Large Muscle Cyst Following Use of Non-absorbable Suture for Strabismus Surgery.

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.].

Pituitary Dysfunction in Pediatric Patients with Optic Nerve Hypoplasia: A Retrospective Cohort Study (1975-2014).

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.

Planning Strabismus Surgery: How to Avoid Pitfalls and Complications.

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.

Eye Findings on Vigabatrin and Taurine Treatment in Two Patients with Succinic Semialdehyde Dehydrogenase Deficiency.

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.

Treatment of refractory uveitic macular edema with dexamethasone intravitreal implants in a pediatric patient with bilateral granulomatous idiopathic panuveitis: a case report.

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.

Ophthalmic complications of otitis media in children.

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.

Acquired myelinated nerve fibers in association with optic disk drusen.

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.

Diffuse corneoscleral limbal neuromas with prominent corneal nerves in multiple endocrine neoplasia syndrome type IIB.

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.

Ocular neuromyotonia in a 15-year-old girl after radiation therapy.

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.

Possible venous air embolism during open eye surgery in a child.

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.

Morning glory syndrome: clinical, computerized tomographic, and ultrasonographic findings.

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.

Retinoblastoma, microphthalmia and the chromosome 13q deletion syndrome.

A 10-month-old boy, known to have the 13q deletion syndrome, presented with leukocoria in a microphthalmic right eye. He was found to have bilateral retinoblastoma requiring enucleation of the right eye and laser, cryotherapy and external beam radiation to the left eye. This case demonstrates that retinoblastoma may be present in a microphthalmic eye and that more than one imaging modality should be used in the evaluation of suspected retinoblastoma, as calcification may be absent on the computed tomography scan but present on B-mode ultrasound. Children with a known 13q deletion require immediate and serial ophthalmological examinations.