Pediatric Eyelid Cutaneous Horns: A Case Series and Literature Review.

PURPOSE: To describe the clinical and histopathologic features of pediatric eyelid cutaneous horns. DESIGN: Retrospective observational case series and review of literature. SUBJECTS: Five pediatric patients with eyelid cutaneous horns Methods: Five cases with eyelid cutaneous horns were retrospectively identified using departmental databases. Patients' records were analyzed for demographic data, clinical appearance, histologic findings, and clinical course. An excisional biopsy of the lesion was performed in 3 patients. The remaining 2 patients were managed conservatively. MAIN OUTCOME MEASURES: Clinical outcome and histopathologic evaluation with emphasis on excluding malignancy. RESULTS: All 5 cutaneous horn lesions resolved surgically or conservatively. The average age at presentation was 6.6 years (range 5-11 years). Clinically, 4 lesions were preceded by a hordeolum or chalazion and all excised lesions had benign features on histologic examination. Mitotic figures or atypia were not observed. None of the patients developed recurrence during the follow-up period ranging from 1 to 96 months. Five previous reports of five cases were found on review of the literature. Our case series doubles this number to support the benign nature of these lesions in children. CONCLUSIONS: Pediatric eyelid cutaneous horns are closely related to eyelid margin inflammatory disease and appear to follow a benign course. This contrasts with the adult population where cutaneous horns are frequently associated with neoplasia.

Pre-term Familial Exudative Vitreoretinopathy: A Novel Nonsense LRP5 Mutation.

PURPOSE: This case report details the diagnosis and management of a pre-term infant with aggressive bilateral retinal pathology. METHODS: A 4-week-old preterm baby girl, born at 28 weeks and 6 days to consanguineous parents, was referred for suspected aggressive posterior retinopathy of prematurity (ROP). She had a family history of bilateral retinal detachments and intellectual disability in an older sister. Clinical assessment included retinal examination, fluorescein angiography, optical coherence tomography, dual-energy X-ray absorptiometry (DEXA), and genetic testing. The genetic testing involved sequence analysis and copy number variation analysis of 25 genes related to vitreoretinopathy. RESULTS: Retinal examination and fluorescein angiography revealed extensive non-perfusion and telangiectatic vessels in both eyes, and a macula-involving tractional retinal detachment in the left eye. Despite treatment with intravitreal bevacizumab and laser photocoagulation, they progressed to total retinal detachment and no light perception in both eyes. Genetic testing revealed a pathogenic homozygous nonsense mutation in the LRP5 gene (c.3259C>T, p.(Gln1087*)), a mutation not previously reported in association with familial exudative vitreoretinopathy (FEVR). At 10 months of age, DEXA demonstrated normal bone density, diverging from the typical presentation of osteoporosis pseudoglioma syndrome associated with LRP5 mutations. CONCLUSION: This case describes a novel mutation in a complex retinal disease and underscores the necessity of considering pre-term FEVR in the differential diagnosis of atypical or aggressive ROP in preterm infants. The overlap in clinical features between ROP and FEVR highlights the complexity of diagnosis and management and the importance of genetic testing in preterm infants with retinal vascular abnormalities.

A Comprehensive Evaluation of 16 Old and New Intraocular Lens Power Calculation Formulas in Pediatric Eyes.

Purpose: To compare the accuracy of 16 intraocular lens (IOL) power calculation formulas in pediatric cataract eyes. Patients and Methods: The data records of pediatric patients who had been implanted with three IOL models (SA60AT, MA60AC, and enVista-MX60) between 2012 and 2018 were analyzed. The accuracy of 16 IOL power calculation methods was evaluated: Barrett Universal II (BUII), Castrop, EVO 2.0, Haigis, Hill-RBF 3.0, Hoffer Q, Hoffer QST, Holladay 1, Kane, LSF AI, Naeser 2, Pearl-DGS, SRK/T, T2, VRF, and VRF-G. The non-optimized (ULIB/IOLcon) and optimized constants were used for IOL power calculation. The mean prediction error (PE), Performance Index (FPI), and all descriptive statistics were calculated. Results: Ninety-seven eyes of 97 pediatric patients aged 13.2 (IQR 11.2-17.1) were included. No statistically significant difference (HS-test) was observed (p > 0.818) except for the Hoffer Q, and Naeser 2 (P = 0.014). With optimized lens constants, the best FPI indices were obtained by Hoffer Q (0.256) and VRF-G (0.251) formulas, followed by Hill-RBF 3.0 and BUII, with an index of 0.248. The highest FPI indices with non-optimized constants showed SRK/T and T2 formulas (0.246 and 0.245, respectively), followed by VRF-G and Holladay 1, with an index of 0.244. The best median absolute error values (MedAE) were achieved by Hoffer Q (0.50 D), VRF-G (0.53 D), and Hill-RBF 3.0 (0.54 D), all P ≥ 0.074. Conclusion: Our results place the Hoffer Q, VRF-G, Hill-RBF 3.0, and BUII formulas as more accurate predictors of postoperative refraction in pediatric cataract surgery.

