INTRODUCCIÓNLa lipomatosis simétrica múltiple (también denominada enfermedad de Madelung, enfermedad de Launois-Bensaude o adenolipomatosis) es una entidad infrecuente caracterizada por la presencia de tejido graso no encapsulado localizado preferentemente en cuello, nuca, región supraclavicular y porción superior de extremidades. Afecta sobre todo a varones de mediana edad del área mediterránea con hábito etílico prolongado. La etiopatogenia es desconocida, aunque se ha sugerido un trastorno del metabolismo lipídico inducido por la ingesta de alcohol. CASOS APORTADOSEn el presente trabajo describimos tres casos de esta enfermedad, con dos formas de presentación clínicamente diferentes. Caso 1. Mujer de 46 años con prolongada historia de etilismo que consultó por aumento simétrico del perímetro de la porción superior de ambas extremidades superiores de varios meses de evolución, con aspecto de obesidad simple (Fig. 1). El cuadro se acompañaba de discreto dolorimiento e impotencia funcional en extremidades superiores.En la analítica destacó una severa hipercolesterolemia (350 mg/dl). La radiografía de tórax resultó ser normal. La electromiografía demostró la existencia de una polineuropatía mixta de predominio axonal en ambas extremidades superiores. Se comprobó mediante biopsia la naturaleza lipomatosa de sus lesiones.Tras abstinencia alcohólica permanecen estables las masas lipomatosas y la clínica de polineuropatía.Caso 2. Varón de 52 años que presenta bultomas en región cervical posterior desde hace más de 5 años, los cuales han ido aumentando progresivamente de tamaño. Es fumador de más de 40 cigarrillos/día y bebedor de más de 100 g alcohol/día. Diagnosticado de hipertensión arterial de larga evolución, sufrió un accidente cerebrovascular lacunar izquierdo en 1999; actualmente sigue tratamiento con doxazosina y losartán. RESUMENLa lipomatosis simétrica múltiple o enfermedad de Launois-Bensaude es una entidad infrecuente caracterizada por el desarrollo de depósitos grasos subcutáneos en el cuello, hombros y porción superior de extremidades. La polineuropatía periférica es un hallazgo frecuente así como su asociación a alcoholismo, hepatopatía e intolerancia a la glucosa. La patogénesis es desconocida. Describimos tres casos con dos formas clíni-cas distintas de presentación y revisamos la literatura médica.

Purpose To identify factors associated with best-corrected visual acuity (BCVA) presentation and two-year outcome in 479 intermediate, posterior, and panuveitic eyes. Design Cohort study using randomized controlled trial data Methods Multicenter Uveitis Steroid Treatment (MUST) Trial masked BCVA measurements at baseline and 2 years’ follow-up used gold standard methods. Twenty-three clinical centers documented characteristics per protocol, which were evaluated as potential predictive factors for baseline BCVA and two-year change in BCVA. Results Baseline factors significantly associated with reduced BCVA included: age ≥50 vs. <50 years; posterior vs. intermediate uveitis; uveitis duration >10 vs. <6 years; anterior chamber (AC) flare > grade 0; cataract; macular thickening; and exudative retinal detachment. Over two years, eyes better than 20/50 and 20/50 or worse at baseline improved, on average, by 1 letter (p=0.52) and 10 letters (p<0.001) respectively. Both treatment groups and all sites of uveitis improved similarly. Factors associated with improved BCVA included resolution of active AC cells, of macular thickening, and cataract surgery in an initially cataractous eye. Factors associated with worsening BCVA included longer duration of uveitis (6–10 or >10 vs. <6 years), incident AC flare, cataract at both baseline and follow-up, pseudophakia at baseline, persistence or incidence of vitreous haze, and incidence of macular thickening. Conclusions Intermediate, posterior and panuveitis have a similarly favorable prognosis with both systemic and fluocinolone acetonide implant treatment. Eyes with more prolonged/severe inflammatory damage and/or inflammatory findings initially or during follow-up have a worse visual acuity prognosis. The results indicate the value of implementing best practices in managing inflammation.

Purpose To describe fluocinolone acetonide implant dissociations in the Multicenter Uveitis Steroid Treatment (MUST) Trial. Design Randomized clinical trial with extended follow-up. Methods Review of data collected on the first implant in the eye(s) of participants. Dissociation was defined as the drug pellet no longer being affixed to the strut and categorized as spontaneous or surgically-related. Results 250 eyes (146 patients) had at least one implant placed. Median time follow-up time after implant placement was 6 years (range 0.5 to 9.2). Thirty-four dissociations were reported in 30 participants. There were 22 spontaneous events in 22 participants; 6-year cumulative risk of a spontaneous dissociation was 4.8% (95% confidence interval (CI): 2.4%–9.1%). The earliest event occurred 4.8 years after placement. Nine of 22 eyes with data had a decline in visual acuity ≥5 letters temporally related to the dissociation. 39 implant removal surgeries were performed, 33 with replacement. Twelve dissociations were noted during implant removal surgeries in 10 participants (26%, 95% CI 15%–48%); 5 of these eyes had a decline in visual acuity ≥5 letters after surgery. The time from implant placement to removal surgery was longer for the surgeries at which dissociated implants were identified than for those without one (5.7 vs 3.7 years, p < 0.001). Overall, visual acuity declined 15 or more letters from pre-implant values in 22% of affected eyes; declines were frequently associated with complications of uveitis or it’s treatment. Conclusion There is an increasing risk of dissociation of Retisert implants during follow-up, the risk is greater with removal/exchange surgeries, but both the risk of spontaneous and surgically related events increase with longevity of the implants. In 22% of affected eyes visual acuity declined by 15 letters. In the context of eyes with moderate to severe uveitis for years, this rate is not unexpected.