Ocular manifestations and long-term complications of rhabdomyosarcoma in children.

BACKGROUND/OBJECTIVES: The purpose of the study was to describe the ocular manifestations of rhabdomyosarcoma in a large cohort of children. SUBJECT/METHODS: This was a retrospective observational cohort study. The medical records of all pediatric patients with head and neck rhabdomyosarcoma diagnosed between 1997 and 2021 at a tertiary-care pediatric hospital were analyzed. The main outcome measures were the incidence and prognostic role of ocular findings at presentation and long-term ocular complications. RESULTS: There were 77 children with head and neck rhabdomyosarcoma in the study cohort with 38 patients showing ocular manifestations at presentation. Median age at diagnosis was 6.0 years, the median follow-up was 5.7 years and 54.5% were male. At last follow-up, 70.1% had no evidence of progression, 26.0% were deceased, and 2.6% were on palliative treatment. Orbital signs were common (44.2%). The most common ocular findings were proptosis (18.2%), restriction of extraocular motility (28.6%), strabismus/diplopia (22.1%) and ptosis (16.9%). The most common long-term complications were bony hypoplasia/facial asymmetry (40.3%) and keratopathy/dry eye (31.2%). Poor visual acuity (≤20/200) was noted in 13 (16.9%) patients with 5 (6.5%) patients requiring an exenteration. Survival was 100% in primary orbital RMS (p = 0.02), whereas any or a combination of cranial nerve palsies carried a poor prognosis (42% survival, p = 0.008). CONCLUSIONS: In our cohort, half of children with rhabdomyosarcoma had ocular manifestations at presentation with about one-third showing orbital tumor involvement. Cranial nerve involvement carried a significantly worse prognosis for survival.

Assessment of the effectiveness of weight-adjusted antibiotic administration, for reduced duration, in surgical prophylaxis of primary hip and knee arthroplasty.

BACKGROUND: Prophylactic antibiotics have significantly led to a reduction in the risk of post-operative surgical site infections (SSI) in orthopaedic surgery. The aim of using antibiotics for this purpose is to achieve serum and tissue drug levels that exceed, for the duration of the operation, the minimum inhibitory concentration of the likely organisms that are encountered. Prophylactic antibiotics reduce the rate of SSIs in lower limb arthroplasty from between 4% and 8% to between 1% and 3%. Controversy, however, still surrounds the optimal frequency and dosing of antibiotic administration. AIM: To evaluate the impact of introduction of a weight-adjusted antibiotic prophylaxis regime, combined with a reduction in the duration of administration of post-operative antibiotics on SSI incidence during the 2 years following primary elective total hip and knee arthroplasty. METHODS: Following ethical approval, patients undergoing primary total hip arthroplasty (THA)/total knee arthroplasty (TKA) with the old regime (OR) of a preoperative dose [cefazolin 2 g intravenously (IV)], and two subsequent doses (2 h and 8 h), were compared to those after a change to a new regime (NR) of a weight-adjusted preoperative dose (cefazolin 2 g IV for patients < 120 kg; cefazolin 3g IV for patients > 120 kg) and a post-operative dose at 2 h. The primary outcome in both groups was SSI rates during the 2 years post-operatively. RESULTS: A total of n = 1273 operations (THA n = 534, TKA n = 739) were performed in n = 1264 patients. There was no statistically significant difference in the rate of deep (OR 0.74% (5/675) vs NR 0.50% (3/598); fishers exact test P = 0.72), nor superficial SSIs (OR 2.07% (14/675) vs NR 1.50% (9/598); chi-squared test P = 0.44) at 2 years post-operatively. With propensity score weighting and an interrupted time series analysis, there was also no difference in SSI rates between both groups [RR 0.88 (95%CI 0.61 to 1.30) P = 0.46]. CONCLUSION: A weight-adjusted regime, with a reduction in number of post-operative doses had no adverse impact on SSI incidence in this population.

