Do infants with isolated congenital sixth nerve palsy require comprehensive work-up? A retrospective cohort and review of the literature.

PURPOSE: The purpose of this study is to describe a case series of infants with isolated congenital sixth nerve palsy (ICSNP) and suggest a management algorithm based on our experience and a review of the literature. METHODS: A retrospective cohort design was used. The clinical database of a single tertiary medical center was reviewed to identify all patients diagnosed with ICSNP from January 2020 to November 2022. Data were collected as follows: demographic parameters, age at initial presentation, presenting symptoms and signs, findings on ophthalmic and neurologic examinations, findings on follow-up, and outcome. RESULTS: Six patients were included. All were born at term. The average gestational weight was 3675.7 ± 262.7 g. Three mothers had gestational diabetes. Five deliveries necessitated labor induction either by oxytocin (n = 4) or by membrane stripping followed by oxytocin (n = 1). One had also gone a forceps assisted delivery. Symptoms were noticed in all newborns by their parents within the first week of life. Ophthalmological and neurological examinations were otherwise unremarkable apart of one patient with a head turn to the side of the involved eye. Four patients underwent brain imaging that were unremarkable. All abduction deficits resolved by 1 to 3 months of age. Follow up examinations were unremarkable (mean follow up 14.3 ± 5.0 months, range 4-23). CONCLUSIONS: This case series, together with previous reports, support ICSNP's benign nature. We suggest an initial basic work-up that solely includes ophthalmological and neurological examinations which will be elaborated in case of any additional pathologic findings or if ICSNP does not fully resolve by 3 months.

Long-term retinal vasculature abnormalities following intravitreal bevacizumab for retinopathy of prematurity.

PURPOSE: To report long-term fluorescein angiography (FA) findings in consecutive patients with type 1 retinopathy of prematurity (ROP) treated with intravitreal bevacizumab (IVB), whose ROP seemed to have resolved clinically. METHODS: Data were retrospectively collected for all patients with IVB-treated type 1 ROP who underwent an exam under anesthesia (EUA) and FA at 60 weeks post-gestational age (PGA) or older at a tertiary medical center between 2011 and 2020. FA results were reviewed for pathological vascular findings. RESULTS: Twenty-nine eyes of 16 patients were included. Mean gestational age and birth weight were 25.3 ± 1.5 weeks and 762.2 ± 189.8 g, respectively. The mean age at the time of EUA and FA was 23.4 ± 15.8 months. All eyes had a peripheral avascular zone and irregular peripheral branching. Vascular loops were seen in 27 eyes (93.1%) and vascular bulbs and anastomoses in 16 eyes each (55.2%). Additional abnormal findings included leakage (10 eyes, 34.5%), vessels crossing the fovea (5 eyes, 17.2%), tortuous arteries and veins (9 eyes, 31%, and 5 eyes, 17.2%, respectively), and neovascularization (2 eyes, 6.9%). When comparing patients who were less than or greater than 70 weeks PGA at follow-up, FA findings in the group with shorter follow-up were significant for more anastomoses and vascular bulbs (p = 0.002 and p = 0.024, respectively) and trended towards more leakage (45.5% vs. 27.8%, p = 0.331). CONCLUSION: The vast majority of IVB-treated type 1 ROP eyes suffered from vascular pathologies long after treatment. There may be long-term progression in the vascularization process of the retina in some cases.

Histological and molecular characterization of glaucoma model induced by one or two injections of microbeads to the anterior chamber of mice.

