Malignant Hypertensive Retinopathy in an Infant with Mid-Aortic Occlusion.

Purpose. Case report describing an eight-month-old infant presenting with intermittent esotropia and irritability who was found to have malignant (grade 4) hypertensive retinopathy and mid-aortic syndrome. Methods. Visual acuity was 6/140 in the right eye and not recordable in the left eye. Blood pressure was as high as 230/120 mmHg. Fundoscopy revealed bilateral optic disc swelling, macular stars, and serous retinal detachment in the left eye, findings that are consistent with malignant (grade 4) hypertensive retinopathy. CT abdominal angiogram revealed a severe mid-aortic syndrome with occlusion of the abdominal aorta at T12. Results. The patient was treated with medical management of his hypertension, improving the subretinal exudate. Binocular visual acuity improved to 6/9.5 over 9 months. There was a persistent left relative afferent pupillary defect and moderate left esotropia. Conclusion. This is the first reported case of malignant hypertensive retinopathy in an infant with concomitant mid-aortic occlusion. The authors emphasize the need for an ophthalmological and pediatric examination in a child presenting with intermittent squint and irritability. The esotropia was found to be a false localizing sign of raised intracranial pressure secondary to the severe mid-aortic syndrome.

Outcomes in retinoblastoma, 1974-2005: the Children's Hospital, Westmead.

BACKGROUND: To report the 31-year experience of outcomes in retinoblastoma from a single centre. METHODS: A retrospective analysis of consecutive cases of retinoblastoma diagnosed and treated at the Westmead Children's Hospital, Sydney between 1974 and 2005 was performed. The subjects were analysed as two groups: those diagnosed between 1974 and 1989 (series alpha) and those diagnosed between 1990 and 2005 (series beta). RESULTS: There were a total of 142 patients included in the study, with a median follow up of 72 months. There were 84 patients with unilateral disease and 58 patients (116 eyes) with bilateral disease. The total enucleation rate remained high throughout both series for those with unilateral disease: 89% (series alpha) and 95% (series beta). There was a reduction in enucleations performed for those with bilateral disease from 68.4% (series alpha) to 43.6% (series beta) (P < 0.025). There were no bilateral enucleations performed after 1995. Actuarial Kaplan-Meier curves showed that 56% of all preserved eyes had not recurred at a median follow up of 95 months and 78.1% had avoided enucleation. Overall 43% of preserved eyes attained a visual acuity better than or equal to 6/12 and 55% achieved a visual acuity better than 6/60. There were four deaths due to retinoblastoma. Five patients were diagnosed with a second non-ocular malignancy. The most common treatment-related complications were cataracts, facial deformity, sepsis and febrile neutropaenia. CONCLUSIONS: The introduction of newer globe-preserving treatments for retinoblastoma was associated with equivalent visual outcomes, stable mortality rate and a greater number of short-term complications but avoided the late side-effects associated with external beam radiotherapy.

The Ahmed drainage implant in the treatment of pediatric glaucoma.

PURPOSE: To report the safety and efficacy of Ahmed Glaucoma Valve (New World Medical, Inc., Rancho Cucamonga, California, USA) implantation for the management of pediatric glaucoma in the early and intermediate follow-up period. DESIGN: Consecutive interventional case series. METHOD: A retrospective chart review was conducted on 60 eyes (44 patients, age range at surgery 1.5 months to 16 years, average 6 +/- 4.9 years), with pediatric glaucoma that underwent Ahmed Glaucoma Valve implantation for medically uncontrolled intraocular pressure (IOP) between the years 1995 and 2000. Outcome measures were control of IOP below 21 mm Hg, the need for antiglaucoma medications after surgery, and loss of 2 or more lines of Snellen acuity. Complications were monitored. RESULTS: The postoperative follow-up period for each eye averaged 24.3 +/- 16 months (range, 3 to 60 months). At last follow-up, IOP was controlled in 44 eyes (73%), 11 of which did not need antiglaucoma therapy. Kaplan-Meier life-table analysis showed probability of success with or without medications of 93% (95% confidence interval [CI], 86%-100%), 86% (95% CI, 77%-96%), 71% (95% CI, 59%-87%), and 45% (95% CI, 28%-80%) after 12, 24, 36, and 48 months of follow-up. Average IOP decreased from 32.8 +/- 6.2 before surgery to 16.6 +/- 8.0 postoperatively (P <.0001). The average number of medications used decreased from 4.4 +/- 1.97 to 2.0 +/- 2.0 (P <.0001). Kaplan-Meier survival analysis did not reveal any difference in survival profiles related to specific diagnosis of glaucoma, age (above or below 18 months), or prior surgery. Complications occurred in 30 eyes (50%). Although most resolved or were treated successfully, four patients had severe visual loss during the follow-up. Uveitis was a significant risk factor for tube exposure (Fisher exact test, P =.006). CONCLUSIONS: Ahmed Glaucoma Valve implantation is an effective treatment for pediatric glaucoma, although patients frequently require antiglaucoma medications. However, a high rate of potentially sight-threatening postoperative complications warrants ongoing close follow-up.