External dacryocystorhinostomy in consultants and fellows - a comparison of the causes of failure.

PURPOSE: Failure of primary dacryocystorhinostomy (DCR) often requires revision surgery to inspect the cause of failure and re-establish anatomic patency. This study aims to specifcally compare the anatomical causes of failure noted during revision DCR of primary external DCR (EX-DCR) and compare the difference between consultants and fellows. METHODS: A retrospective review of 37 patients who underwent revision of a primary external approach DCR over a 7-year-period in a University Hospital. All primary surgery was performed by either a consultant surgeon or senior oculoplastic fellow. Details of the initial pathology prior to primary DCR and grade of operating surgeon were collected along with perioperative surgical findings. The cause of failure of the initial surgery was classified according to perioperative findings. Failure was classified as either inappropriately sized/located ostium or fibrous/membranous soft tissue obstruction of the newly created ostium. RESULTS: The cause of failure of the initial surgery was soft tissue obstruction in 43.3% and an inappropriately sized/located ostium in 56.7%. In those patients whose primary surgery was performed by a consultant, 73.3% were found to have a soft tissue obstruction and 26.7% were found to have an inappropriately sized/ located ostium. In contrast, if initial surgery was performed by a fellow, 22.7% were found to have a soft tissue obstruction and 77.3% an inappropriately sized/ located ostium (p =0.002). CONCLUSIONS: Where the primary surgeon has been a trainee there is a trend toward inadequately sized or located ostium being the most likely causative factor in failure of primary EX-DCR.  Hippokratia 2015; 19 (3): 216-218.

Phenotypic progression in X-linked retinitis pigmentosa secondary to a novel mutation in the RPGR gene.

PURPOSE: To report phenotypic progression for a novel mutation in the RPGRgene causing X-linked retinitis pigmentosa (RP), and describe the phenotype in affected males and females. METHODS: Bidirectional fluorescent sequencing analysis was used to screen for mutations in RPGR. Five affected males and eight affected females from two English families underwent refraction, ETDRS visual acuity, OCT imaging, and Goldmann visual field testing. RESULTS: DNA analysis identified a novel c.350G>A sequence change in exon 5 of RPGR. The change segregated with disease in both families. For affected males there was a significant correlation between age and visual acuity (r=-0.91, P=0.034), and a non-significant correlation between age and visual field area (r=-0.56, P=0.4). For affected females, there was a significant correlation between age and visual acuity (r=-0.8, P=0.018), and between age and visual field area (r=-0.94, P=0.005). All affected females were highly myopic. No correlation between retinal thickness, and either age or sex was noted. CONCLUSION: This novel mutation in RPGRcauses X-Linked RP with complete penetrance in males and females. Affected females are highly myopic but retain better visual function than affected males. The phenotypic data can be used to provide a mutation-specific visual prognosis, and may also help recognition of the genotype.