Optic Nerve Aplasia.

OBJECTIVE: Optic nerve aplasia (ONA) is a rare ocular anomaly. We report ophthalmologic, systemic, and genetic findings in ONA. METHODS: Patients were identified through an International Pediatric Ophthalmology listserv and from the practice of the senior author. Participating Listserv physicians completed a data collection sheet. Children of all ages were included. Neuroimaging findings were also recorded. RESULTS: Nine cases of ONA are reported. Patients' ages ranged from 10 days to 2 years (median 9 months). Seven cases were bilateral. All patients had absence of the optic nerve and retinal vessels in the affected eye or eyes. Ophthalmologic findings included glaucoma, microcornea, persistent pupillary membrane, iris coloboma, aniridia, retinal dysplasia, retinal atrophy, chorioretinal coloboma, and persistent fetal vasculature. Systemic findings included facial dysmorphism, cardiac, genitourinary, skeletal, and developmental defects. A BCOR mutation was found in one patient. One patient had rudimentary optic nerves and chiasm on imaging. CONCLUSION: ONA is a unilateral or bilateral condition that may be associated with anomalies of the anterior or posterior segment with or without systemic findings. Rudimentary optic nerve on neuroimaging in one case suggests that ONA is on the continuum of optic nerve hypoplasia.

Refractive error in unilateral Duane syndrome.

PURPOSE: To investigate whether chronic co-contraction of the medial and lateral rectus muscles leads to higher against-the-rule astigmatism in affected versus fellow eyes in Duane syndrome. METHODS: A database of 4,103 patients of Dr. Arthur Jampolsky was queried for diagnosis of Duane syndrome. Inclusion criteria were unilateral Duane syndrome and complete data set. Refractive error was compared between Duane and fellow eyes. RESULTS: A total of 84 patients with unilateral Duane syndrome were identified. The spherical equivalent of Duane eyes and fellow eyes was similar (0.4 vs. 0.2 [P = 0.14]). Mean cylinder power was higher in Duane eyes compared to fellow eyes (0.7 vs 0.4 [P = 0.0003]). There was modest evidence of more against-the-rule astigmatism in Duane eyes compared to fellow eyes (P = 0.04) but no difference for with-the-rule astigmatism (P = 0.83). Duane compared to fellow eyes were, however, significantly more likely to have oblique astigmatism (P = 0.004) or any astigmatism at all (P = 0.0005). CONCLUSIONS: Duane eyes compared to fellow eyes had higher astigmatism that was more likely to be oblique. Our study lends support to the hypothesis that extraocular muscles may influence refractive error.

Endophthalmitis after strabismus surgery: incidence and outcome in relation to age, operated eye muscle, surgical technique, scleral perforation and immune state.

PURPOSE: Identify risk factors for endophthalmitis after strabismus surgery (EASS) and relate these to incidence and outcome. METHODS: Ophthalmologists, who had operated, diagnosed or treated EASS, completed a case record form with 71 questions in six domains: Preoperative, Surgery, Perforation, Postoperative, Outcome and Experts' opinion. To estimate the age-specific incidence per number of strabismus operations in the Netherlands during 1994-2013, the age distribution of Dutch cases was compared with the age-specific rates of strabismus surgery in the Dutch Registry of Strabismus Operations and with population data. Exploratory data analysis was performed. The immune state was evaluated in six patients. Five enucleated eyes were studied histopathologically. RESULTS: None of the 26 patients (27 eyes with EASS) were between 9 and 65 years old, except for one patient with retinal haemorrhage followed by endophthalmitis. In the Netherlands during 1994-2013, the rate of EASS was approximately one per 11 000 strabismus operations, but one per 4300 for children aged 0-3 and one per 1000 for patients 65 and older. Endophthalmitis was diagnosed on postoperative day 1-4 in children aged 0-3. In all 15 children aged 0-5, the 16 affected eyes were phthisical, eviscerated or enucleated. The involved eye muscle had been recessed in 25 of 27 cases. It was a medial rectus in 15 of 16 children aged 0-6. It was a lateral (6), inferior (2) or medial (1) rectus in elderly. Scleral perforation went unnoticed in all children (no record in three) and in two of seven elderly (no record in two). Histopathology showed transscleral scarring compatible with scleral perforation in four patients but, in a two-year-old girl who had EASS together with a transient medial rectus palsy, the sclera underneath the former suture tract was not perforated but did contain the long posterior ciliary artery. CONCLUSIONS: Endophthalmitis after strabismus surgery (EASS) affects children and elderly, with a grave outcome in young children. It occurs after recession of the medial rectus muscle in children, and it may occur without scleral perforation. Age and perforation are key determinants that interact with other factors that determine the occurrence and fulminance of EASS.

