Publications by authors named "Bharti R Nihalani"

Orbital myositis is a rare, commonly idiopathic, inflammatory condition that affects one or more extraocular muscles. We present a case of unilateral orbital myositis affecting the lateral rectus muscle presenting with gaze-evoked amaurosis, pain, and diplopia, with restrictive limitation of adduction. With improvement in adduction after initiating treatment, we noted narrowing of the palpebral fissure on attempted adduction, mimicking Duane retraction syndrome (DRS). Reported cases of "pseudo-DRS" are associated with multiple etiologies and are characterized by retraction on attempted abduction rather than adduction, as occurs in true DRS. In this case, pseudo-DRS occurred in the setting of idiopathic orbital inflammatory syndrome (orbital myositis) with a motility pattern more consistent with true DRS.

Pediatric genetic disorders of lens include various cataractous and non-cataractous anomalies. The purpose of this review is to help determine the genetic cause based on the lens appearance, ocular and systemic associations. Children with bilateral cataracts require a comprehensive history, ophthalmic and systemic examination to guide further genetic evaluation. With advancements in genetics, it is possible to determine the genetic mutations and assess phenotype genotype correlation in different lens disorders. The genetic diagnosis helps the families to better understand the disorder and develop realistic expectations as to the course of their child's disorder.

Purpose: To report outcomes of esotropia surgery with a goal-determined tool.

Methods: A goal-determined outcomes analysis tool was devised to address a quality improvement initiative at Boston Children's Hospital. Surgeons preoperatively ranked four possible goals for intervention: enhancement of binocular potential, restoration of eye contact (reconstructive), management of diplopia, and resolution of torticollis. Criteria for success were goal specific; the primary outcome measure was surgical success at 2-4 months. Secondary outcomes included appraisal of risk factors and a comparison of outcomes with this methodology versus traditional criteria for success based on motor alignment. No patients were excluded based on diagnosis, systemic and ocular risk factors, or intervention performed.

Results: A total of 824 patients underwent esotropia surgery from 2006 to 2012 and returned for evaluation at 2-4 months' follow-up. Of these, 777 had sufficient documentation for inclusion: 372 procedures were performed primarily to improve binocular potential; 238, to restore eye contact; 124, to resolve diplopia; and 43, to remediate torticollis. Excellent (71%) or good (13.7%) results were obtained in 84% of cases. Without associated risk factors, 75% had excellent and 14% had good outcomes. Risk factors were present in 444 (57%). Success diminished with prior strabismus surgery (P = 0.004), preoperative angle ≥50(Δ) (P = 0.002), and surgery before 12 months of age (P = 0.003). Patients having surgery to remediate diplopia had the best outcomes (excellent, 79%; good, 8%). Preoperative ranking of goals allowed demonstration of better results than would have been reported with requirement of "traditional" motor alignment criteria (P = 0.009).

Conclusions: Goal-determined methodology can be useful for monitoring outcomes of esotropia surgery in diverse populations.

Purpose: To report uncorrected distance and near visual acuity in pediatric eyes treated with primary monofocal intraocular lens (IOL) implantation.

Setting: Boston Children's Hospital, Boston, Massachusetts, USA.

Design: Retrospective chart review.

Methods: Records of children older than 5 years who had uneventful monofocal IOL implantation targeted for emmetropia within ± 1.00 diopter (D) spherical equivalent were reviewed. Eyes with secondary IOL placement and sulcus-fixated IOLs were excluded. Vision was tested using the Mentor BVAT or M&S system for distance and the standard Jaeger near card for near visual acuity. Keratometry, axial length, type of IOL, and preoperative and postoperative refractions were recorded. The main outcome measure was uncorrected visual acuity at distance and near. Good visual acuity was defined as 20/40 or better.

Results: Forty-one eyes of 25 children had uncorrected distance and near visual acuity recorded in the early postoperative period. The mean age was 11.2 years ± 3.6 (SD). Twenty eyes (49%) had good visual acuity at distance and near, 11 had good visual acuity at distance only, 6 had good visual acuity at near only, and 4 had worse than 20/40 at distance and near. Thirty eyes (70%) had with-the-rule astigmatism. All but 2 eyes (95%) had 20/70 or better uncorrected acuity at distance and near. In children with bilateral pseudophakia, 12 (75%) of 16 had 20/40 or better uncorrected acuity at distance and near.

Conclusion: Monofocal IOL placement resulted in good uncorrected distance and near visual acuity in almost 50% of pediatric eyes and in 75% of patients when targeted within ± 1.00 D of emmetropia.

