Etiology of white pupillary reflex in pediatric age group.

Background: Leukocoria means white pupil. Normal pupil appears black in children and adults. The typical red reflex is due to retro-illumination of choroidal vessels reflected via the retina, vitreous humor, crystalline lens, aqueous humor, pupil, and cornea. If there is interference in these structures, it would result in a changed red reflex, or leukocoria. Immediate family members are highly likely to detect the first indicator and the pediatrician or general ophthalmologist is usually the first to be visited. The aim of the study was to find out the prevalence of common causes of white pupillary reflex in children, to undertake early diagnosis and treatment, and to reduce morbidity and death. This study aimed to see how common it is for children to have a white pupillary reflex when they visit a pediatric ophthalmologist. Objective: Determine the incidence of conditions that cause a white pupillary reflex in children who visited Hayatabad Medical Complex Hospital in Peshawar. Materials and methods: This study was carried out in the Ophthalmology unit of HMC Hospital Peshawar, from January 2021 to December 2021. 168 patients were enrolled in the study. We included all patients of up to 10 years and both genders with the above findings. Workup for leukocoria was done to find the exact cause that included fundoscopy, B-Scan, MRI, and CT scans. Examination under anesthesia (EUA) was carried out for uncooperative children for detailed fundus examination. Patient data was recorded and a proforma was made to collect all the necessary information. Family history was taken in detail during this study. Results: The most common cause of aberrant pupillary reflex in children aged 1 to 10 years was cataract, 79.76 percent of patients having it. Retinoblastoma (12.5%), Coats disease (3.5%), retinal detachment (2.9%) and persistent hyperplastic vitreous (PHPV) (1.1%) were other notable causes found. Conclusion: Leukocoria is a critical clinical finding, and if parents or primary care physicians notice it, the patient requires a complete follow-up examination by a pediatric ophthalmologist to determine the etiology.

Síndrome de Marcus-Gunn / Marcus Gunn syndrome

Introducción: El síndrome de Marcus-Gunn se manifiesta con retracción o elevación del párpado ptótico ante la estimulación del músculo pterigoideo del mismo lado y el término "guiño mandibular" es inapropiado ya que el párpado no siempre desciende. Puede producirse en la apertura bucal, masticación, avance mandibular, al sonreír, silbar, bruxar, sacar la lengua, deglutir, chupar, cantar, en la contracción esternocleidomastoidea, maniobra de Valsalva, respirar o inhalar. Es congénito y de modalidad rara y autosómica dominante. Objetivo: Contribuir al conocimiento de esta rara enfermedad y a su relación con el ámbito odontológico. Presentación del caso: Se trata de un niño de 3 años diagnosticado de síndrome de Marcus-Gunn en el periodo neonatal, por la observación de la madre de una apertura palpebral izquierda durante la succión nutritiva. No presenta otras enfermedades ni antecedentes de interés. En la exploración extraoral se advierte una ptosis palpebral derecha y apertura palpebral izquierda en los movimientos mandibulares y deglución. La intensidad del reflejo se incrementa en estados de ansiedad derivados del tratamiento dental. Conclusiones: el síndrome de Marcus-Gunn es una entidad rara en pediatría, en la cual sus hallazgos clínicos determinan el diagnóstico. Teniendo en cuenta que en ocasiones presentan alteraciones oculares, nada nos hace sospechar la presencia de una enfermedad oral específica(AU)

Introduction: Marcus Gunn syndrome manifests with retraction or elevation of the eyelid ptotico while stimulation of the pterygoid muscle on the same side and the term jaw-winking is inappropriate because the eyelid does not always goes down. It can occur during mouth opening, mastication, mandibular advancement, while smiling, whistling, bruxing, sticking out the tongue, swallowing, sucking, singing, during the sternocleidomastoid contraction, the Valsalva maneuver, breathing or inhaling. This syndrome is congenital and rare, and of autosomal dominant modality. Objective: To contribute to the knowledge of this rare disease and its relationship with the odontologic field. Presentation of the case: 3 years old boy diagnosed with Marcus Gunn syndrome in the neonatal period by the observation of the mother of a left palpebral opening during the nutritive sucking. He does not present other diseases or a background of interest. In the extraoral exploration, it is noticed a right palpebral ptosis and a left palpebral opening in the jaw movements and in swallowing. The intensity of the reflex increases in anxiety states arising from the dental treatment. Conclusions: Marcus Gunn syndrome is a rare entity in pediatrics, in which its clinical findings determine the diagnosis. Taking into account that sometimes it presents ocular alterations, nothing makes us suspect the presence of a specific oral disease(AU)

Deep coma does not always predict poor outcomes among patients with polytrauma.

