[Retinal neovascularization with myelinated nerve fibers]. / Néovaisseaux rétiniens compliquant des fibres à myéline.

INTRODUCTION: The prevalence of myelinated retinal nerve fibers is 0.3%-0.6% of eyes. Although they often constitute benign lesions, they are rarely associated with retinal vascular abnormalities including preretinal neovascularization. CASE REPORT: A 31-year-old patient, with no previous pathological antecedents, consulted us for myodesopsia in her left eye. The exam found a visual acuity of 10/10 and a normal anterior segment in the two eyes. At the ophthalmoscopic examination of the left eye, we noted myelinated nerve fibers in the inferotemporal quadrant that were associated with a temporal neovascular bouquet covering area of 1.5 optic disks. A small amount of intravitreal hemorrhage was found. The ophthalmoscopic examination of the right eye was normal. The treatment consisted in sector-based photocoagulation with argon laser and cryotherapy. DISCUSSION: The source of neovascularization in the myelinated retinal nerve fibers was discussed. Underlying retinal ischemia is the most probable mechanism. The treatment was based on sector-based or panretinal photocoagulation. CONCLUSION: Preretinal neovascularization is a rare complication of myelinated nerve fibers. The earlier the care is given, the more complications can be avoided.

[Allgrove syndrome. Report on a family]. / Syndrome d'Allgrove: à propos d'une famille.

Triple A or Allgrove syndrome is a rare autosomal recessive disease with alacrima, achalasia, and ACTH-resistant adrenal insufficiency. It is usually associated with neurological disorders. Recently, mutations in the AAAS, a candidate gene mapped to chromosome 12q13, were identified. We report a family with seven affected siblings. All of them have signs of alacrima, four were operated on for achalasia, five have neurological abnormalities including cranial nerve abnormalities, amyotrophic lateral sclerosis, pyramidal syndrome, distal motor neuropathy, and amyotrophy, and two have adrenal insufficiency. Triple A syndrome should be considered in any young patient with alacrima.