Bilateral severe microphthalmia in a neonate with trisomy 8 mosaicism: A new finding.

Mosaic Trisomy 8 is a rare chromosomal abnormality estimated to occur one in 30,000 newborns. The phenotype is highly variable and the severity does not appear to be correlated with the proportion of cells that contain the additional chromosome. Ocular involvement in Trisomy 8 mosaicism has previously been described to include corneal opacities, retinal dystrophy, coloboma, and unilateral microphthalmia. We report a case of severe bilateral microphthalmia in a neonate with Trisomy 8 mosaicism, a previously unrecognized ophthalmic manifestation.

Management of vernal keratoconjunctivitis: Navigating a changing treatment landscape.

Vernal keratoconjunctivitis (VKC) is a chronic, progressive, and potentially sight-threatening form of ocular inflammatory disease that primarily affects children and young adults. Prevalence varies by region, ranging from <2 per 10,000 in the United States to as high as 1,100 per 10,000 in parts of Africa. The rarity of VKC in developed countries can make differential diagnosis challenging, and treatment is often delayed until the disease is advanced, and symptoms are significantly impacting patients' quality of life. Although once viewed primarily as an immunoglobulin E-mediated condition, approximately 50% of patients with VKC do not exhibit allergic sensitization. It is now recognized that the immunopathology of VKC involves multiple inflammatory pathways that lead to the signs, symptoms, and conjunctival eosinophilic and fibroproliferative lesions that are a hallmark of the disease. We examine the evolution of our understanding of the immunopathology of VKC, the expanding VKC treatment armamentarium, the clinical implications of emerging treatment approaches, and future directions for VKC research and practice.

Effect of vertical central plication on vertical deviations.

PURPOSE: To evaluate the surgical effect of the Wright central plication on vertical rectus muscles to correct vertical strabismus. METHODS: In this multicenter, retrospective, observational outcomes study, data were collected from two surgeons in different practice settings (2017-22). All patients who underwent vertical rectus central plication were included; those undergoing any concurrent strabismus surgery for vertical strabismus were excluded. Primary outcome was amount of strabismus correction in prism diopters per vertical rectus central plication. Secondary outcome was to determine factors associated with better or worse surgical outcomes and patient and patient responses. Data were analyzed using descriptive and bivariate statistics. RESULTS: A total of 36 patients were included. Mean age was 60 years. Mean follow-up was 8.4 months. Of the 36 patients, 11 (31%) had idiopathic strabismus, and 7 (19%) had congenital superior oblique palsy. The remainder had a history that included prior ocular surgery, trauma, and Brown syndrome; 16 (44 %) had prior strabismus surgery. Of 31 patients with preoperative diplopia, 23 (74%) had postoperative resolution of diplopia, and 10 of 16 patients with preoperative prisms (63%) no longer required prisms postoperatively. Mean vertical deviation change was 4.7Δ. Subgroup analysis removing patients with congenital superior oblique palsy showed a larger response of 5.5Δ. 78% of patients had a final deviation <5Δ. No complications or induced postoperative diplopia was reported. CONCLUSIONS: In our study cohort, vertical rectus central plication corrected approximately 5Δ (range, 4.5Δ-5.5Δ) of vertical strabismus due to a variety of causes.

Missed appointments in a tertiary academic pediatric ophthalmology and adult strabismus service: cross-sectional study and literature review.

PURPOSE: To investigate the rate of missed appointments in a Canadian academic hospital-based pediatric ophthalmology and adult strabismus practice and the demographic and clinical factors associated with missed appointments. METHODS: This cross-sectional study included all consecutive patients seen from June 1, 2018, to May 31, 2019. Multivariable logistic regression model assessed associations between clinical and demographic variables with no-show status. A literature review on evidence-based interventions to reduce no-show appointments in ophthalmology was performed. RESULTS: Of 3,922 visits, 718 (18.3%) were no-shows. Characteristics associated with no-shows included new patient (OR = 1.4; 95% CI, 1.1-1.7 [P = 0.001]), age 4-12 years (OR = 1.6; 95% CI, 1.1-2.3 [P = 0.011]) or age 13-18 years (OR = 1.8; 95% CI, 1.2-2.7 [P = 0.007]) compared with age 19+ years, history of previous no-shows (OR = 2.2; 95% CI, 1.8-2.7 [P = 0.001]), referrals from nurse practitioners (OR = 1.8; 95% CI, 1.0-3.2 [P = 0.037]), nonsurgical diagnoses such as retinopathy of prematurity (OR = 3.2; 95% CI, 1.8-5.6 [P < 0.001]), and winter season (OR = 1.4; 95% CI, 1.2-1.7 [P < 0.001]). CONCLUSIONS: Missed appointments in our pediatric ophthalmology and strabismus academic center are more likely new patient referrals, prior no-shows, referrals from nurse practitioners, and nonsurgical diagnoses. These findings may facilitate targeted strategies to help improve utilization of healthcare resources.

