Updated clinical practice recommendations for managing children with 22q11.2 deletion syndrome.

This review aimed to update the clinical practice guidelines for managing children and adolescents with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society, the international scientific organization studying chromosome 22q11.2 differences and related conditions, recruited expert clinicians worldwide to revise the original 2011 pediatric clinical practice guidelines in a stepwise process: (1) a systematic literature search (1992-2021), (2) study selection and data extraction by clinical experts from 9 different countries, covering 24 subspecialties, and (3) creation of a draft consensus document based on the literature and expert opinion, which was further shaped by survey results from family support organizations regarding perceived needs. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text reviews, including 1545 meeting criteria for potential relevance to clinical care of children and adolescents. Informed by the available literature, recommendations were formulated. Given evidence base limitations, multidisciplinary recommendations represent consensus statements of good practice for this evolving field. These recommendations provide contemporary guidance for evaluation, surveillance, and management of the many 22q11.2DS-associated physical, cognitive, behavioral, and psychiatric morbidities while addressing important genetic counseling and psychosocial issues.

Deep Learning Approach for Differentiating Etiologies of Pediatric Retinal Hemorrhages: A Multicenter Study.

Retinal hemorrhages in pediatric patients can be a diagnostic challenge for ophthalmologists. These hemorrhages can occur due to various underlying etiologies, including abusive head trauma, accidental trauma, and medical conditions. Accurate identification of the etiology is crucial for appropriate management and legal considerations. In recent years, deep learning techniques have shown promise in assisting healthcare professionals in making more accurate and timely diagnosis of a variety of disorders. We explore the potential of deep learning approaches for differentiating etiologies of pediatric retinal hemorrhages. Our study, which spanned multiple centers, analyzed 898 images, resulting in a final dataset of 597 retinal hemorrhage fundus photos categorized into medical (49.9%) and trauma (50.1%) etiologies. Deep learning models, specifically those based on ResNet and transformer architectures, were applied; FastViT-SA12, a hybrid transformer model, achieved the highest accuracy (90.55%) and area under the receiver operating characteristic curve (AUC) of 90.55%, while ResNet18 secured the highest sensitivity value (96.77%) on an independent test dataset. The study highlighted areas for optimization in artificial intelligence (AI) models specifically for pediatric retinal hemorrhages. While AI proves valuable in diagnosing these hemorrhages, the expertise of medical professionals remains irreplaceable. Collaborative efforts between AI specialists and pediatric ophthalmologists are crucial to fully harness AI's potential in diagnosing etiologies of pediatric retinal hemorrhages.

Prevalence and Causes of Subconjunctival Hemorrhage in Children.

OBJECTIVE: Subconjunctival hemorrhage (SCH) is a reported sign of occult abusive injury, but there are limited published data about SCH during childhood. We sought to determine the prevalence and causes of SCH in children. METHODS: This is a retrospective cross-sectional study of children seen by pediatric ophthalmologists in an outpatient setting over 4 years. Primary outcomes were prevalence and causes of SCH, based on history, physical ocular and nonocular findings, and laboratory and imaging studies. Subconjunctival hemorrhage prevalence was determined including and excluding eye surgery to reduce bias in the prevalence estimate. RESULTS: We studied 33,990 children, who underwent 86,277 examinations (median age, 5 years; range, 2 days to 18 years; 9282 younger than 2 years, 13,447 age 2-7 years, 11,261 age 8-18 years). There were 949 cases of SCH (1.1%; 95% confidence interval, 1.0-1.2). When surgery was excluded, there were 313 cases (prevalence, 0.4%; 95% confidence interval, 0.3-0.4), of which 261 (83%) were due to trauma; 40 (13%) ocular surface inflammation, including infectious conjunctivitis; 7 (2%) orbital or conjunctival lesion; 3 (1%) vessel rupture from choking or cough; and 2 (1%) coagulopathy related. Across all ages, including less than 2 years, trauma and inflammation together accounted for 94% to 97% of all cases of SCH. CONCLUSIONS: Subconjunctival hemorrhage is uncommon in children. The great majority of cases are due to trauma. All children with SCH, including infants and young children, should be closely examined to identify other ocular or nonocular signs of trauma.