Efficacy of the Subtenon Block in Children Undergoing Strabismus Surgery: A Systematic Review and Meta-Analysis.

PURPOSE: To investigate the efficacy of the subtenon block in preventing postoperative complications in children undergoing strabismus surgery. DESIGN: Systematic review and meta-analysis. METHODS: We searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, Scopus, Web of Science, and clinicaltrials.gov. All randomized controlled trials investigating the efficacy of the subtenon block in children undergoing strabismus surgery were included. Outcomes included severity of pain after surgery, number of children requiring postoperative opioid and nonopioid analgesia, and the incidences of postoperative nausea and vomiting, oculocardiac events, and block-related complications. We pooled continuous outcomes using a random-effects model to calculate the mean difference (MD) and/or standardized MD and corresponding 95% confidence intervals (CI). Dichotomous outcomes were pooled using a random-effect model to calculate the relative risk (RR) and corresponding 95% CI. Risk of bias was assessed using the Cochrane Risk of Bias instrument and quality of evidence was assessed using a Grading of Recommendations Assessment, Development, and Evaluation approach. RESULTS: Nine randomized controlled trials (607 participants) were included. We found evidence for an effect of the subtenon block in reducing pain at 20 to 40 minutes after surgery (MD -1.9, 95% CI -2.2 to -1.5; high-quality evidence) and on admission to PACU (MD -1.8, 95% CI -2.2 to -1.4; moderate-quality evidence); however, there was no difference in pain scores at 6 hours after surgery. In addition, evidence was found that the subtenon block decreased the number of children requiring postoperative opioid (RR 0.59, 95% CI 0.37-0.92; high-quality evidence) and nonopioid (RR 0.52, 95% CI 0.27-0.98; moderate-quality evidence) analgesia, and the incidences of postoperative vomiting (RR 0.31, 95% CI 0.12-0.7; high-quality evidence) and intraoperative oculocardiac events (RR 0.40, 95% CI 0.26-0.60; high-quality evidence). Other secondary outcomes had low or moderate-quality evidence. Risk of bias was low in six trials and high in three. There were no reports of block-related complications. CONCLUSIONS: Our findings suggest that the subtenon block reduces the severity of early postoperative pain and the incidences of postoperative vomiting and intraoperative oculocardiac events compared with controls in children undergoing strabismus surgery. REVIEW REGISTRATION: PROSPERO (CRD42015025443).

Nonsurgical Consecutive Exotropia Following Childhood Esotropia: A Multicentered Study.

PURPOSE: Nonsurgical consecutive exotropia (NCX) occurs when an esotropia (ET) spontaneously converts to exotropia (XT) without surgical intervention. Although NCX is considered to occur in early-onset accommodative ET with high hyperopia, consensus on causation is lacking. We report the clinical characteristics of NCX and assess the response to conservative management. DESIGN: Retrospective, multicenter, observational case series. METHODS: Patients aged 6 months and older with an initial diagnosis of ET who converted to XT without surgical intervention. Sensory strabismus was excluded. Age, visual acuity, cycloplegic refraction, glasses prescriptions, deviation, and binocular vision were collected. RESULTS: Forty-nine children were included with a mean age of 3.5 ± 1.6 years and 8.4 ± 3.6 years at the time of ET and NCX, respectively. Mean refractive error was +4.40 ± 2.13 diopters (D) and +4.05 ± 2.74 D at the time of ET and NCX, respectively. Accommodative ET occurred in 60% of cases, and only 35.7% were high hyperopes. All but 1 patient presented with XT at distance. In response to the XT, a mean decrease in hyperopic prescription of 1.55 ± 0.48 D was given (N = 17); only 1 case reverted to ET. Eventually, 43% underwent XT surgery, with similar rates between those who had refractive management and those who did not. CONCLUSIONS: NCX occurs in both accommodative and nonaccommodative ET; high hyperopia is present in only one-third of cases. On average, drift to XT occurs within 5 years. Refractive management has a modest result. No predictive risk factors were identified. Our findings challenge hyperopia-linked theories of causation. Nonrefractive explanations, such as the role of the vergence system, deserve further study.

