Ophthalmologic Findings in Children with Neurofibromatosis Type 1.

The purpose of this study was to evaluate the ophthalmologic findings in children with neurofibromatosis type 1 (NF1) and compare these findings in eyes with and without optic pathway gliomas (OPGs). We carried out a retrospective chart review of children with NF1. We recorded demographic characteristics, clinical manifestations of disease, and ophthalmologic findings including visual acuity, intraocular pressure, cup-to-disc ratio, visual field testing, and optical coherence tomography findings. Ophthalmologic findings were examined for the cohort for initial and final appointments. These findings were also compared between eyes with and without OPGs. The study included 119 participants with 238 total eyes. The most common clinical manifestations of NF1 in this cohort were café au lait macules (98%), axillary or inguinal freckling (91%), Lisch nodules (66%), and cutaneous neurofibromas (57%). Thirty-seven participants had imaging that allowed evaluation for choroidal abnormalities, and 28 (76%) had choroidal lesions. Twenty-seven participants (23%) had OPGs, and 44 eyes were affected. On initial assessment, eyes with OPGs had worse visual acuity. On final examination, eyes with OPGs were more likely to have a worse visual acuity and a thinner generalised retinal nerve fibre layer (RNFL) thickness, inferior RNFL thickness, and temporal RNFL thickness. This study provides longitudinal follow-up of children affected by NF1 with and without OPGs. Eyes with OPGs were found to be associated with worse visual acuity and thinner RNFLs overall on final testing.

Conversion to Glaucoma in Pediatric Glaucoma Suspects.

PRCIS: In this retrospective review of pediatric glaucoma suspects, 11.5% of eyes progressed to glaucoma over an average of 6.5 years; eyes with ocular hypertension had an 18-fold increased risk of progression compared with eyes with suspicious disc appearance. PURPOSE: The purpose of this study was to describe the rate of progression to glaucoma of a large cohort of pediatric glaucoma suspects at a quaternary academic center. DESIGN: Retrospective case series. PARTICIPANTS: One thousand three hundred seventy-five eyes (824 individuals) followed as pediatric glaucoma suspects at the Wilmer Eye Institute between 2005 and 2016. METHODS: Retrospective study of pediatric patients monitored as glaucoma suspects at the Wilmer Eye Institute between 2005 and 2016. MAIN OUTCOME MEASURES: Progression to glaucoma, defined according to Childhood Glaucoma Research Network criteria or by surgical intervention; initiation of intraocular pressure-lowering therapy. RESULTS: One hundred fifty-eight (11.5%) eyes from 109 unique patients met the criteria for conversion to glaucoma during follow-up; rates of conversion ranged between 34.1% for eyes monitored for ocular hypertension, 16.2% for eyes with prior lensectomy, 12.1% for eyes monitored for other ocular risk factors, 2.4% for eyes with suspicious disc appearance, and 0.4% for eyes monitored for systemic risk factors. The first criterion met for conversion to glaucoma was ocular hypertension in 149 eyes (94.3%) and enlarged cup-to-disc ratio (CDR) in 9 eyes (5.7%); the most common second criterion met was the enlargement of CDR since initial presentation (45 eyes, 28.5%), surgical intervention (33 eyes, 20.9%), visual field changes (21 eyes, 13.3%), and asymmetric CDR change compared with fellow eye (20 eyes, 12.7%). The Kaplan-Meier survival curves across the different indications for being monitored as a glaucoma suspect significantly differed ( P <0.0001). Eyes being monitored for ocular hypertension had an 18-fold increased risk of conversion to glaucoma than those followed for suspicious disc appearance [hazard ratio (HR) 18.33, 95% CI, 10.05-33.41). Eyes monitored for prior lensectomy and for other ocular risk factors had a 6-fold and 5-fold increased risk of conversion to glaucoma than those followed for suspicious disc appearance, respectively (HR: 6.20, 95% CI, 3.66-10.51; HR: 5.43, 95% CI, 3.00-9.84). Eyes followed for ocular hypertension were nearly 4 times more likely to convert to glaucoma than those followed for prior lensectomy (HR: 3.72, 95% CI, 2.28-6.07). CONCLUSIONS: Eyes being followed as pediatric glaucoma suspects for ocular hypertension had higher rates of progression to glaucoma than eyes being monitored for prior lensectomy, other ocular risk factors, suspicious disc appearance, or systemic risk factors.