The decline of pneumococcal resistance after cessation of mass antibiotic distributions for trachoma.

After 6 biannual mass distributions of oral azithromycin for trachoma in Ethiopian communities, 76.8% (95% confidence interval [CI], 66.3%-85.1%) of nasopharyngeal Streptococcus pneumoniae isolates from children aged 1-5 years were resistant to macrolides. Twelve and 24 months after the last azithromycin treatment, resistance decreased to 30.6% (95% CI, 18.8%-40.4%; P <.001 ) and 20.8% (95% CI, 12.7%-30.7%; P < .001), respectively. Macrolide resistance decreases after antibiotic pressure is removed.

Risk factors for intraoperative complications in resident-performed phacoemulsification surgery.

PURPOSE: To determine the risk factors for intraoperative complications in resident-performed phacoemulsification surgery and the effect of complications on postoperative visual acuity. DESIGN: Retrospective case series. PARTICIPANTS: A total of 320 consecutive eyes of predominantly male (96.6%) and elderly patients (mean age +/-1 standard deviation = 73.1+/-10.0 years) undergoing phacoemulsification surgery by ophthalmology residents at a Veterans Administration Hospital between January 2006 and 2007. There were no exclusion criteria for type of cataract undergoing phacoemulsification surgery. METHODS: Data were collected by review of patients' electronic medical records. Collected data included the patient demographics, ocular comorbidities, cataract features, resident, resident experience, attending, right or left eye, anesthesia type, wound type, phacoemulsification technique, preoperative and postoperative visual acuities, and presence of any intraoperative complication. Multivariate models were constructed to determine potential risk factors for intraoperative complications. MAIN OUTCOME MEASURES: Major intraoperative complication rate, risk factors for major intraoperative complications, and best-corrected postoperative visual acuity. RESULTS: The major intraoperative complication rate was 4.7%, which included 3.1% of cases with vitreous loss. The strongest association with a major complication was the presence of a case identified as challenging preoperatively, which had an odds ratio of 6.0 (95% confidence interval [CI], 1.5-24.1, P=0.01). The challenging features most strongly associated with major complications were mature 4+ nuclear sclerotic cataracts and zonular pathology (antecedent trauma and pseudoexfoliation), which had odds ratios of 18.9 (95% CI, 3.1-117, P=0.002) and 26.2 (95% CI, 4.3-159, P=0.003), respectively. A major complication decreased the likelihood of achieving 20/40 or better visual acuity within 90 days of surgery (95% of uncomplicated eyes vs. 71% of complicated eyes; P=0.009). CONCLUSIONS: Residents performing phacoemulsification surgery achieved a low overall rate of major complications. However, specific features of cataracts, such as mature nuclei and zonular pathology, carried increased intraoperative risk. Anticipating risk may help to decrease surgical complications further and to counsel patients appropriately.

Acute angle-closure glaucoma due to iris transfixation of a subluxated posterior chamber intraocular lens-capsular bag complex.

An 80-year-old woman with pseudoexfoliation syndrome developed subluxation of a posterior chamber intraocular lens (IOL)-capsular bag complex due to zonular rupture. She underwent a limited 25-gauge pars plana vitrectomy and iris transfixation of the subluxated IOL-capsule complex. The postoperative course was uneventful until the patient developed acute angle-closure glaucoma 3 weeks postoperatively. Ultrasound biomicroscopy confirmed pupillary block by the IOL-capsule complex. The patient was treated with intraocular pressure-lowering medications and a vitreous tap before a laser peripheral iridotomy could be performed successfully. The vision returned to normal, and the angle closure resolved. We recommend that a prophylactic surgical iridectomy be performed in patients with iris transfixation of a posterior chamber IOL-capsular bag complex.

