Intraocular foreign bodies in children: A retrospective case series.

INTRODUCTION: Open globe injuries are a major cause of visual impairment in children, related to the severity of the trauma or secondary to induced amblyopia. Intraocular foreign bodies (IOFB) have been reported in approximately one third of cases of open globe injuries. As clinical presentation and management may differ between adults and children, data is lacking about IOFBs in children under 18years of age. The purpose of this study was to assess the clinical characteristics and visual prognosis of ocular trauma associated with intraocular foreign bodies in children. MATERIALS AND METHODS: This single-center retrospective study included patients under 18years of age treated for ocular trauma with IOFB. Demographic characteristics, complete initial and final ophthalmological examination, imaging data and details of medical and surgical management were collected. RESULTS: Fourteen patients were included (78.6% boys), with a mean age of 10.3years (min 7months-max 17years). In 92.9% of cases, patients were found to have a single IOFB, mostly metallic (71.4%). Posterior segment IOFBs were found in 50% of cases, anterior segment IOFBs in 28.6% and orbital IOFBs in 21.4%. The clinical examination permitted detection of the IOFBs in 50% of cases, while they were visible on CT scan in all cases. The mean initial visual acuity was 20/320, and the mean final visual acuity was 20/125. Endophthalmitis occurred in 2 cases (14%). DISCUSSION: Open globe injuries associated with IOFB are severe and sight-threatening. Localization of the IOFB in the posterior segment has a worse prognosis. CT scan is mandatory, especially in children, as the trauma history is often missing. Retinal detachment and endophthalmitis appear to be the main prognostic factors requiring urgent specialized pediatric ophthalmology management.

Intubating conditions and adverse events during sevoflurane induction in infants.

BACKGROUND: The aim of this study was to compare intubating conditions and adverse events after sevoflurane induction in infants, with or without the use of rocuronium or alfentanil. METHODS: Seventy-five infants, aged 1-24 months, undergoing elective surgery under general anaesthesia were randomly assigned to receive 8% sevoflurane with either placebo (i.v. saline 0.5 ml kg⁻¹), rocuronium (0.3 mg kg⁻¹), or alfentanil (20 µg kg⁻¹). The primary outcome measure was intubating conditions evaluated 90 s after test drug injection by an anaesthetist unaware of the patient's group. The secondary outcome criteria were respiratory (Sp(O2) <90%, laryngospasm, closed vocal cords preventing intubation, bronchospasm) and haemodynamic adverse events (heart rate and mean arterial pressure variations ≥30% control value). RESULTS: Intubating conditions were significantly better in the rocuronium group, with clinically acceptable intubating conditions in 92%, vs 70% in the alfentanil group and 63% in the placebo group (P=0.044). Adverse respiratory events were significantly less frequent in the rocuronium group: 0% vs 33% in the placebo group and 30% in the alfentanil group (P=0.006). Haemodynamic adverse events were more frequent in the alfentanil group: 48% vs 7% in the placebo group and 16% in the rocuronium group (P=0.0019). CONCLUSIONS: In 1- to 24-month-old infants, the addition of 0.3 mg kg⁻¹ rocuronium to 8% sevoflurane improved intubating conditions and decreased the frequency of respiratory adverse events. Alfentanil provided no additional benefit in this study.

[Vision screening for children: Recommended practices from AFSOP]. / Le dépistage visuel chez l'enfant : les recommandations de l'Association Francophone de Strabologie et d'Ophtalmologie Pédiatrique (AFSOP).

In light of the international literature, a workgroup of experts from the AFSOP met in February 2019 to formulate updated recommendations for visual screening in children. An ophthalmologic examination during the first month of life is recommended for children at risk of developing infantile organic amblyopia. An ophthalmologic examination including cycloplegic refraction between 12 and 15 months of age is recommended for children at risk of developing functional amblyopia. At any age, a prompt ophthalmologic examination is recommended for a child suspected of functional or organic ocular pathology. In children without risk factors or warning signs, a systematic orthoptic screening examination is recommended during the third year of life, including a monocular visual acuity test, a cover-test and a refraction by photoscreener. The child is referred to the ophthalmologist only in the case of an abnormal screening result, according to the following criteria: visual acuity <5/10, or >1 difference between eyes, abnormal cover test, photodetection refraction values <-3D or>+2.5D for the sphere,>1.5D for astigmatism and>1D for anisometropia. Finally, we review normal childhood refractive errors as a function of age as well as the correct use of photo screening devices.