Unplanned Return to the Operating Room After Tube Shunt Surgery.

PURPOSE: The purpose of this study was to determine the rate of unplanned returns to the operating room (OR) within 180 days and at any time postoperatively after valved and non-valved tube shunt surgery. DESIGN: Retrospective case-control study. METHODS: A review of 357 eyes that underwent tube shunt surgery (151 valved, 206 non-valved) was conducted at an academic glaucoma service between January 2014 and December 2016. A control eye was time matched for each eye that underwent reoperation. RESULTS: The reoperation rate within 180 days was 16 of 151 (10.6%) for valved and 25 of 206 (12.1%) for non-valved tube shunts and at any time postoperatively was 31 of 151 (20.5%) for valved, and 47 of 206 (22.8%) for non-valved tube shunts. Mean postoperative follow-up was 2.8 ± 1.1 years. The most common reoperations within 180 days and at any time postoperatively after valved tube shunt surgery were tube revisions (43.8% within 180 days, 38.7% any time) and external cyclophotocoagulation (CPC) (31.3% within 180 days, 38.7% anytime). The most common reoperations within 180 days after non-valved tube shunt surgery were tube revisions (32.0%), external CPC (12.0%), and vitrectomy with anterior chamber washout (12.0%) and at any time postoperatively were tube revision (34.0%), external CPC (31.9%), and tube explant (12.8%). At last follow-up, eyes that returned to the OR and controls were similar in terms of mean intraocular pressure (IOP), proportion of eyes meeting target IOP, and change in visual acuity. CONCLUSIONS: More than 20% of eyes undergoing tube shunt surgery returned to the OR at any time postoperatively with a mean follow-up of nearly 3 years, with more than 10% of eyes undergoing reoperation within the first 180 days. Rates of reoperation were similar between valved and non-valved tube shunts.

Unplanned Return to the Operating Room After Trabeculectomy.

PURPOSE: To determine the unplanned return to operating room (OR) rate within 180 days and at any time postoperatively after trabeculectomy performed or supervised by an attending surgeon and to identify associated factors. DESIGN: Retrospective case-control study. METHODS: Review of 881 eyes that underwent trabeculectomy at an academic glaucoma service between January 2014 and December 2016. Inclusion criteria included adult patients with postoperative follow-up >180 days and no other glaucoma-related surgery within the prior year. For each eye that underwent reoperation, a control was time-matched within 1 month. RESULTS: The reoperation rate within 180 days was 9.5% (84/881) and at any time postoperatively was 23.3% (205/881). When intraoperative bleb needling cases were excluded, the reoperation rate was 6.5% (57/881) within 180 days and 13.6% (120/881) at any time postoperatively. Mean postoperative follow-up was 2.9 ± 1.1 years. The most common reoperations within 180 days were bleb revision (32.1%) and intraoperative bleb needling (28.6%) and at any time postoperatively were bleb needling (36.1%), bleb revision (23.9%), and tube shunt implant (13.2%). At last follow-up, eyes that returned to OR and control eyes were similar in terms of mean intraocular pressure (IOP), the proportion of eyes meeting target IOP, and change in visual acuity since the original trabeculectomy. CONCLUSIONS: Nearly 10% of eyes returned to OR within 180 days and over 20% of eyes required reoperation at any time postoperatively with a mean follow-up of nearly 3 years. Additional studies should evaluate unplanned return to OR as a measure of surgical quality within ophthalmology.

Long-term Functional Outcomes of Trabeculectomy Revision Surgery.