A Comparison of Chemodnervation to Incisional Surgery for Acute, Acquired, Comitant Esotropia: An International Study.

PURPOSE: To compare the efficacy of botulinum toxin injections to strabismus surgery in children with acute, acquired, comitant esotropia (ACE), and to investigate factors predicting success. DESIGN: International, multi-center nonrandomized comparative study METHODS: Setting: Cloud-based survey. STUDY POPULATION: Children aged 2 to 17 years who underwent a single surgical intervention for ACE. INTERVENTIONS: Botulinum toxin injection ("chemodenervation" group) or strabismus surgery ("surgery" group). MAIN OUTCOME MEASURES: Primary measure: success rate at 6 months in propensity-matched cohort, defined as total horizontal deviation of 10 prism diopters or less with evidence of binocular single vision. Secondary measure: Risk factors for poor outcomes in the full cohort. RESULTS: Surgeons from 19 centers contributed. There were 74 patients in the chemodenervation group and 97 patients in the surgery group. In the propensity-matched data (n = 98), success rate was not significantly different at 6 months (70.2% vs 79.6%; P = .2) and 12 months (62.9% vs 77.8%; P = .2), but was significantly lower in the chemodenervation group at 24 months (52% vs 86.4%; P = .015). Irrespective of treatment modality, treatment delay was associated with lower success rates at 6 months, with median time from onset to intervention 4.5 months (interquartile range (IQR): 2.1, 6.7) in the success group and 7.7 months (IQR: 5.6, 10.1) in the failure group (P < .001). CONCLUSIONS: In children with ACE, success rate after chemodenervation was similar to that of surgery for up to 12 months but lower at 24 months. Those with prompt intervention and no amblyopia had the most favorable outcomes, regardless of treatment modality.

Diagnostic utility of point-of-care ultrasound and optical coherence tomography for papilloedema in children: a prospective pilot study.

BACKGROUND/AIMS: Papilloedema is an important sign of serious neurological disease, but it can be difficult to detect on funduscopy. The purpose of this study was to determine the diagnostic accuracy of point-of-care ultrasound (POCUS) and optical coherence tomography (OCT) for detecting papilloedema in children. METHODS: This was a prospective observational study at a tertiary care paediatric hospital. Patients were eligible for the study if they underwent a lumbar puncture with opening pressure and had high-quality POCUS and OCT imaging. RESULTS: A total of 63 eyes from 32 patients were included in the study, 41 (65%) with papilloedema and 22 (35%) without. There were statistically significant differences between the groups in the optic disc elevation (ODE) (p<0.001) and optic nerve sheath diameter (ONSD) (p<0.001) on POCUS, and in the average retinal nerve fibre layer (rNFL) thickness on OCT (p<0.001). Average rNFL thickness had the highest diagnostic accuracy with an area under the curve (AUC) of 0.999 and a 100% sensitivity and 95% specificity for papilloedema (threshold value of ≥108 µm). ODE had an AUC of 0.866 and a 93% sensitivity and 55% specificity (threshold value of ≥0.5 mm). ONSD had an AUC of 0.786 and a 93% sensitivity and 45% specificity (threshold value of ≥5.5 mm). CONCLUSION: Both OCT and POCUS are potentially useful tools to help diagnose papilloedema in children. Larger studies are needed to further define the role and accuracy of POCUS and OCT in assessing papilloedema in children.

Acquired myelinated retinal nerve fiber layer in a child with neurofibromatosis 1-related optic pathway glioma.

Myelinated retinal nerve fiber layer (RNFL) is a rare structural anomaly that occurs from abnormal myelination extending anterior to the lamina cribrosa. Clinically, myelinated RNFL is characterized as a gray-white lesion with feathered, well-demarcated borders obscuring the retinal vasculature. Myelinated RNFL is typically congenital, benign, and asymptomatic. It is most commonly noted as an incidental finding on ophthalmic examination. However, cases of acquired myelinated RNFL have been reported. We report the case of a patient with neurofibromatosis type 1 and optic pathway glioma with unilateral acquired myelinated RNFL.

