOP control. Ahmed glaucoma valve implantation is a safe and effective surgical option to be considered as both first- and second-line treatment. Functional outcome was more favorable in those eyes with medically controlled glaucoma. [J Pediatr Ophthalmol Strabismus. 2020;57(5):292-300.].

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.

Conjunctival lymphangiectasia presenting as pediatric pseudopterygium.

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. Cycloplegic refraction was +1.50sph -0.50cyl×93° and +9.00sph -9.00cyl×180° in the right and left eyes, respectively. RESULTS: Resection of the lesion with amniotic membrane implantation and conjunctival autograft was performed. The histologic examination revealed conjunctival lymphangiectasia. Ten months later, the patient did not show any signs of recurrence. Refraction in the left eye was +0.5sph -1.5cyl×70°, and spontaneous VA was 20/20 in both eyes. CONCLUSIONS: Pediatric pseudopterygium may be the clinical presentation of several ocular surface disorders. Thus, pathologic analysis of this lesion should be considered to determine its exact nature. Surgery lessens the refractive defect and hastens visual recovery.

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.

Epidemiology of open-globe trauma in the southeast of Spain.

PURPOSE: 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 6.76 per 100,000 inhabitants/year. The majority of patients were male (89%) and young (80% of patients < 50 years old), with an average age of 37 +/- 20 years (mean +/- SD). The most common causes and location of injury were wire-induced trauma (50%) and accidents at work (56%), respectively. The types of injury encountered were ruptures, intraocular foreign bodies, perforating injury, penetrating injury, and mixed injury. Sixty-six percent of these injuries were penetrating in zone I (55%). The injuries found were vitreous hemorrhage (33%), cataracts (47%), vitreous prolapse (30%), retinal detachment (8%), endophthalmitis (2%), and associated with a facial trauma (7%). Eighty percent of surgery was carried out under general anesthesia. Fifty-one percent of the eyes underwent one operation only (5% were enucleated). Sixty-one percent of the eyes resulted in visual acuity of less than 50%. CONCLUSIONS: The incidence of OGT in the southeast of Spain is very high, being in most cases produced by accidents while using wire in greenhouses. This provokes severe monocular visual loss among the young population.

Actualización en cirugía oftálmica pediátrica

Libro del año 2000, en el que han participado gran número de colaboradores, no sólo oftalmólogos infantiles, también otorrinolaringólogos, maxilofaciales, neuro-cirujanos, radiólogos, anatomopatólogos y pediatras. Esta obra consta de 63 capítulos, clasificados en: Introducción, anestesia para cirugía oftálmica pediátrica, Segmento anterior, glaucoma, retinopatía del prematuro, retina y vítreo, retinoblastoma, estrabismo y órbita; además de contar con un apartado de colaboradores, agradecimientos y prólogo.El texto completo está accesible en formato html.