Childhood hypertension, sixth nerve palsy, and renal artery stenosis.

This short report demonstrates an uncommon presentation of intracranial bleeding and sixth nerve palsy in a child with systemic hypertension due to long-standing unilateral renal artery stenosis of unknown etiology that resulted in contracture of the affected kidney and compensatory hypertrophy of the contralateral kidney. Structural renal damage and cardiac ventricular hypertrophy suggested the pathology and ensuing hypertension to be long-standing; however, renal function tests were normal at presentation. The importance of routine outpatient evaluation of systemic blood pressure in children, similar to adults, so as to diagnose and treat secondary hypertension and its causes early, before further systemic and neurological complications set in, is emphasized. The primary neuro-ophthalmological clinical presentation of the child with subsequent confluence of major pediatric domains of medicine, surgery, neurology, cardiology, nephrology, interventional radiology, and ophthalmology toward optimum multidisciplinary etiological and symptomatic management of the condition and its sequelae and subsequent rehabilitation is described.

Persistent hyperplastic primary vitreous in a child with incontinentia pigmenti and infantile spasms.

Incontinentia pigmenti (IP) is a rare neurocutaneous syndrome of X-linked dominant inheritance (1:40000 births in the Caucasian population) which is usually lethal in males. It commonly presents with skin, central nervous system, and dental anomalies. Ophthalmic associations of IP include intra-ocular anomalies such as leukocoria, megalocornea, corneal edema, band keratopathy, bullous keratopathy, iridocorneal attachments, macular capillary dropout, peripheral arteriovenous shunts, retinal neovascularization, vitreous hemorrhage, preretinal fibrosis, traction retinal detachment as well as strabismus. We report an 18-month-old developmentally delayed female child with diagnosed IP and infantile spasms conforming to the west syndrome triad, who presented with left eye microphthalmia and persistent hyperplastic primary vitreous and discuss this rare ophthalmic presentation.

Worldwide inequality in access to full text scientific articles: the example of ophthalmology.

BACKGROUND: The problem of access to medical information, particularly in low-income countries, has been under discussion for many years. Although a number of developments have occurred in the last decade (e.g., the open access (OA) movement and the website Sci-Hub), everyone agrees that these difficulties still persist very widely, mainly due to the fact that paywalls still limit access to approximately 75% of scholarly documents. In this study, we compare the accessibility of recent full text articles in the field of ophthalmology in 27 established institutions located worldwide. METHODS: A total of 200 references from articles were retrieved using the PubMed database. Each article was individually checked for OA. Full texts of non-OA (i.e., "paywalled articles") were examined to determine whether they were available using institutional and Hinari access in each institution studied, using "alternative ways" (i.e., PubMed Central, ResearchGate, Google Scholar, and Online Reprint Request), and using the website Sci-Hub. RESULTS: The number of full texts of "paywalled articles" available using institutional and Hinari access showed strong heterogeneity, scattered between 0% full texts to 94.8% (mean = 46.8%; SD = 31.5; median = 51.3%). We found that complementary use of "alternative ways" and Sci-Hub leads to 95.5% of full text "paywalled articles," and also divides by 14 the average extra costs needed to obtain all full texts on publishers' websites using pay-per-view. CONCLUSIONS: The scant number of available full text "paywalled articles" in most institutions studied encourages researchers in the field of ophthalmology to use Sci-Hub to search for scientific information. The scientific community and decision-makers must unite and strengthen their efforts to find solutions to improve access to scientific literature worldwide and avoid an implosion of the scientific publishing model. This study is not an endorsement for using Sci-Hub. The authors, their institutions, and publishers accept no responsibility on behalf of readers.

Cerebral venous thrombosis with auto-immune hyperthyroidism.

Sudden deterioration of visual functions warrants comprehensive ophthalmic examination with evaluation for systemic association. Cerebral venous thrombosis (CVT) is an uncommon disorder that can present with neurological deficits. We report a young female patient aged 28 years who presented with severe headache and sudden diminution of vision and was subsequently diagnosed with hyperthyroidism and CVT. Management of CVT and hyperthyroidism hastened full recovery of visual functions.