PURPOSES: This study aimed to clarify the prognosis of polytrauma patients presenting to the emergency department (ED) with a Glasgow Coma Scale score (GCS) of 3. METHODS: A trauma registry system has been established at our institution since 2009. The current study reviewed patients in the registry who presented to the ED with a GCS of 3 from January 2011 to December 2015. Surviving and non-surviving patients were compared to identify the prognostic factors of patient survival. The study also aimed to determine the factors contributing to patients who survived with a GCS > 13 at discharge. RESULTS: During the study period, 145 patients were enrolled in the study, 119 of whom (82.1%) did not survive the traumatic insult. Of the 26 survivors, 13 (9.0%) had a GCS of 14 or 15 at discharge. The multiple logistic regression revealed that a lack of bilateral dilated and fixed pupils (BFDP) (OR 5.967, 95% CI 1.780-19.997, p = 0.004) and a GCS > 3 after resuscitation (OR 6.875, 95% CI 2.135-22.138, p = 0.001) were independent prognostic factors of survival. Based on the multiple logistic regression, an age under 40 years (OR 16.405, 95% CI 1.520-177.066, p = 0.021) and a GCS > 3 after resuscitation (OR 12.100, 95% CI 1.058-138.352, p = 0.045) were independent prognostic factors of a GCS > 13 at discharge. CONCLUSION: Aggressive resuscitation still provided benefit to polytrauma patients presenting with a GCS of 3, especially those with a rapid response to the resuscitation. Young patients with a deep coma on arrival had a higher probability of functional recovery after resuscitation in the ED.

[Rare causes of childhood leukocoria]. / Les causes rares de leucocorie chez l'enfant.

PURPOSE: The purpose was to record the causes of leukocoria among children under 10years of age and to determine the proportion of rare causes of leukocoria. PATIENTS AND METHODS: This retrospective study was conducted over a period of ten years, from January 1, 2004 to December 31, 2013, in patients under 10years of age who were referred for leukocoria. RESULTS: Leukocoria represented one of the ten reasons for consultation among children under 10years of age. The mean age of our patients was 42.5months. In 76 % of cases, the leukocoria patients were children under 6years of age. Male patients were affected more commonly, with a sex-ratio of 1.5. Patients coming from Dakar and its suburbs represented two thirds of the total. Bilateral involvement represented 53.7 % of the total. Cataracts were responsible for 74.3 % of cases, retinoblastoma 20.58 %, retinal detachment 0.96 %, retinopathy of prematurity 0.96 %, pupillary membrane persistence 0.96 %, persistent hyperplastic primary vitreous 0.64 %, endophthalmitis 0.64 %, optic nerve coloboma 0.32 %, iris heterochromia 0.32 % and ametropia 0.32 %. DISCUSSION: The total percentage of rare causes was 5.12 % in our study, including one case of hyperopia. These etiologies, although rare, do exist. CONCLUSION: Rare causes of leukocoria require special attention. The discovery of leukocoria necessitates rigorous etiological work-up. Ametropia must be a diagnosis of exclusion.

Pupillary Abnormalities in Descemet Membrane Endothelial Keratoplasty After Nearly Full Tamponade.

PURPOSE: To describe the incidence, risk factors, and management of pupillary abnormalities after anterior chamber (AC) full air or gas tamponade in Descemet membrane endothelial keratoplasty (DMEK) without pupillary block. METHODS: In this retrospective case series, clinical records of 25 patients (32 eyes) who underwent DMEK were reviewed for pupillary abnormalities and iris morphology. All patients had nearly full intracameral tamponade with air or 20% SF6 at the end of surgery without default air release postoperatively. RESULTS: Pupillary abnormalities ranging from mild ovalization to mid-mydriasis were seen in 56% of the cases. These abnormalities were not related to morphometric changes in the iris volume, or in the iris dilator or iris sphincter muscle, but were probably due to posterior synechiae. Combining DMEK and cataract surgery increases by 5-fold the odds of developing this complication. Surgical revision of the posterior surface of the iris and synechiolysis reversed these pupillary abnormalities. CONCLUSIONS: Nonischemic pupillary abnormalities can be seen in patients with DMEK using a nearly full air/gas tamponade in the AC after surgery despite patent iridectomy especially when combined with cataract surgery. Ensuring complete mydriasis in the immediate postoperative period and a free-floating bubble in the AC above the inferior pupillary margin may reduce its incidence.

Iris alterations after DSAEK. / Alteraciones iridianas tras Queratoplastia endotelial automatizada con stripping de la Descemet.