[Diagnosis and treatment of tearing in infancy]. / Larmoiement du nourrisson : conduite à tenir et thérapeutique.

Tearing in infancy is a very frequent symptom, particularly during the first year of life. A definite diagnosis can be made easily, only by questioning the parents about the characteristics and the evolution of the tearing since the birth and with a simple examination. The main causes are congenital nasolacrimal duct obstruction account of his high frequency in infant and congenital glaucoma account of his seriousness.

[Computer-assisted diagnosis and therapy for glaucoma]. / Diagnostic et traitement des glaucomes.

Boolean algebra, or combinatory analysis and their related computer routines, can provide invaluable help in resolving classic diagnostic problems. However complex each case may be, the diagnosis is always made from a finite set of data, and the fundamental problem is thus how to exploit this data. Invention no longer has a place in ascertaining a diagnosis. Traditional ways of reasoning are numerous, personal, and fragile, but fortunately redundant. They may give rise to four types of error: omission or mistake (an error of judgment), either during the semiotic or the dialectic stages. Whereas the physiological capacity of the human brain and memory only enables it to make a limited number of hypotheses concerning certain aspects of glaucoma, computer programs can take the total number of hypotheses into account, i.e., 3000. For every input the program explores each of the 3,000 items, thus eliminating the four types of error. The probabilistic nature of data, which compromises the confidence one can have in conclusions resulting from such complex reasoning, is treated by the adjusted probabilities. The use of such diagnostic aids, whose thesaurus is updated regularly, is reserved for ophthalmologists, the only authority capable of assessing the pertinence of the computer responses. Consequently, the specialist can rest assured that the patient has benefited from the most comprehensive and updated knowledge in medical science.

Identification of novel VMD2 gene mutations in patients with best vitelliform macular dystrophy.

ABSTRACT We report five novel VMD2 mutations in Best's macular dystrophy patients (S16F, I73N, R92H, V235L, and N296S). An SSCP analysis of the VMD2 11 exons revealed electrophoretic mobility shifts exclusively in exons 2, 3, 4, 6 and 8. Direct sequencing indicated that these shifts are caused by mono-allelic transition in exons 2, 4, 6, 8 and transversion in exons 3 and 6. Five novel "silent" polymorphisms are also reported: 213T>C, 323C>A, 1514A>G, 1661C>T, and 1712T>C. Hum Mutat 17:235, 2001.

PAX2 mutations in renal-coloboma syndrome: mutational hotspot and germline mosaicism.

The renal-coloboma syndrome (RCS, MIM 120330) is an autosomal dominant disorder caused by PAX2 gene mutations. We screened the entire coding sequence of the PAX2 gene for mutations in nine patients with RCS. We found five heterozygous PAX2 gene mutations: a dinucleotide insertion (2G) at position 619 in one sporadic RCS case, a single nucleotide insertion (619 + G) in three unrelated cases, and a single nucleotide deletion in a familial case. In this familial case, three affected sibs showed a striking ocular phenotypic variability. Each of the sibs carried a 619insG mutation, whilst unaffected parents did not, suggesting the presence of germline mosaicism. Interestingly, the 619insG mutation has been previously reported in several patients and is also responsible for the Pax21Neu mouse mutant, an animal model of human RCS. This study confirms the critical role of the PAX2 gene in human renal and ocular development. In addition, it emphasises the high variability of ocular defects associated with PAX2 mutations ranging from subtle optic disc anomalies to microphthalmia. Finally, the presence of PAX2 germline mosaicism highlights the difficulties associated with genetic counselling for PAX2 mutations.

Prominent beta-5 gene expression in the cardiovascular system and in the cartilaginous primordiae of the skeleton during mouse development.