Neurodevelopmental disorders in children aged 2-9 years: Population-based burden estimates across five regions in India.

BACKGROUND: Neurodevelopmental disorders (NDDs) compromise the development and attainment of full social and economic potential at individual, family, community, and country levels. Paucity of data on NDDs slows down policy and programmatic action in most developing countries despite perceived high burden. METHODS AND FINDINGS: We assessed 3,964 children (with almost equal number of boys and girls distributed in 2-<6 and 6-9 year age categories) identified from five geographically diverse populations in India using cluster sampling technique (probability proportionate to population size). These were from the North-Central, i.e., Palwal (N = 998; all rural, 16.4% non-Hindu, 25.3% from scheduled caste/tribe [SC-ST] [these are considered underserved communities who are eligible for affirmative action]); North, i.e., Kangra (N = 997; 91.6% rural, 3.7% non-Hindu, 25.3% SC-ST); East, i.e., Dhenkanal (N = 981; 89.8% rural, 1.2% non-Hindu, 38.0% SC-ST); South, i.e., Hyderabad (N = 495; all urban, 25.7% non-Hindu, 27.3% SC-ST) and West, i.e., North Goa (N = 493; 68.0% rural, 11.4% non-Hindu, 18.5% SC-ST). All children were assessed for vision impairment (VI), epilepsy (Epi), neuromotor impairments including cerebral palsy (NMI-CP), hearing impairment (HI), speech and language disorders, autism spectrum disorders (ASDs), and intellectual disability (ID). Furthermore, 6-9-year-old children were also assessed for attention deficit hyperactivity disorder (ADHD) and learning disorders (LDs). We standardized sample characteristics as per Census of India 2011 to arrive at district level and all-sites-pooled estimates. Site-specific prevalence of any of seven NDDs in 2-<6 year olds ranged from 2.9% (95% CI 1.6-5.5) to 18.7% (95% CI 14.7-23.6), and for any of nine NDDs in the 6-9-year-old children, from 6.5% (95% CI 4.6-9.1) to 18.5% (95% CI 15.3-22.3). Two or more NDDs were present in 0.4% (95% CI 0.1-1.7) to 4.3% (95% CI 2.2-8.2) in the younger age category and 0.7% (95% CI 0.2-2.0) to 5.3% (95% CI 3.3-8.2) in the older age category. All-site-pooled estimates for NDDs were 9.2% (95% CI 7.5-11.2) and 13.6% (95% CI 11.3-16.2) in children of 2-<6 and 6-9 year age categories, respectively, without significant difference according to gender, rural/urban residence, or religion; almost one-fifth of these children had more than one NDD. The pooled estimates for prevalence increased by up to three percentage points when these were adjusted for national rates of stunting or low birth weight (LBW). HI, ID, speech and language disorders, Epi, and LDs were the common NDDs across sites. Upon risk modelling, noninstitutional delivery, history of perinatal asphyxia, neonatal illness, postnatal neurological/brain infections, stunting, LBW/prematurity, and older age category (6-9 year) were significantly associated with NDDs. The study sample was underrepresentative of stunting and LBW and had a 15.6% refusal. These factors could be contributing to underestimation of the true NDD burden in our population. CONCLUSIONS: The study identifies NDDs in children aged 2-9 years as a significant public health burden for India. HI was higher than and ASD prevalence comparable to the published global literature. Most risk factors of NDDs were modifiable and amenable to public health interventions.

Amniotic Band Syndrome: A Review of 2 Cases.

Amniotic band syndrome is a rare congenital disorder caused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while in utero that presents with complex multisystem anomalies. The authors report 2 children with amniotic band syndrome who presented to the ophthalmic unit of the authors' pediatric hospital. One of them presented with telecanthus, syndactyly, amputated toes, and unilateral epiphora diagnosed as congenital nasolacrimal duct obstruction. She was managed conservatively with lacrimal sac massage and provided with refractive correction while she simultaneously underwent multiple surgeries for correction of clubfoot and craniosynostosis. The second patient presented with cleft lip, cleft palate, multiple constriction bands in upper limbs and fingers with unilateral microphthalmos, microcornea, typical iris coloboma, and retinochoroidal coloboma, very similar to a case reported in literature. These 2 cases provide an overview of the clinical spectrum of ophthalmic manifestations along with their staged optimum rehabilitation.

