[Exophthalmos and convergence insufficiency in Camurati-Engelmann disease].
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Biomedical subjects
Publications and source records attributed to M Stirpe.
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BACKGROUND: Optic disc pits represent a rare congenital abnormality frequently associated with macular detachment. Several theories on the pathogenesis of the detachment have been proposed, but the issue still remains unclear. Treatment remains speculative as well; advocated treatments include eye-patching, bed rest, steroids, laser photocoagulation, pars plana vitrectomy with fluid-air exchange and optic nerve sheath decompression. METHODS: The Authors report on the case of an optic pit with associated macular detachment which developed an inner layer macular hole and, after an apparently insignificant skull trauma, a large macular break. The patient underwent a standard three-port pars plana vitrectomy, fluid gas-exchange, posterior scleral buckling and laser treatment. RESULTS: Twelve months after surgery the retina was flat, posterior buckling was evident and laser treatment pigmented. Visual acuity was 20/600. CONCLUSIONS: The presence of strands of condensed vitreous strands crossing the posterior lacuna and exerting traction over the macula and optic disc, support the theory of a possible tractional role of anomalous vitreous adhesion in the pathogenesis of the detachment and macular break. Anomalous vitreous adhesion over the posterior retina could be related to the malformative genesis of the syndrome, and the anomalous persistence of the Cloquet's canal or primary vitreous could be responsible for the traction.
This report on 496 highly myopic eyes that underwent transcleral or vitreoretinal surgery for retinal detachment (RD) focuses particularly on how changes in the vitreous gel and the resulting modifications of the vitreoretinal interface produce typical characteristics and complications. According to the pattern of vitreous modifications the 496 eyes were divided into five groups: 1) eyes with uniform PVD (108 eyes) 2) eyes with PVD spreading towards the upper quadrants (231 eyes) 3) eyes with extensive vitreous liquefaction (EVL) and condensations of the vitreous base (51 eyes) 4) eyes with posterior vitreous lacuna (PVL, 87 eyes) 5) eyes with very limited PVD (19 eyes). Age, degree of myopia, surgical procedures and final results are reported for each group. A strong correlation was observed between vitreous changes and clinical picture of RD especially in the group of PVL and EVL. In the PVL group a higher degree of myopia was found and more pronounced posterior staphyloma. Frequently the posterior hyaloid, in the form of a thin, extremely smooth membrane, was hard to separate from the inner posterior retina during surgery. Posterior retinal breaks, including macular holes, were found in 56% of eyes. The presence of EVL with condensation of the vitreous base was correlated with giant retinal tear (GRT) in 70% of cases (36 of 51 eyes). Sixteen GRT were also found in the group of uniform PVD, but these were less extensive and located more posteriorly than in the EVL group. In the former group there were better surgical results because of a lower incidence of PVR. In 46% of the eyes of our series (group 2), PVD extended mostly in the upper quadrants with no vitreous detachment inferiorly. In these cases there was a clinical appearance of inferior vitreous collapse. These eyes had 92% of peripheral superior retinal breaks. Relapses of RD in this group almost invariably occurred in the inferior quadrants.
PURPOSE: To assess the efficacy of optical coherence tomography (OCT) for the morphological evaluation of idiopathic full-thickness macular holes and for detecting any morphological changes with time. METHODS: Serial sagittal tomographs through the macula were taken by OCT in a consecutive series of 34 eyes of 34 patients with diagnosis of idiopathic full-thickness macular hole. The patients were divided into two groups on the basis of "recent" (group 1, 25 patients) or "not-recent" (group 2, 9 patients) onset of symptoms related to the macular hole. Fourteen of the 25 patients in group 1 and all nine in group 2 underwent vitrectomy. The 11 in group 1 who refused surgery were observed by OCT examination with follow-up from 6 to 13 months. RESULTS: In most eyes OCT scans revealed two different anatomical features of macular holes depending on the time of onset of symptoms. Eleven of the 14 "recent-onset" holes that underwent surgery showed "sharp", undermining edges at preoperative OCT; the other three had "rounded" edges. Seven of the nine eyes operated for long-standing full-thickness macular holes had preoperative "rounded" edges, while the edges in the remaining two eyes were "sharp". OCT of eight of the 11 non-operated eyes in group 1 showed a morphological evolution of the macular hole edges from a "sharp" to a "rounded" contour and an increase in the diameter of the hole. CONCLUSIONS: OCT can help in the morphological evaluation of idiopathic full-thickness macular holes and in the detection of morphological changes with time.
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