PURPOSE: To characterize glaucoma-induced damage following injections of plastic microbeads into the anterior chamber of mice. METHODS: Mice were divided into three groups: a single plastic microbeads injection (n = 21); two consecutive plastic microbead injections to the right eye at 1-week intervals, 4 of which with two consecutive saline injections in the left eye (n = 15); and an additional control group of two consecutive saline injections at 1-week intervals (n = 6). Intraocular pressure (IOP) was measured weekly. Retinal thickness, ganglion cells (RGCs) and axonal loss, inflammatory and gliosis reactions were measured at week four. Molecular analysis using qRT-PCR in the microbeads injection groups focused on expression levels of inflammation and glaucoma-related genes. RESULTS: Mean IOP following single injection at 4 weeks was significantly elevated compared to baseline in injected eyes (14.5 ± 3.3 mmHg vs. 11.1 ± 2.5 mmHg, respectively, p = 0.003) and not in fellow eyes (13.2 ± 2.9 mmHg vs. 12.2 ± 2.9, respectively, NS). Six (35.3%) bead-injected eyes had IOP ≥ 17 mmHg compared with 2 (11.8%) saline-injected control eyes. Retinal thickness in injected and fellow eyes was 193.7 ± 15.5 µm and 223.9 ± 15.5 µm, respectively (p = 0.03). RGC loss in injected and fellow eyes was 16.0 ± 0.5 and 17.6 ± 0.7 cells per 200 µm, respectively (p = 0.005). Retinal gliosis, axonal loss and inflammatory cell infiltration to the bead-injected eyes were noted. Molecular analysis following double injection showed STAT3 expression decreased in the glaucoma-induced optic nerves (0.69 ± 0.3 vs. 1.16 ± 0.3, p = 0.04), but increased in the glaucoma-induced retinae (p = 0.05) versus saline; retinal IL-1ß decreased significantly (0.04 ± 0.04 vs. 0.36 ± 0.2, p = 0.02). TNF-α, NFkB and SOD-1 expression did not change. CONCLUSION: One/two injections of microbeads elevated IOP, with measurable neuronal damage. An inflammatory response was detected in the injured retina and optic nerve. The therapeutic significance of these findings should be explored.

Diagnosis of Pseudoexfoliation Syndrome in Pseudophakic Patients.

OBJECTIVE: The aim of this study was to evaluate the sensitivity and specificity of pseudoexfoliation syndrome diagnosis in pseudophakic patients and potential means of improving it. METHODS: This prospective, nonrandomized study comprised 41 consecutive patients (41 eyes) scheduled for cataract surgery at a tertiary medical center during 2016 and 2017. Preoperatively, all patients underwent a detailed slit-lamp examination, including gonioscopic assessment of the iridocorneal angle. The examination was performed by a glaucoma specialist who completed an assessment form documenting the presence/absence of clinical signs of pseudoexfoliation syndrome. It was repeated 1-2 weeks postoperatively by a second, masked, glaucoma specialist. RESULTS: Sixteen patients (39.0%) were diagnosed with pseudoex-foliation syndrome preoperatively. The diagnosis was confirmed postoperatively in 11/16 patients (68.8% sensitivity) and in an additional patient not diagnosed preoperatively (96% specificity). The ability to diagnose pseudoexfoliation syndrome postoperatively was significantly worse than preoperatively (Z = 12.161, p < 0.0001). Pupillary border deposits (75% of cases) and the Sampaolesi line (83.3%) were the cornerstones of the postoperative diagnosis; anterior capsular deposits were evident in only 41.6% of cases diagnosed postoperatively (31.3% of the originally diagnosed cases). CONCLUSIONS: Underdiagnosis of pseudoexfoliation syndrome is common in pseudophakic patients and may have significant implications for future management. Careful attention to pupillary border anatomy and meticulous gonioscopic assessment of the iridocorneal angle are essential for accurate diagnosis. Preoperative documentation of pseudoexfoliation syndrome could help prevent this diagnostic pitfall.

Prevention of Exposure Keratopathy in Critically Ill Patients: A Single-Center, Randomized, Pilot Trial Comparing Ocular Lubrication With Bandage Contact Lenses and Punctal Plugs.