A Meta-Analysis of Neurodevelopmental Outcomes following Intravitreal Bevacizumab for the Treatment of Retinopathy of Prematurity.

BACKGROUND: Retinopathy of prematurity (ROP) is the most common cause of preventable blindness in preterm infants. First-line treatments include intravitreal bevacizumab (IVB) or laser photocoagulation (LPC). OBJECTIVES: The aim of the study was to evaluate neurodevelopmental safety of IVB compared to LPC for ROP. METHODS: MEDLINE, Embase, and Cochrane library were searched up to September 2022. Studies were included with at least 12-month follow-up of primary outcomes such as severe neurodevelopmental impairment (sNDI), cerebral palsy (CP), and hearing impairment (HI). Secondary outcomes were moderate-to-severe neurodevelopmental impairment (msNDI), Bayley Scores of Infant Development (BSID-III), and visual impairment. RESULTS: 1,231 patients from 11 comparative studies were included. Quality of evidence was rated low for all outcomes. IVB was associated with a higher risk for sNDI (risk ratio [RR] = 1.25, 95% confidence interval [CI]: [1.01, 1.53], p = 0.04); and CP (RR = 1.40, CI: [1.08, 1.81], p = 0.01) compared to LPC. There was no significant difference between IVB and LPC for msNDI (RR = 1.15, CI: [0.98, 1.35], p = 0.08) and HI (RR = 1.43, CI: [0.86, 2.39], p = 0.17). BSID-III percentile scores were similar between IVB and LPC, with weighted mean differences of 1.51 [CI = -1.25, 4.27], 2.43 [CI = -1.36, 6.22], and 1.97 [CI = -1.06, 5.01] for cognitive, language, and motor domains, respectively (p > 0.05). CONCLUSION: To our knowledge, this is the largest meta-analysis on neurodevelopmental outcomes and the first to rigorously examine IVB monotherapy in ROP treatment. Compared to LPC, there was a marginally increased risk for sNDI and CP with IVB but little or no difference in the risk of msNDI and HI. Further randomized studies are needed to strengthen these findings.

Subdural hemorrhages and severe retinal hemorrhages in a short fall with a rotational component.

We present the case of a 5-month-old referred for child abuse investigation with subdural hemorrhages and extensive retinal hemorrhages following a short fall from a swivel chair seen on video footage. Subdural hemorrhages with extensive retinal hemorrhages are not typically seen as the result of short household falls. Reviewing the footage, contributing factors may have included increased rotational and deceleration forces.

Rise in the incidence of severe pediatric blepharokeratoconjunctivitis during the COVID-19 pandemic.

Severe blepharokeratoconjunctivitis (BKC) is associated with vision loss and ocular morbidity; hence, early diagnosis and treatment are crucial. Retrospective data collection using electronic patient and billing database records of all patients <18 years of age with severe BKC between March 2010 and March 2022 was performed at the Eye Clinic at the Hospital for Sick Children in Toronto, Canada. Severe BKC was defined as including corneal inflammation, new vessel formation, scarring, thinning and lipid deposits. We excluded patients with mild or no corneal involvement, and those with other corneal pathologies. Over the study period, 257 patients were diagnosed with severe BKC (161 females), with an average age of 8 years. There was a statistically significant threefold increase in the diagnosis of severe BKC since the beginning of the COVID-19 pandemic in 2020 compared to the previous years. We speculate that the increased use of facial masks during the pandemic contributed to this significant increase.

Outcomes of cataract surgery in children who present with cataract at uveitis diagnosis.

