Congenital iris ectropion and glaucoma associated with intestinal neuronal dysplasia: a manifestation of a neural crest syndrome.
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Biomedical subjects
Publications and source records attributed to Matthias C Grieshaber.
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PURPOSE: To describe a hypothesized relationship between optic disc haemorrhages (ODHs) and primary vascular dysregulation (PVD). METHODS: Observational case report of a patient with classical PVD and five bilateral recurrent ODHs RESULTS: The ODHs were superotemporal in the right eye and inferotemporal in the left; the eyes were otherwise normal. Intraocular pressure (IOP) never exceeded 17 mmHg. Visual fields were normal. Increased blood flow resistivity, a reduced blood flow of the extraocular vessels, a low systemic blood pressure, a cold-induced flow stop of the nailfold capillaries, and elevated endothelin-1 plasma levels were found, all confirming the diagnosis of vascular dysregulation. CONCLUSIONS: Optic disc haemorrhages may be due to a disturbed blood-retina barrier rather than to a mechanical rupture of the vessel. This barrier dysfunction may occur in the context of PVD.
PURPOSE: To present a modified surgical technique for preventing posterior capsule opacification (PCO) in children with congenital cataracts and to evaluate its long-term efficacy. SETTING: Department of Ophthalmology, Medical University of Southern Africa, Medunsa, South Africa. METHODS: Anterior and posterior vertical capsulotomy, with optic entrapment of the intraocular lens (IOL) by maintaining the anterior hyaloid, was performed in 68 cataractous eyes of children aged 2 months to 8 years (mean 3 years, 1 month). The posterior capsule was evaluated for at least 5 years for secondary opacification, IOL position, pigmentary deposits on the IOL optic, and the presence of synechias. RESULTS: Sixty-eight eyes maintained a clear visual axis for 5 to 12 years (mean 9 years, 1 month) postoperatively. No secondary procedure was necessary. In all eyes, the IOL remained well centered and entrapped. CONCLUSIONS: Posterior capsulotomy with optic entrapment of the IOL proved to be a safe and efficient surgical procedure for preventing PCO in children with congenital cataracts. Uniting the anterior and posterior capsule in front of the IOL limits the proliferation and migration of Elschnig pearls. An intact anterior hyaloid does not induce capsule opacification in association with optic entrapment; therefore, a vitrectomy is not indicated even in infants under age 5 years. Clear visual axis, centered IOL, and intact vitreous were achieved in this series; this enables a promising long-term prognosis for binocular visual development, especially because surgery was performed early.
PURPOSE OF REVIEW: Glaucoma, one of the leading causes of blindness in the world, is characterized by progressive visual field loss and distinctive excavation of the optic nerve head. Although elevated intraocular pressure is the major risk factor, there is increasing evidence that the pathogenesis of glaucoma is also linked to altered ocular blood flow. This review summarizes the recent publications relevant to blood flow in glaucoma. RECENT FINDINGS: Several studies indicate that a perfusion instability, rather than a steady reduction of ocular blood flow, might contribute to glaucomatous optic neuropathy. The main cause of the instability is a disturbed autoregulation in the context of a general vascular dysregulation. The underlying mechanism of such a vascular dysregulation is not known. A dysfunction of both the autonomic nervous system and vascular endothelial cells is discussed. SUMMARY: The mechanical and vascular theories are not mutually exclusive; on the contrary, a vascular dysregulation increases the susceptibility to intraocular pressure. Therapeutically, therefore, both an intraocular pressure reduction and an improvement of the ocular blood flow might be considered.