Managing the apparently blind child presenting in the first year of life: A review.

Severe vision impairment and blindness in childhood have a significant health burden on the child, family and society. This review article seeks to provide a structured framework for managing the apparently blind child presenting in the first year of life, starting from a comprehensive history and examination. Different investigation modalities and the increasingly important role of genetics will also be described, in addition to common causes of severe vision impairment. Crucially, a systematic approach to the blind infant is key to correct diagnoses and timely management. Incorrect diagnoses can be costly to all involved, however it is important to note that diagnoses can change with ongoing follow-up and investigations. Furthermore, the modern age of ophthalmology requires a multi-disciplinary approach and close collaboration with specialists including paediatricians, neurologists and geneticists, in addition to rehabilitation and low vision services, to ensure the best care for these vulnerable infants.

Spectrum of ocular disease in children aged between 0 and 3 years at an Australian paediatric tertiary hospital.

BACKGROUND: Childhood ocular disease can be a significant health burden to the child, family and society. Previous studies have examined the spectrum of paediatric ocular disease presenting to tertiary hospitals; however, these studies have broader age ranges, smaller sample sizes, and are largely based in developing countries. This study aims to assess the spectrum of ocular disease in the first 3 years of life presenting to the eye department of an Australian tertiary paediatric hospital. METHODS: The records of 3337 children who had their initial presentation at the eye clinic between the age of 0 and 36 months were reviewed, spanning 6.5 years from 1st July 2012 to 31st December 2018. RESULTS: The most common primary diagnoses overall were strabismic amblyopia (6.0%), retinopathy of prematurity (5.0%) and nasolacrimal duct obstruction (4.5%). Bilateral visual impairment was more common in younger children, while unilateral visual impairment was more common in older children. The proportion of all children presenting with visual impairment was 10.3%, with 5.7% of all children presenting with bilateral visual impairment and 4.6% presenting with unilateral visual impairment. In children with visual impairment, the most common sites of primary abnormality were lens (21.4%), retina (17.3%), and cerebral and visual pathways (12.1%). The most common primary diagnoses in children with visual impairment were cataract (21.4%), strabismic amblyopia (9.3%) and retinoblastoma (6.5%). CONCLUSIONS: The spectrum of eye disease and vision impairment presenting in the first 3 years of life facilitates health care planning, greater community education about vision impairment and importance of early intervention, and guidance for appropriate resource allocation. Health systems can apply these findings to aid in early identification and intervention to reduce preventable blindness and institute appropriate rehabilitation services.

Acute zonal occult outer retinopathy-like presentation secondary to scleral buckle.

Purpose: To describe a case of acute zonal occult outer retinopathy-like (AZOOR-like) presentation following scleral buckle surgery for rhegmatogenous retinal detachment. Observations: A 48-year-old man underwent successful scleral buckle with cryotherapy for repair of a left eye inferior macula-on rhegmatogenous retinal detachment. Five years later he presented with a six-month history of left peripheral field restriction. Fundus autofluorescence and optical coherence tomography demonstrated degeneration of the photoreceptors in a ring pattern around the left macula. Humphrey visual fields showed functional loss corelating with the imaging, with a paracentral ring scotoma. Electrophysiology demonstrated a delayed 30 Hz flicker latency in the left eye confirming cone system dysfunction. Conclusion and Importance: Scleral buckling surgery for repair of a rhegmatogenous retinal detachment may be associated with a late AZOOR-like presentation.

Neurogenic pulmonary edema.

AIM: We report a case of neurogenic pulmonary edema in a patient who sustained a severe traumatic brain injury in a motorbike accident and review the current literature with regards to the management of neurogenic pulmonary edema (NPE). METHODS: A 17 year old male was involved in a motorbike collision into a tree. Copious amounts of pink frothy sputum was noted on scene. Rapid sequence intubation was performed on scene and video laryngoscopy demonstrated profuse frothy secretions welling up from the larynx. RESULTS: The patient underwent emergency external ventricular drain insertion for intracranial pressure (ICP) monitoring. Intracranial pressures remained refractorily high and a decompressive bifrontal craniectomy was performed on the subsequent day. He was tracheostomised on admission day 24 and discharged from ICU on day 34. DISCUSSION: Neurogenic pulmonary edema is defined as acute respiratory distress triggered by severe sympathetic discharge from acute compromise in the central nervous system. Rapid intervention with intubation is often necessary to protect the airways and facilitate diagnostic evaluation. Reduction of ICP and supportive mechanical ventilation to improve oxygenation is necessary. Positive end-expiratory pressure should be carefully applied to balance recruitment of alveoli and minimisation of ICP. Although NPE is estimated to resolve within 72 h in more than half of patients, prognosis is generally poor due to the severity of the underlying brain injury, with estimated mortality rates of between 60 and 100%. CONCLUSION: Neurogenic pulmonary edema is a potentially life-threatening complication of severe brain injury and should be recognised early to facilitate management.

Parental understanding of relapsing idiopathic nephrotic syndrome-Where are the knowledge gaps?

AIM: To assess parental understanding of idiopathic nephrotic syndrome (INS) and its management, to enable targeted education in areas of deficit. METHODS: Families of children with at least one relapse of INS were interviewed, following a template covering key domains of (a) disease understanding, (b) management of INS and (c) access to information. Common trends and responses were identified and notable observations recorded. RESULTS: Twenty-one parents were interviewed. The mean duration of INS was 4.4 years (range 0.5-14.5 years), with a mean of two steroid-sparing agents used. Although 90% parents self-reported that they understood INS, only 29% could appropriately define relapse and 24% name potential complications. The management of INS was generally good, with most parents appropriately testing urine (81%) and managing relapses (90%). Unnecessary dietary restrictions were imposed on 57% during remission. The Internet was searched by 90% of parents for disease and drug information. Further information was desired in paper form (71%), hospital website (81%) and face-to-face workshop (90%), plus educational materials for schools. CONCLUSION: Parents overestimated their understanding of INS; however, their management was generally well done. Parents desired more information and support in various forms.

International publication trends in hepato-pancreato-biliary surgery: emergency compared to oncology.

BACKGROUND: There is growing interest in publishing trends given the increasing amount of research publication across various specialities. Studies relating to hepato-pancreato-biliary (HPB) surgery show an oncological focus compared to benign, emergency and trauma. METHODS: Analysis of ISI JCR impact factors in the Web of Knowledge and the Scimago Journal Rank through Scopus was performed to select four readily available, relevant and frequently read journals from the surgery category. A total of 5265 articles between 2012 and 2016 were categorized relating to emergency or oncology topics. A secondary analysis of PubMed MeSH term was performed to scrutinize trends of publishing over the period 1960-2016. RESULTS: Of the 5265 articles screened, 2062 related to HPB surgery. Of these, 49% (1007 of 2062) were oncology related. This trend towards oncology-related topics is continued in HPB subspecialty journals where 51% (679 of 1320) of articles are oncology related. Emergency- and trauma-related topics accounted 10% (198 of 2062) overall, whereas in subspecialty journals, they account for 8% (111 of 1320). Secondary analysis of MeSH term trends demonstrated a now stable trend over the last 20 years of liver and pancreatic trauma to oncology ratio of 1:10 publications, and biliary trauma to oncology publishing ratio of 1:5. CONCLUSIONS: Quantitatively oncology topics are published favourably, whereas emergency- and trauma-related HPB articles appear static. This is in keeping with baseline trends over the last five decades. Further analysis could delineate whether publishing in trauma subspecialty journals compensate for this trend.