OBJECTIVES: To compare the effectiveness of bandage contact lenses and punctal plugs with ocular lubricants in preventing corneal damage in mechanically ventilated and sedated critically ill patients. DESIGN: Single-center, prospective, randomized, pilot study. SETTING: Sixteen-bed, general ICU at a tertiary academic medical center. PATIENTS: Adults admitted to the ICU and anticipated to require mechanical ventilation and continuous sedation for greater than or equal to 4 days. INTERVENTIONS: Patients were randomized to receive eye care with ocular lubricants (n = 38), bandage contact lenses (n = 33), or punctal plugs (n = 33). The bandage contact lenses were changed every 4 days, whereas the punctal plugs remained in situ for the entire study. MEASUREMENTS AND MAIN RESULTS: The primary endpoint was the presence or absence of corneal damage as assessed by the grade of keratopathy. Patients were examined by an ophthalmologist blinded to the study group every 4 days and at the time of withdrawal from the study, due to cessation of sedation, discharge from the ICU, or death. The mean duration of the study was 8.6 ± 6.2 days. The grade of keratopathy in the ocular lubricant group increased significantly in both eyes (p = 0.01 for both eyes) while no worsening was noted in either the lens or punctal plugs groups. In a post hoc analysis of patients with an initially abnormal ophthalmic examination, significant healing of keratopathy was noted in the lens group (p = 0.02 and 0.018 for left and right eyes, respectively) and in the right eye of the plugs group (p = 0.005); no improvement was noted in the ocular lubricant group. CONCLUSIONS: Compared with ocular lubrication, bandage contact lenses and punctal plugs were more effective in limiting keratopathy, and their use, particularly of bandage contact lenses, was associated with significant healing of existing lesions.

Bilateral nonarteritic ischemic optic neuropathy and retinal ischemia in a pediatric dialysis patient.

A nearly 3-year-old boy on nightly dialysis presented emergently with sudden loss of vision. On examination, his visual acuity was light perception in the right eye and no light perception in the left eye. There was bilateral optic disk edema, diffuse pallor of posterior poles, and a cherry red spot in the left fundus. The patient was subsequently found to be hemodynamically unstable and admitted to the pediatric intensive care unit with presumed septic shock. Optical coherence tomography revealed paracentral acute middle maculopathy lesions in the right eye and diffusely thick retina in the left eye. Magnetic resonance imaging and magnetic resonance angiography of the brain and vessels did not reveal any acute findings. The patient's presentation was most consistent with bilateral nonarteritic ischemic optic neuropathy and unilateral central retinal artery occlusion. On repeat evaluation 9 months later, vision was largely unchanged.

Long-Term outcomes of pediatric dacryocystorhinostomy in a single medical center- a retrospective study.

PURPOSE: To examine the long-term success rate of pediatric endoscopic DCR surgery via telephone questionnaires, as determined by patients and their parents. METHODS: This is a retrospective cohort study of all patients who underwent DCR surgery at the Schneider Children's Medical Center of Israel between 2010 and 2020. We performed long-term follow-ups to assess the quality of life, surgical complications, and satisfaction with surgical outcomes. RESULTS: Our study includes seventy-nine patients with a total of 108 eyes. The mean age at the time of DCR was 7.05 years (Std = 4, min = 0.3, max = 17.7) Mean follow-up time was 5.7 years (Std =2.5, min = 1.4, max = 11.1). Tubes were inserted for a mean of 129 days (Std = 101). Fifty-seven patients (72%) declared they had no complications after surgery, three patients (4%) reported pain after surgery, and 14 patients (17.7%) reported tube extrusion, which occurred 7-21 days after surgery. Forty-four patients (56%) reported no recurrence of symptoms, 29 (37%) complained of mild epiphora, and 18 (23%) reported some ocular discharge. Sixty-eight patients (86%) stated that they did not undergo additional surgery, while the remaining 11 (14%) reported undergoing a revision operation for symptom control. Satisfaction rate (1-7) mean score reported was 6.15 (Std = 1.6). Sixty-two (78%) reported improved quality of life, while 17 (22%) reported no improvement. Our questionnaire results have been compared with the TEARS scores with similar findings. CONCLUSION: Regardless of its etiology, endoscopic DCR surgery in the pediatric population is safe and efficient, with a high long-term patient satisfaction rate, as reported via a telephone questionnaire.

Incidental Unilateral Macular Lesions in Children.