What is new with 22q? An update from the 22q and You Center at the Children's Hospital of Philadelphia.

22q11.2 deletion syndrome (22q11.2DS) is a disorder caused by recurrent, chromosome-specific, low copy repeat (LCR)-mediated copy-number losses of chromosome 22q11. The Children's Hospital of Philadelphia has been involved in the clinical care of individuals with what is now known as 22q11.2DS since our initial report of the association with DiGeorge syndrome in 1982. We reviewed the medical records on our continuously growing longitudinal cohort of 1,421 patients with molecularly confirmed 22q11.2DS from 1992 to 2018. Most individuals are Caucasian and older than 8 years. The mean age at diagnosis was 3.9 years. The majority of patients (85%) had typical LCR22A-LCR22D deletions, and only 7% of these typical deletions were inherited from a parent harboring the deletion constitutionally. However, 6% of individuals harbored other nested deletions that would not be identified by traditional 22q11.2 FISH, thus requiring an orthogonal technology to diagnose. Major medical problems included immune dysfunction or allergies (77%), palatal abnormalities (67%), congenital heart disease (64%), gastrointestinal difficulties (65%), endocrine dysfunction (>50%), scoliosis (50%), renal anomalies (16%), and airway abnormalities. Median full-scale intelligence quotient was 76, with no significant difference between individuals with and without congenital heart disease or hypocalcemia. Characteristic dysmorphic facial features were present in most individuals, but dermatoglyphic patterns of our cohort are similar to normal controls. This is the largest longitudinal study of patients with 22q11.2DS, helping to further describe the condition and aid in diagnosis and management. Further surveillance will likely elucidate additional clinically relevant findings as they age.

Ocular Complications in Children with Diabetes Mellitus.

PURPOSE: The effectiveness of annual eye examinations in diabetic children is unclear. We sought to determine the prevalence and onset of ocular pathology in children with diabetes mellitus (DM), identify risk factors for ocular disease, and recommend a screening regimen for asymptomatic children. DESIGN: Retrospective, consecutive cohort study. PARTICIPANTS: Children aged less than 18 years with type 1 or 2 DM examined over a 4-year period. METHODS: All children underwent a complete eye examination, including dilated fundoscopy and cycloplegic refraction. A literature review was performed, identifying the youngest reported age and shortest reported duration of DM before the diagnosis of diabetic retinopathy (DR). MAIN OUTCOME MEASURES: Prevalence of DR, cataract, high refractive error, and strabismus. RESULTS: A total of 370 children (mean age, 11.2 years; range, 1-17.5 years) had 693 examinations, with a mean DM duration of 5.2 years (range, 0.1-16.2 years) and a mean hemoglobin A1c (HbA1c) of 8.6 (range, 5-≥14). No children had DR. A total of 12 children had cataract; 5 required extraction but were identified by decreased vision, not diabetic screening. A total of 19 children had strabismus; only 1 was microvascular paralytic strabismus. A total of 41 children had high refractive error. There were no associations between these conditions and duration or control of DM. In the literature, the youngest age at diagnosis of severe DR was 15 years, and the shortest duration of disease was 5 years. CONCLUSIONS: Diabetic retinopathy is rare in children regardless of duration and control of DM. On the basis of our study and literature review, screening examinations for type 1 diabetes could begin at age 15 years or at 5 years after the diagnosis of DM, whichever occurs later, unless the child is judged by the endocrinologist as being at unusually high risk. Other ocular complications are identifiable through existing amblyopia screening methods.

The prevalence of strabismus in unilateral coronal synostosis.