The alpha v beta (alpha(v)beta5) heterodimer has been implicated in many biological functions, including angiogenesis. We report the beta5 gene expression pattern in embryonic and foetal mouse tissues as determined by Northern blotting and in situ hybridization. During the earliest stages, beta5 mRNA is widespread in the mesoderm. During later developmental stages, it remains mostly confined to tissues of mesodermal origin, although probable inductive effects trigger shifts of beta5 gene expression from some mesenchymatous to epithelial structures. This was observed in the teeth, skin, kidneys, and gut. Of physiological importance is the beta5 labeling in the developing cardiovascular and respiratory systems and cartilages. Furthermore, early beta5 gene expression was observed within the intra- and extraembryonic sites of hematopoiesis. This suggests a major role for beta5 in the hematopoietic and angiogenic stem cells and thus in the development of the vascular system. Later, the beta5 gene was expressed in endothelial cells of the vessels developing both by angiogenesis and vasculogenesis in the lung, heart, and kidneys. Moreover, the beta5 hybridization signal was detected in developing cartilages but not in ossified or ossifying bones. beta5-Integrin is a key integrin involved in angiogenesis, vasculogenesis, hematopoiesis, and bone formation.

Long term light-induced retinal degeneration in the miniature pig.

In developing a model of slow light-induced retinal degeneration, ten miniature pigs were submitted to constant lighting for a period ranging from one to three months. Post-lighting survival time ranged from zero to two months. Control and illuminated animals were examined for pupillary reflex, underwent fundus examination and an electroretinogram. After euthanasia, retinas were processed for histology with measure of outer nuclear layer thickness. All animals illuminated one or more months had pupillar reflex alteration. Mean outer nuclear thickness was 24.12 microns in the control and ranged from 18.36 to 21.45 microns in illuminated animals (mean reduction 20%). Despite the pigmentation of miniature pigs, consistent results were obtained in the absence of pharmacologic pupil dilation.

Fluid-regulating and sympathoadrenal hormonal responses to peak exercise following cardiac transplantation.

Orthotopic heart transplantation results in cardiac denervation that can disrupt the normal regulation of hydromineral balance. This study compared the exercise-induced variations in plasma osmolality; atrial natriuretic peptide (ANP), arginine vasopressin (AVP), norepinephrine (NE), epinephrine (E), and dopamine (DA) concentrations; and plasma renin activity (PRA) of six cardiac transplant recipients (HTX) and six healthy age-matched controls (C) submitted to graded upright maximal cycling. Venous blood samples were obtained at rest, at submaximal (70% O2 uptake) and peak exercise, and after 10 and 30 min of sitting recovery. Peak O2 uptake was not different between groups despite lower maximal heart rate in HTX (136 +/- 6 vs. 183 +/- 9 beats/min). Baseline plasma ANP and PRA were higher in HTX (203 +/- 55 pg/ml and 29.9 +/- 7.4 ng.ml-1 x h-1) than in C (71 +/- 17 pg/ml and 5.4 +/- 0.96 ng.ml-1 x h-1); AVP was lower in HTX than in C (1.1 +/- 0.3 vs. 3.2 +/- 0.8 pg/ml; P < 0.05); and circulating E, NE, and DA were not different between groups. Exercise resulted in more marked increases in HTX than in C for ANP (300 vs. 100%), AVP (2,000 vs. 300%), NE (860 vs. 500%), and DA (611 vs. 187%) but not for PRA and a higher E response in C than in HTX (455 vs. 1,258%). These observations confirm that the potential for ANP release to central volume loading is independent of intact cardiac innervation. The exaggerated AVP response in HTX could, however, reflect the absence of inhibitory influences consecutive to denervation.(ABSTRACT TRUNCATED AT 250 WORDS)

SHORT syndrome: a case with high hyperopia and astigmatism.

We describe a case of the SHORT syndrome and compare it with previously published cases. This six-year-old girl shows nearly all the typical manifestations reported in patients with the SHORT syndrome, including lipoatrophy, minor facial anomalies, Rieger anomaly, and short stature. However, she also suffers from high hyperopia and astigmatism associated with poor visual acuity.