BACKGROUND: This study aims to examine the rare entity of unilateral macular lesions in the pediatric population and describe the distinct diagnoses and characterizations related to these findings. METHODS: A retrospective cohort design. The database of the ophthalmology clinic in a tertiary medical center was reviewed to identify all children with incidental unilateral macular findings, examined during 2016 through 2021. RESULTS: Twenty children were included. Mean age was 7.8 ± 3.4 years, 50% were girls. The most common macular lesion was torpedo maculopathy (50%), followed by pigmentary changes (25%), discoid maculopathy (15%), macular scar and combined hamartoma of the retina and retinal pigment epithelium (RPE) (5% each). None of the lesions changed after a mean follow-up duration of 2.3 ± 1.5 years. Visual acuity in the involved eye was equal to that in the contralateral eye in 90% of patients and did not change from initial to final visit. CONCLUSION: Incidental unilateral macular lesions in the pediatric population are usually benign, stable, and do not affect vision. Long-term follow-up is advised, as vision-threatening alterations may appear. [Ophthalmic Surg Lasers Imaging Retina 2023;54:346-352.].

Long-Term Evaluation of Pseudoexfoliation Syndrome Post-Cataract Extraction.

The study aimed to examine the effect of cataract extraction on ophthalmologists' ability to detect pseudoexfoliation (PXF) syndrome. A total of 31 patients admitted for elective cataract surgery were enrolled in this prospective comparative study. Prior to surgery, patients underwent slit-lamp examination and gonioscopy conducted by experienced glaucoma specialists. Subsequently, patients were re-examined by a different glaucoma specialist and comprehensive ophthalmologists. Pre-operatively, 12 patients were diagnosed with PXF on the basis of a Sampaolesi line (100%), anterior capsular deposits (83%), and pupillary ruff deposits (50%). The remaining 19 patients acted as controls. All patients were re-examined 10-46 months post-operatively. Of the 12 patients with PXF, 10 (83%) were correctly diagnosed post-operatively by glaucoma specialists and 8 (66%) by comprehensive ophthalmologists. There was no statistically significant difference in PXF diagnosis. However, detection of anterior capsular deposits (p = 0.02), Sampaolesi lines (p = 0.04), and pupillary ruff deposits (p = 0.01) were significantly lower post-operatively. Diagnosis of PXF is challenging in pseudophakic patients as the anterior capsule is removed during cataract extraction. Therefore, PXF diagnosis in pseudophakic patients relies mainly on the presence of deposits at other anatomical sites, and careful attention to these signs is required. Glaucoma specialists may be more likely than comprehensive ophthalmologists to detect PXF in pseudophakic patients.

Azithromycin and Sildenafil May Have Protective Effects on Retinal Ganglion Cells via Different Pathways: Study in a Rodent Microbead Model.

Decreased blood flow to the optic nerve (ON) and neuroinflammation are suggested to play an important role in the pathophysiology of glaucoma. This study investigated the potential neuroprotective effect of azithromycin, an anti-inflammatory macrolide, and sildenafil, a selective phosphodiesterase-5 inhibitor, on retinal ganglion cell survival in a glaucoma model, which was induced by microbead injection into the right anterior chamber of 50 wild-type (WT) and 30 transgenic toll-like receptor 4 knockout (TLR4KO) mice. Treatment groups included intraperitoneal azithromycin 0.1 mL (1 mg/0.1 mL), intravitreal sildenafil 3 µL, or intraperitoneal sildenafil 0.1 mL (0.24 µg/3 µL). Left eyes served as controls. Microbead injection increased intraocular pressure (IOP), which peaked on day 7 in all groups and on day 14 in azithromycin-treated mice. Furthermore, the retinas and ON of microbead-injected eyes showed a trend of increased expression of inflammatory- and apoptosis-related genes, mainly in WT and to a lesser extent in TLR4KO mice. Azithromycin reduced the BAX/BCL2 ratio, TGFß, and TNFα levels in the ON and CD45 expression in WT retina. Sildenafil activated TNFα-mediated pathways. Both azithromycin and sildenafil exerted a neuroprotective effect in WT and TLR4KO mice with microbead-induced glaucoma, albeit via different pathways, without affecting IOP. The relatively low apoptotic effect observed in microbead-injected TLR4KO mice suggests a role of inflammation in glaucomatous damage.