OBJECTIVE: To evaluate a series of case that developed iris changes after performing Descemet's stripping automated endothelial keratoplasty (DSAEK). METHODS: Retrospective study of eyes that developed iris abnormalities, such as pupil ovalisation, iris atrophy, iridocorneal synechiae, mydriatic pupil, and pigmentary changes after performing DSAEK in a tertiary hospital. RESULTS: In a series of the first 32 DSAEK procedures performed, new single or mixed iris alterations were observed in 12 eyes (37.5%). Iris-corneal synechiae were observed in 7 eyes, corectopias in 9 eyes, iris atrophy in 3 cases, and one case developed an areflexic mydriatic pupil. Long-term pigment dispersion at the edge of the lenticule was observed in 12 eyes. The alterations occurred after three months from the surgery. In the evaluation of the associated factors, malignant glaucoma had occurred in 1 case, 2 eyes had required a second surgery, one case by re-DSAEK, and the other one by removing the intraocular lens due to lens opacification. Two cases had a shallow anterior chamber. No relationship was found between the thickness of the peripheral lenticule and the presence of synechiae. CONCLUSION: Iris changes regarding DSAEK are possible. A discussion is presented on the relationship between increased intraocular pressure due to air in anterior chamber and its relationship with ischaemia and secondary alterations in the iris.

[Impact of follow-up loss over visual deficiency in open-globe ocular trauma]. / Impacto de la pérdida de seguimiento sobre la deficiencia visual en trauma ocular con globo abierto.

INTRODUCTION: Open-globe ocular trauma causes visual deficiency; calculating the magnitude of the latter often misses the estimation in patients without follow-up. AIM: to identify the modification of the postoperative proportion of visual deficiency in open-globe ocular trauma, which would introduce considering the proportion estimated in patients without follow-up. METHODS: Non-interventional, retrospective, longitudinal, analytical study. Visual outcome in eyes with open-globe trauma, with and without follow-up, was calculated using the Ocular Trauma Score. The observed postoperative proportion of visual deficiency was identified in eyes with follow-up; in eyes without follow-up, the postoperative proportion of visual deficiency was estimated using an analysis of scenarios: best (Ocular Trauma Score), mean (that of eyes with follow-up) and worst (last observation/no visual improvement). The estimated proportion of visual deficiency was added to that observed in eyes with follow-up, and the resulting proportion was compared with that expected in the sample, using the Ocular Trauma Score (χ(2)). RESULTS: 104 eyes, 70 without follow-up and 34 without it. In eyes with follow-up the expected proportion of visual deficiency was 58.6%, and the observed one was 71.4% (p = 0.1); the estimated proportion of visual deficiency in eyes without follow-up was 76.5%. The resulting postoperative proportion of visual deficiency in the sample would be 73.1%, which would overcome that expected by the Ocular Trauma Score (59.6%, p = 0.04). CONCLUSIONS: In open-globe ocular trauma, the efficacy of surgery to reduce the proportion of visual deficiency would decrease with regard to the standard expected by the Ocular Trauma Score, if the deficiency estimated in eyes without follow-up were considered.

Retinoblastoma: a recent experience at the National University Hospital, Singapore.

INTRODUCTION: Retinoblastoma is a very rare disease. There were 30 cases of retinoblastoma diagnosed and treated at National University Hospital (NUH). MATERIALS AND METHODS: A retrospective chart review was performed on the medical records of 30 patients who were diagnosed with retinoblastoma between 1995 and 2008 at the Department of Paediatrics, National University Hospital, Singapore. RESULTS: The median age at diagnosis was 1.6 years (range, 0-5.9) with a median follow-up of 1.8 years (range, 0.1 to 11.6). The median time from presenting signs to the time of diagnosis was 5.2 months (range, 0-25.2). Common presenting signs of retinoblastoma were identified; the most common of which were leukocoria (50.0%), squinting (13.3%), poor vision (10.0%), strabismus (6.6%) and unknown (33.3%). Of the 30 patients, 10 were from Singapore whilst the other 20 patients were from the surrounding countries. Twelve patients had bilateral disease at the time of diagnosis, while 18 had unilateral disease. Staging information was available in 27 patients. Enucleation was performed in 25 of 30 patients. Radiation therapy was given in 3 patients in 1995 (bilateral disease), 2001 (bilateral disease) and 2003 (unilateral disease). At the time of analysis, 19 patients were alive with no evidence of disease. Overall 5-year survival for the cohort was 88.1% [95% confidence interval (CI), 88.0-100] and event-free survival for the whole cohort was 74.2% (95% CI, 55.8-92.6). CONCLUSION: In our limited experience, the importance of collaboration and standardisation of the staging system, raising awareness and education of primary healthcare providers and parents are strongly stressed.

Rapid assessment of avoidable blindness in Kunming, china.