Long-term Surgical Outcomes in the Sagging Eye Syndrome.

BACKGROUND/AIMS: Strabismus due to sagging eye syndrome (SES) caused by age-related connective tissue involution is now an established cause of diplopia in older people. High suspicion of the condition results in early recognition, often obviating extensive neurological investigations and enabling surgical correction of the strabismus. METHODS: This retrospective study reviewed surgical results in 93 patients (40 males) of average age 68 ± 12 years, who had small-angle strabismus due to SES, during the 20-year period 1994-2014. RESULTS: In central gaze, mean distance pre-operative esotropia was 4.2 ± 7.5Δ (mean, SD), while mean hypertropia was 4.7 ± 5.9Δ. Surgeries comprised medial rectus (MR) recession; lateral rectus (LR) resection; plication, imbrication, and superior transposition of the LR to the superior rectus (SR) for esotropia; and graded vertical rectus tenotomy (GVRT) or vertical muscle recession for hypertropia. Mean post-operative immediate and long-term deviation after 316 ± 265 months average follow-up was 0.2 ± 1.2Δ and 1.1 ± 2.7Δ esotropia (both distance), respectively, and 0.00Δ and 1.1 ± 2.7Δ, respectively, for hypertropia. Strabismus recurred in 19 cases. CONCLUSIONS: Progressive connective tissue involution in SES may occasionally result in symptomatic recurrences of the small-angle strabismus in about 20% of patients, irrespective of surgical procedures performed, possibly because of progressive involutional changes. This risk should be disclosed pre-operatively.

Pedigree Analysis of Familial Primary Concomitant Horizontal Strabismus in Northern India.

PURPOSE: Familial clustering of common forms of primary strabismus like esotropia (ET) and exotropia (XT) is observed in a proportion of the strabismus cohort. The genetic components of this remain unidentified. Linkage studies have demonstrated susceptibility locus for primary strabismus at the STBMS1 locus on 7p22.1 as well as other loci on 4q28.3 and 7q31.2. Recently next generation sequencing (NGS) technology has emerged as a powerful tool in discovery genomics and a large number of novel disease-causing variants are being reported. In this study, we recruited informative families for subsequent genetic analysis for disease-causing variant identification. METHODS: All consecutive families with two or more affected members with primary concomitant horizontal strabismus were prospectively recruited at the ophthalmic outpatients department (OPD) of Lady Hardinge Medical College, New Delhi, from August 2014 to February 2017. Detailed phenotypic evaluation and pedigree documentation was performed. RESULTS: Of the 39 recruited families of north Indian origin, 18 families each had affected family members demonstrating either ET or XT. 100% concordance of the phenotype in the affected family members was observed in these families. While vertical transmission was observed in 17/18 families with XT, 7 with ET had affected members across one generation, 2 demonstrated consanguineous pedigree, and 2 comprised identical twin families. In 3 families, a combination of ET and XT was noted. This comprised one family with the ET and XT patients being from 2 separate arms of the family related by marriage, one family where one sibling had XT and the other had ET, and another family where the maternal aunt of the affected proband with ET had XT. CONCLUSIONS: Subjects with familial primary concomitant strabismus recruited in this study may provide a valuable resource to unravel the genetic determinants of this condition, which is a common disorder of early childhood with high ophthalmic morbidity.

Graded vertical rectus tenotomy for small-angle cyclovertical strabismus in sagging eye syndrome.