Risk of serious intracranial pathology in children presenting with acute acquired comitant esotropia.

OBJECTIVE: Acute acquired comitant esotropia (AACE) is defined as a sudden-onset constant nonaccommodative esodeviation. The purpose of this study was to determine the risk of serious intracranial pathology in children presenting with AACE. DESIGN: Retrospective observational cohort study. SETTING: Tertiary care pediatric hospital. METHODS: The study included consecutive children who met the diagnostic criteria for AACE and had neuroimaging at a tertiary care pediatric hospital between 2000 and 2020. Patients were identified by searching the radiology database for all children who underwent neuroimaging for esotropia. The primary outcome measure was the proportion of patients with serious intracranial pathology. Secondary outcomes included risk factors for finding likely causative intracranial pathology and the proportion of patients with incidental findings. RESULTS: A total of 107 patients met the inclusion criteria. Most of the patients (75.7%) had normal neuroimaging. The next most common result was an incidental finding unrelated to the esotropia (18.7%). Five patients (4.7%) had findings with uncertain contribution to esotropia, including 3 cases of type I Chiari malformation. A serious intracranial pathology was found in 1 patient (0.9%) who had a cerebellar medulloblastoma. CONCLUSION: In this large series of pediatric patients with AACE who underwent neuroimaging, there was a small but nontrivial risk of serious intracranial pathology. In these patients, it is advisable either to obtain neuroimaging or to monitor closely for the development of concerning signs or symptoms.

Ensemble of deep convolutional neural networks is more accurate and reliable than board-certified ophthalmologists at detecting multiple diseases in retinal fundus photographs.

AIMS: To develop an algorithm to classify multiple retinal pathologies accurately and reliably from fundus photographs and to validate its performance against human experts. METHODS: We trained a deep convolutional ensemble (DCE), an ensemble of five convolutional neural networks (CNNs), to classify retinal fundus photographs into diabetic retinopathy (DR), glaucoma, age-related macular degeneration (AMD) and normal eyes. The CNN architecture was based on the InceptionV3 model, and initial weights were pretrained on the ImageNet dataset. We used 43 055 fundus images from 12 public datasets. Five trained ensembles were then tested on an 'unseen' set of 100 images. Seven board-certified ophthalmologists were asked to classify these test images. RESULTS: Board-certified ophthalmologists achieved a mean accuracy of 72.7% over all classes, while the DCE achieved a mean accuracy of 79.2% (p=0.03). The DCE had a statistically significant higher mean F1-score for DR classification compared with the ophthalmologists (76.8% vs 57.5%; p=0.01) and greater but statistically non-significant mean F1-scores for glaucoma (83.9% vs 75.7%; p=0.10), AMD (85.9% vs 85.2%; p=0.69) and normal eyes (73.0% vs 70.5%; p=0.39). The DCE had a greater mean agreement between accuracy and confident of 81.6% vs 70.3% (p<0.001). DISCUSSION: We developed a deep learning model and found that it could more accurately and reliably classify four categories of fundus images compared with board-certified ophthalmologists. This work provides proof-of-principle that an algorithm is capable of accurate and reliable recognition of multiple retinal diseases using only fundus photographs.

Impact of extended-infusion piperacillin-tazobactam in a Canadian community hospital.