Purpose: To describe phenotypic characteristics of two pedigrees manifesting early onset crystalline cataract with mutations in the γD-crystallin gene (CRYGD).

Methods: A detailed medical history was obtained from two Caucasian pedigrees manifesting autosomal dominant congenital cataracts. Genomic DNA was extracted from saliva (DNA Genotek). Single Nucleotide Polymorphism (SNP) based genome analysis of the larger pedigree revealed linkage to an 8.2 MB region on chromosome 2q33-q35 which encompassed the crystallin-gamma gene cluster (CRYG). Exons and flanking introns of CRYGA, CRYGB, CRYGC and CRYGD were amplified and sequenced to identify disease-causing mutations.

Results: A morphologically unique cataract with extensive refractile "crystals" scattered throughout the nucleus and perinuclear cortex was found in the probands from both pedigrees. A heterozygous C→A mutation was identified at position 109 of the coding sequence (R36S of the processed protein) in exon 2 of CRYGD and this missense mutation was found to cosegregate with the disease in the larger family; this mutation was then identified in affected individuals of pedigree 2 as well.

Conclusions: The heterozygous 109C→A CRYGD missense mutation is associated with a distinct crystalline cataract in two US Caucasian pedigrees. This confirms crystalline cataract formation with this mutation, as previously reported in sporadic childhood case from the Czech Republic and in members of a Chinese family.

Purpose: To describe our technique for secondary intraocular lens (IOL) implantation in aphakic children and report patient outcomes.

Patients And Methods: In our technique for in-the-bag IOL implantion, we used a microvitreoretinal (MVR) blade to separate the capsular leaflets for aspiration of reproliferated lens material within Soemmering's ring. When sufficient capsular opening was not possible or capsular support deemed inadequate, an IOL was implanted in the sulcus. Records of patients with secondary IOL implantation between 1999 and 2009 were retrospectively reviewed with regard to intra- and postoperative complications and visual and refractive outcomes.

Results: A total of 50 consecutive eyes of 32 patients were evaluated. Mean age at secondary IOL implantation was 9.1 years. Of these, 26 eyes had in-the-bag IOLs and 24 had sulcus IOLs. Forty-four eyes (88%) had an AcrySof IOL (Alcon Inc, Ft. Worth, TX). The prevalence of any corneal edema (54% vs 19%, P = 0.04) and uveal inflammation >2+ (92% vs 31%, P < 0.05) in immediate postoperative period was greater with sulcus IOL than in-the-bag placement. Late inflammation at 6 months developed in 1 eye, and glaucoma developed in 2 eyes with sulcus IOLs. All eyes maintained or gained lines of best-corrected visual acuity. Mean absolute prediction error was 0.9 ± 0.9 with no significant difference between in-the-bag and sulcus placement.

Conclusions: Eyes achieving secondary in-the-bag IOL showed less inflammation and corneal edema in the early postoperative period partly as the result of greater surgical and anatomic challenges in some eyes receiving sulcus IOL. Visual and refractive outcomes were satisfactory in eyes with secondary IOL implantation. Mean prediction error was similar for in-the-bag and sulcus IOLs.

Purpose: Managing pediatric cataracts is often challenging. It is technically difficult to perform surgery in these small complaint eyes and there is higher prevalence of postoperative complications. The outcomes of surgery depend on technique and technology. The purpose of this study is to review the current literature on technological advances in pediatric cataract surgery.

Methods: Review of literature on management of pediatric cataract surgery.

Results: The advent of vitrectomy machines and intraocular lenses (IOLs) has revolutionized pediatric cataract surgery. "Vitrectorhexis" has become a good alternative to manual capsulorhexis. Primary management of posterior capsule and limited anterior vitrectomy has reduced the incidence of visual axis opacification. Primary IOL implantation is becoming a standard of care in the youngest children. Single piece Acrysof® is preferred for in-the-bag implantation and 3-piece Acrysof® for sulcus implantation. Newer IOLs are being evaluated in pediatric eyes. Precise measurement of intraocular lens power and predicting refractive change are major challenges in long term care of children after surgery.

Conclusion: New technology and surgical techniques have refined pediatric cataract surgery. It is critical to focus our efforts on precise biometry measurements, IOL power calculation, and designing IOLs that may address refractive changes in the growing pediatric eye.

Purpose: To evaluate accuracy of intraocular lens (IOL) power calculation formulae (SRK II, SRK/T, Holladay 1, Hoffer Q) in pediatric eyes.

Design: Retrospective case series.

Participants: One hundred thirty-five eyes of 96 children with congenital, developmental, or acquired cataracts who underwent uncomplicated cataract surgery and IOL implantation by a single surgeon over a 10-year period.