PURPOSE: To describe the clinical and demographic characteristics of patients presenting with cataract at uveitis diagnosis treated at a single institution between 2005 and 2019 and to analyze postoperative outcomes following cataract surgery. METHODS: We retrospectively reviewed the medical records of children (<18 years of age) diagnosed with cataract at their initial uveitis presentation who subsequently underwent cataract extraction. Outcome measures were best-corrected visual acuity, number of uveitis flare-ups (cells ≥1+), and postoperative complications. RESULTS: A total of 14 children (17 eyes) were included. Mean patient age was 7.2 ± 3.9 years. Methotrexate was initiated preoperatively in 11 patients; adalimumab, in 3. Primary intraocular lens was implanted in 4 eyes. Best-corrected visual acuity improved from a mean of 0.90 ± 0.40 logMAR preoperatively to 0.50 ± 0.35 logMAR at 1 year and 0.57 ± 0.40 logMAR at mean of 6.3 ± 3.4 years postoperatively. Four eyes (24%) had a single episode of uveitis flare-up during the first postoperative year. Macular and/or disk edema was discovered in 6 eyes following cataract removal. Only 3 eyes (18%) had ocular hypertension in the first year, but glaucoma developed in subsequent years in 7 eyes (41%), 5 of which required surgery. CONCLUSIONS: In our study cohort, surgery for preexisting cataract at uveitis diagnosis resulted in improved visual acuity. Postoperative uveitis flare-ups were relatively uncommon, occurring in 4 of 17 eyes. Glaucoma was the main long-term complication.

Bilateral keratouveitis associated with COVID-19 multisystem inflammatory syndrome in children.

We report the case of a 16-year-old girl admitted to hospital with multisystem inflammatory syndrome in children (MIS-C) secondary to COVID-19. Conjunctivitis-like symptoms prompted ocular examination, which demonstrated peripheral confluent corneal opacities and anterior uveitis. Uveitis laboratory investigations were negative, and with topical steroid treatment her signs and symptoms resolved completely. These features may be overlooked in the setting of MIS-C, where patients are systemically unwell and are typically examined at the bedside.

Long-term outcomes of type 1 retinopathy of prematurity following monotherapy with bevacizumab: a Canadian experience.

OBJECTIVE: To report long-term structural, visual, and refractive outcomes after monotherapy with intravitreal bevacizumab injection. DESIGN: Cohort retrospective chart review. PARTICIPANTS: A total of 56 premature infants with type 1 retinopathy of prematurity. METHODS: This is a chart review at 2 Canadian institutions. Inclusion criteria were single injection of 0.625 mg  intravitreal bevacizumab and minimum age at last follow-up of 3 years. Primary outcome was retinal structure. Secondary outcomes were refractive error in spherical equivalent, monocular visual acuity, strabismus, and amblyopia. RESULTS: Fifty-six infants (101 eyes) met inclusion criteria. Mean birth weight was 707 ± 178 g (range, 420-1520 g). Mean gestational age was 25.0 ± 1.3 weeks (range, 22.9-29.7 weeks). Twenty-four eyes were in zone I (24%) and 77 in zone II (76%). Mean postmenstrual age at treatment was 36.9 ± 2.1 weeks (range, 32.8-42.0 weeks). At a mean age of 5.4 ± 1.6 years (range, 3.0-8.0 years), all eyes had a favourable structural outcome with no reactivation requiring treatment. Mean monocular visual acuity was 0.29 ± 0.27 logMAR (range, 0.0-1.3 logMAR; 89 of 101 eyes). Mean spherical equivalent was -1.98 ± 4.91 D (range, -16.63 to +5.38 D; 101 of 101 eyes). Prevalence of emmetropia (>-1.0 to ≤1 D) was 43.6%; low myopia (≥1.0 to <5 D) was 17.8%; high myopia (≥5 to <8 D) was 8.9 %; very high myopia (≥8.0 D) was 12.9%; and hyperopia (>1 D) was 16.8%. Twelve children (23%) had amblyopia, and 17 (32%) developed strabismus. CONCLUSIONS: All patients demonstrated a favourable structural outcome with a single bevacizumab injection without the need for additional laser. We suggest regular monitoring following regression of acute retinopathy of prematurity as an alternative to universal, preplanned delayed prophylactic laser treatment. Future studies to evaluate other aspects of visual function are needed.

Children with high astigmatism: tomographic and refractive characteristics and the ability of current indices to rule out keratoconus.