Outcomes of Combined Scleral Buckling Plus Pneumatic Retinopexy Vs. Scleral Buckling for Primary Rhegmatogenous Retinal Detachment.

PURPOSE: To evaluate the outcomes and complications of scleral buckle surgery alone or combined with pneumatic retinopexy (pneumatic buckle) for the treatment of primary rhegmatogenous retinal detachment. DESIGN: Retrospective chart review. PARTICIPANTS: Two hundred thirteen patients with rhegmatogenous retinal detachment of whom 101 underwent primary scleral buckle surgery at Rabin Medical Center in 2005-2015 (SB group) and 112 underwent pneumatic buckle surgery at Royal Alexandra Hospital in 2013-2015 (PB group). METHODS: All patients were followed for ≥12 months. Data on clinical and surgical parameters, outcome, and complications were collected from the medical files. MAIN OUTCOME MEASURES: Best corrected visual acuity and anatomical outcomes. RESULTS: At 12 months, average best corrected visual acuity was 0.3 logMar in the SB group and 0.42 logMar in the PB group (P < 0.05). Rates of anatomical reattachment were high and similar in the two groups (99% and 97%, respectively, P = 0.623). The SB group had a higher percentage of patients requiring additional laser applications (21% vs. 7%; P < 0.01) and buckle readjustment surgery (6% vs. 0; P = 0.01), and the PB group had a higher percentage of patients who required postoperative pars plana vitrectomy (30% vs. 17%; P = 0.03). CONCLUSION: Scleral buckle surgery alone is efficient for the treatment of rhegmatogenous retinal detachment. Its combination with pneumatic retinopexy usually has no significant added value in terms of anatomical reattachment rate. Outcomes of Pneumatic buckling vs Scleral Buckling for RRD.

Functional and Structural Findings in Infants Treated for Retinopathy of Prematurity Using Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: To assess the foveal microvascular structure of children with retinopathy of prematurity (ROP) treated with diode laser photocoagulation using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: OCTA was performed at a tertiary medical center in 17 children (27 eyes) aged 4 to 16 years with a history of diode laser photocoagulation treated ROP. OCTA parameters were compared with those of 12 healthy age-matched controls (23 eyes) attending the orthoptics clinic and correlated with clinical factors. RESULTS: Compared with controls, the ROP group had a smaller foveal avascular zone area (P < .001), lower deep vascular plexus density (P < .001), lower flow density (P = .025), and greater central macular thickness (P < .001). High intraventricular hemorrhage grade correlated with smaller foveal avascular zone area (P = .008) and greater inner macular thickness (P = .047). There was no impact of gestational age, birth weight, or refractive status. CONCLUSION: OCTA can identify significant quantifiable long-term macular microvascular and structural changes in this patient population. [Ophthalmic Surg Lasers Imaging Retina. 2022;53(4):194-201.].

A unique case of benign intermittent upbeat nystagmus triggered by light stimulation.

A 3-month-old girl with no past medical history presented with new-onset intermittent upbeat nystagmus. Episodes were triggered by movement into the supine position as well as by bright light flashes. They lasted a few seconds and were not associated with any distress. Neurological examination and work-up, including magnetic resonance imaging of the brain and abdominal ultrasonography, were normal. The eye movements completely resolved after 3 months.

Effect of Penetration Angle and Velocity During Intravitreal Injection on Pain.

Purpose: To evaluate the effect of velocity and angle of the intravitreal injection of anti-vascular endothelial growth factors on pain sensation.Methods: Patients were randomly assigned to one of four injection methods: straight and fast, straight and slow, tunneled and fast, and tunneled and slow. Later, they graded their pain sensation on a Visual Analog Scale (range 0-10).Results: The cohort included 180 patients. Mean pain score was 2.81 ± 2.34. There was no statistically significant difference in mean pain score among the four groups (p = .858); between the slow-injection (straight and tunneled) and fast-injection groups (p = .514); and between the straight-injection (fast and slow) and tunneled-injection groups (p = .992), nor other background variables.Conclusion: Velocity and angle of intravitreal injections are unrelated to the pain sensation. Therefore, the method may be left to the clinician's discretion. This implies that the sensation is mostly subjective.