Background: Studies have demonstrated improved clinical outcomes with extended infusion (EI) piperacillin/tazobactam (TZP) compared to standard infusion (SI). However, there is less evidence on its benefits in noncritically-ill patients. Hospital-wide EI TZP was implemented at our site on February 21, 2012. Our objectives were to compare clinical, safety and economic outcomes between EI and SI TZP. Methods: A retrospective cohort study of all adult patients who received EI TZP (3.375 g IV q8h infused over 4 hours and SI TZP for ≥ 48 hours during 3 years pre-and postimplementation was conducted. The primary study outcome was 14-day mortality while secondary outcomes included length of hospital stay (LOS), nursing plus pharmacy cost, occurrence of Clostridioides difficile infection, readmission within 30 days and change in Pseudomonas aeruginosa minimum inhibitory concentration (MIC) distribution for TZP. The primary outcome and binary secondary outcomes were analyzed using a logistic regression model. LOS was examined using time to event analysis. Cost was examined using linear regression modelling. Results: Overall, 2034 patients received EI TZP and 1364 patients received SI TZP. EI TZP was associated with lower odds of mortality (OR 0.76, 95% CI 0.63-0.91), lower odds of C. difficile infection (OR 0.59, 95% CI 0.41-0.84) and 8% lower cost (estimate 0.92, 95% CI 0.87-0.98) compared to SI TZP. Conclusions: Hospital-wide implementation of EI TZP was associated with lower odds of 14-day mortality and incidence of C. difficile infection with cost savings at our institution.

Association Between Vitamin Deficiencies and Ophthalmological Conditions.

Vitamin deficiencies can have adverse effects on health, including on the visual system. The ocular manifestations of a vitamin deficiency are related to the underlying biochemical function of the particular nutrient. While vitamin deficiencies are not common in developed counties, they are still prevalent in parts of the developing world and in specific, vulnerable populations. Vitamin deficiencies can cause or contribute to many ophthalmological conditions and eye diseases may even be the first presenting finding of a vitamin deficiency. As such, it is important for ophthalmologists to be aware of the ocular manifestations of vitamin deficiencies, especially given that the complications can be severe and effectively treated if identified early. This review summarizes the literature on the main vitamins known to have characteristic ocular manifestations: vitamins A, B1, B2, B9, B12, C, D, E and K. The function, epidemiology, manifestations, workup, and management of each vitamin is discussed in detail.

Evaluating a pilot, structured, face-to-face, antimicrobial stewardship, prospective audit-and-feedback program in emergency general surgery service in a community hospital.

Background: Prospective audit and feedback (PAF) is an established practice in critical care settings but not in surgical populations. We pilot-tested a structured face-to-face PAF program for our acute-care surgery (ACS) service. Methods: This was a mixed-methods study. For the quantitative analysis, the structured PAF period was from August 1, 2017, to April 30, 2019. The ad hoc PAF period was from May 1, 2019, to January 31, 2021. Interrupted time-series segmented negative binomial regression analysis was used to evaluate change in antimicrobial usage measured in days of therapy per 1,000 patient days for all systemic and targeted antimicrobials. Secondary outcomes included C. difficile infections, length of stay and readmission within 30 days. Each secondary outcome was analyzed using a logistic regression or negative binomial regression model. For the qualitative analyses, all ACS surgeons and trainees from November 23, 2015, to April 30, 2019, were invited to participate in an email-based anonymous survey developed using implementation science principles. Responses were measured using counts. Results: In total, 776 ACS patients were included in the structured PAF period and 783 patients were included in the in ad hoc PAF period. No significant changes in level or trend for antimicrobial usage were detected for all and targeted antimicrobials. Similarly, no significant differences were detected for secondary outcomes. The survey response rate was 25% (n = 10). Moreover, 50% agreed that PAF provided them with skills to use antimicrobials more judiciously, and 80% agreed that PAF improved the quality of antimicrobial treatment for their patients. Conclusion: Structured PAF showed clinical outcomes similar to ad hoc PAF. Structured PAF was well received and was perceived as beneficial by surgical staff.

A dynamical graph-based feature extraction approach to enhance mental task classification in brain-computer interfaces.