Methods: Axial length (AL), keratometry (K), and manufacturer's A constant were employed in 4 common IOL power calculation formulae to predict the refractive outcome. Retinoscopy was measured at 4 to 8 weeks postoperatively and converted to spherical equivalent. For analysis, eyes were grouped by age at surgery, AL, and mean K.

Main Outcome Measures: We determined the prediction error (PE) = predicted refraction - actual refraction and the absolute PE = |predicted refraction - actual refraction|. The formula that gave the best prediction (minimum PE) was determined.

Results: The mean age at surgery was 6.4 years. Mean absolute PE was 1.11 for the SRK II, 0.84 for SRK/T, 0.76 for Holladay, and 0.76 for Hoffer Q formulae. There was a trend toward greater PE in eyes of younger children (< or =2 years), shorter AL (AL < or = 22 mm) and steeper corneas (mean K > 43.5 diopters [D]). On comparing absolute PE obtained with 4 formulae in each patient, Hoffer Q gave the minimum PE in 46% of eyes compared with 23% with SRK II, 18.5% with SRK/T, and 12.5% with Holladay 1. The SRK/T, Holladay 1, and Hoffer Q were similar in accurately predicting refractive error within +/-0.5 D in about 43% eyes. When clinically significant deviation in PE occurred (>0.5 D), there was usually an undercorrection (72%), except for Hoffer Q, which was almost as likely to overcorrect as undercorrect (44% vs 56%). The PE was lower with office measurements when compared with anesthesia measurements, owing probably to better fixation in older children with higher ALs.

Conclusion: The PE was insignificant (PE < or = 0.5 D) in 43% eyes, and similar for all formulae. However, the Hoffer Q was predictable for the highest number of eyes. When the PE was >0.5 D, most formulae gave an undercorrection, except for the Hoffer Q, which the surgeon may want to consider when targeting postoperative refractions.

Objective: To present and evaluate a new technique that allows the second-stage suture adjustment in strabismus surgery to be skipped or delayed if the immediate postoperative alignment is satisfactory.

Methods: The "short tag noose" technique replaces long sutures with short tags that can be left under the conjunctiva after adjustment. Retrospectively, the medical records of all patients treated by a single surgeon with this approach between January 1, 2005, and December 31, 2008, were evaluated for success rate (< or =10 prism diopters [PD] horizontal and < or =6 PD vertical), reoperation rate, and complications.

Results: Of 120 procedures reviewed, 27 (22.5%) were performed in children; 97 procedures (80.8%) were performed in patients with complex strabismus. Mean follow-up was 6 months. The success rate was 81.0% for horizontal strabismus and 70.7% for vertical strabismus at 2 months. The reoperation rate was 10.0% for horizontal strabismus and 19.0% for vertical strabismus. The noose slipped in 1 patient (0.8%) and was corrected by readjustment on day 4. Two patients (1.7%) required in-office excision of cysts or granulomas.

Conclusions: The short tag noose technique simplifies the logistics of suture adjustment and avoids the need for sedation in children who do not require adjustment. It provides the ability to defer adjustment for days after surgery.

The treatment modalities for neurofibromatosis type 1-associated optic gliomas include chemotherapy, radiation therapy, and surgical excision. The current recommendation is to consider treatment for an optic nerve glioma only if there is clear evidence of either ophthalmologic or radiographic progression with significant visual dysfunction. We report a case of a child with neurofibromatosis type 1 and an isolated optic nerve glioma with documented progression and visual loss in which clinical signs improved and visual deterioration stabilized after optic nerve sheath fenestration.

Purpose: To evaluate surgical outcomes of 1-piece AcrySof SA30AL intraocular lens (IOL) (Alcon Laboratories) implantation in children having surgery for congenital and developmental cataracts.

Setting: Iladevi Cataract & IOL Research Centre, Raghudeep Eye Clinic, Ahmedabad, India.

Methods: This prospective observational study comprised 134 consecutive eyes of 84 children from 2 to 15 years old who had surgery for congenital and developmental cataract. Two groups were formed depending on the age of the child at surgery. Primary posterior continuous curvilinear capsulorhexis (PCCC) was performed in children younger than 6 years (Group 1, 66 eyes), and no PCCC was performed in children older than 6 years (Group 2, 68 eyes). Vitrectomy was not performed. An AcrySof IOL was implanted in the bag in all eyes except 1 in Group 1, which received a sulcus-fixated IOL. The study's primary outcome measures were the incidence of visual axis obscuration and the need for a secondary procedure to clear the axis. Secondary observations included the incidence of posterior synechias, cell deposits, and haptic compression. A test of proportion was applied to determine whether age was a risk factor for the development of visual axis obscuration.