BACKGROUND/AIMS: Graded vertical rectus tenotomy (GVRT) is postulated as effective for small-angle vertical heterotropia. We aimed to determine the dosing recommendations for GVRT in sagging eye syndrome (SES). METHODS: This was a retrospective, observational study of surgical outcomes for GVRT from 2009 to 2014 in a single surgeon's academic practice. There were 37 (20 women) patients of average age 68±10 (SD) years with comitant or incomitant hypertropia ≤10Δ caused by SES. The main outcome measure was the dose-effect of GVRT required to correct intraoperative hypertropia. RESULTS: Preoperative average central gaze hypertropia measured 4.7±2.2Δ. Three patients underwent repeat GVRT for residual or consecutive hypertropia, one undergoing it twice. All surgeries were analysed, increasing the total operations to 41. The inferior rectus tendon in the hypotropic eye was operated in 32 eyes, and the superior rectus tendon in the hypertropic eye in 9 eyes. Mean tenotomy was 68±19% of tendon width. Hypertropia was always eliminated intraoperatively by progressive GVRT. Mean hypertropia was 1.1±1.6Δ at average 93 days postoperatively. Linear regression demonstrated that 3-6Δ hypertropia correction requires 30%-90% graded tenotomy (R(2)=0.32, p<0.0001), but with substantial individual variability. Undercorrection necessitated reoperation in 10% of cases. CONCLUSION: GVRT precisely corrects hypertropia of up to 10Δ, but because of variable effect, it should be performed with intraoperative monitoring under topical anaesthesia.

Magnetic resonance imaging of bilateral split lateral rectus transposition to the medial globe.

PURPOSE: Medial transposition of the split or intact lateral rectus (LR) muscle in oculomotor palsy improves extreme exotropia. We studied rectus pulley positions using high-resolution surface coil MRI before and after LR split with medial transposition surgery in a patient with bilateral oculomotor and trochlear nerve palsies. METHODS: This is a report of a 14-year-old girl with 90Δ exotropia due to bilateral oculomotor and trochlear nerve palsies following traumatic midbrain infarction at age 6 years. Surgery comprised longitudinal division of each LR into a superior and inferior, threading of both halves between the inferior rectus (IR) and inferior oblique inferiorly, and inferior to the superior oblique and the superior rectus superiorly, with suturing of each 10 mm posterior to the medial rectus (MR) insertion. RESULTS: Pre-operative MRI of the orbit and extraocular muscles (EOMs) with thin, 2-mm slices revealed bilateral atrophy of all EOMs supplied by the oculomotor and trochlear nerves. Post-operative MRI at 2 months demonstrated no significant changes in rectus EOM pulley positions compared with pre-operative values. CONCLUSIONS: The LR pulley does not change position even after split LR transposition to the MR insertion, confirming the profound constraint of the connective tissue pulley system on the LR path.

Surgical outcomes following rectus muscle plication: a potentially reversible, vessel-sparing alternative to resection.

IMPORTANCE: Extraocular muscle strengthening is a common treatment for strabismus. Plication is an alternative procedure for strengthening muscles with less tissue trauma than resection. OBJECTIVE: To compare the surgical dose effect of plication with that of resection. DESIGN, SETTING, AND PARTICIPANTS: Retrospective comparison of surgical outcomes in an academic pediatric ophthalmology and strabismus practice from July 25, 2005, through March 28, 2013, with a mean follow-up of 137 days for plication and 1243 days for resection. A single surgeon performed all procedures. Twenty-two consecutive patients underwent bilateral horizontal rectus plication or plication combined with antagonist recession (13 with esotropia and 9 with exotropia; mean [SD] age, 38 [21] years). Thirty-one consecutive patients underwent bilateral resection or resection combined with antagonist recession (12 with esotropia and 19 with exotropia; mean [SD] age, 28 [24] years). Six patients underwent vertical rectus plication. EXPOSURES: Rectus resection or plication performed by folding the anterior tendon posteriorly under the muscle margins oversewn to the poles of the scleral insertion, avoiding the anterior ciliary arteries. MAIN OUTCOMES AND MEASURES: Postoperative binocular alignment at the first postoperative and last available examinations. RESULTS: Lateral rectus plication (mean [SD], 6.5 [2.2] mm) and resection (6.6 [1.6] mm) were performed for similar magnitudes of esotropia (27.9 [13.4] prism diopters [PD] for plication, 29.0 [15.2] PD for resection; P = .44). Mean (SD) initial correction by lateral rectus plication was 5.17 (0.29) PD/mm, slightly less than the 95% CI (5.51-7.75 PD/mm) for resection (6.63 [0.50] PD/mm). Medial rectus plication (mean [SD], 4.9 [0.9] mm) vs resection (5.1 [1.1] mm) was performed for similar magnitudes of exotropia (32.8 [14.2] PD for plication, 31.2 [15.6] PD for resection; P = .33). Mean (SD) initial correction by medial rectus plication (7.10 [1.65] PD/mm) was within the 95% CI (4.65-9.87 PD/mm) for resection (7.26 [1.23] PD/mm). Initial and late effects were similar. Ciliary circulation observed at surgery remained patent after plication. Plication was cosmetically acceptable and did not produce conspicuous tissue elevations. CONCLUSIONS AND RELEVANCE: Horizontal rectus muscle plication is a rapidly performed, technically simple surgical procedure to strengthen the rectus muscles, with an equivalent (per millimeter) in surgical effect to that of medial rectus resection for treatment of esotropia and exotropia. Plication diminishes surgical trauma and does not intentionally sacrifice ciliary circulation, with the potential for reversal by suture release in the first postoperative days.

