Optical coherence tomography angiography findings in patients with Alport syndrome / Alterações encontradas na angiografia por tomografia de coerência óptica (OCT-A) em pacientes com síndrome de Alport

ABSTRACT Purpose: To describe the findings on optical coherence tomography angiography associated with Alport syndrome. Methods: Descriptive study from a referral ophthalmology service (Hospital Evangélico de Vila Velha, Brazil). Patients diagnosed with Alport syndrome were included. Results: The study group consisted of four patients (one female and three males) diagnosed with Alport syndrome. Visual acuity in the worst eye was between 20/40 and 20/60. All male patients had anterior lenticonus on biomicroscopy. The observed retinal findings included dots and flecks and pigmentary changes in the macula. On optical coherence tomography angiography, the inner retinal layers of all patients displayed thinning (especially in the temporal quadrant of the macula) and an increase in the foveal avascular zone. A thick choroid was observed in both eyes of the two youngest patients. Conclusions: In patients with Alport syndrome, the inner retinal layers suffer changes due to type IV collagen mutations. Optical coherence tomography angiography makes it possible to visualize and document these findings, making it a useful tool in the detection of early retinal findings associated with Alport syndrome.

RESUMO Objetivos: Descrever os achados na angiografia por tomografia de coerência óptica associada à síndrome de Alport. Métodos: Estudo descritivo de um serviço de referência em Oftalmologia (Hospital Evangélico de Vila Velha, Brasil). Os pacientes diagnosticados com síndrome de Alport, foram incluídos. Resultados: O grupo de estudo foi composto por quatro pacientes (um feminino e três homens) com diagnóstico de síndrome de Alport. A acuidade visual no pior olho estava entre 20/40 a 20/60. Todos os pacientes do sexo masculino apresentaram lenticone anterior à biomiscroscopia. Os achados da retina observados incluíram pontos e manchas e alterações pigmentares na mácula. Na angiotomografia de coerência óptica, as camadas internas da retina de todos os pacientes apresentaram afinamento (especialmente na região temporal da mácula) e aumento da zona avascular foveal. Uma coroide espessa foi observada em ambos os olhos dos dois pacientes mais jovens. Conclusões: Em pacientes com síndrome de Alport, as camadas internas da retina sofrem alterações devido à mutação do colágeno tipo IV. A angiotomografia de coerência óptica permite visualizar esses achados, tornando-o uma ferramenta útil na detecção de achados iniciais da retina associados à síndrome de Alport.

Effect of preoperative use of topical prednisolone acetate, ketorolac tromethamine, nepafenac and placebo, on the maintenance of intraoperative mydriasis during cataract surgery: A randomized trial.

Purpose: To compare the effects of preoperative use of topical anti-inflammatory prednisolone acetate, ketorolac tromethamine, nepafenac and placebo, on the maintenance of intraoperative mydriasis during cataract surgery. Design: Randomized clinical trial. Materials and Methods: This single-center, masked, randomized clinical study comprised 140 patients scheduled for cataract surgery. Patients (35 in each group) were randomized to receive placebo, prednisolone acetate, ketorolac tromethamine 0.4% or nepafenac. These eye drops were administered three times daily for the two days prior to surgery. The pupillary diameters were measured by the surgeon using a compass prior to the corneal section and at the end of surgery. The primary outcome was the number of patients with pupil ≥ 6mm at the end of the surgery; the secondary outcome was the number of patients with pupil ≥ 6mm at the beginning of the surgery. Results: All the patients achieved pupil ≥ 6mm at the beginning of the surgery. The number of patients in the prednisolone (29/35), nepafenac (31/35) and ketorolac (30/35) groups with pupil ≥ 6mm was greater than in the placebo group in the maintenance of intraoperative mydriasis (19/35 – P =0.003). There was no statistical difference among the prednisolone, nepafenac and ketorolac groups in the maintenance of intraoperative mydriasis (P =.791). There were no complications during surgery or related to the preoperative use of the eye drops. Conclusion: Preoperative use of ketorolac, prednisolone and nepafenac was effective in maintaining intraoperative mydriasis when compared with placebo.

Growth of the lower spleen pole remaining after subtotal splenectomy in rats

PURPOSE: To determine whether the lower pole of the spleen grows after subtotal splenectomy following ligature of major spleen blood vessels. METHODS: Thirty-nine Wistar rats (328.8 ± 27.8 g) submitted to subtotal splenectomy with preservation of the lower splenic pole were divided into two groups: group 1 (control, n=20), immediate removal of the lower pole; group 2 (n=19), removal of the pole on postoperative day 80. The length, width and thickness of the pole were measured. In the control group, mean percent pole weight was calculated immediately after surgery in a direct and indirect manner. In the first case, the weight of the lower pole was divided by overall spleen weight; in the second case, pole weight was divided by the ideal weight of the spleen obtained by linear regression analysis. The results of the two calculations were compared. Macro- and microscopic examinations of the pole were performed. RESULTS: In group 1, no significant difference in mean percent pole weight was observed between the direct and indirect method. In group 2, mean percent pole weight obtained by indirect calculation on day 80 was higher than in group 1 (p<0.001). In group 2, mean length, width and thickness of the pole remnant increased from the first to the 80th day (p<0.05). Histological analysis showed preserved tissue architecture and features compatible with cell hyperplasia in group 2. CONCLUSION: The lower pole splenic remnant presented statistically significant growth up to postoperative day 80 after subtotal splenectomy, even after ligature of the major spleen vessels. Light microscopy revealed changes compatible with cell hyperplasia.

OBJETIVO: Verificar se o pólo inferior do baço cresce após a esplenectomia subtotal mesmo com a ligadura dos vasos esplênicos principais. MÉTODOS: 39 ratos, Wistar, pesando 328,8g ± 27,79 foram submetidos à esplenectomia subtotal com preservação do pólo inferior e distribuídos em dois grupos de acordo com a época da retirada do referido pólo: 1- controle (n=20)-retirada imediata; 2(n=19); retirada no 80º dia de pós-operatório. Foram medidos o comprimento, largura e espessura do pólo inferior. No grupo controle, o percentual médio do pólo inferior foi calculado imediatamente após a cirurgia de forma direta e indireta, e no grupo 2 de forma indireta. No cálculo direto dividiu-se o peso do pólo inferior pelo peso global do baço. No cálculo indireto dividiu-se o peso do pólo inferior pelo peso ideal do baço obtido por análise de regressão linear. Foi realizada a comparação entre esses dois cálculos. Foi realizado o exame macro e microscópico do pólo inferior. RESULTADOS: Não houve diferença significante entre o cálculo direto e indireto do percentual médio do pólo inferior no grupo 1. No grupo 2 o percentual médio do pólo inferior, por cálculo indireto, no 80º dia foi maior que no grupo 1 (p <0,001). A média do comprimento, largura e espessura desse remanescente no grupo 2 aumentou do 1º para o 80º dia( p<0,05). A análise histológica mostrou no grupo 2 manutenção da arquitetura esplênica e sinais compatíveis com hiperplasia celular. CONCLUSÃO: O pólo inferior do baço remanescente da esplenectomia subtotal, mesmo com a ligadura dos vasos esplênicos principais, apresentou média de crescimento significante no 80º dia de pós-operatório e à microscopia óptica de luz houve sinais compatíveis com hiperplasia celular.