RESUMEN
Polyfibromatosis is a rare fibrosing condition characterized by fibromatosis in different body areas and by keloid formation, and which can be associated with arthropathy and osteolysis. Familial occurrence has been described, but the cause remains unknown. Here, we describe a patient with characteristics of polyfibromatosis with arthropathy who had in addition severe conjunctival fibrosis, distinctive face, gingival overgrowth, and pigmented keloids. We discuss the resemblances and differences with polyfibromatosis and descriptions of other, similar patients. We conclude that at present it remains uncertain whether the patient has a variant of polyfibromatosis or a separate entity.
Asunto(s)
Enfermedades de la Conjuntiva/patología , Fibroma/patología , Fibromatosis Gingival/patología , Artropatías/patología , Osteólisis/patología , Anomalías Múltiples/diagnóstico por imagen , Anomalías Múltiples/genética , Anomalías Múltiples/patología , Artrografía , Fisura del Paladar/diagnóstico por imagen , Fisura del Paladar/genética , Fisura del Paladar/patología , Hibridación Genómica Comparativa , Enfermedades de la Conjuntiva/diagnóstico por imagen , Enfermedades de la Conjuntiva/genética , Contractura/diagnóstico por imagen , Contractura/genética , Contractura/patología , Análisis Citogenético , Diagnóstico Diferencial , Fibroma/diagnóstico por imagen , Fibroma/genética , Fibromatosis Gingival/diagnóstico por imagen , Fibromatosis Gingival/genética , Fibrosis/diagnóstico por imagen , Fibrosis/genética , Fibrosis/patología , Articulaciones del Pie/diagnóstico por imagen , Articulaciones del Pie/patología , Sobrecrecimiento Gingival/diagnóstico por imagen , Sobrecrecimiento Gingival/genética , Sobrecrecimiento Gingival/patología , Articulaciones de la Mano/diagnóstico por imagen , Articulaciones de la Mano/patología , Humanos , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/genética , Hidrocefalia/patología , Artropatías/diagnóstico por imagen , Artropatías/genética , Queloide/diagnóstico por imagen , Queloide/genética , Queloide/patología , Deformidades Congénitas de las Extremidades/diagnóstico por imagen , Deformidades Congénitas de las Extremidades/genética , Deformidades Congénitas de las Extremidades/patología , Masculino , Persona de Mediana Edad , Osteólisis/diagnóstico por imagen , Osteólisis/genéticaAsunto(s)
Enfermedades de la Conjuntiva/inducido químicamente , Endotaponamiento , Granuloma de Cuerpo Extraño/etiología , Aceites de Silicona/efectos adversos , Adolescente , Enfermedades de la Conjuntiva/diagnóstico por imagen , Enfermedades de la Conjuntiva/cirugía , Enucleación del Ojo , Ojo Artificial , Femenino , Granuloma de Cuerpo Extraño/diagnóstico por imagen , Granuloma de Cuerpo Extraño/cirugía , Humanos , Implantes Orbitales , Retina/lesiones , Desprendimiento de Retina/etiología , Desprendimiento de Retina/cirugía , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Avascularity of the bleb is regarded as a risk of bleb infection, which is the most serious complication after filtration surgery with mitomycin C (MMC). There is no perfect way to prevent avascular bleb formation. We hypothesized that keeping the conjunctiva away from direct exposure to aqueous filtration may suppress avascular bleb formation after filtration surgery with MMC. In order to prove our hypothesis, we investigated whether a thin biodegradable, honeycomb-patterned film (HPF) can reduce bleb avascularity in a rabbit model of filtration surgery with mitomycin C (MMC). METHODS: A fornix-based full-thickness filtration surgery was performed in one eye of each of five rabbits (control group). The same surgery with intraoperative MMC treatment was done in both eyes of six rabbits, with one eye receiving a 14-µm-thick HPF made from poly(L-lactide-co-ε-caprolactone), which was placed subconjunctivally over the filtration site with the honeycomb surface facing up. Intraocular pressure (IOP) measurements, bleb evaluations using ultrasound biomicroscopy (UBM), and in vivo confocal microscopy (IVCM) were performed periodically for 4 weeks postoperatively followed by histological examination. RESULTS: The postoperative IOP decrease and bleb survival were significantly greater in MMC-treated eyes than in control eyes, and were similar between MMC-only and MMC + HPF eyes. The avascular area in the bleb persisted for 4 weeks in MMC-only eyes. Postoperative IVCM showed morphological changes of the conjunctival epithelial cells (i.e., enlarged and variable in size and shape) and epithelial defects in MMC-only eyes, and significantly lower density of connective tissue and vascularity in the subepithelial space in MMC-only eyes compared to the control eyes. These IVCM findings agreed with those by UBM and histology. Bleb avascularity shown by clinical bleb appearance, IVCM and histology, conjunctival epithelial damage shown by IVCM and histology, and loose subepithelial connective tissue shown by UBM, IVCM, and histology were all reduced in MMC + HPF eyes compared to MMC-only eyes. CONCLUSIONS: IVCM successfully showed the characteristic conjunctival damage in MMC-treated blebs. The concomitant use of a thin biodegradable HPF reduced avascularity and conjunctival damage in blebs, without compromising filtration in rabbits following filtration surgery with MMC.
Asunto(s)
Implantes Absorbibles , Alquilantes/administración & dosificación , Enfermedades de la Conjuntiva/prevención & control , Cirugía Filtrante , Mitomicina/administración & dosificación , Poliésteres/uso terapéutico , Animales , Materiales Biocompatibles , Enfermedades de la Conjuntiva/diagnóstico por imagen , Modelos Animales de Enfermedad , Células Epiteliales/patología , Presión Intraocular/fisiología , Microscopía Acústica , Microscopía Confocal , Conejos , Estructuras Creadas Quirúrgicamente , Tonometría OcularRESUMEN
PURPOSE: To report a patient with intraocular silicone oil migration into the subconjunctival space and orbit through an Ahmed glaucoma valve implant. METHODS: Observational case report. A 29-year-old female with diabetes mellitus and proliferative diabetic retinopathy underwent three left eye pars plana vitrectomies and ultimately intraocular silicone oil placement for tractional retinal detachment. Subsequently, she developed neovascular glaucoma and underwent an inferotemporal Ahmed glaucoma tube implant, left eye. RESULTS: Intraocular silicone oil migrated through the Ahmed glaucoma valve into the subconjunctival space and orbit. Left eye evisceration with silicone oil removal was performed for a blind painful left eye and improvement of appearance. CONCLUSION: Silicone oil migration through an Ahmed valve may be observed after retinal detachment repair with intraocular silicone oil. An Ahmed valve may be contraindicated in eyes of patients with intraocular silicone oil.