Orbital Lymphatic-Venous Malformation Accompanied by an Intraocular Vascular Malformation: A Rare Case Study.

Lymphatic-venous malformations (LVMs) are development defects that result in abnormal connections between the lymphatic and venous systems. The authors describe a 7-weeks-old female infant who presented with a right orbital LVM extending to the ipsilateral cheek and subconjunctiva of the right eye, intracranial developmental venous anomalies in the right cerebellum, and a significant right eye intraocular retinal vascular malformation. Since orbital LVM is usually diagnosed in infancy or childhood, pediatric ophthalmologists should actively look for intraocular vascular malformations as such findings can poorly affect a patient's vision.

A Single Low-Dose of Methylphenidate Improves Abnormal Visual Field Testing.

PURPOSE: To evaluate the effect of methylphenidate on visual field testing in healthy adults with abnormal visual field results. METHODS: This prospective, randomized, controlled interventional clinical trial comprised all patients who had abnormal visual field test results and normal eye examination and ophthalmic history. Eligible patients were randomly assigned to either the study group or the control group. All patients repeated their visual field testing. Study group patients received a single dose of 10 mg methylphenidate prior to that. The main outcome measures were the percent difference in mean deviation and pattern standard deviation between the second and first visual fields. RESULTS: The methylphenidate group had greater improvement in all parameters. Mean deviation improved by median 68% (IQR 19%-78%) in the methylphenidate group vs. 27% [-5% to 55%] in the controls. However, this was not statistically significant (p = .83). Pattern standard deviation improved by median 49% (22%-59%) vs. 7% [-9% to 45%], respectively (p = .012). The visual fields were also reviewed by 3 masked experienced ophthalmologists. They indicated that the second visual field improved in 76.2% of the methylphenidate group vs. 48.5% of the controls (p = .04). A normal repeat visual field occurred in 57.7% vs. 21.2%, respectively. A subgroup analysis of patients with prior experience in visual field testing yielded an even more striking improvement in the methylphenidate group vs. controls. CONCLUSIONS: A single low dose of methylphenidate can improve visual field testing in subjects without ocular pathology, and even more in those with prior experience in perimetry.

Effect of different lens status on intraocular pressure elevation in patients treated with anti-vascular endothelial growth factor injections.

AIM: To assess the effect of lens status on sustained intraocular pressure (IOP) elevation in patients treated intravitreally with anti-vascular endothelial growth factor (VEGF) agents. METHODS: Data were retrospectively collected for all patients treated with intravitreal injections of anti-VEGF medication at a tertiary medical center in July 2015. Findings were analyzed by lens status during 6 months' follow-up. The main outcome measure was a sustained increase in IOP (≥21 mm Hg or change of ≥6 mm Hg from baseline on ≥2 consecutive visits, or addition of a new IOP-lowering medication during follow-up). RESULTS: A total of 119 eyes of 100 patients met the study criteria: 40 phakic, 40 pseudophakic, and 39 pseudophakic after Nd:YAG capsulotomy. The rate of sustained IOP elevation was significantly higher in the post-capsulotomy group (23.1%) than in the phakic/pseudophakic groups (8.1%; P=0.032), with no statistically significant differences among the 3 groups in mean number of injections, either total (P=0.82) or by type of anti-VEGF mediation (bevacizumab: P=0.19; ranibizumab: P=0.13), or mean follow-up time (P=0.70). CONCLUSION: Nd:YAG capsulotomy appears to be a risk factor for sustained IOP elevation in patients receiving intravitreal anti-VEGF injections. This finding has important implications given the growing use of anti-VEGF treatment and the irreversible effects of elevated IOP.

Ahmed Valve Tube Extension in Pediatric Glaucoma.

