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Giant orbital cysts are a rare complication of strabismus surgery or orbital foreign body. A patient with a history of multiple surgeries presented with an orbital cyst that developed in the medial rectus muscle. Surgical exploration found a non-absorbable suture inside the cyst. The conjunctiva was closed using amniotic membrane. [J Pediatr Ophthalmol Strabismus. 2020;57:e74-e77.]., (Copyright 2020, SLACK Incorporated.)

Background/aims: The risk factors for pituitary hormone dysfunction (PHD) in children with optic nerve hypoplasia (ONH) are not well understood. This study identified the type, timing, and predictors of PHD in children with ONH., Methods: ONH patient charts were reviewed retrospectively. The incidence rate of PHD was calculated assuming a Poisson distribution. Predictors of PHD were identified through a multivariable Cox proportional hazards model., Results: Among 144 subjects with ONH, 49.3% (n = 71) developed PHD over 614.7 person-years of follow-up. The incidence was 11.55 (95% confidence interval [CI]: 9.02-14.57/100 person-years). The median time to first PHD was 2.88 (interquartile range: 0.02-18.72) months. Eighty-two percent developed their first PHD by their 5th and 90% by their 10th birthday, and 89% within 5 years of ONH diagnosis. Prematurity (adjusted hazard ratio [aHR]: 0.33; 95% CI: 0.1-1.07), blindness (aHR: 1.72; 95% CI: 1.03-2.86), maternal substance abuse (aHR: 1.51; 95% CI: 0.91-2.48), abnormal posterior pituitary (aHR: 3.8; 95% CI: 2.01-7.18), and hypoplastic/absent anterior pituitary (aHR: 2.52; 95% CI: 1.29-4.91) were significant predictors of PHD., Conclusions: The clinical predictors of PHD included blindness, pituitary gland abnormalities, and maternal substance abuse. These predictors help clinical decision-making related to the need for and frequency of hormone testing in pediatric patients with ONH., (© 2017 S. Karger AG, Basel.)

We describe for the first time two patients with succinic semialdehyde dehydrogenase (SSADH) deficiency, who were found to have abnormal electroretinogram (ERG) examinations at baseline, or 6 months after vigabatrin treatment was started. This was somewhat reversible with L-taurine treatment, or minimally progressive. The mechanism of injury to the retina may be induced by elevations of γ-aminobutyric acid causing peripheral photoreceptor and ganglion cell damage, and this can be exacerbated by the use of vigabatrin. The use of taurine supplementation in tandem with vigabatrin may allow reversal of retinopathy and mitigate or slow down further deterioration. Further prospective clinical trials are required to evaluate this further. We recommend starting L-taurine therapy together with vigabatrin if a trial of vigabatrin is commenced in a patient with SSADH deficiency. Close monitoring of visual fields or ERG is also recommended at baseline and during vigabatrin therapy., (Georg Thieme Verlag KG Stuttgart · New York.)

Background and Purpose: Good surgical results following strabismus surgery depend on several factors. In this article, detailed steps for planning strabismus surgery will be reviewed for basic horizontal strabismus surgery, vertical, and oblique muscle surgeries. The thought process behind each case will be presented to help in selecting the best surgical approach to optimize postoperative results., Patients and Method: The surgical planning for strabismus will be developed with clinical examples from easy cases to more complex ones. Preoperative pictures of the ocular alignment are an integral part of planning surgery and help in documenting the strabismus before and after surgery., Results: Three cases of strabismus cases will be reviewed with several key factors for planning surgery, including visual acuity, refractive error, potential for stereovision, and risk of postoperative diplopia. The most important factor is accurate orthoptic measurements. The surgical planning for each patient is detailed along with preoperative pictures., Conclusion: Strabismus surgery results can be improved by careful preoperative planning. The surgeon has the ability to discern potential pitfalls that can alter the surgical outcome. Surgical planning allows a dedicated time of reflection before surgery, foreseeing potential problems, and avoiding them during the surgery., (© 2016 Board of regents of the University of Wisconsin System, American Orthoptic Journal, Volume 66, 2016, ISSN 0065-955X, E-ISSN 1553-4448.)

Background: Macular edema is a common complication of uveitis and represents a therapeutic challenge, especially in children. Recently, intravitreal dexamethasone implants have been shown to decrease intraocular inflammation and to control uveitic macular edema in patients with non-infectious intermediate or posterior uveitis., Findings: An 11-year-old boy with bilateral granulomatous idiopathic panuveitis and orbital inflammation experienced macular edema refractory to topical steroids and subcutaneous methotrexate. He was treated with off-label bilateral injections of dexamethasone intravitreal implant. Three months later, his vision had improved from 20/200 in both eyes to 20/30 in the right eye and 20/40 in the left eye. Optical coherence tomography showed complete resolution of the cystoid macular edema and subretinal fluid in both eyes., Conclusions: This is a rare report of the use of bilateral dexamethasone intravitreal implant in a pediatric patient. The implants achieved complete resolution of the uveitic macular edema with no adverse events 3 months post-implantation.