BACKGROUND: While there is a clear correlation between unilateral coronal synostosis (UCS) and ocular motility abnormalities, the literature provides little information as to the true epidemiology of strabismus, or the underlying etiology of these paralleled pathologies. The purpose of this study is to investigate the rate of oculomotor abnormalities associated with UCS and its management. METHODS: A retrospective review of all patients identified to have single-suture, nonsyndromic UCS treated by fronto-orbital advancement at a tertiary craniofacial referral center from 1977 to 2013 was performed. Inclusion criteria mandated complete medical, surgical, and ophthalmological records. Patients were evaluated for strabismus both preoperatively and postoperatively, and as to whether eye muscle surgery was performed. RESULTS: A total of 181 patients underwent treatment for UCS at our institution during the study period, of which 79 met the inclusion criteria. Twenty-nine patients had strabismus prior to any craniofacial surgical intervention. Following fronto-orbital advancement, 23 patients (46 %) developed a new onset strabismus. Fifty-five patients had no change in their preoperative ocular examination, and one patient had resolution of preoperative strabismus. Of the 51 patients who had postoperative strabismus, 30 went on to have eye muscle surgery. There were no statistically significant differences in gender (p=0.477), race (p=0.395), sidedness of suture involvement (p=0.552), or age at intervention (p=0.66) in comparing the group with new postoperative strabismus and those without. CONCLUSIONS: This study sheds new light on the prevalence of strabismus in UCS, and more importantly, the risk of developing strabismus in the setting of conventional fronto-orbital advancement. This data will allow more accurate preoperative counseling and reinforces the important role of ophthalmologists as members of the multidisciplinary craniofacial team.

The eye in child abuse: key points on retinal hemorrhages and abusive head trauma.

This review presents an up-to-date overview of ocular injuries resulting from child abuse, with a spotlight on abusive head trauma. Retinal hemorrhage is a principle finding of inflicted head trauma. The specific pattern of hemorrhages holds valuable diagnostic information, which can help to guide multidisciplinary assessments of the likelihood of abuse. Indirect ophthalmoscopy through dilated pupils by an ophthalmologist is necessary for adequate examination and documentation of retinal findings. Initial pediatrician evaluation of the eye and indications for ophthalmological consultation are reviewed. Focus is then placed upon understanding retinal hemorrhage patterns, their diagnostic significance and likely pathophysiological mechanisms. The differential diagnosis of retinal hemorrhage in young children is discussed, highlighting key distinctions among retinal hemorrhage patterns, severity and frequencies, as well as other ocular findings. The most common cause of retinal hemorrhage in an infant is trauma, and most other causes can be identified by considering the hemorrhage pattern, ocular or systemic signs and the results of laboratory and imaging tests, when indicated.

Retinal hemorrhage after pediatric neurosurgical procedures.

BACKGROUND: Neurosurgical procedures may occur prior to eye examination in children with suspected abusive head trauma and raise questions by child abuse physicians and ophthalmologists regarding the contribution of neurosurgery to retinal hemorrhage found postoperatively. The purpose of this study was to determine the prevalence and patterns of retinal hemorrhage attributable to neurosurgical intervention in children. METHODS: We conducted a retrospective cohort study of children undergoing neurosurgery who had postoperative ophthalmoscopy. Some children were also examined preoperatively. Primary outcome measures were the prevalence and patterns of retinal hemorrhage attributable to neurosurgical intervention. Medical records were reviewed to identify confounding coexistent diseases. RESULTS: Among 267 children undergoing 289 neurosurgical procedures, there were no cases in which children had post-procedural retinal hemorrhage that could be attributed to neurosurgery. Retinal hemorrhage was seen in 32 (12%) cases, but in every case they were either already present on preoperative examination (13 cases) or matched the pattern of a coexistent known cause of retinal hemorrhage, including head trauma with unambiguous history and/or nonocular signs (13), hydrocephalus-related increased intracranial pressure with papilledema-associated peripapillary retinal hemorrhage (5), and retinopathy of prematurity ridge-associated retinal hemorrhage (1). No retinal hemorrhage could be attributed only to neurosurgery. CONCLUSIONS: Although children undergoing child abuse evaluations may have intracranial hemorrhage requiring neurosurgery that occurs before a dilated retinal examination can be performed, our data suggest that neurosurgery independently is unlikely to produce retinal hemorrhage and therefore is not a significant confounding factor in the interpretation of retinal hemorrhage patterns in child abuse evaluations.

Patterns of retinal hemorrhage associated with cardiac arrest and cardiopulmonary resuscitation.