PRéCIS:: Ahmed valve tube extender showed good long-term outcomes for retracted tubes in pediatric glaucoma. The procedure is safe with limited complications. PURPOSE: To analyze the long-term outcomes of Ahmed valve tube extension in pediatric glaucoma patients. PATIENTS AND METHODS: This study analyzed the records of all children who underwent surgery involving extension of a pediatric Ahmed valve tube, treated at a tertiary medical center between 2007 and 2018. Surgical success was defined as intraocular pressure between 6 and 22 mm Hg and reduced by at least 20% from its preoperative value, with or without intraocular pressure lowering medications, without additional surgical procedures and without vision loss. RESULTS: Fourteen eyes of 11 children were treated with the Ahmed tube extender during the study period. Mean age of all children was 5.7±4.7 years (range, 3 mo to 16 y). The most common type of glaucoma was congenital glaucoma (n=6, 42.9%). The mean age at the time of initial valve implant was 2.39±3.89 years and the mean duration from initial insertion to the extension was 3.39±2.95 years. The mean follow-up was 72.8±43.3 months (range, 12 to 140 mo), with 85.7% followed for >2 years. The mean survival was 34.1±37.2 months after the extension. Three surgeries (21.4%) were primary failures, 8 patients (57.1%) failed during follow-up (mean, 40.6±44.4 mo after the extension; range, 1 to 125 mo), whereas 3 patients (21.4%) maintained the successful outcome until their last follow-up visit (mean, 37.7±24.1 mo; range, 12 to 70 mo). CONCLUSIONS: The Ahmed valve extender can often save a retracted or obstructed tube. The procedure has limited complications and long-term efficacy in one third of the patients.

Secondary intraocular lens implantation using the flanged intrascleral fixation technique in pediatric aphakia: case series and review of literature.

PURPOSE: To present a pediatric case series in which the flanged intrascleral intraocular lens (IOL) fixation technique (Yamane technique) was used to correct aphakia. METHODS: The surgical database of a single tertiary children's hospital was reviewed to identify all patients who underwent secondary IOL implantation by a single surgeon from May 2018 to January 2020. The medical records and operative reports of all patients operated on using the Yamane technique were reviewed retrospectively. Intra- and postoperative complications and pre- and postoperative vision and refraction were documented to assess outcomes. RESULTS: A total of 12 eyes of 10 consecutive patients were included. Mean age at surgery was 10 ± 6 years. Indications for scleral fixation were ectopia lentis (secondary to Marfan syndrome [n = 3] or idiopathic [n = 1]), lens subluxation with intermittent pupillary block secondary to Weill- Marchesani syndrome (n = 2), early childhood lensectomy with insufficient residual capsular support (n = 5), and traumatic aphakia after an open globe (n = 1). Mean follow-up was 8 ± 5 months. No major intraoperative complications occurred. Postoperatively 1 patient required IOL repositioning 1 week after surgery. The location of one haptic was noted to be intrascleral but superficial in another patient, who did not require surgical intervention. The mean postoperative astigmatism was 2 ± 2 D. Good visual results were achieved in all eyes. CONCLUSIONS: With slight modifications, the Yamane technique can be adapted to the pediatric eye. This technique has significant surgical and anatomic advantages and provides stable IOL fixation. Visual outcomes and adverse events compare favorably with older techniques.

Gene-Related Response of Basal Cell Carcinoma to Biologic Treatment with Vismodegib.

We aimed to characterise the response of locally advanced basal cell carcinoma (BCC) to systemic treatment with Vismodegib, a Hedgehog pathway inhibitor, by changes in the expression levels of Hedgehog pathway genes. Data were collected prospectively on 12 patients treated systemically for locally advanced BCC. Biopsy samples taken on admission and after treatment cessation were analysed pathologically and with the NanoString nCounter system to quantify the expression of 40 Hedgehog signaling pathway genes. Findings were compared before and after treatment, between complete and partial responders, and with localised BCC samples from 22 patients. Sixteen Hedgehog pathway genes changed significantly from before to after treatment. GAS1 was the only gene with a significantly different expression at baseline between complete responders (6 patients) and partial responders (4 patients) to Vismodegib (P = 0.014). GAS, GLIS2 and PRKACG1 showed different expression before treatment between the locally advanced and localised BCCs. The baseline expression level of GAS1 appears to be predictive of the response of locally advanced BCC to systemic Vismodegib treatment. A change in expression of many Hedgehog pathway genes, albeit expected by the known activity of Vismodegib, may nevertheless serve as an indicator of the response potential of the tumour.