Residues in the first transmembrane domain of the Caenorhabditis elegans GABA(A) receptor confer sensitivity to the neurosteroid pregnenolone sulfate.

The GABA(A) receptor is a target of endogenous and synthetic neurosteroids. Little is known about the residues required for neurosteroid action on GABA(A) receptors. We have investigated pregnenolone sulfate (PS) inhibition of the Caenorhabditis elegans UNC-49 GABA receptor, a close homolog of the mammalian GABA(A) receptor. The UNC-49 locus encodes two GABA receptor subunits, UNC-49B and UNC-49C. UNC-49C is sensitive to PS but UNC-49B is not sensitive. By analyzing chimeric receptors and receptors containing site-directed mutations, we identified two regions required for PS inhibition. Four residues in the first transmembrane domain are required for the majority of the sensitivity to PS, but a charged extracellular residue at the end of the M2 helix also plays a role. Strikingly, mutation of one additional M1 residue reverses the effect of PS from an inhibitor to an enhancer of receptor function. Mutating the M1 domain had little effect on sensitivity to the inhibitor picrotoxin, suggesting that these residues may mediate neurosteroid action specifically, and not allosteric regulation in general.

Ophthalmic manifestations of infections caused by the USA300 clone of community-associated methicillin-resistant Staphylococcus aureus.

PURPOSE: To report the microbiological, clinical, and pathological characteristics of community-associated methicillin-resistant Staphylococcus aureus (CAMRSA) infections of the eye and orbit. DESIGN: Prospective case series. PARTICIPANTS: Nine patients with CAMRSA infections of the eye and orbit were identified during a 6-month period at 2 tertiary care hospitals in San Francisco. METHODS: Case identification was by prospective case selection and retrospective laboratory review of 549 MRSA cultures collected in the 2 hospitals. Ophthalmic microbial isolates were analyzed by pulsed-field gel electrophoresis and compared with a control CAMRSA clone (USA300). Clinical characteristics of patients infected with CAMRSA were reviewed, and all surgical specimens underwent pathological examination. MAIN OUTCOME MEASURES: Pulsed-field gel electrophoresis banding patterns of MRSA isolates, antibiotic sensitivity profiles, patient demographics, systemic and ocular complications of infection, and posttreatment visual acuities. RESULTS: Nine ophthalmic isolates were CAMRSA clone USA300. The infections included orbital cellulitis, endogenous endophthalmitis, panophthalmitis, lid abscesses, and septic venous thrombosis. Patients were treated with trimethoprim-sulfamethoxazole, rifampin, clindamycin, or vancomycin based on microbial sensitivity studies and severity of infection. Eight of the 9 patients had no history of hospitalization. Seven patients required hospitalization, 3 required surgery, and an additional 4 required invasive procedures. Eight patients had good visual outcomes, but 1 deteriorated to no light perception. Pathological analyses showed extensive necrosis in eyelid and orbital specimens, and disorganized atrophy bulbi in an enucleated eye. CONCLUSION: The USA300 CAMRSA clone, which carries Panton-Valentine leukocidin genes, can cause aggressive infections of the eye and orbit in hospital-naive patients. Treatment of infections often required debridement of necrotic tissues in addition to non-beta-lactam class antibiotics. In communities where CAMRSA is prevalent, ophthalmologists should obtain microbial cultures and sensitivity studies to help guide antibiotic therapy for severe ophthalmic infections.

Periorbital zygomycosis (mucormycosis) treated with posaconazole.

PURPOSE: To report on the successful treatment of periorbital zygomycosis (mucormycosis) with posaconazole, a broad-spectrum oral antifungal available for compassionate use. DESIGN: Interventional case report. METHODS: Review of a medical record. RESULTS: A 22-year-old male undergoing induction chemotherapy for acute lymphoblastic leukemia presented with periorbital cellulitis attributable to Rhizopus. The patient was initially treated with liposomal amphotericin B, surgical debridement, and reversal of immune compromise. The patient was switched to posaconazole because of amphotericin side effects and lack of improvement. He took posaconazole for five months while undergoing additional cycles of chemotherapy. Despite recurrent profound neutropenia, the periorbital infection resolved, he tolerated reconstructive procedures, and he did not develop orbital invasion. His Rhizopus isolate was highly susceptible to posaconazole in vitro. CONCLUSIONS: Drug toxicities can limit the use of amphotericin in some patients with zygomycosis. Posaconazole shows promise as an alternative antifungal agent in the treatment of periorbital zygomycosis.

