Biomedical subjects
Publications and source records attributed to H Deconinck.
We report an unusual case of ophthalmoplegia after maxillofacial surgery. A thirteen year old girl with unilateral left cleft lip and palate underwent maxillary advancement by distraction osteogenesis. Postoperatively she developed diplopia. The diplopia was not due to orbital lesions, most often seen after maxillofacial surgery, but to a haemorrhage posterior to the cavernous sinus.
The cause of amblyopia by ametropia is evaluated, hypoaccommodation plays an important role and also astigmatism. The treatment of hypermetropia by full spectacle correction is contested and the tests used to detect amblyopia and hypoaccommodation are described. The use of videorefraction and photoscreening is important in the follow-up of our patients.
Megalocornea (corneal diameter > or = 13 mm) is associated with mental and neurological impairment, and minor anomalies in Neuhäuser syndrome (megalocornea-mental retardation syndrome). Here we report 4 new cases of megalocornea and mental retardation. Those unrelated patients have a consistent pattern of anomalies with possible recessive inheritance which clearly differs from that of the original patients of Neuhäuser et al. [1975]. We discuss the heterogeneity of the syndromes with megalocornea and mental retardation. Based on these cases and on a review of the literature, we suggest a provisional clinically oriented classification in 5 subtypes: (1) a recessive form type Neuhäuser (with iris hypoplasia and minor anomalies), (2) a recessive form type Frank-Temtamy (with camptodactyly, scoliosis and growth retardation), (3) a recessive type 3, including our 4 personal cases (with normal irides, severe hypotonia, relative or absolute macrocephaly and minor anomalies), (4) a possible Frydman type (with normal irides, megalencephaly and obesity), and (5) provisionally unclassifiable cases.
We report a case of atypical bilateral endogenous mycotic endophthalmitis in an infant. A diagnostic vitrectomy was done on the left eye. Electron microscope examination of the vitreous demonstrated fungal mycelia. Intravenous treatment with amphotericin B seemed effective for treating the infection of the right eye, but a vitrectomy was necessary to prevent deprivation amblyopia and retinal traction, secondary to persistence of a dense vitreous membrane.
A family with hereditary optic neuropathy was investigated. Visual acuity, Goldmann perimetry, colour vision test, fundoscopy and electrophysiological examination (flash and pattern ERG and VER) were performed. The pattern of inheritance and the results of all these tests are discussed in this article.
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