BACKGROUND: Hypoxia and cardiopulmonary resuscitation (CPR) have been proposed as causes of retinal hemorrhage (RH) in children evaluated for abusive head trauma (AHT). We sought to determine the prevalence and characteristics of RH in children who underwent CPR after cardiac arrest. METHODS: This was a prospective, single-center, consecutive observational study of 38 children (<18 years of age). Indirect ophthalmoscopic examination was completed by an ophthalmologist within 48 hours of CPR. Extensive medical records data were collected to assess for potential confounding factors. Outcomes included the presence and pattern of RH. RESULTS: Of the 38 children, 20 had in-hospital arrest; 18 had out-of-hospital arrest. The median duration of CPR was 10 minutes. Seven children had RH, of whom 6 had an RH pattern consistent with coexistent medical conditions: 4 AHT diagnosable with nonocular findings, including subdural and subarachnoid hemorrhage, rib fractures, abdominal injury (RH pattern: diffuse, numerous, intraretinal and/or multilayered RH); 1 septic shock (RH pattern: 1-2 posterior pole RH); 1 ruptured arteriovenous malformation (RH pattern: 4-8 peripapillary RH). The seventh child had unwitnessed cardiac arrest due to nonfatal drowning and a single superficial intraretinal peripapillary hemorrhage. CONCLUSIONS: CPR for cardiac arrest is rarely associated with RH, which, absent coexisting conditions causing retinal hemorrhage, are intraretinal, few in number, and located in the posterior pole. In children who have undergone CPR, when RH are multilayered, or are more than a few in number, or extend outside the posterior pole, another etiology for the RH should be sought.

Periocular ulcerative dermatitis associated with gentamicin ointment prophylaxis in newborns.

A recent shortage of erythromycin ointment has resulted in the use of alternative agents for newborn ocular infection prophylaxis in the United States. We report a series of 26 newborns in whom a characteristic periocular ulcerative dermatitis developed after gentamicin ointment administration at 2 Philadelphia hospitals.

Congenital craniofacial anomalies.

PURPOSE OF REVIEW: Congenital abnormalities of the cranium and face present complex diagnostic and therapeutic challenges to the ophthalmologist. RECENT FINDINGS: Patients with craniofacial anomalies are best treated by a multidisciplinary team that includes specialists from plastic surgery, neurosurgery, ophthalmology, otolaryngology, oromaxillofacial surgery, orthodontics, anesthesia, and genetics as well as specialists in support disciplines such as nursing, social work, and nutrition. Other subspecialty needs may be important to address behavioral and developmental issues as well as associated systemic anomalies and medical conditions. The specialists must work together to provide for the overall well being of the patient. To assist in the evaluation and treatment of these patients, the ophthalmologist must possess an understanding of craniofacial syndromes as well as the necessary medical and surgical interventions required to improve ocular and adnexal problems. SUMMARY: This article systematically reviews the major craniofacial anomalies of ophthalmic importance and highlights salient treatment issues.

Cut and paste: sutureless conjunctival closure in strabismus surgery.

PURPOSE: This study investigated the use of fibrin glue and compared its effect with traditional sutures for conjunctival closure in strabismus surgery. METHODS: The study included 12 patients undergoing horizontal strabismus surgery, of whom 5 underwent bilateral medial rectus muscle recessions and 7 underwent bilateral lateral rectus muscle recessions. For each patient, fibrin glue was used to close the conjunctiva of one eye and 6-0 plain sutures were used to close the other. RESULTS: All eyes maintained adequate closure of the conjunctiva postoperatively and there were no intraoperative or postoperative complications for an eye. However, the average surgical time needed to apply fibrin glue was considerable less than that required for closure with sutures. Furthermore, eyes closed with fibrin glue were associated with significantly less postoperative inflammation and patient discomfort than those closed with sutures. All patients and parents reported significantly less discomfort from the eyes treated with fibrin glue. CONCLUSION: These results are promising and demonstrated a safe and effective alternative to traditional suture closure in strabismus surgery. When compared to traditional suture closure, conjunctival closure with glue includes the following advantages: less postoperative patient discomfort, diminished postoperative inflammation, and potentially reduced surgical time with corresponding reduced time under general anesthesia.