Bilateral blindness from orbital cellulitis caused by community-acquired methicillin-resistant Staphylococcus aureus.

PURPOSE: To describe bilateral blindness resulting from infection with community-acquired methicillin-resistant Staphylococcus aureus (MRSA). DESIGN: Observational case report. METHODS: A 44-year-old man developed proptosis, ptosis, ophthalmoplegia, and no light perception vision after attempting to lance a nasal pustule. A nasal culture grew MRSA. Imaging showed bilateral orbital cellulitis, pansinusitis, and cavernous sinus thrombosis. The right fundus showed severe ischemia, but the left fundus was essentially normal. RESULTS: Despite initiation of appropriate antibiotics early in the course of infection, the patient lost sight in both eyes. Surgical drainage of the paranasal sinuses and use of intravenous corticosteroids and heparin led to the resolution of orbital cellulitis. CONCLUSIONS: MRSA orbital cellulitis can progress to irreversible blindness despite antibiotic treatment. A new, community-acquired clone of this organism has exhibited increased potential for tissue invasion.

Ophthalmic manifestations of perinatally acquired HIV in a US cohort of long-term survivors.

BACKGROUND/AIMS: To determine the ophthalmic manifestations of HIV in a cohort of long-term survivors of perinatally acquired HIV. METHODS: Twenty-two patients with perinatally acquired HIV who were aged ≥12 years were prospectively studied at a university clinic. They underwent complete ophthalmic examinations and fundus photography. Their medical histories, medications and CD4 counts were abstracted from the medical records. To evaluate for keratoconjunctivitis sicca, both HIV patients and 44 healthy controls (matched by age, gender and contact lens wear) underwent Schirmer testing and ocular surface staining. RESULTS: Nine male and 13 female HIV patients with mean age of 16.6 years (SD, 3.4) were examined. Of the 22 HIV patients, 21 had been treated with highly active antiretroviral therapy (HAART). Only one patient had a CD4 count nadir of <200 cells/µL. The mean visual acuity of the eyes of the HIV subjects was 20/22 (SD, 1.6 lines). No patient had cytomegalovirus retinitis. Four of the 22 (18%) HIV patients had strabismus. HIV subjects and controls had similar rates of abnormal Schirmer (9% and 14%, p=0.62) and ocular staining scores (p=0.29). CONCLUSIONS: In the post-HAART era, long-term survivors of perinatally acquired HIV exhibited little vision-threatening disease, but had a high prevalence of strabismus.

Introduced Plasmodium vivax malaria in a Bolivian community at an elevation of 2,300 meters.

Tuntunani, Bolivia, a community of 199 persons situated at an elevation of 2,300 meters, experienced its first malaria outbreak in 1998. Blood smears from 63 of 183 symptomatic residents were examined, and 52 showed Plasmodium vivax. An investigation two years later indicated that the epidemic resulted from introduced transmission, since persons of all ages and both sexes were infected, and there had been no travel to low-lying endemic areas in the five months preceding the epidemic. Treatment became available only two months into the epidemic, at which time 58% of the people had been ill for three weeks or longer. This outbreak demonstrates the vulnerability of highland populations with poor access to health care to introduced malaria.

Clinical problems in strabismus: the Smith-Kettlewell Eye Research Institute symposium.

The Smith Kettlewell Eye Research Institute (SKERI), celebrating its 50th anniversary in 2014, hosted a symposium to identify the most pressing clinical problems in strabismus and binocular vision. Forty-five experts from around the world shared their perspectives at the San Francisco meeting, held November 6-9, 2012. Prior to the meeting, the organizers (TR, APW, RH, JB, AJ) asked attendees to identify the most pressing clinical problems in strabismus and to discuss them in a workshop-based format. The clinical problems were organized into the following six areas: (1) esotropias; (2) binocular vision: amblyopia, suppression, and diplopia; (3) intermittent exotropia; (4) "oblique dysfunctions"; (5) dissociated vertical deviation (DVD); and (6) new approaches to strabismus management. Herein we highlight of some of the clinical problems discussed at the meeting.