Results: The mean age at surgery was 6.0 years +/- 3.2 (SD). The mean follow-up was 2.6 +/- 0.6 years. In Group 1, 20 eyes (30.3%) developed visual axis obscuration but only 6 (9.1%) required a secondary procedure. In Group 2, 20 eyes (29.4%) developed visual axis obscuration and 10 (14.7%) required secondary procedures. Posterior synechias were observed in 2 eyes (3.0%) in Group 1 and none in Group 2. Cell deposits were seen in 8 eyes (12.1%) in Group 1 and 8 eyes (11.8%) in Group 2. Haptic compression was noted in 1 eye in Group 2. Mild IOL decentration was observed in the 1 eye with a sulcus-fixated IOL.

Conclusion: The 1-piece AcrySof IOL provided satisfactory visual axis clarity, produced an acceptable inflammatory response, and maintained centration in pediatric eyes.

Purpose Of Review: Pediatric cataract surgery remains a very important and difficult problem to manage. While dramatic advances have occurred in this field over the past 10 years, some technical aspects of surgery, changing refraction and functional outcome continue to pose significant problems. The aim of the present review is to update the reader on advances reported on the topic during the past year.

Recent Findings: Manual capsulorhexis still remains a gold standard for the successful outcome of pediatric cataract surgery. Primary management of the posterior capsule is mandatory depending on the age of the child at surgery. Primary implantation of the intraocular lens after cataract removal is gaining popularity even in infants and young children. Short-term results of single-piece Acrysof in pediatric eyes are encouraging. Predicting axial growth and the refractive change that accompanies it is one of the major challenges for long-term care of children after surgery. The evaluation of rate of axial growth and its correlation with age at surgery, laterality, aphakia/pseudophakia and visual-axis obscuration is a positive step in the right direction. Despite satisfactory technical outcomes, the functional outcomes remain unpredictable.

Summary: With refinements in surgical techniques, improvisation of intraocular lenses and better understanding of growth of the pediatric eye, in the coming years intraocular lens implantation is likely to become an established mode of treatment of children even in the youngest age group.

Purpose: To determine the prevalence of relative anterior microphthalmos (RAM) and evaluate intraoperative performance and surgical outcome in eyes with RAM undergoing cataract surgery.

Design: Nested case-control series.

Participants: One thousand four hundred consecutive patients undergoing cataract surgery were evaluated to determine the prevalence of RAM. Relative anterior microphthalmos is defined as horizontal corneal diameter (HCD) < or =11 mm, anterior chamber depth (ACD) < or =2.2 mm, and axial length (AL) >20 mm, with no other morphologic malformation.

Methods: Patients were examined preoperatively for HCD, ACD, and AL. Horizontal corneal diameter was measured with calipers. Anterior chamber depth and AL were measured with immersion shell with water. Associated ocular pathologic conditions were recorded. Two control groups were identified. Group I (normal eyes; n = 84) had HCD >11 mm, ACD >2.2 mm, and AL >20 mm. Group II (eyes with small corneal diameter; n = 84) had HCD < or =11 mm, ACD >2.2 mm, and AL >20 mm.

Main Outcome Measures: Patients with RAM and controls were evaluated for intraoperative performance and postoperative outcome. The 2-tailed Fisher exact test was applied to compare the performance of RAM with each of the control groups. The odds ratio (OR) with 95% confidence intervals (95% CI) was determined.

Results: The prevalence of RAM was 6% (84 of 1400 eyes; 95%CI, 0.048-0.074). Relative anterior microphthalmos was associated with the presence of small pupil, 34 (40.48%); corneal guttae, 31 (36.9%); glaucoma, 29 (34.5%); and pseudoexfoliation, 6 (7.14%). Intraoperatively, RAM was associated with overall surgical difficulty because of less working space in 59 eyes (70.24%; OR, 63.7; 95% CI, 18.3-221; P<0.001) compared with control groups I and II; uveal trauma in 12 (14.28%); Descemet's detachment in 5 (5.95%); and posterior capsule rupture in 2 (2.38%). Postoperatively, RAM was associated with transient corneal edema in 63 eyes (75%; OR, 9.0; 95% CI, 4.4-18.0; P<0.001; OR, 5.4; 95% CI, 2.7-10.5; P<0.001) on the first postoperative day.

Conclusions: The prevalence of RAM was 6%. Relative anterior microphthalmos with its associations posed significant intraoperative difficulties. The occurrence of transient corneal edema was frequent.