PURPOSE: To describe the long-term results of revision surgery for complications from trabeculectomy in a case series from an academic glaucoma service. DESIGN: Retrospective case series. PARTICIPANTS: A total of 310 eyes (310 individuals) who underwent revision of trabeculectomy at the Wilmer Eye Institute between 2007-2015. METHODS: Retrospective study of patients who underwent revision of trabeculectomy at the Wilmer Eye Institute between 2007-2015. MAIN OUTCOME MEASURES: Success of revision, defined as maintenance of long-term intraocular pressure (IOP) control at or below target, no further glaucoma-related reoperations, no major complications during revision surgery, and no dysfunctional hypotony; need for additional surgery after revision; change in visual acuity (VA) between original trabeculectomy; revision surgery; and last follow-up visit. RESULTS: The mean time between original surgery and revision was 3.1±4.0 years, and the mean follow-up from revision to last examination was 4.4±2.9 years. The overall successes at 1, 2, 5, and 8 years after revision were 76%, 68%, 57%, and 50%, respectively (Kaplan-Meier). The success was similar among revision indications (P = 0.43). The 5-year Kaplan-Meier survival was 60% for eyes undergoing revision for hypotony, 57% for bleb leak, 63% for dysesthesia, and 44% for uncontrolled IOP. Revisions in which the original surgery was performed by surgeons other than present Wilmer faculty were 5 times more likely to fail than cases in which the original surgery was performed by the Wilmer surgeon performing the most trabeculectomy and revision surgeries (P = 0.04). Further surgery after revision was required in 30% of cases during follow-up. Approximately half were additional IOP-lowering procedures, and half were further revisions. Duration of symptoms before revision was not consistently associated with the loss of VA between original surgery and last follow-up. Eyes that lost the most VA before revision surgery for hypotony tended to be the same eyes that regained the most vision after revision; eyes that underwent revision for hypotony with shorter duration of symptoms showed a greater improvement of VA between revision surgery and last follow-up (P = 0.01). CONCLUSIONS: Revision of trabeculectomy provided successful resolution of complications associated with trabeculectomy surgery in many cases.

Long-Term Functional Outcomes of Glaucoma Tube Shunt Revision Surgery.

PURPOSE: To characterize long-term outcomes of tube shunt revision surgeries and identify factors associated with their failure. DESIGN: Retrospective chart review. PARTICIPANTS: One eye from each of 179 patients who underwent tube shunt revision surgery at the Wilmer Eye Institute between 2004 and 2015 with a minimum follow-up of 6 weeks. METHODS: Eligible eyes were identified from billing records and data related to their care were extracted from electronic medical records. Eyes were analyzed in aggregate and by indication for revision, including hypotony, high intraocular pressure (IOP), tube reposition, and tube exposure. MAIN OUTCOME MEASURES: Surgical failure, defined as a need for further tube shunt revision or other glaucoma surgery, unsatisfactory IOP at last follow-up, or both. Secondary outcomes included postoperative infection and functional visual impairment. RESULTS: With a median follow-up of 4.2 years, 126 failures occurred among 179 eyes. By Kaplan-Meier analysis, the cumulative rates of surgical failure at 1, 2, and 5 years after revision were 49%, 59%, and 74%, respectively. Most revision failures (105/126) were the result of the need for additional surgery, whereas 11 eyes showed IOP above target levels and 10 eyes showed dysfunctionally low IOP at the last follow-up. Factors associated with failure in a stepwise regression model were revision for hypotony (hazard ratio [HR], 6.79; P = 0.002), different surgeons performing the original and revision surgeries (HR, 2.80; P = 0.002), longer duration of symptoms before revision (P = 0.01), revision of a right eye (HR, 1.92; P = 0.03), and presumed preoperative infection (HR, 2.47; P = 0.04). In univariate analysis, success varied significantly by prior surgeon (P = 0.01), but not by revision surgeon. There was a 16% cumulative incidence of postoperative infection, with the highest risk in those with presumed preoperative infections (P = 0.01) and in persons of non-African, non-European derivation (P = 0.03). CONCLUSIONS: The estimated rate of failure of tube shunt revision is 75% by 5 years, most often because of a need for further surgery. The major potentially modifiable feature associated with success is tube shunt revision being performed by the original surgeon.