Purpose: To evaluate the outcome of ophthalmic complications in children with otitis media., Methods: The records of children with ophthalmic complications arising from otitis media who presented to the British Columbia Children's Hospital between August 2006 and March 2008 were reviewed retrospectively., Results: Of 1,400 patients presenting to the emergency department for otitis media during the study period, 7 with ophthalmic complications were identified (age range, 1-11 years). All patients had abducens nerve palsy on presentation. Other notable ophthalmic complications included papilledema, Horner syndrome, and proptosis. Extracranial and intracranial complications included mastoiditis, petrositis, parapharyngeal abscess, hydrocephalus, epidural abscess, and cerebral venous thrombosis, including cavernous sinus thrombosis in 2. Of the 7 patients, 6 were treated with surgery, including myringotomy and tube placement (6 patients) and mastoidectomy (3 patients). All patients were initially anticoagulated and received intravenous antibiotics. Satisfactory final visual outcomes and stereopsis ranging from 40 to 100 seconds were achieved in all patients., Conclusions: Ophthalmic complications of otitis media in children are likely to include abducens palsy. All patients in our series required anticoagulation and intravenous antibiotics. Most required otolaryngologic surgery, but none required strabismus surgery, and all patients had satisfactory visual and ocular motility outcomes., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Myelinated retinal nerve fibers are a well-recognized anomaly of the ocular fundus associated with many ocular and systemic conditions. Myelination is almost always congenital and stable, but progression has been documented in rare cases. Optic disk drusen are the result of a degenerative process at the optic nerve head and are often found incidentally on ophthalmologic examination. To our knowledge, optic disk drusen have only been reported once in association with acquired and progressive myelinated retinal nerve fibers. We present 2 such cases and consider the implications for the pathogenesis of myelinated nerve fibers., (Copyright © 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

This report describes a 7-year-old boy with multiple endocrine neoplasia type 2B who presented to the ophthalmology department because of peripheral corneal changes. In addition to prominent corneal nerves and eyelid neuromas, the patient was found to have substantial corneoscleral limbal thickening with corneal neovascularization.

A 15-year-old girl, previously treated with radiation, chemotherapy, and surgery for a posterior fossa medulloblastoma and parasellar metastasis at age 8, presented with a 10-month history of episodic horizontal diplopia. She was diagnosed with ocular neuromyotonia and successfully treated with oral carbamazepine. Given the strong association between peripheral neuromyotonia and the presence of autoimmune antivoltage-gated potassium channels, the patient's blood was tested and found negative for these autoantibodies. This is the first time this has been verified in a person with ocular neuromyotonia.

Purpose: Although venous air embolism is a well recognized complication within most surgical subspecialties, it has only been reported recently during eye surgery in one adult, and never before in a child. We describe the case of a 17-month-old boy who sustained a possible air embolism during repair of an open trauma of his right eye., Clinical Features: A vitrectomy through the pars plana with air-fluid exchange of the posterior chamber was performed under general anesthesia to remove a foreign body which had impaled the optic nerve. A few minutes after removal of the foreign body, a notable decrease in end-tidal CO(2), oxygen saturation and arterial blood pressure occurred, requiring administration of a high inspired oxygen fraction and vasopressors. Venous air embolism was strongly suspected after other causes of a decrease of end-tidal CO(2) and hypoxemia had been excluded. An arterial blood gas confirmed hypoxemia and respiratory acidosis., Conclusion: Although venous air embolism is a rare complication of eye surgery, it should nevertheless be considered as a possible complication during air-fluid exchange vitrectomy in children.

Objective: To evaluate the ophthalmic, radiologic, and ultrasonographic findings in morning glory syndrome., Patients and Methods: Retrospective review of patients' charts., Results: Twenty-one patients were included. Visual acuity was 20/200 or better in 50% of the eyes. Three eyes developed a retinal detachment. When computerized tomography of the orbit demonstrated peripapillary scleral staphyloma and intraocular calcifications, this correlated with poor visual acuity. Computerized tomography of the brain revealed abnormalities in three asymptomatic patients. B-scan ultrasonography demonstrated retinal tissue overhanging the peripapillary scleral staphyloma., Conclusions: Morning glory syndrome has a spectrum of severity, with most patients retaining useful vision. Orbital and cerebral computerized tomography scan as well as B-scan ultrasonography may help with diagnosis and management.

