TY - JOUR T1 - The nanophthalmic macula JF - British Journal of Ophthalmology JO - Br J Ophthalmol SP - 276 LP - 279 DO - 10.1136/bjo.82.3.276 VL - 82 IS - 3 AU - J C Serrano AU - P R Hodgkins AU - D S I Taylor AU - G A Gole AU - A Kriss Y1 - 1998/03/01 UR - http://bjo.bmj.com/content/82/3/276.abstract N2 - AIMS To define an unusual macular appearance found in association with nanophthalmos. METHODS A case review. RESULTS Seven children (aged 8 months to 17 years) with nanophthalmos were examined. They all exhibited the same clinical findings of an unusual yellow macula appearance with retinal folds and crowded optic discs. Visual electrophysiology performed in four cases was normal. CONCLUSION A distinctive yellow macular pigmentation with associated chorioretinal folds and crowded optic discs is present in nanophthalmos. It is proposed that the retinal folds are due to a disparity between scleral and retinal growth while the macula discoloration is due to a congenital abnormality in arrangement or position of the luteal pigment and is not degenerative. Included in this case series is the second case in the literature of nanophthalmos associated with Kenny’s syndrome. Inheritance of nanophthalmos appears to be autosomal recessive. ER -