Sagging eye syndrome: connective tissue involution as a cause of horizontal and vertical strabismus in older patients.

IMPORTANCE: Recognition of sagging eye syndrome (SES) as the cause of chronic or acute acquired diplopia may avert neurologic evaluation and imaging in most cases. OBJECTIVES: To determine whether SES results from inferior shift of lateral rectus (LR) extraocular muscle (EOM) pulleys and to investigate anatomic correlates of strabismus in SES. DESIGN AND SETTING: We used magnetic resonance imaging to evaluate rectus EOMs, pulleys, and the LR-superior rectus (SR) band ligament at an eye institute. PARTICIPANTS: Patients with acquired diplopia suspected of having SES. We studied 56 orbits of 11 men and 17 women (mean [SD] age of 69.4 [11.9] years) clinically diagnosed with SES. Data were obtained from 25 orbits of 14 control participants age-matched to SES and from 52 orbits of 28 younger controls (23 [4.6] years). MAIN OUTCOME MEASURES: Rectus pulley locations compared with age-matched norms and lengths of the LR-SR band ligament and rectus EOMs. Data were correlated with facial features, binocular alignment, and fundus torsion. RESULTS: Patients with SES commonly exhibited blepharoptosis and superior sulcus defect. Significant inferolateral LR pulley displacement was confirmed in SES, but the spectrum of abnormalities was extended to peripheral displacement of all other rectus pulleys and lateral displacement of the inferior rectus pulley, with elongation of rectus EOMs (P < .001). Symmetrical LR sag was associated with divergence paralysis esotropia and asymmetrical LR sag greater than 1 mm with cyclovertical strabismus. The LR-SR band was ruptured in 91% of patients with SES. CONCLUSIONS AND RELEVANCE: Widespread rectus pulley displacement and EOM elongation, associated with LR-SR band rupture, causes acquired vertical and horizontal strabismus. Small-angle esotropia or hypertropia may result from common involutional changes in EOMs and orbital connective tissues that may be suspected from features evident on external examination.

Functional magnetic resonance imaging of horizontal rectus muscles in esotropia.

PURPOSE: Monkey neurophysiology suggests that changes in neural drive rather than extraocular muscle structure underlie sensory-induced strabismus. If this is true, then extraocular muscle structure should be normal. We used magnetic resonance imaging to measure horizontal rectus muscle size and contractility to determine whether muscle structure is a factor in human concomitant esotropia. METHODS: High-resolution, quasicoronal plane magnetic resonance imaging was performed in target-controlled central gaze, abduction, and adduction in 13 orthotropic controls (mean age, 38 ± 19 years) and 12 adults (mean age, 52 ± 16 years) who had concomitant esotropia averaging 28(Δ) ± 18(Δ) at distance. Thyroid ophthalmopathy was excluded. Horizontal rectus muscle cross sections were determined in 6 contiguous, 2-mm-thick midorbital image planes. Contractility was computed in each plane as the difference in cross section from contraction to relaxation. RESULTS: Medial rectus muscle cross sections in multiple planes averaged up to 39% larger in esotropic patients than in controls (P < 0.005), whereas lateral rectus muscle cross sections in esotropia were up to 28% larger but only significantly larger in one plane (P < 0.02). Medial rectus contractility was increased by up to 60% in esotropic patients (P < 0.005), whereas lateral rectus contractility in esotropia was slightly but not significantly supernormal. CONCLUSIONS: Medial rectus muscle size is supernormal and lateral rectus muscle size is not subnormal in concomitant esotropia. This finding indicates that human concomitant esotropia is associated with peripheral muscular abnormality.