Graph theoretic approaches in analyzing spatiotemporal dynamics of brain activities are under-studied but could be very promising directions in developing effective brain-computer interfaces (BCIs). Many existing BCI systems use electroencephalogram (EEG) signals to record and decode human neural activities noninvasively. Often, however, the features extracted from the EEG signals ignore the topological information hidden in the EEG temporal dynamics. Moreover, existing graph theoretic approaches are mostly used to reveal the topological patterns of brain functional networks based on synchronization between signals from distinctive spatial regions, instead of interdependence between states at different timestamps. In this study, we present a robust fold-wise hyperparameter optimization framework utilizing a series of conventional graph-based measurements combined with spectral graph features and investigate its discriminative performance on classification of a designed mental task in 6 participants with amyotrophic lateral sclerosis (ALS). Across all of our participants, we reached an average accuracy of 71.1%±4.5% for mental task classification by combining the global graph-based measurements and the spectral graph features, higher than the conventional non-graph based feature performance (67.1%±7.5%). Compared to using either one of the graphic features (66.3%±6.5% for the eigenvalues and 65.9%±5.2% for the global graph features), our feature combination strategy shows considerable improvement in both accuracy and robustness performance. Our results indicate the feasibility and advantage of the presented fold-wise optimization framework utilizing graph-based features in BCI systems targeted at end-users.

Bilateral compressive optic neuropathy and outer retinopathy due to optic canal hyperostosis in a child with isolated vitamin a deficiency.

PURPOSE: Vitamin A plays a crucial role in rod phototransduction, with deficient levels manifesting as night blindness. Animal models have demonstrated bone dysplasia in the setting of hypovitaminosis A. We present a rare case of bony overgrowth leading to bilateral compressive optic neuropathy, combined with outer retinopathy, in a paediatric patient secondary to isolated vitamin A deficiency. METHODS: A single case report was conducted from Toronto, Canada. RESULTS: A 12-year-old boy with known autism spectrum disorder presented with a 9-month history of progressive painless vision loss. Vision was 20/300 and hand motion in the right and left eye, respectively. Fundus photography demonstrated bilateral optic atrophy and yellow lesions notably in the right eye far periphery. Optical coherence tomography (OCT) imaging demonstrated thinning of the retinal nerve fibre layer, alterations in the ellipsoid zone, as well as retinal pigment epithelium deposits. Computed tomography imaging demonstrated sphenoid bone thickening with narrow optic canals and moderate optic atrophy bilaterally. Full-field electroretinogram (ERG) demonstrated mildly reduced dark adapted (DA) 0.01 b-wave amplitudes and electronegative configuration of DA 3.0 and DA 10.0 ERG; the light adapted ERGs were normal. The patient was treated with pulse vitamin A therapy. Subsequently, the DA ERG normalized, outer retinal changes reversed and vision stabilised; no surgical intervention was conducted. CONCLUSION: This case represents a rare presentation of compressive optic neuropathy with concomitant outer retinopathy secondary to isolated vitamin A deficiency. Despite improvement in outer retinal integrity on OCT imaging and ERG testing results following vitamin A supplementation, no functional improvement was obtained due to severe optic atrophy.

Trends in hospital antibiotic utilization during the coronavirus disease 2019 (COVID-19) pandemic: A multicenter interrupted time-series analysis.

Objective: To describe the evolution of respiratory antibiotic prescribing during the coronavirus disease 2019 (COVID-19) pandemic across 3 large hospitals that maintained antimicrobial stewardship services throughout the pandemic. Design: Retrospective interrupted time-series analysis. Setting: A multicenter study was conducted including medical and intensive care units (ICUs) from 3 hospitals within a Canadian epicenter for COVID-19. Methods: Interrupted time-series analysis was used to analyze rates of respiratory antibiotic utilization measured in days of therapy per 1,000 patient days (DOT/1,000 PD) in medical units and ICUs. Each of the first 3 waves of the pandemic were compared to the baseline. Results: Within the medical units, use of respiratory antibiotics increased during the first wave of the pandemic (rate ratio [RR], 1.76; 95% CI, 1.38-2.25) but returned to the baseline in waves 2 and 3 despite more COVID-19 admissions. In ICU, the use of respiratory antibiotics increased in wave 1 (RR, 1.30; 95% CI, 1.16-1.46) and wave 2 of the pandemic (RR, 1.21; 95% CI, 1.11-1.33) and returned to the baseline in the third wave, which had the most COVID-19 admissions. Conclusions: After an initial surge in respiratory antibiotic prescribing, we observed the normalization of prescribing trends at 3 large hospitals throughout the COVID-19 pandemic. This trend may have been due to the timely generation of new research and guidelines developed with frontline clinicians, allowing for the active application of new research to clinical practice.