PURPOSE: To assess corneal tomographic, topographic, and refractive changes in children with high astigmatism and their ability to exclude keratoconus. METHODS: In this longitudinal observational study, the medical records of children with high regular cylindrical refraction of ≥3.50 D referred to the Hospital for Sick Children, Toronto, to exclude keratoconus between January 2009 and June 2020 were reviewed retrospectively. Corneal tomography records (Scheimpflug imaging) were reviewed for subjects with total astigmatism of ≥ +3.50 D by retinoscopy. Children with abnormal anterior segment examination and/or other risk factors for corneal disease or ectasia and those with unreliable corneal tomography were excluded. Baseline demographic, longitudinal tomographic, topographic, and refractive data were analyzed. RESULTS: A total of 67 eyes of 37 children (mean age, 9.1 ± 3.5 years) were included. Mean cylindrical refraction at presentation was 5.10 ± 1.30 D. Mean follow-up was 2.3 ± 1.8 years. Twenty-nine eyes had Kmax ≥47.20 D at baseline, with no change at last follow-up. Ksteep, Kmax, and thinnest pachymetry were 46.44 ± 2.33 D, 47.06 ± 2.57 D, and 525.86 ± 35.45 µm, respectively, at baseline compared with 46.40 ± 2.28 D, 46.98 ± 2.40 D, and 527.61 ± 37.67 µm at last follow-up (P > 0.05). All eyes were predicted as not having keratoconus using inferior-superior dioptric asymmetry ratio (I-S ratio), and the keratometry, inferior-superior, and astigmatism index (KISA%), which also incorporates skew percentage. The other tomographic indices predicted keratoconus or subclinical keratoconus in at least 22.4% of eyes. All parameters did not significantly change during follow-up. CONCLUSIONS: In the absence of other risk factors for keratoconus, children with high regular astigmatism demonstrated clinical and tomographic stability over time. Based on our results, we recommend that I-S ratio and KISA% be primarily used when monitoring normal children with high astigmatism to rule out keratoconus-related changes.

A typical anterior retinoblastoma: diagnosis by aqueous humor cell-free DNA analysis.

BACKGROUND: Aqueous humor from eyes with active retinoblastoma contains tumor-derived cell-free DNA. MATERIALS AND METHODS: Single retrospective case report. RESULTS: A 13-year-old girl with acute right eye pain and redness was diagnosed with hypertensive anterior uveitis. Following initial management, she was referred to ocular oncology for an atypical clinical picture. Multiple seeds were noted 360 degrees in the anterior chamber, at the equator of the lens and canal of Petit, and ultrasound biomicroscopy identified a temporal pars plana lesion. While aqueous humor cytology was inconclusive for malignancy, targeted next-generation sequencing of aqueous cell-free DNA identified biallelic RB1 full gene deletion, confirming the diagnosis of retinoblastoma. Partial regression followed three cycles of systemic carboplatin, etoposide, and vincristine and three intracameral melphalan injections. Four months later, she had recurrence of the primary tumor and increase in seeding and received the investigational sustained release episcleral topotecan chemoplaque. Stable regression was achieved to 28-month follow-up, with no detectable aqueous cell-free DNA. CONCLUSIONS: RB1 sequencing analysis of tumor-derived cell-free DNA from aqueous humor can confirm the diagnosis of retinoblastoma in cases of diagnostic uncertainty.

Late surgery for unilateral persistent fetal vasculature: long-term visual and anatomic outcomes.

BACKGROUND: Persistent fetal vasculature (PFV) is a congenital anomaly caused by failure of the ocular vasculature to regress. We report the visual and anatomic outcomes in patients who underwent surgery for PFV between 7-48 months of age. METHODS: The medical records of patients who underwent lensectomy and anterior or core vitrectomy for unilateral PFV, with or without intraocular lens implantation, were reviewed retrospectively. Inclusion criteria were surgery between 7-48 months of age and at least 12 months of follow-up. Patients with severe posterior segment involvement were excluded. The primary outcome was the final visual acuity using age-appropriate tests converted to logMAR. Secondary outcomes included the rate of adverse events and number of subsequent intraocular procedures. RESULTS: A total of 20 patients with a mean age at surgery of 19.3 ± 10.5 months and mean follow-up of 73.7 ± 46.7 months were included. Sixteen patients had delayed surgery due to late presentation; the remaining 4 were managed initially with refractive correction and occlusion. Eight patients (40%) achieved a final visual acuity better than 1.0 logMAR. Four eyes had adverse events, including one retinal detachment. No patient developed glaucoma. Four eyes underwent subsequent procedures. CONCLUSIONS: In our study cohort, surgery for unilateral PFV between 7-48 months of age achieved functional visual acuity in over one-third of patients. This is comparable to results achieved with surgery before 7 months of age, but with fewer adverse events. Differences in disease severity could account for later PFV presentation and surgery as well as outcomes.

Unilateral cataract and congenital stationary night blindness in a child with novel variants in TRPM1.

