What's new AAPOS 2008 - The Private Eye Clinic

PEDIATRICS
Visual Manifestations of Craniofrontonasal Dysplasia.
JPOS 2007; 44:251-254.
Cranifrontal dysplasia is a rare, inheritable syndrome with features of coronal
craniosynostosis and frontonasal dysplasia. Clinical features include brachycephaly,
frontal bossing, orbital hypertelorism, thick, curly hair, longitudinally grooved nails, and
skeletal abnormalities. High prevelance of strabismus has previously been reported. A
44% prevalence of visual impairment was observed in the nine patients identified with
this syndrome. More than half of these patients were identified with potentially
correctable causes of visual loss, including amblyopia and anisometropia. Regular eye
exams in these individuals are recommended in order to identify not only strabismus
(88.9%) but other potentially amenable forms of visual impairment.
Neuroradiographic, endocrinologic, and ophthalmic correlates of adverse
developmental outcomes in children with optic nerve hypoplasia: A prospective
study.
Garcia-Fillon P, Epport K, Nelson M, Azen C et al.
Pediatrics 2008 Mar; 121(3):e653-e659.
The authors conducted a prospective study of 73 children who were diagnosed with
optic nerve hypoplasia prior to 36 months of age. They found that 71% of subjects had
developmental delay. Corpus callosum hypoplasia and hypothyroidism were
significantly associated with poor developmental outcomes. The authors conclude that
because there is this correlation with developmental delay, all children with optic nerve
hypoplasia should have neuroradiologic and endocrinologic testing for risk factors in
delay and developmental assessment so they may receive early intervention planning if
necessary.
Omega-3 polyunsaturated fatty acid intake and islet autoimmunity in children at
increased risk for Type 1 diabetes.
Norris J, Yin X, Lamb M, Barriga K et al.
JAMA 2007 Sep; 298(12):1420-1428.
Type 1 diabetes is an autoimmune disease that leads to the destruction of insulin
producing beta cells in the pancreas. A previous retrospective study from Norway
showed that children with diabetes were less likely to have been given Cod Liver Oil
during infancy than children without diabetes. In this longitudinal observational study,
children at risk for the development of type 1 diabetes were followed prospectively to
see if there was a correlation between Omega-3 fatty acid intake and the development
of islet autoimmunity. The investigators found that dietary intake with Omega-3 fatty
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