Br J Ophthalmol 1998;82:276-279 ( March )
The nanophthalmic macula
J C Serrano,a
P R Hodgkins,a
D S I Taylor,a
G A Gole,b
A Krissa
a Department of Ophthalmology, Great Ormond Street
Hospital for Children, Great Ormond Street, London WC1N 3JH, b Department Of Ophthalmology, Royal Children's Hospital,
Brisbane, Queensland, Australia
Correspondence to: Mr D Taylor.
Accepted for publication 29 September 1997
AIMS
To define an unusual macular appearance found
in association with nanophthalmos.
METHODSA case review.
RESULTSSeven 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.
CONCLUSIONA 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.
Keywords:
nanophthalmos;
maculopathy;
Kenny's syndrome
© 1998 by British Journal of Ophthalmology
