Ta C. Chang

General anesthesia in the pediatric population.

Purpose of review This article reviews the pertinent perioperative, intraoperative, and short- and long-term postoperative risks associated with general anesthesia in children undergoing ocular surgery. Recent findings Animal studies suggest an association between general anesthesia and neurodevelopmental delay; however, animal pharmacodynamics and pharmacokinetics do not directly correlate with human metrics. Retrospective human studies present conflicting data. Further, prospective studies in humans are underway, with projected results available within the next 3–5 years. Summary All surgeons should be aware of current practices in pediatric anesthetic care, as well as the immediate- and long-term risks of general anesthesia. Ophthalmologists with pediatric patients should be aware of the potentially life-threatening conditions associated with general anesthesia. Additionally, the relative lifelong risks and benefits of general anesthetic exposure should be considered when recommending surgery, especially in light of the childs age, health status, and necessity of multiple anesthetic events.

Utilizing optical coherence tomography in diagnosing a unique presentation of chiasmal hypoplasia variant of septo-optic dysplasia.

W e evaluated a patient whose findings relate to 2 recent reviews in the Journal, that of Borchert (1) on optic nerve hypoplasia and Fraser et al (2) dealing with nonglaucomatous optic disc cupping. A 13-year-old adopted boy was referred for abnormal optic nerve appearance. On examination, the visual acuity was 20/20 in each eye and intraocular pressure was 13 mm Hg, right eye, and 14 mm Hg, left eye. Funduscopy revealed bilateral excavated and pale optic nerves (Fig. 1). Retinal nerve fiber layer (RNFL) analysis by spectral-domain optical coherence tomography showed a pattern consistent with bow-tie atrophy in each eye (Fig. 2). Automated visual field testing revealed bitemporal hemianopia. Magnetic resonance imaging (MRI) of the brain was remarkable for absence of septum pellucidum and chiasmal atrophy (Fig. 3). Based on these findings, the diagnosis of chiasmal hypoplasia-variant septo-optic dysplasia (SOD) was made. Our patient presented with optic nerve cupping, raising suspicion for juvenile open-angle glaucoma. However, the RNFL analysis revealed thinning of nasal and temporal quadrants, unlike glaucomatous damage, which typically involves thinning of the superior and inferior quadrants. The relatively preserved superior and inferior RNFL bundles on optical coherence tomographic analysis correlated well with the findings of bitemporal hemianopia and the MRI findings of chiasmal hypoplasia. To our knowledge, this is the first description of RNFL analysis in the chiasmal hypoplasia variant of SOD. This

Unilateral foveal hypoplasia in a child with bilateral anterior segment dysgenesis

In patients with foveal hypoplasia, anterior segment dysgenesis and an absence of systemic findings, consider a recently described syndrome of foveal hypoplasia, optic nerve decussation defects and anterior segment dysgenesis (FHONDA) in the differential diagnosis.

A novel characterization of posterior keratoconus using anterior segment optical coherence tomography in an infant: a case report

BackgroundPosterior keratoconus is a rare cause of a corneal opacity in an infant. It is characterized by thinning of the posterior cornea without ectasia of the anterior cornea. Imaging of this condition with anterior segment optical coherence tomography (AS-OCT) has not been reported in the literature.Case presentationA six week old African-American male presented with a congenital corneal opacity of the right eye. He underwent an examination under anesthesia in which photography and AS-OCT were performed. AS-OCT confirmed the diagnosis of posterior keratoconus. The patient subsequently underwent an optical iridectomy for visual development.ConclusionAS-OCT is a useful tool in cases when a child presents with a corneal opacity of unknown or unclear etiology. In our patient, AS-OCT showed the classic description of central corneal thinning seen in this condition. Additionally, it revealed an associated detached Descemet membrane, a feature which has not been previously described in posterior keratoconus.

Anesthesia considerations in pediatric glaucoma management.

Purpose of review This article reviews the potentially adverse neurodevelopmental effects of early exposure to general anesthesia and examines a changing paradigm in the management of pediatric glaucoma. Recent findings Literature across multiple subspecialties has examined the potentially neurotoxic effects of general anesthesia on the developing childs brain. Associations between general anesthesia exposure early in life and attention deficit hyperactivity disorder, language processing, and cognition have been suggested but not confirmed. Several population studies support the conclusion that early anesthetic exposure may increase the risk of neurodevelopmental deficits, although this is unsupported in sibling cohorts. Newer technology such as rebound tonometry may decrease the frequency of examination under anesthesia in the long-term management of patients with pediatric glaucoma and may decrease the risk of these potentially adverse neurodevelopmental outcomes. Summary As the potential long-term adverse neurodevelopmental effects of general anesthesia become better understood, pediatric glaucoma specialists should be cognizant of the relative lifelong risks and benefits of repeat examinations under anesthesia in young patients.

Retinal pigment epithelium changes in pediatric patients with glaucoma drainage devices

Purpose Retinal changes secondary to hypotony are usually described as wrinkling or folding of the inner portion of the choroid, the retinal pigment epithelium (RPE), and the outer retinal layers in the macular area due to scleral wall collapse. We describe a new retinal finding in children with suspected hypotony after implantation of Baerveldt Glaucoma Implant (BGI). Observations Four patients in our series developed significant RPE defects after BGI implant. The RPE defects appeared as elongated white lines observed solely in the posterior pole, in no particular pattern, and seemed to be worse in infants with anterior segment dysgenesis and with collagen disorders. Conclusion and importance Children have thinner and more elastic scleral walls than adults. This characteristic may cause the inward scleral wall to collapse when the eye is hypotonic. The resulting redundancy of the retina leads to wrinkling and RPE defects characterized by hypopigmented lines predominantly in the macular area. Such findings, to our knowledge, have not been previously reported in pediatric patients.