Pathophysiology of retinopathy of prematurity.

Retinopathy of prematurity (ROP) is a vasoproliferative disease occurring in premature infants that affects the blood vessels of the developing retina. ROP results in the development of vascular shunts, neovascularization, and in its most severe form tractional retinal detachment. The development of retinal vascular shunts and neovascularization in ROP is related to local ischemia in the immature and incompletely vascularized retina. Understanding the pathophysiology of ROP helps physicians both in the prevention and treatment of ROP and will be discussed in this review article. The role of oxygen in the pathophysiology of ROP will be reviewed with recent studies discussed.

Amniotic membrane transplantation for restrictive strabismus.

PURPOSE: To report the use of amniotic membrane transplant in patients with restrictive strabismus. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Patients with restrictive strabismus treated with amniotic membrane transplantation. METHODS: Chart review of 7 consecutive patients (8 eyes) who developed restrictive strabismus after periocular surgery and were treated with surgical removal of restrictive adhesions and placement of an amniotic membrane transplant. MAIN OUTCOME MEASURES: Intraoperative findings to explain the mechanism of restrictive strabismus, and clinical postoperative results, including ocular alignment, ductions and versions, symptom relief, and resolution of diplopia. RESULTS: Restrictive strabismus occurred after surgery for pterygium, retinal detachment, orbital floor fracture, dermoid cyst, and dermatochalasis. Restrictive strabismus was due to a combination of conjunctival contracture, fat adherence, or rectus muscle contracture. All patients developed postoperative scarring, with failed additional standard surgery to remove the adhesions, including 1 patient treated with mitomycin C for recurrent scarring after pterygium. Reoperation using amniotic membrane transplantation was associated with improvement of ocular motility in 6 of the 7 patients; 1 patient had recurrence of scarring with persistent diplopia. The remaining 6 of 7 patients had no significant recurrence of scarring, and motility remained stable during the follow-up period of 5 to 13 months. CONCLUSIONS: Amniotic membrane transplantation seems to help prevent recurrence of adhesions in patients with restrictive strabismus caused by conjunctival scarring, fat adherence syndrome, or rectus muscle contracture. Use of an amniotic membrane transplant should be considered as a treatment option for these difficult cases of restrictive strabismus. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Use of neuromuscular blocking drugs in strabismus surgery: systematic review and ophthalmology survey study.

OBJECTIVE: Nondepolarizing neuromuscular blocking drugs have been advocated for accurate forced duction testing (FDT) during strabismus surgery. The purpose of this study was to evaluate the literature on use of muscle relaxants (MRs) in strabismus surgery and to evaluate the current practice of pediatric ophthalmologists. DESIGN: Systematic review and survey study. PARTICIPANTS: Seventy-seven pediatric ophthalmologists. METHODS: MEDLINE, EMBASE, CENTRAL, Web of Science, and OpenGrey were searched to August 2019. Reporting followed the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines. Primary studies on depolarizing or nondepolarizing MRs in patients undergoing strabismus surgery were included. Primary outcomes included indications and prevalence of use. A 23-question survey was emailed to pediatric ophthalmologists worldwide to collect practice pattern information involving MRs in strabismus surgery. RESULTS: Three studies were included in the systematic review. MRs were used in 34%-45% of patients undergoing strabismus surgery in one 1999 study for FDT. No evidence exists to support the use of MRs for facilitating FDT. Seven of 77 (9.1%) pediatric ophthalmologists requested MRs for FDT. Those who use MRs were more likely to be practicing outside the United States (p < 0.05) and in academic hospitals or private centres rather than in community settings. Drawbacks appeared to outweigh benefits, with the biggest drawback identified as increased turnover time between surgical cases (44%). CONCLUSIONS: Inadequate evidence exists in the literature to support the use of nondepolarizing MRs to maintain paralysis of extraocular muscles during strabismus surgery, and this is an uncommon practice among pediatric ophthalmologists.

Wright hang-back recession with fibrin glue compared with standard fixed suture recession for the treatment of horizontal strabismus.

