Cleft palate morphology, genetic etiology, and risk of mortality in infants with Robin sequence.

Robin sequence (RS) has many genetic and nongenetic causes, including isolated Robin sequence (iRS), Stickler syndrome (SS), and other syndromes (SyndRS). The purpose of this study was to determine if the presence and type of cleft palate varies between etiologic groups. A secondary endpoint was to determine the relationship of etiologic group, cleft type, and mortality. Retrospective chart review of patients with RS at two high-volume craniofacial centers. 295 patients with RS identified. CP was identified in 97% with iRS, 95% with SS, and 70% of those with SyndRS (p < .0001). U-shaped CP was seen in 86% of iRS, 82% with SS, but only 27% with SyndRS (p < .0001). At one institution, 12 children (6%) with RS died, all from the SyndRS group (p < .0001). All died due to medical comorbidities related to their syndrome. Only 25% of children who died had a U-shaped CP. The most common palatal morphology among those who died was an intact palate. U-shaped CP was most strongly associated with iRS and SS, and with a lower risk of mortality. RS with submucous CP, cleft lip and palate or intact palate was strongly suggestive of an underlying genetic syndrome and higher risk of mortality.

Long-term survival following diabetic vitrectomy.

AIM: To update long-term survival data on patients with proliferative diabetic retinopathy undergoing vitrectomy and to identify associated risk factors. METHODS: Retrospective clinical record review at a single New Zealand tertiary referral centre. A total of 182 eyes that underwent a vitrectomy for a diabetic vitreous haemorrhage and/or tractional retinal detachment between March 2000 and December 2010 were included. Kaplan-Meier survival curves and Cox-regression analyses were performed for survival rates and associated risk factors. RESULTS: The mean age of patients was 55 years (range 22 to 85) at time of surgery. The three-year survival rate following diabetic vitrectomy was 83.5%, and the five-year survival rate (N=154) was 70.1%. Increasing age, dialysis and high serum creatinine were associated with poorer survival on multivariate Cox regression analyses (hazard ratio of 1.035, 4.216 and 1.930 respectively with p-values of 0.018, <0.001 and 0.046). CONCLUSION: Survival rates after diabetic vitrectomy remain relatively poor but comparable to earlier New Zealand and international reports. However, there remain significant differences between ethnic groups within New Zealand that need to be addressed in addition to renal disease, which appears to be a major risk factor for poor survival. Overall, the contemporary survival outcomes observed in this study may influence decision making by patients and clinicians as well as encourage a review of current healthcare resource allocation in diabetes care.

Long-Term Changes in Intraocular Pressure after Vitrectomy for Rhegmatogenous Retinal Detachment, Epi-Retinal Membrane, or Macular Hole.

PURPOSE: To determine the long-term changes in the intraocular pressure (IOP) following vitrectomy for rhegmatogenous retinal detachment (RRD), epiretinal membrane (ERM), and macular hole (MH), and to investigate the relationship between the retinal disease and the incidence of late-onset IOP elevation. METHODS: This was a retrospective, observational, comparative study. We reviewed the medical records of 54 eyes of 54 RRD patients, 117 eyes of 117 ERM patients, and 75 eyes of 75 MH patients who underwent 20-, 23- or 25-gauge vitrectomy. The IOPs before surgery and 1, 3, 6, and 12 months following vitrectomy, and also at the final visit (average, 23.95 months) were evaluated. We defined a significant increase in the IOP as an increase of ≥4 mmHg from the preoperative IOP, and this increase was taken to be a 'death' event for the Kaplan-Meier survival analyses. RESULTS: The mean follow-up period was not significantly different among the groups. The mean IOP at 3 (P = 0.001) and 12 (P = 0.011) months following the vitrectomy and at the final visit (P = 0.002) were significantly higher than that before the vitrectomy in the RRD group. The mean IOP in the RRD group was significant higher than that in the ERM group at 1 (P = 0.005), 3 (P = 0.009), and 12 (P = 0.013) months following vitrectomy, and at the final visit (P = 0.032). Kaplan-Meier survival analyses showed that the RRD group had a significantly higher risk of an IOP increase following vitrectomy than the other groups (P<0.001 by log-rank test). Multivariate logistic regression analyses showed that a preoperative diagnosis of RRD was the only risk factor that was significantly associated with a postoperative IOP elevation after excluding eyes with a low preoperative IOP (odds ratio, 3.208; P = 0.003). CONCLUSIONS: A late-onset IOP elevation following vitrectomy was observed only in eyes that underwent RRD surgery. The elevation was probably caused by the specific characteristics and surgical procedures of RRD. Clinicians should pay more attention to the IOP elevation for long times after vitrectomy especially in eyes with RRD.

Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment.

BACKGROUND: Superselective delivery of chemotherapy through the ophthalmic artery, i.e. ophthalmic artery chemosurgery, has been used for the treatment of advanced intraocular retinoblastoma. Herein, we evaluate the efficacy of ophthalmic artery chemosurgery for retinoblastoma associated with >50% retinal detachment. PROCEDURE: Retrospective review of 37 eyes of 34 retinoblastoma patients who had extensive (>50%) bullous non-rhegmatogenous retinal detachments and received ophthalmic artery chemosurgery either as primary treatment or as "salvage" treatment after failed multi-cycle intravenous chemotherapy and/or external beam radiation (mean follow-up, 21 months). Data on patient and ocular survival, complications of ophthalmic artery chemosurgery treatments, time course of retinal reattachment, and serial electroretinograms (ERG) were collected. RESULTS: A total of 134 ophthalmic artery chemosurgery injections were performed. All children survive. Five eyes (5/37; 14%) were enucleated for progression of disease. The Kaplan-Meier enucleation-free survival rate at 2 years was 87.9% (95% confidence interval, 76.5-99.3%). The retina reattached in 28 eyes (28/37; 76%) and the 30-Hz flicker ERGs improved by >25 µV in seven eyes (7/37; 19%), remained stable (change < 25 µV) in 26 eyes (26/37; 70%), and decreased by >25 µV in four eyes (4/37; 11%). The Kaplan-Meier retinal reattachment rate was 58% after 3 months and three ophthalmic artery chemosurgery infusions (95% confidence interval, 41.9-74.1%). CONCLUSIONS: Ophthalmic artery chemosurgery is effective in preventing enucleation, promoting retinal reattachment and preserving or improving retinal function in the majority of eyes with advanced retinoblastoma and >50% retinal detachment that would otherwise be considered for enucleation.

Repair of retinal detachment caused by cytomegalovirus retinitis in patients with the acquired immunodeficiency syndrome.

Twenty-two eyes of 19 patients with the acquired immunodeficiency syndrome who had pars plana vitrectomy and silicone-oil injection after retinal detachment caused by cytomegalovirus retinitis were studied. All patients but one were monitored until time of death. The postoperative survival time and the factors that predicted anatomic success (retinal attachment) and functional success (visual acuity) were analyzed. No intraoperative complications were encountered. The mean survival time after surgery was four months. Of all of the preoperative and intraoperative factors studied, only the duration of cytomegalovirus retinitis was predictive of survival (P less than .03). The anatomic success rate was 89.5% (17 of 19 patients). None of the factors showed a trend or statistical significance in relation to anatomic success. Fifteen of 19 patients (79%) had lost at least two lines of Snellen visual acuity at time of death. Vision declined in a bimodal pattern (within the first postoperative month and after four months postoperatively). The optic nerve was pink and well perfused preoperatively in 16 of 19 patients (81.8%), but optic-nerve atrophy was observed postoperatively in 18 of 19 patients (95.5%). There was a trend for functional success to be influenced by increased intraocular pressure and optic-nerve atrophy, although our sample size was too small for statistical significance.