Independent passive mechanical behavior of bovine extraocular muscle compartments.

PURPOSE: Intramuscular innervation of horizontal rectus extraocular muscles (EOMs) is segregated into superior and inferior (transverse) compartments, while all EOMs are also divided into global (GL) and orbital (OL) layers with scleral and pulley insertions, respectively. We sought evidence of potential independent action by examining passive mechanical coupling between EOM compartments. METHODS: Putative compartments of each of the six whole bovine anatomical EOMs were separately clamped to a physiologically controlled, dual channel microtensile load cell (5-mN force resolution) driven by independent, high-speed, linear motors having 20-nm position resolution. One channel at a time was extended or retracted by 3 to 5 mm, with the other channel stationary. Fiducials distributed on the EOM global surface enabled optical tracking of local deformation. Loading rates of 5 to 100 mm/sec were applied to explore speeds from slow vergence to saccades. Control loadings employed transversely loaded EOM and isotropic latex. RESULTS: All eom bellies and tendons exhibited substantial compartmental independence when loaded in the physiologic direction, both between OL and GL, and for arbitrary transverse parsings of EOM width ranging from 60%: 40% to 80%:20%. Intercompartmental force coupling in the physiologic direction was less than or equal to 10% in all six EOMS even for saccadic loading rates. Coupling was much higher for nonphysiologic transverse EOM loading and isotropic latex. Optical tracking demonstrated independent strain distribution between EOM compartments. CONCLUSIONS: Substantial mechanical independence exists among physiologically loaded fiber bundles in bovine EOMs and tendons, providing biomechanical support for the proposal that differential compartmental function in horizontal rectus EOMs contributes to novel torsional and vertical actions.

Medial rectus recession is as effective as lateral rectus resection in divergence paralysis esotropia.

OBJECTIVE: To propose medial rectus (MR) recession to be equally as effective as lateral rectus (LR) resection, which has heretofore been the preferred treatment for divergence paralysis esotropia (DPE). METHODS: We examined a 17-year surgical experience comparing LR resection with MR recession in adults with DPE, defined as symptomatic distance esotropia (ET) at least double the asymptomatic ET of 10 or less prism diopters() at near. RESULTS: Twenty-four patients with DPE underwent surgery. Six patients underwent bilateral LR resection and 2 underwent unilateral LR resection (group L), while 13 underwent bilateral MR recession and 3 underwent unilateral MR recession,with the target angle double the distance ET (group M). One of 8 patients in group L and 15 of 16 patients in group M underwent intra operative adjustable surgery under topical anesthesia. Mean (SD) preoperative central gaze ET measured 15.0 (7.7) Δ at distance and 4.1 (3.4) Δ at near in group L, but 10.4 (6.8) Δ at distance and 0.6 (1.7) Δ at near in group M (P=.15; distance, 0.003, near). Postoperatively, no patient in either group had symptomatic diplopia or convergence insufficiency in follow-up from 8.5 to 40 months. Twice the usual surgical dose of MR recession per prism diopter was required to achieve correction of the distance deviation in DPE as compared with that recommended for ET generally and also for LR resection in the same condition. CONCLUSIONS: Recession of the MR provides binocular single vision in DPE without convergence insufficiency at near, and it is convenient for intraoperative adjustment under topical anesthesia.

Bilateral retrobulbar neuritis in an adolescent boy with lymphoma.

A 13-year-old boy presented with bilateral retrobulbar neuritis. Three months later he presented with unilateral proptosis and cervical lymphadenopathy. Lymph node biopsy confirmed non-Hodgkin's lymphoma, B-cell type. To the best of our knowledge, this is the first reported case of bilateral retrobulbar neuritis as the presumptive initial presentation of non-Hodgkin's lymphoma.