We present ocular findings of 20 patients with the recessively inherited muscle-eye-brain (MEB) disease, characterised by severe visual failure, mental retardation, a pachygyria-polymicrogyria type neuronal migration disorder and congenital muscular dystrophy. The ocular findings consisted of myopia ranging from -6 to -27 D, retinal degeneration and optic atrophy. Five infants had congenital glaucoma, and juvenile cataracts developed in 9 children. The visual evoked potentials were abnormally high (> 50 microV) and delayed in 70% of patients. The electroretinogram was abolished in 12 patients. The changes were progressive during the follow-up time, which was up to 20 years.

Adolescent↗

PubMedDate not supplied

Transillumination guided cyclocryotherapy in the treatment of secondary glaucoma.

A consecutive series of 38 eyes of 35 glaucoma patients treated with transillumination guided cyclocryotherapy (CCT) either to obtain pain relief or to achieve IOP control was studied at Helsinki University Eye Hospital. Transillumination was used to guide the applications to the correct site. The follow-up range was 1-43 months (mean 9 months). Pretreatment IOP was 39 +/- 13 mm Hg and postoperative 26 +/- 16 mm Hg (mean +/- SD). Twenty-three eyes underwent one and 15 eyes 2-5 procedures. IOP control (IOP 9-23 mm Hg) was achieved in 50%. The response was more favorable in eyes without iris neovascularisation (59% achieved IOP control) than in eyes with neovascular glaucoma (38% achieved IOP control). The best response was achieved in eyes with chronic uveitis (5/7 eyes achieved IOP control). Repeated procedures improved the IOP control rate. Hypotony (IOP < 9 mm Hg) without clinical signs of phthisis occurred in three eyes (8%). Thirteen eyes needed primarily pain relief and 92% achieved it. Postoperatively vision was better in 8%, unchanged in 37% and worse in 55%. Visual acuity was 0.05 or better in nine eyes pretreatment and in eight eyes after treatment.

Adult↗

PubMedDate not supplied

Management and prognosis of gliomas of the optic pathways in children.

A follow-up study was made of 24 children with glioma of the optic pathways. In gliomas restricted to one optic nerve, total excision should be performed. The prognosis is excellent. Radiotherapy is not indicated. In gliomas of the anterior chiasm, a biopsy should be taken; occasionally, partial removal is indicated. The prognosis is good. Radiotherapy is indicated only if the follow-up shows a progression in the visual signs. In most cases, the growth of the tumor seems to be arrested. The vision remains stable and most patients have a useful degree of vision in at least one eye. In gliomas of the posterior chiasm with hypothalamic signs, the prognosis is poor. A biopsy and radiotherapy are indicated.

Adolescent↗