The reliability of the angle of deviation measurement from the Photo-Hirschberg tests and Krimsky tests.

OBJECTIVE: To compare the angle of deviation measured from Photo-Hirschberg testing and Krimsky testing, with that from an alternate prism cover test (APCT) in strabismus patients. METHODS: A cross-sectional study was conducted in Songklanagarind Hospital, Thailand. Thirty-three strabismus patients were photographed for analysis by Photo-Hirschberg testing using computer software. The corneal light reflex displacement, converted into prism diopter (PD), was compared to the angle of deviation measured with APCT. Twenty-eight strabismus patients were tested with the Krimsky test. Data were analyzed using Pearson correlation and paired t-tests. The study excluded 4 intermittent exotropia cases, 1 intermittent esotropia case and 2 which cases missing data for krimsky test. RESULTS: The mean±SD of the deviation angle, measured by APCT with a fixation target at 30 cm and 6 m; were 48.09±16.34PD and 47.82±15.73 PD, respectively. At 1 m, the difference in the angle of deviation measured from APCT and the Photo-Hirschberg test within 10 PD were 58.8% and 63.6%, for ET and XT, respectively. The difference in the angle of deviation measured from APCT and Krimsky tests within 10 PD in ET and XT were 86.7% and 80.0%, respectively. At 4 m, the difference in angle of deviation measured from APCT and Photo-Hirschberg tests within 10 PD in ET and XT were 58.8% and 54.5%, respectively; whereas, the difference in the angle of deviation measured from APCT and Krimsky tests within 10 PD in ET and XT were 80.0% and 70.0%, respectively. CONCLUSION: The reliability of Krimsky test was better than Photo-Hirschberg test for measuring an angle of deviation.

Two Thai families with Norrie disease (ND): association of two novel missense mutations with severe ND phenotype, seizures, and a manifesting carrier.

We describe two Thai families with Norrie disease (ND) in three generations, including 10 affected males and one manifesting female. All affected males in each family had severely defective eye development with complete loss of vision. In addition, three male patients (one from family 1 and two from family 2) suffered from epilepsy, and one female carrier from one family manifested blindness with phthisis bulbi in her right eye. Mutation analysis of the ND gene (NDP) revealed two different novel missense mutations (L16P and S75P) that co-segregated with ND in each family, suggesting that the newly appearing proline at codon 16 or codon 75 alters the conformation of the ND protein and contributes to the severe phenotype of ND in each family. Other studies suggest that epileptic seizures or growth retardation that is associated with ND is the consequence of loss of contiguous genes, because most such patients had deletions extending beyond the Norrie locus. Our finding that the three affected males in the two families with the missense mutations had epilepsy does not support a contiguous gene effect, but favors the pleiotropism of NDP, at least as far as the epileptic manifestation is concerned. The unilateral blindness in the female carrier may have been due to non-random X-inactivation.

Persistent infra-orbital nerve hyperaesthesia after blunt orbital trauma.

BACKGROUND: Hyperaesthesia involving the infra-orbital nerve is rarely associated with orbital floor fracture. METHODS: An 11-year-old boy and a 34-year-old man had persistent and severe hyperaesthesia in the distribution of the infra-orbital nerve 12 and 26 months, respectively, after blunt orbital trauma. RESULTS: Surgical decompression of the infra-orbital nerve resulted in rapid and complete resolution of the hyperaesthesia in both cases. CONCLUSION: Persistent hyperaesthesia of the infra-orbital nerve is another indication for exploration of the orbital floor following blunt orbital trauma.