A 2-year-old boy meeting the criteria for autistic disorder was diagnosed 2 years later with a visual agnosia characterised by a combination of certain aspects of associative and apperceptive agnosia. MRI then revealed a severe encephalomalacia of the right temporal lobe and bilateral temporo-occipital areas. This association is discussed in terms of a clinical and aetiological relation between autistic disorder and visual agnosia.

Objective: To determine the ability of the Hardy-Rand-Rittler (HRR) and Ishihara colour plates to detect acquired colour vision defects in patients with nonglaucomatous optic neuropathy (NGON)., Design: Prospective study., Setting: Neuro-Ophthalmology Unit of the Wilmer Eye Institute, Baltimore., Patients: A total of 178 consecutive patients (349 eyes) referred to the Neuro-Ophthalmology Unit and the General Eye Service of the Wilmer Eye Institute and examined by two of the authors were enrolled from July 1992 to June 1993., Outcome Measures: Results of testing with HRR and Ishihara plates., Results: Among the 202 eyes that were found to have no ocular disease on neuro-ophthalmologic testing, the HRR plates gave a normal result in 168 (83.2%), compared with 196 (97.0%) with the Ishihara plates (p < 0.0001). The HRR plates detected an acquired colour vision deficit in 48 (87.3%) of the 55 eyes with NGON, compared with 38 (69.1%) for Ishihara plates (p = 0.001). The values for the eyes with NGON with a visual acuity of 20/25 or better were 76.5% (13/17) and 35.3% (6/17) respectively (p = 0.008) and with a visual acuity less than 20/25, 92.1% (35/38) and 84.2% (32/38) respectively., Conclusions: For patients with unilateral or bilateral NGON, HRR plates are more likely than Ishihara plates to detect a colour vision defect, particularly when the visual acuity is 20/25 or better. However, neither test is sensitive enough to be used as the sole criterion for the diagnosis of NGON. The results of comparison of colour perception of the two eyes may be more useful than absolute colour vision responses, particularly in patients with unilateral disease.

This study evaluated the ophthalmological outcome following sixth nerve palsy or paresis in 64 children 7 years of age and younger. The outcomes considered were vision, residual strabismus and the need for strabismus surgery. Etiologies included tumor, hydrocephalus, trauma, infection, malformation, and idiopathic and miscellaneous causes. Strabismus surgery was performed on 24% of the patients, with residual strabismus present in 66% of the patients. Neurologists and ophthalmologists should monitor visual acuity in these young children at frequent intervals be prepared to institute amblyopia therapy early in the course of the ocular misalignment if permanent visual disability is to be avoided.

A 16-year-old girl developed headaches and bilateral papilledema while taking minocycline for acne. The initial neuro-ophthalmologic evaluation was normal except for enlarged blind spots OU. An MRI scan demonstrated subtle abnormalities. A lumbar puncture was entirely normal except for an increased opening pressure. A tentative diagnosis of pseudotumor cerebri was made and the patient was treated with Diamox. A second MRI was unchanged, and a lumbar puncture performed while the patient was taking Diamox was entirely normal. The patient subsequently lost vision in both eyes, and a third MRI now revealed a supracellar enhancing mass. Biopsy and subtotal resection of the mass showed it to be a glioblastoma multiforme. This case emphasizes pitfalls in the diagnosis of pseudotumor cerebri. Careful follow-up and a high index of suspicion in pseudotumor cerebri syndromes are essential.

A patient who presented with bilateral loss of all pupillary reactions and normal ocular motor function is reported. Investigation revealed the presence of massive hydrocephalus. The syndrome developed shortly after transsphenoidal surgery for a suprasellar craniopharyngioma. Pupillary function returned to normal following the insertion of a ventriculo-peritoneal shunt. It is suggested that the syndrome was due to compression of the visceral oculomotor nuclei by a dilated sylvian aqueduct.

The authors review the pathophysiology of gastroesophageal reflux, pointing out the predominant role of the lower esophageal sphincter in antireflux mechanisms. The diagnosis of gastroesophageal reflux must be based on the results of detailed examination and other tests of which endoscopy and esophageal pH monitoring are the most important. The authors describe how to manage operation for gastroesophageal reflux so as to prevent complications. Although the Hill, Belsey and Nissen operations, referred to as valvuloplasties, have proved their effectiveness in curing gastroesophageal reflux, a review of the literature shows that better results are achieved by Nissen total fundoplication.