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.

Evaluation of different antimicrobial stewardship models at a rehabilitation hospital: An interrupted time series (ITS) study.

Objective: To evaluate different prospective audit-and-feedback models on antimicrobial prescribing at a rehabilitation hospital. Design: Retrospective interrupted time series (ITS) and qualitative methods. Setting: A 178-bed rehabilitation hospital within an academic health sciences center. Methods: ITS analysis was used to analyze monthly days of therapy (DOT) per 1,000 patient days (PD) and monthly urine cultures ordered per 1,000 PD. We compared 2 sequential intervention periods to the baseline: (1) a period when a dedicated antimicrobial stewardship (AMS) pharmacist performed prospective audit and feedback and provided urine culture education followed by (2) a period when ward pharmacists performing audit and feedback. We conducted an electronic survey with physicians and semistructured interviews with pharmacists, respectively. Results: Audit and feedback conducted by an AMS pharmacist resulted in a 24.3% relative reduction in total DOT per 1,000 PD (incidence rate ratio [IRR], 0.76; 95% confidence interval [CI], 0.58-0.99; P = .04), whereas we detected no difference between ward pharmacist audit and feedback and the baseline (IRR, 1.20; 95% CI, 0.53-2.70; P = .65). We detected no statistically significant change in monthly urine-culture orders between the AMS pharmacist period and the baseline (level coefficient, 0.81; 95% CI, 0.65-1.01; P = .07). Compared to baseline, the ward pharmacist period showed a statistically significant increase in urine-culture ordering over time (slope coefficient, 1.04; 95% CI, 1.01-1.08; P = .02). The barrier most identified by pharmacists was insufficient time. Conclusions: Audit and feedback conducted by an AMS pharmacist in a rehabilitation hospital was associated with decreased antimicrobial use.

Prevalence and Characteristics of Cytomegalovirus Ocular Disease in Children: A Multi-Center Study.

Purpose: The objective of this study was to identify the prevalence of CMV ocular disease in children and to identify associated risk factors for ocular involvement. Design: Retrospective multicenter, cross-sectional study. Methods: Setting: Hospitalized patients screened for CMV viremia by PCR between 2005 and 2018 at four pediatric referral centers. Participants: Seven-hundred and ninety-three children showed CMV viremia (>135 copies/mL by polymerase chain reaction; PCR). Main Outcomes and Measures: (1) Occurrence of ophthalmologic examination. (2) Presence of CMV ocular disease, defined as retinitis, vasculitis, hemorrhage, optic nerve atrophy, or anterior uveitis in the setting of CMV viremia without other identifiable causes. Results: A total of 296/793 (37%) underwent ophthalmologic examination following CMV viremia. A total of23/296 patients (8%) had ocular symptoms prompting evaluation while the rest had eye exams for baseline screening unrelated to CMV viremia. Of these, 13 cases (4% of those with an eye exam) with ocular disease were identified (three congenital CMV, five severe combined immunodeficiency disorder (SCID) status post-stem cell transplantation, three hematologic malignancy status post-stem cell transplantation for two of them, one Evans syndrome status post-stem cell transplantation, and one medulloblastoma status post-bone marrow transplantation). No patients with solid organ transplantation developed CMV ocular disease in our cohort. Conclusion: CMV ocular disease was a rare occurrence in this cohort without an identifiable pattern across sub-groups. Excluding the three congenital CMV cases, nine out of ten patients with CMV ocular disease were status post-stem cell transplantation. We provide integrated screening guidelines based on the best available evidence for this rare condition.

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.