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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Ectopic lacrimal glandular tissue in a neonate with giant congenital melanocytic nevi and cutis verticis gyrata.

This report describes an unusual case of a neonate with giant congenital melanocytic nevi covering more than 80% of the body surface area, with cutis verticis gyrata and abnormal lacrimal glandular tissue rests in the epibulbar area. The patient's mother had pigmented lesions in the periorbital region and sclera.

Optical coherence tomography of traumatic maculopathy following blunt trauma with sugarcane.

An unusual case of traumatic maculopathy following blunt trauma with a sugarcane stick is reported in a 12-year-old boy. An area of parafoveal exudates associated with decrease in visual acuity was observed in the affected eye of the patient. Baseline parameters related to the best-corrected visual acuity, visual fields, fundus photographs, fluorescein angiography, and macular optical coherence tomography were performed and the patient was observed for 6 months with conservative management.

Congenital eyelid colobomas in 51 patients.

Purpose: To review the clinical observations in the management of congenital eyelid coloboma.

Methods: Fifty-one cases of congenital eyelid colobomas including the functional and cosmetic rehabilitation of patients who were treated over a period of 18 years with an average follow-up of 4.7 years were retrospectively reviewed.

Pediatric keratoplasty.

Penetrating keratoplasty in children is a highly challenging and demanding procedure associated with a high risk of graft failure or failure of amblyopia therapy in clear grafts. Nonetheless, keratoplasty remains the surgery of choice for the management of pediatric corneal stromal opacities or edema. Allograft rejection, graft infection, corneal neovascularization, glaucoma, trauma to the anterior segment, vitreous pathology, and additional surgical interventions, especially those related to glaucoma management, are important risk factors.

Abnormal ocular electrophysiology in Sjögren-Larsson syndrome.

Two siblings who were born out of consanguineous marriage in an Indian family and had typical clinical manifestations of Sjögren-Larsson syndrome were found to have normal electroretinogram and abnormal visual evoked potential results. Abnormal visual evoked potential results along with the presence of distinctive foveal and parafoveal deposits, which could have a prognostic bearing on the visual acuity of the patients, emphasize the importance of a complete ophthalmological and electrophysiological evaluation in all patients with Sjögren-Larsson syndrome.