Correlation of echographic and photographic assessment of optic nerve head cupping in children

PURPOSE To determine the diagnostic value of B-scan echography in optic nerve head (ONH) cupping estimation in children. METHODS The medical records of pediatric patients who had previously undergone examination under anesthesia and for whom both adequate B-scan echography images and optic nerve head (ONH) photographs and were available were reviewed retrospectively. The cup:disk ratio was estimated with a grading scale of 0-1.0 and rounded to the nearest tenth; degree of cupping was estimated from B-scan echography (small, medium, or large) by 5 masked graders (3 glaucoma specialists and 2 ophthalmic sonographers) on 2 separate occasions. Inter- and intraobserver agreement in echographic and photographic cupping assessment by the masked graders as well as correlation of echographic and photographic cup size estimation was evaluated. RESULTS A total of 36 children were included. Glaucoma specialists reliably assessed cup:disk ratio with moderately good consistency across specialists (average intraclass correlation coefficient [ICC] for intraobserver agreement, 0.86; average ICC for interobserver agreement, 0.71). Sonographers were extremely reliable in assessment of cup size when examining echographic images (ICC for both inter- and intrarater variability, 1.0). Echographic estimate of cup size correlated poorly with cup:disk ratio (ICC, 0.34). CONCLUSIONS B-scan echography is a reliable and consistent diagnostic tool in estimating the degree of ONH cupping in children and can be very useful in patients in whom direct visualization is not feasible. Failure to account for disk size may have contributed to the poor correlation between echographic cup size and photographic cup:disk ratio.

Presumed solar retinopathy in child with juvenile open-angle glaucoma

A 12-year-old girl with well-controlled, severe juvenile openangle glaucoma presented to the ophthalmology emergency department with blurred vision in both eyes. The onset of visual symptoms began after staring at the sun for approximately 1 minute the day prior to presentation. Her best-corrected visual acuity was 20/60 OD and 20/40 OS, decreased from a prior visit in which the visual acuity was 20/30 OD and 20/20 OS. On dilated fundus examination, she had unchanged severe glaucomatous cupping with new foveal hypopigmentation in both eyes, which corresponded to anatomic changes visualized on macular optical coherence tomography imaging (Figure). The remainder of her ocular examination findings were unremarkable. The child was observed and followed up by pediatric glaucoma and retina specialists. On follow-up, her visual acuity remained stable and no further changes on examination or macular optical coherence tomography were noted.

B-Scan Echography in Cases of Confirmed Persistent Fetal Vasculature

To the Editors: Persistent fetal vasculature (PFV) results from failure of the intraocular hyaloid vasculature to involute during lens development. It is a common cause of unilateral congenital cataracts and has a reported prevalence of 20% of monocular cataracts.1,2 Although the visual outcome of PFV cataract surgery is not inferior to other unilateral cataract surgeries, the presence of PFV increases the risk of intraoperative complications and has a higher incidence of postoperative adverse events.3,4 Echography is often employed in the preoperative assessment because the preoperative diagnosis of PFV can be made with visualization of the persistent hyaloid vessel (Figure 1). Although the falsenegative rate of echography in PFV has been evaluated, the true positive rate has not.5 To evaluate the true positive and false-negative rates of B-scan echography in intraoperatively confirmed PFV cases, we obtained institutional review board approval and performed a retrospective review of medical records at Bascom Palmer Eye Institute between 1994 and 2014. We included all patients younger than 18 years of age who had undergone B-scan echography prior to congenital cataract surgery and had a PFV diagnosis. PFV was diagnosed if the postoperative diagnosis in the operative report was “persistent fetal vasculature” or “persistent hyperplastic primary vitreous,” or if visualization of a persistent hyaloid vessel and/ or ciliary body stretching was described. Eyes were excluded if only a “retrolental membrane” was described but none of the other findings were present or if evidence of posterior disease precluded adequate echographic examination of the vitreous cavity. A technician experienced in ophthalmic echography interpretation reviewed archived images of each study and a forced choice of “PFV present” or “PFV absent” was made. If a patient had bilateral disease, only the eye with the better quality echographic images was included. Twenty-five eyes of 25 patients were included. Twenty-three had positive preoperative echographic findings indicative of PFV (92%, “true positives”), whereas 2 had negative echographic findings (8%, “false negatives”). Of these patients, cataracts were unilateral in 23 (92%) and bilateral in 2 (8%). Dilated fundus examination was attempted in all eyes and the fundus was visible in only 7 (28%) eyes. Tractional retinal detachment was present preoperatively in 3 (12%) eyes. Retinal detachment occurred intraoperatively in 1 (4%) eye. The limitations of our study include the retrospective design and relatively small number of patients; however, given the relative rarity of congenital cataracts and PFV, a prospective study with a large population would be unlikely. Second, there was no standardization of echography technique. Because the eyes included in the analysis had either a preoperative diagnosis of PFV or echographic examination performed specifically to rule out PFV, the

Incarceration of Lens Capsular Material in a Tube Shunt in an Aphakic, Vitrectomized Infant Eye.

An aphakic child with glaucoma associated with infantile cataract surgery and a previously functioning nonvalved glaucoma drainage device presented with sudden-onset pain and increased eye pressure. Meticulous vitrectomy was performed at the time of tube shunt surgery. Examination under anesthesia revealed obstruction of the tube with capsular remnants (Figure), which likely loosened and became mobile at the time of tube opening. The child underwent a repeated vitrectomy with removal of capsular remnants from the tube. On the first day after surgery, the intraocular pressure normalized.