OBJECTIVE: To evaluate the clinical safety and efficacy of the novel Wright hang-back recession with fibrin glue for the treatment of horizontal strabismus. STUDY DESIGN: Retrospective, case-controlled clinical study comparing surgical outcomes of the Wright hang-back rectus recession with fibrin glue (WHBG) versus standard fixed suture rectus recession (SFR). METHODS: Medical records of all patients who underwent strabismus surgery by one strabismus surgeon between 2016 and 2018 for horizontal deviations only, including cases of WHBG (group 1) or SFR (group 2), were reviewed. Good surgical outcome was defined as a postoperative deviation ≤10 prism diopters (PD) at a minimum 2 months of follow-up. RESULTS: 32 eyes of 17 patients underwent WHBG and 32 eyes of 17 patients underwent SFR; in each group, 35% had esotropia and 65% had exotropia. Mean preoperative deviations between groups were similar: esotropia 25.5 PD and exotropia 26.6 PD in WHBG; esotropia 28.3 PD and exotropia 23.8 PD in SFR. The mean postoperative deviation was <7 PD for both groups. Good surgical outcomes were similar between groups, 16/17 (94%) in WHBG and 15/17 (88%) in SFR, with no complications. CONCLUSIONS: WHBG was safe and effective with postoperative results similar to SFR. WHBG has an important advantage, eliminating the complication of retinal perforation that can occur with SFR while avoiding under- or overcorrection that can occur with traditional hang-back recession. This technique increases patient safety without sacrificing surgical outcomes and is especially useful in patients with thin sclera such as patients with high myopia or with difficult posterior exposure.

Validation of a novel strabismus surgery 3D-printed silicone eye model for simulation training.

PURPOSE: To demonstrate the validity of a new 3D-printed silicone model for practicing strabismus surgery, compared with the rabbit head, in terms of simulator fidelity. METHODS: In this multicenter study, a validated questionnaire was developed to assess fidelity of the model and rabbit head. Participants were asked to rate overall globe, conjunctiva, muscle, and scleral fidelity using a 5-point scale. The survey instrument was disseminated at three strabismus instruction courses: at two meetings, participants practiced on the model and rabbit head prior to completing the questionnaire; at the third, instructors demonstrated advanced surgical skills using only the model and then completed the questionnaire. Repeated measures analysis of variance compared ratings. Pearson's or Spearman's correlation evaluated correlation between years of experience to participants' responses. Qualitative data were coded into themes. RESULTS: A total of 47 participants completed the questionnaire. The model rated 18% higher than rabbit head for anatomical accuracy (mean difference, 0.667; P = 0.001) and 25% higher for position of eyes within the head (mean difference, 0.867; P = 0.006). More experienced participants were more likely to strongly agree that the silicone conjunctiva effectively mimics real conjunctiva (ρ = 0.337; P = 0.036) and that scleral tissue effectively mimics real sclera (ρ = 0.298, P = 0.042). Qualitative data supported the model. CONCLUSIONS: This study demonstrated the validity of the surgical model in terms of fidelity compared to the rabbit head.

Silicone oil migration into periorbital space surrounding an extraocular muscle after sutureless 23-gauge vitrectomy in a child.

We report the case of an 11-year-old girl with a rare finding of silicone oil migration into the periorbital space surrounding an extraocular muscle, discovered during strabismus surgery for a sensory exotropia that had developed after retinal detachment repair by pars plana vitrectomy with intraocular silicone oil.

Forceps trauma in a newborn presenting as iris heterochromia.

Birth-associated ocular trauma is common and often minor, including subconjunctival and retinal haemorrhage and eyelid edema. Significant ocular trauma during birth, usually associated with forceps-assisted delivery, is rarer and can include Descemet's membrane rupture, lid lacerations, hyphema, Purtcher retinopathy, facial nerve palsy, corneal edema, and corneal laceration. We report the first case of ocular birth trauma from forceps presenting as isolated iris heterochromia and a pseudo rubeosis iridis, which completely resolved by 1 month of age with no known adverse ocular sequelae.

Fourth-generation fluoroquinolones in the treatment of mycobacterial infectious keratitis after laser-assisted in situ keratomileusis surgery.

CASE REPORT: Infectious keratitis after laser-assisted in situ keratomileusis (LASIK) is a rare complication that may be challenging to diagnose and treat. We discuss some of the reasons for difficulties diagnosing postoperative atypical or nontuberculous mycobacterial infection and establishing an effective treatment, with reference to reports of comparative antibacterial activity of the most commonly used antibiotics. COMMENTS: To the best of our knowledge, we are the first to report the use of the fourth-generation fluoroquinolone moxifloxacin in combination therapy for the successful treatment of this condition.