Unilateral cataract can cause pediatric vision impairment. Although the majority of unilateral cataracts are idiopathic in nature, genetic causes have been reported. We present the case of a 4-week-old child of nonconsanguineous parents who was affected with unilateral cataract. Whole-genome sequencing using DNA extracted from blood and the lens epithelial cells following cataract surgery revealed two presumed pathogenic variants in the TRPM1 gene, the founding member of the melanoma-related transient receptor potential (TRPM) subfamily. TRPM1 is responsible for regulating cation influx to hyperpolarized retinal ON bipolar cells, and mutations in this gene are a major cause of autosomal recessive congenital stationary night blindness (CSNB). Electroretinography revealed findings consistent with CSNB, a phenotype that was not initially suspected, and which would likely have been missed without genome sequencing. It remains unclear whether the TRPM1 variants are associated with the cataract phenotype.

Evaluation of IOL power calculation with the Kane formula for pediatric cataract surgery.

PURPOSE: To assess the accuracy of the Kane formula for intraocular lens (IOL) power calculation in the pediatric population. METHODS: The charts of pediatric patients who underwent cataract surgery with in-the-bag IOL implantation with one of two IOL models (SA60AT or MA60AC) between 2012 and 2018 in The Hospital for Sick Children, Toronto, Ontario, CanFada, were retrospectively reviewed. The accuracy of IOL power calculation with the Kane formula was evaluated in comparison with the Barrett Universal II (BUII), Haigis, Hoffer Q, Holladay 1, and Sanders-Retzlaff-Kraff Theoretical (SRK/T) formulas. RESULTS: Sixty-two eyes of 62 patients aged 6.2 (IQR 3.2-9.2) years were included. The SD values of the prediction error obtained by Kane (1.38) were comparable with those by BUII (1.34), Hoffer Q (1.37), SRK/T (1.40), Holaday 1 (1.41), and Haigis (1.50), all p > 0.05. A significant difference was observed between the Hoffer Q and Haigis formulas (p = 0.039). No differences in the median and mean absolute errors were found between the Kane formula (0.54 D and 0.91 ± 1.04 D) and BUII (0.50 D and 0.88 ± 1.00 D), Hoffer Q (0.48 D and 0.88 ± 1.05 D), SRK/T (0.72 D and 0.97 ± 1.00 D), Holladay 1 (0.63 D and 0.94 ± 1.05 D), and Haigis (0.57 D and 0.98 ± 1.13 D), p = 0.099. CONCLUSION: This is the first study to investigate the Kane formula in pediatric cataract surgery. Our results place the Kane among the noteworthy IOL power calculation formulas in this age group, offering an additional means for improving IOL calculation in pediatric cataract surgery. The heteroscedastic statistical method was first implemented to evaluate formulas' predictability in children.

Ophthalmic involvement in PHACES syndrome: prevalence, spectrum of anomalies, and outcomes.

PURPOSE: To highlight prevalence, spectrum of anomalies, and outcome of ophthalmic involvement in PHACES syndrome (posterior fossa malformations, infantile hemangiomas, arterial, cardiac, eye, and sternal anomalies). METHODS: A retrospective, noncomparative, single-institution observational case series of children with PHACES was conducted from 2000 to 2019. Data on ocular presentations, interventions and visual outcomes were collected. Primary outcome measures were the frequency and spectrum of ocular involvement. Secondary outcomes were final visual acuity, long-term ocular sequelae, and frequency of surgical interventions. RESULTS: A total of 43 infants had PHACES, of whom 29 (67%) had periocular infantile facial hemangiomas (IFH) and 6 (14%) had primary ocular anomalies that were always ipsilateral to the IFH. Five patients (12%) met ocular PHACES-specific diagnostic criteria, including optic nerve (3), retinal vascular (1) and lenticular (2) anomalies. Non-PHACES-specific abnormalities were Peters anomaly (1), persistent pupillary membranes (2), dysmorphic optic nerves (1), and iris/choroidal hemangiomas (2). IFH-related periocular abnormalities were frequent: ptosis (29), proptosis (9), strabismus (6). Surgery was required in 8 of the 29 children: (strabismus [6], entropion [2], ptosis [2], and optical iridectomy [1]), all of whom had orbital/conjunctival hemangioma (P = 0.03). Final visual acuity (follow-up, 8.7 years) ranged between 20/20 and 20/80 in 26 of 29 patients. All patients with visual acuity worse than 20/200 (3/29 [10%]) had structural anomalies. CONCLUSIONS: Two-thirds of infants with PHACES have periocular IFH causing vision compromising complications of amblyopia and strabismus. Structural ocular anomalies exist in 1 of 7 patients and are always ipsilateral to the IFH. Long-term ophthalmic monitoring and management is required, and the majority of patients obtain good visual outcomes.