Healthcare disparities for women hospitalized with myocardial infarction and angina.

AIMS: Ischaemic heart disease persists as the leading cause of death in both men and women in most countries and sex disparities, defined as differences in health outcomes and their determinants, may be relevant. We examined sex disparities in presenting characteristics, treatment and all-cause mortality in patients hospitalized with myocardial infarction (MI) or angina. METHODS AND RESULTS: We conducted a cohort study of all patients admitted with MI or angina (01 October 2013 to 30 June 2016) from a secondary care acute coronary syndrome e-Registry in NHS Scotland linked with national registers of community drug dispensation and mortality data. A total of 7878 patients hospitalized for MI or angina were prospectively included; 3161 (40%) were women. Women were older, more deprived, had a greater burden of comorbidity, were more often treated with guideline-recommended therapy preadmission and less frequently received immediate invasive management. Men were more likely to receive coronary angiography [adjusted odds ratio (OR) 1.52, confidence interval (CI) 1.37-1.68] and percutaneous coronary intervention (adjusted OR 1.68, CI 1.52-1.86). Women were less comprehensively treated with evidence-based therapies post-MI. Women had worse crude survival, primarily those with ST-elevation myocardial infarction (14.3% vs. 8.0% at 1 year, P < 0.001), but this finding was explained by differences in baseline factors. Men with non-ST-elevation myocardial infarction had a higher risk of all-cause death at 30 days [adjusted hazard ratio (HR) 1.72, CI 1.16-2.56] and 1 year (adjusted HR 1.38, CI 1.12-1.69). CONCLUSION: After taking account of baseline risk factors, sex differences in treatment pathway, use of invasive management, and secondary prevention therapies indicate disparities in guideline-directed management of women hospitalized with MI or angina.

Amblyopia treatment: 1998 versus 2004.

PURPOSE: To determine whether there has been a change in treatment practice patterns of patients with amblyopia between the late 1990s and 2004. METHODS: A questionnaire survey was mailed to 1,200 AAPOS members listed in the 2004 AAPOS directory. Seven scenarios were presented that described patients with amblyopia and the clinician was asked to choose from six treatment options. Respondents were asked to indicate their preferred initial treatment in 1998 (or during their initial year of practice if later than 1998) and in 2004. The scenarios were not necessarily those of patients who would meet the eligibility criteria for the Amblyopia Treatment Studies because they also included scenarios to assess the impact of amblyopia treatments in general. RESULTS: Three hundred eighty-nine surveys (33.1%) were returned. In four of the seven scenarios, comments suggested that a change in practice was attributable to recent publications of Pediatric Eye Disease Investigator Group trials. In all seven scenarios, atropine would have been offered in 2004 as an alternative to patching in 1998, and in five of the seven scenarios the combination of simultaneous atropine and patching would have been prescribed. In six of the seven scenarios, some type of nonspecific near work would now be prescribed as an adjunct treatment. CONCLUSION: A change in practice patterns was observed for some, but not all, scenarios. In many scenarios, this change was directly attributed to the recent Pediatric Eye Disease Investigator Group trials.

An update of ophthalmic management in craniosynostosis.

Craniosynostosis has a varied clinical spectrum, ranging from isolated single suture involvement to multisutural fusions. Syndromic and nonsyndromic patients require orchestrated and multidisciplinary care from birth to adulthood. Advances in our understanding of craniosynostosis over the last quarter-century have resulted in more systematic management of the problems associated with the syndromic and nonsyndromic forms of this condition. This review provides an update on the genetic basis of, management of strabismus and oculoplastic manifestations in, and visual surveillance of patients with craniosynostosis.

Retinal Findings in Young Children With Increased Intracranial Pressure From Nontraumatic Causes.

