Coats' Disease in a Patient With Cornelia de Lange Syndrome: Management With Laser and Bevacizumab.

Cornelia de Lange syndrome is a congenital disorder with multisystem abnormalities including multiple ocular findings. The authors report a case of Coats' disease in a patient with Cornelia de Lange syndrome who was successfully treated with laser and intravitreal bevacizumab. This case demonstrates the importance of fluorescein angiography in making the diagnosis and directing treatment and the efficacy of combined laser with intravitreal anti-vascular endothelial growth factor therapy for persistent vascular leakage associated with Coats' disease in Cornelia de Lange syndrome.

Use of sweep visual evoked potential in preverbal children with optic nerve hypoplasia.

Purpose: To evaluate sweep VEP (sVEP) in preverbal children with optic nerve hypoplasia (ONH) and to assess associations between sVEP results, patient clinical characteristics and future recognition visual acuity.

Methods: The medical records of children with ONH who had sVEP testing and documented recognition visual acuity at the University of Wisconsin from 2005 to 2013 were reviewed retrospectively. Optic nerve size, amblyopia treatment, and neurologic diagnoses were collected.

Opioid prescribing to preteen children undergoing ambulatory surgery in the United States.

Background: Overuse and misuse of opioids is a continuing crisis. The most common reason for children to receive opioids is postoperative pain, and they are often prescribed more than needed. The amount of opioids prescribed varies widely, even for minor ambulatory procedures.

SVEP1 as a Genetic Modifier of TEK-Related Primary Congenital Glaucoma.

Purpose: Affecting children by age 3, primary congenital glaucoma (PCG) can cause debilitating vision loss by the developmental impairment of aqueous drainage resulting in high intraocular pressure (IOP), globe enlargement, and optic neuropathy. TEK haploinsufficiency accounts for 5% of PCG in diverse populations, with low penetrance explained by variable dysgenesis of Schlemm's canal (SC) in mice. We report eight families with TEK-related PCG, and provide evidence for SVEP1 as a disease modifier in family 8 with a higher penetrance and severity.

Angiopoietin-1 is required for Schlemm's canal development in mice and humans.

Primary congenital glaucoma (PCG) is a leading cause of blindness in children worldwide and is caused by developmental defects in 2 aqueous humor outflow structures, Schlemm's canal (SC) and the trabecular meshwork. We previously identified loss-of-function mutations in the angiopoietin (ANGPT) receptor TEK in families with PCG and showed that ANGPT/TEK signaling is essential for SC development. Here, we describe roles for the major ANGPT ligands in the development of the aqueous outflow pathway.

Cervical lymphadenopathy secondary to rhabdomyosarcoma presenting as Horner syndrome in an infant.

A 4-week-old boy with left ptosis, anisocoria, and a mass on his left hand was diagnosed with Horner syndrome. The diagnosis precipitated a work-up for a possible malignant etiology. Magnetic resonance imaging demonstrated enlarged left cervical and axillary lymph nodes.

Intraobserver reliability of contact pachymetry in children.

Background: Central corneal thickness (CCT) is an important measurement in the treatment and management of pediatric glaucoma and potentially of refractive error, but data regarding reliability of CCT measurement in children are limited. The purpose of this study was to evaluate the reliability of CCT measurement with the use of handheld contact pachymetry in children.

Methods: We conducted a multicenter intraobserver test-retest reliability study of more than 3,400 healthy eyes in children aged from newborn to 17 years by using a handheld contact pachymeter (Pachmate DGH55; DGH Technology Inc, Exton, PA) in 2 clinical settings--with the use of topical anesthesia in the office and with the patient under general anesthesia in a surgical facility.

Identification and treatment of amblyopia.

Amblyopia is the leading cause of vision loss in children. It is treatable if diagnosed early, making identification of affected children critical. The American Association for Pediatric Ophthalmology and Strabismus and the American Academy of Pediatrics recommend that clinicians routinely perform age-appropriate vision chart testing, red reflex testing, and examination for signs of strabismus.

Outcomes of Harada-Ito surgery for acquired torsional diplopia.

Purpose: To evaluate the outcomes of Harada-Ito surgery in correcting various types of torsional diplopia.

Methods: The medical records of patients who underwent Harada-Ito surgery at two academic institutions were retrospectively reviewed. Data collected included etiology of torsional diplopia, strabismus and torsion measurements, reoperation rate, patient symptoms, and use of prism.

Comparison of Tono-Pen and Goldmann applanation tonometers for measurement of intraocular pressure in healthy children.

Purpose: To assess the agreement of intraocular pressure (IOP) measured with the Tono-Pen and the Goldmann applanation tonometer (GAT) in normal children and adolescents.

Methods: A total of 439 subjects from birth to <18 years of age without anterior segment anomalies or glaucoma had their IOP measured with the two instruments by separate, masked examiners in the office or under general anesthesia.

Results: On average, the Tono-Pen measured values slightly lower than the GAT for IOP <11 mm Hg and slightly higher than the GAT for IOP >11 mm Hg in the office setting.

Central corneal thickness in children.

Objectives: To determine the central corneal thickness (CCT) in healthy white, African American, and Hispanic children from birth to 17 years of age and to determine whether CCT varies by age, race, or ethnicity.

Design: Prospective observational multicenter study. Central corneal thickness was measured with a handheld contact pachymeter.

Chorioretinopathy and microcephaly with normal development.

Purpose: To report a pediatric patient with bilateral chorioretinopathy and microcephaly who from birth to 2 years of age is reaching appropriate developmental milestones.

Design: Retrospective case report with clinical findings and literature review.

Main Outcome Measures: Clinical findings and visual acuity estimated by sweep visual evoked potentials (VEP), electroretinogram (ERG) and fundoscopic exam.

Sweep visual evoked potential testing as a predictor of recognition acuity in albinism.

Objective: To determine if sweep visual evoked potential (VEP) acuity is predictive of recognition acuity in children with albinism.

Methods: A retrospective review was performed in children with albinism who underwent sweep VEP testing from 1992 to 2003. All patients had a complete ophthalmologic examination with either binocular or monocular sweep VEP testing and at least 5 years of follow-up.

Pseudotumor cerebri in children with Down syndrome.

Purpose: To report 4 pediatric Down syndrome patients with optic nerve elevation and pseudotumor cerebri.

Design: Retrospective observational case series.

Participants: Four pediatric Down syndrome patients found to have optic nerve elevation on ophthalmologic examination at a single institution.

Factors associated with recurrence of amblyopia on cessation of patching.

Purpose: In a prospective observational study, we previously reported that weaning (tapering or gradually reducing) treatment in children treated with 6 to 8 hours of daily patching for amblyopia resulted in a 4-fold reduction in odds of recurrence. We now report the association of additional factors with recurrence or regression of amblyopia in this same cohort.

Design: Prospective, nonrandomized, observational study.

Successful treatment of anisometropic amblyopia with spectacles alone.

Background: The efficacy of treating anisometropic amblyopia with occlusion therapy is well known. However, this form of treatment can be associated with risks. Spectacle correction alone may be a successful and underutilized form of treatment.

Ocular complications after organ and bone marrow transplantation in children.

Background: Organ and bone marrow transplantation commonly are performed in children. Ocular complications usually are described as secondary to post-transplantation medications. The complication rate is unknown.

Astigmatism after small-incision clear corneal cataract extraction and intraocular lens implantation in children.

Purpose: To investigate the magnitude of postoperative astigmatism in children having cataract extraction with intraocular lens (IOL) implantation through a 3.0 mm superior clear corneal incision.

Setting: Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, Indiana, USA.