OBJECTIVE: To estimate the magnitude and causes of visual impairment (VI) in people aged > or = 50 years in Kunming using the Rapid Assessment for Avoidable Blindness methodology, and to assess the prevalence of a pupillary defect in participants diagnosed as cataract visually impaired. DESIGN: Population-based cross-sectional survey. PARTICIPANTS: We enrolled 2760 residents of Kunming prefecture in southwest China, >50 years of age. METHODS: Forty-six clusters of 60 people were selected based on population proportional to size. Households from each cluster were selected using compact segment sampling (CSS) or quota sampling when CSS was not feasible. Visual acuity (VA) was assessed using a tumbling E chart. Lens status and cause of VI were determined by ophthalmologists using direct ophthalmoscopy through a dilated pupil where necessary. The pupillary reaction was assessed on undilated pupils when VI was detected. MAIN OUTCOME MEASURES: Prevalence of blindness (VA<3/60), severe VI (SVI) (VA<6/60), and VI (VA<6/18) using presenting VA (PVA). The causes of blindness and VI and prevalence of a pupillary defect in the cataract visually impaired were also assessed. RESULTS: Of 2760 enumerated residents, 2588 were examined. The sample prevalence of bilateral blindness was 3.7% (95% confidence interval [CI], 2.8-4.6%). The prevalence of SVI was 3.0% (95% CI, 2.2-3.8%), and of VI was 9.1% (95% CI, 7.5-10.7%). The main cause of blindness was cataract (63.2% of blindness), followed by nontrachomatous corneal scar (14.7%), glaucoma (7.4%), and other posterior segment disease/neurologic disorders (4.2%). A pupillary defect was detected in 16% of those diagnosed with cataract VI. The cataract surgical coverage in the bilaterally blind was 58.9%, and 45% of operated eyes had good outcome with available correction (VA>6/18). The main barrier to cataract surgery was cost. CONCLUSIONS: Cataract remains the most important cause of preventable blindness in this poor region of China, and affordable provision of surgery would help to address this problem. Some cases of cataract blindness may not be preventable owing to preexisting comorbidity, as detected by the presence of a pupillary defect.

Outbreak of toxic anterior segment syndrome associated with glutaraldehyde after cataract surgery.

PURPOSE: To present clinical findings of a cluster of cases of toxic anterior segment syndrome (TASS) after uneventful phacoemulsification cataract surgery. SETTING: Department of Ophthalmology, Akdeniz University, Antalya, Turkey. METHODS: Six eyes of 6 patients developed TASS after uneventful phacoemulsification cataract surgery with implantation of a 3-piece acrylic IOL performed by 2 ophthalmologists on the same day. Clinical findings included corneal edema, Descemet's membrane folds, anterior chamber reaction, fibrin formation, and irregular, dilated, and unreactive pupils. RESULTS: Glutaraldehyde 2% solution was used inadvertently by the operating room staff who cleaned and sterilized reusable ocular instruments before autoclaving. None of the affected corneas improved. Additional surgical procedures were required and included penetrating keratoplasty, trabeculectomy, and glaucoma tube implantation. CONCLUSIONS: Glutaraldehyde in concentrations generally used for cold sterilization is highly toxic to the corneal endothelium. The operating room staff involved in sterilizing instruments should be well educated about and careful to follow the protocols to properly clean and sterilize reusable ocular instruments.

Surgery for patients with severe supratentorial intracerebral hemorrhage.

INTRODUCTION: Little information is available on the efficacy of aggressive treatment such as surgery in improving the outcome of severely affected patients after supratentorial intracerebral hemorrhage (ICH). Our objective was to assess the effect of hematoma removal and ventricular drainage on the mortality of patients with severe primary supratentorial ICH. METHODS: We studied 103 consecutive patients who were admitted to the intensive care unit and diagnosed with primary supratentorial ICH. The impacts of clinical factors on 30-day mortality were assessed, including surgery, Glasgow Coma Scale (GCS) score and pupillary abnormality at admission, hematoma volume, and other related factors. RESULTS: The 30-day mortality rate was 42%, and the median time between admission and death was 3 days (range: 1 to 27 days). Hematoma removal and ventricular drainage, within the first 24 hours of admission, were performed on 11 and 17 patients, respectively. Two patients who were treated with removal and four with drainage died. A logistic regression model for predicting 30-day mortality was performed. After controlling for GCS score, pupillary abnormality, hydrocephalus, and hematoma volume, hematoma removal was identified as an independent predictor of survival (odds ratio [OR], 0.12; 95% confidence interval [CI], 0.02 to 0.92). Ventricular drainage also tended to decrease mortality rate greatly (OR, 0.31; 95% CI, 0.06 to 1.76). Patients with GCS scores of 3 or 4 were 4.01 times more likely to die (95% CI, 1.13 to 14.26) than those with GCS of at least 5. CONCLUSIONS: Hematoma removal may reduce the mortality rate of patients with severe supratentorial ICH.