OBJECTIVES: Increased intracranial pressure (ICP) has been suggested in legal settings as an alternative cause of retinal hemorrhages (RHs) in young children who may have sustained abusive head trauma. We assessed the prevalence and characteristics of RHs in children with increased ICP. METHODS: We conducted a prospective, multicenter study of children <4 years old with newly diagnosed increased ICP as determined by using direct measurement and/or clinical criteria. Infants who were premature, neonates, and suspected survivors of abusive head trauma were excluded on the basis of nonocular findings. Fundus examinations were performed; extent, number, and type of RH in each of 4 distinct retinal zones were recorded. RESULTS: Fifty-six children (27 boys) were studied (mean age 15.4 months; range 1-43 months). All of the children had elevated ICP that required intervention. One child had papilledema. No child (0%; 95% confidence interval: 0%-6.4%) or eye (0%; 95% confidence interval: 0%-3.3%) was found to have an RH. Causes of increased ICP included hydrocephalus, intraventricular hemorrhage, congenital malformations, malfunctioning shunts, and the presence of intracranial space-occupying lesions. CONCLUSIONS: Although acute increased ICP can present in children with a pattern of peripapillary superficial RHs in the presence of papilledema, our study supports the conclusion that RHs rarely occur in the absence of optic disc swelling and do not present beyond the peripapillary area in the entities we have studied.

Duarte (DG) galactosemia: a pilot study of biochemical and neurodevelopmental assessment in children detected by newborn screening.

UNLABELLED: Newborn screening for galactosemia has shown a high prevalence of partial galactose uridyl transferase deficiencies such as Duarte (DG) galactosemia. STUDY OBJECTIVE: To determine whether (a) there is any clinical impact of DG galactosemia on development (b) there is a relationship between outcome and biochemical parameters in patients who receive no treatment. STUDY POPULATION: Twenty-eight children with DG galactosemia. Group-I-17 children had a lactose restricted diet in the first year of life. Group-II-11 children had a regular diet since birth. METHODS: Developmental, physical, and ophthalmologic assessments were completed on both DG groups. RBC gal-1-p and urine galactitol were monitored during the follow-up visits in every child with DG galactosemia. Gal-1-p, urine galactitol, liver function tests, and FSH were tested at the time of study visit. RESULTS: The groups had statistically significant differences on RBC gal-1-p and urine galactitol at the 2 week, 1 month, 6 month, and 1 year time points. There was no statistical difference of gal-1-p or urine galactitol in group-I and -II at the time of study. The groups had statistically significant differences on adaptive scores, but not on language or IQ. None of the DG subjects had abnormal liver function at the time of diagnosis or the study visit. The FSH levels were normal. There were no statistically significant relationships between the first year metabolic values and developmental outcomes. CONCLUSIONS: The data presented here indicate that clinical and developmental outcomes in DG galactosemics are good regardless of any diet changes.

Sclerocornea associated with the chromosome 22q11.2 deletion syndrome.

Reported ocular findings in the 22q11.2 deletion syndrome (which encompasses the phenotypes of DiGeorge, velocardiofacial, and Takao (conotruncal-anomaly-face) syndromes) have included posterior embryotoxon (prominent, anteriorly displaced Schwalbe's line at the corneal limbus or edge), retinal vascular tortuosity, eyelid hooding, strabismus, and astigmatism. We present seven 22q11.2 patients from multiple centers with sclerocornea, an eye finding previously unreported in the literature. Four boys and three girls were identified with sclerocornea, systemic DGS/VCFS findings, and fluorescence in situ hybridization (FISH)-confirmed microdeletion at chromosome 22q11.2. FISH diagnosis was perinatal in six patients but at 2 years of age in one child. Sclerocornea was bilateral in five patients. Findings included descemetocele (five eyes), microophthalmos (one eye), iridocorneal adhesions (one bilateral case), and severe anterior segment dysgenesis (one eye). Two patients underwent bilateral corneal transplantation; another two were scheduled for possible unilateral transplant. Sclerocornea is a static congenital condition in which the cornea is opaque and vascularized and resembles the sclera. The novel finding of sclerocornea suggests that a genetic locus at 22q11.2 may be involved in anterior segment embryogenesis. In most of our patients, the diagnostic process was underway, but in one patient 22q11.2 deletion was not suspected until after the child had already been undergoing treatment for sclerocornea for 2 years. Sclerocornea should be added to the clinical manifestations of the 22q11.2 deletion syndrome. Ophthalmologists diagnosing sclerocornea in children with systemic findings suggestive of 22q11.2 deletion should ensure appropriate genetic referral.