Errors in strabismus surgery.

OBJECTIVES: To determine the prevalence of and contributing factors for errors in strabismus surgery. METHODS: Five hundred seventeen of 1103 strabismus surgeons (46.87%) completed a survey administered during the 2011 American Association for Pediatric Ophthalmology and Strabismus national meeting or e-mailed to members of the association. RESULTS: One hundred seventy-three strabismus surgeons (33.5%) self-reported having operated on the wrong eye or muscle or performed the wrong procedure at least once. The mean error rate was 1 in 2506 (95% CI, 2128-2941) operations. Surgeons who performed fewer than the median 1500 procedures had an error rate 5.9 (95% CI, 4.1-8.2) times higher than surgeons who performed more than the median (P < .001). The most common factors contributing to errors were confusion between the type of deviation (esotropia/exotropia) and/or the surgical procedure (recession/resection) (34 of 114 responses [29.8%]), globe torsion (20 [17.5%]) leading primarily to inadvertent operation on the inferior rectus rather than the intended medial rectus muscle, and inattention and/or distraction (19 [16.7%]). Running more than 1 operating room (P = .02) and failing to mark eye muscles preoperatively (P = .03) were associated with an increased likelihood of error. CONCLUSIONS: Self-reported error in strabismus surgery is a complication approximately as common as periorbital cellulitis. Reducing error in strabismus surgery might entail confirming that the deviation matches the surgical plan preoperatively, more elaborate site marking, and involving an assistant in a preoperative verification of the specific eye muscles and surgical procedure.

Pathological findings in poststrabismus surgery endophthalmitis.

Bacterial entry into the vitreous cavity via inadvertent scleral perforation is one postulated mechanism for poststrabismus surgery endophthalmitis. In a review of 746 cases, we identified 2 pediatric enucleation specimens related to complications of strabismus surgery. In both cases, the patients developed postoperative endophthalmitis and no light perception vision, and the eyes were enucleated when they became phthisical or painful. In both submitted cases, pathology showed a thick band of scar tissue emanating focally from the sclera into the vitreous. Although no needle tracts were visualized, pathological findings were consistent with scleral perforation.

Effect of timing and duration of tamsulosin exposure on complications in resident-performed phacoemulsification.

BACKGROUND AND OBJECTIVES: Tamsulosin can cause intraoperative floppy iris syndrome and increase the risk of phacoemulsification complications. This study evaluated whether the risk of complications was associated with the timing or duration of preoperative tamsulosin exposure. PATIENTS AND METHODS: The current study was a retrospective review of electronic medical records of resident-performed phacoemulsification surgeries from 1998 to 2008 at a Veterans Administration Hospital. There were 73 eyes with recent tamsulosin exposure (within 30 days preoperatively) and 28 eyes with remote exposure (> 30 days preoperatively but within 3 years of surgery). RESULTS: There was a trend toward more total complications in recent compared with remote tamsulosin exposure cases (31.5% vs 14.3%, P = .09). A longer duration of tamsulosin use was not statistically associated with an increased risk of total complications. CONCLUSION: The authors were unable to detect a statistically significant effect of duration of tamsulosin use on the risk of intraoperative complications in phacoemulsification surgery.

Chorioretinal architecture in Aicardi syndrome: an optical coherence tomography and fluorescein angiography study.

Aicardi syndrome is a rare congenital disorder with a classic triad of infantile spasms, chorioretinal lacunae, and agenesis of the corpus callosum. We report the use of fluorescein angiography and spectral domain optical coherence tomography to examine the posterior segment structures in an 8-month-old girl with Aicardi syndrome. Most of the observed features correlated with previously published histopathological findings, but inner nuclear layer cysts have not been previously described. To our knowledge, this is the first study of the tomographic and angiographic chorioretinal features in vivo.

Severe zone III retinopathy of prematurity in an infant with a birth weight of more than 1,500 grams.

The authors report zone III stage 3 retinopathy of prematurity requiring treatment in a former 1,692-g 30 6/7-week twin cared for in a modern intensive care nursery. This case highlights the need for identifying biomarkers that increase the risk of severe retinopathy of prematurity so that screening guidelines can be optimized.