Pediatric adenoid cystic carcinoma of the lacrimal gland treated with intra-arterial cytoreductive chemotherapy.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is the most common primary malignant tumor of the lacrimal gland. It typically affects patients in the fifth decade of life and presents with rapid progression of pain, ptosis, motility disturbances, and sensory deficits of less than 1 year's duration. ACC is rare in children. Due to early, aggressive perineural and bony spread, there is a high risk of intracranial extension. Additionally, due to frequent hematogenous and lymphatic spread, there is a high rate of distant metastases even after treatment, which can occur late up to a decade or more. The currently accepted treatment for ACC is radical exenteration with orbitotomy and adjuvant chemotherapy and/or radiation therapy. Recently, intra-arterial cytoreductive chemotherapy (IACC) has been investigated as a neoadjuvant treatment modality. It has the advantage of increasing local concentration at the target tissue and decreasing systemic distribution. We report the first known pediatric case of ACC treated with IACC, followed by exenteration, radiation, and adjuvant intravenous chemotherapy. The patient was followed for 4 years, with no recurrence and no known complications.

A child with rapidly progressive necrotizing group a streptococcal Tenon's capsule infection one day after strabismus surgery.

Periorbital infections after strabismus surgery are rare. We describe the first reported case of necrotizing group A streptococcal infection of the conjunctiva and Tenon's capsule complicating uneventful strabismus surgery in a 23-month-old boy, successfully managed with conservative intraoperative debridement and with targeted local and systemic antibiotics.

Strabismus surgical subspecialization: a population-based analysis.

IMPORTANCE: The growing complexity of medical and surgical care has resulted in increasing subspecialization. To date, data have been lacking regarding the degree to which subspecialization has affected the provision of strabismus surgical services. This gap is important to address given the implications for health care human resources planning and educational programs. OBJECTIVE: To investigate the effect of subspecialization on the provision of strabismus surgery services. DESIGN, SETTING, AND PARTICIPANTS: Population-based study in Ontario, Canada, which provides universal health care coverage to the provincial population. Participants included all ophthalmologists in Ontario and the provincial population of approximately 12 million persons from January 1, 1994, through December 31, 2011. MAIN OUTCOMES AND MEASURES: Surgeon-level rates of strabismus surgery. RESULTS: From January 1, 1994, through December 31, 2011, the percentage of ophthalmologists who provided strabismus surgery decreased from 37.7% (156 of 414 surgeons) to 12.5% (54 of 432 surgeons; difference, 25.2%; 95% CI, 19.3%-30.9%; P < .001), a 66.8% decline from the baseline level. Of ophthalmologists who provided strabismus surgery during the same period, the mean number of strabismus procedures per surgeon grew from 16.2 to 55.3 per year, a 241.4% increase (95% CI, 20.4%-461.6%; P < .001). These trends occurred at all career stages. CONCLUSIONS AND RELEVANCE: Strabismus surgery has evolved into a subspecialized field of ophthalmology during the past 15 years. These findings may have important implications for health care professionals and health care system leaders, including the need to account for subspecialization in physician human resources decisions to ensure access to quality strabismus surgery across regions. Furthermore, residency education programs and their governing organizations may need to account for strabismus subspecialization when designing curriculum and accreditation requirements.

Simultaneous bilateral cataract surgery.

PURPOSE: To evaluate the safety and outcomes of simultaneous bilateral cataract surgery (SBCS). SETTING: York Finch Eye Associates and Humber River Regional Hospital, Toronto, Ontario, Canada. METHODS: This retrospective study reviewed the results of 1020 consecutive patients (2040 eyes) who had SBCS by endolenticular phacoemulsification through a clear corneal incision on the corneal steep axis with foldable posterior chamber intraocular lens implantation. The surgeries were performed by the same surgeon from January 1996 to January 2002 as 2 consecutive independent procedures under topical and intracameral anesthesia. Outcome measures included intraoperative and postoperative complications, postoperative uncorrected and best spectacle-corrected visual acuities, refractive error, and patient satisfaction. RESULTS: Complications were few and would not likely have been prevented had the surgery been performed monocularly. CONCLUSIONS: Simultaneous bilateral cataract surgery did not lead to an increased incidence of intraoperative or postoperative complications. The visual acuity results were good, and the patients were pleased. No complications were observed that could be attributed to the procedures being done bilaterally.