Linking hospital patient records for suspected or established acute coronary syndrome in a complex secondary care system: a proof-of-concept e-registry in National Health Service Scotland.

Aims: To implement secondary care electronic record linkage for patients hospitalized with suspected or known acute coronary syndrome (ACS) in a complex regional health care system and evaluate this e-Registry in terms of patterns of service delivery and 1-year outcomes. Methods and results: Existing electronic hospital records were linked to create episodes of care using (i) a patient administration system, (ii) invasive cardiovascular procedure referrals, and (iii) a catheter laboratory record. Data were extracted for admissions (1 October 2013-30 September 2014) with International Classification of Disease (ICD)-10 diagnosis of angina (I200-I209), myocardial infarction (I210-I229), other ischaemic heart disease (I240-I249) or heart failure (I50), linked to other sources to develop a secondary care ACS e-registry and analysed within a Safe Haven. Episodes of care were categorized into care pathways and evaluated in terms of patient characteristics, as well as service delivery metrics and outcomes including mortality. In all, 2327 patients had 2472 episodes of care. Diagnoses were hierarchically classified as ST-elevation myocardial infarction (STEMI) (586, 25.2%), non-ST-elevation myocardial infarction (NSTEMI) (1068, 45.9%), unspecified myocardial infarction (146, 6.3%), unstable angina (527, 22.6%) for the first hospitalization for each patient within the study period. Six care pathways were mapped. Percutaneous coronary intervention rate for STEMI was 80.2% and for NSTEMI 33.1%. Unadjusted all-cause mortality was 9.0% and 3.0% for STEMI and NSTEMI at 30 days, rising to 11.9% and 11.6% at 1 year. Analyses were validated by independent source data verification. Conclusion: The e-registry has enabled analysis of ACS hospitalizations in a complex health care system with implications for quality improvement and research.

Factors associated with recurrence of amblyopia on cessation of patching.

PURPOSE: In a prospective observational study, we previously reported that weaning (tapering or gradually reducing) treatment in children treated with 6 to 8 hours of daily patching for amblyopia resulted in a 4-fold reduction in odds of recurrence. We now report the association of additional factors with recurrence or regression of amblyopia in this same cohort. DESIGN: Prospective, nonrandomized, observational study. PARTICIPANTS: Sixty-nine children aged <8 years with successfully treated anisometropic or strabismic amblyopia (improved > or =3 logarithm of the minimum angle of resolution [logMAR] lines). METHODS: Patients were enrolled at the time they stopped patching for amblyopia. Patients were classified according to whether patching was stopped abruptly or weaned before cessation. They were followed off treatment for 52 weeks to assess recurrence of amblyopia. MAIN OUTCOME MEASURE: Recurrence of amblyopia defined as a > or =2-logMAR level reduction of visual acuity from enrollment (cessation of patching) confirmed by a second examination. Recurrence was also considered to have occurred if treatment was restarted with a > or =2-logMAR level reduction of visual acuity, even if it was not confirmed by a second examination. RESULTS: The risk of recurrence was higher with better visual acuity at the time of cessation of treatment (adjusted risk ratio [RR], 0.68 per line of worse visual acuity; 95% confidence interval [CI], 0.51-0.90), a greater number of lines improved during the previous treatment (adjusted RR, 1.5 per line increase; 95% CI, 1.1-2.0), and a history of recurrence (adjusted RR, 2.7; 95% CI, 1.5-4.9). Orthotropia or excellent stereoacuity at the time of patching cessation did not appear to have a protective effect on the risk of recurrence. CONCLUSIONS: The higher risk of recurrence in the most successfully treated children with amblyopia and absence of protection from orthotropia and excellent random dot stereoacuity suggests that careful and prolonged follow-up is needed for all children who have been previously treated for amblyopia.