Thirty-nine patients who were current or recent users of PPS. Using standardized visual acuity (VA) testing and multimodal imaging, we investigated the impact of PPS therapy on vision and described an expanded spectrum of imaging findings among PPS users. There is growing evidence of a direct association between pentosan polysulfate (PPS) therapy and the development of macular changes. Sclerotherapy had a role in treating macrocystic LM and OVM3. A pure transnasal approach with mass removal and reconstruction of the medial wall with polyethylene sheets was chosen for OVM1 (intra- or extraconal) located in the medial or superomedial compartment. Overall, surgery was the technique of choice for OVM1, microcystic lymphatic malformations (LM) and arteriovenous malformations (AVM). These behave differently from each other, and a prompt differential diagnosis is mandatory to pose correct indications, minimize risks and improve results. Moreover, the authors have analyzed in detail venous malformations, identifying three different types, named orbital venous malformation (OVM) 1, 2 and 3. Each type of malformation has been evaluated separately in terms of diagnosis, indications for treatment, techniques and outcomes. ![]() ![]() In this study, two institutions have retrospectively collected their cases, consisting in a total of 69 vascular malformations of the orbit. The complex anatomy of the orbit and the fact that its content easily suffers from compartmental syndrome explain why treating orbital vascular malformations can be challenging and technically demanding. However, also lymphatic and arteriovenous malformations can affect the orbit. Isolated venous malformations (frequently misnamed as cavernous hemangiomas) are the most frequent among these. Identifikatorer URN: urn:nbn:se:uu:diva-104914 DOI: 10.1111/j. ISI: 000257020600016 PubMedID: 18162058 OAI: oai:DiVA.Vascular malformations are often found inside the orbit. 329-337Ĭhorioretinal atrophy, electroetinography, LCHAD deficiency, myopia HSV kategori Unusual chorioretinal findings should alert the ophthalmologist to the long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive. LCHAD deficiency, a potentially lethal disease, is sometimes difficult to diagnose. Late diagnosis with severe symptoms at diagnosis, neonatal hypoglycaemia and frequent decompensations may increase the progression rate of the chorioretinopathy. In one patient, unilateral visual impairment was associated with fibrosis.Įarly diagnosis and adequate therapy might delay but not prevent the progression of retinal complications. The chorioretinopathy often started in the peripapillary or perimacular areas. Milder chorioretinopathy with or without subnormal visual acuity was present in all other children. Profound chorioretinal atrophy, severe visual impairment and progressive myopia had developed in two teenagers. Regardless of age at diagnosis, initiation of treatment and age at examination, inter-individual differences were present. Ten children with LCHAD deficiency underwent repeated ophthalmological evaluations including ERG.Īll 10 children developed chorioretinal pathology. To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency - a life-threatening metabolic disease - and the relation to age at diagnosis, treatment and other clinical parameters. 329-337 Artikkel i tidsskrift (Fagfellevurdert) Published Abstract Vise andre og tillknytning 2008 (engelsk) Inngår i: Acta Ophthalmologica Scandinavica, ISSN 1395-3907, E-ISSN 1600-0420, Vol.
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