Use of denaturing HPLC and automated sequencing to screen the VMD2 gene for mutations associated with Best's vitelliform macular dystrophy.

We identified three novel VMD2 mutations in patients with Best's macular dystrophy. DHPLC analysis of the 11 VMD2 exons revealed abnormal profiles in exon 8. Direct sequencing showed that these abnormal profiles were due to monoallelic transitions and transversions. We also found three polymorphic sequence changes that have been reported previously and annotated to an online database (http://www.uni-wuerzburg.de/humangenetics/vmd2.html).

Quantitative analysis of intravitreal injections in the rat.

Intravitreal injections are currently used in the rat to introduce a therapeutic factor in the eye, especially for experimental treatments of retinal degenerations. The injected volume and its location can influence the quantification of results. We have investigated the quantitative effect of a single intravitreal injection in rats at different ages and for different volumes. Albinos rats aged three weeks or two months received intravitreal injections of 1, 3, 5 or 10 microl China ink. Animals were sacrificed immediately after injection, eyes were enucleated, fixated, embedded in paraffin and microtomy was performed in a sagittal plane. Regularly spaced sections were analyzed to reconstruct the vitreous and injected dye volumes. The measured vitreous volume was 6.76 +/- 0.37 mm(3) in three weeks old rats and 13.36 +/- 0.64 mm(3) in two months old rats. Mean intravitreal ink volumes immediately after injection were 0.8 mm(3) for 1 ml injections, 2 mm( 3) for 3 ml, 2.3 to 2.6 mm(3) for 5 microl and 3.2 mm(3 ) for 10 microl. The percentage of vitreous volume involved by the injection ranged from 4.4% to 33.2%. The injected volume is limited by the large lens size of the rat. Extraocular loss of injected solution increases for higher injected volumes, with larger standard deviations. In this model, the dye tends to localize behind the lens. A 3 or 5 microl volume appears to have the best reproducibility with minimum loss of solution.

Long-term results of trabeculectomy for congenital glaucoma.

BACKGROUND: To evaluate long-term results of intraocular pressure after trabeculectomy for congenital glaucoma. METHODS: Data concerning 55 eyes (30 patients) who underwent trabeculectomy for congenital glaucoma were recorded. Mean age at diagnosis was 3.4 months (range: 2 days to 10 months). Mean follow up was 56.8 months. Associated anterior segment abnormalities, need for one or more new trabeculectomy procedures during follow up, and intraocular pressure at the last examination were noted. RESULTS: Of the 55 eyes, 48 met the success criteria (87.3%). A second and sometimes third or fourth trabeculectomy were necessary during follow up in 17 eyes (31%). Of the seven failures at final examination, six (85%) had been diagnosed and operated on before the age of 1 month, whereas 15 of the 48 eyes with good results (31.2%) were in this group (p < 0.02). Of the seven failures at final examination, six (85%) were operated on two to four times, whereas 10 of the 48 eyes with good results (20.1%) were in this group (p < 0.01). An associated anterior segment abnormality was present in 13 eyes (23%), and did not seem to influence the final outcome. CONCLUSION: Trabeculectomy is an effective procedure for long-term control of intraocular pressure in congenital glaucoma. The early diagnosis and surgical treatment correspond to a poor long-term prognosis, probably related to initially severe cases. In these cases, intraocular pressure is difficult to control despite repeated surgical procedures.

[Ocular injuries and childbirth]. / Traumatismes oculaires et accouchement.

INTRODUCTION: Severe ocular traumatisms related to childbirth are rare and often subordinate to maneuvers with instruments. The aim of this study was to report on the different severe ocular injuries that might occur during childbirth and their possible consequences. There can be several of these ocular injuries. PATIENTS AND METHODS: Since 1984, we have examined 11 cases of this type of traumatism. For each case, the childbirth circumstances, the affected side, the different injuries observed such as ocular, orbital, cranial and even cerebral lesions, and the results of the ophthalmological follow-up examinations were recorded. RESULTS: In most cases, lesions resulted from forceps traumatisms, and the injuries were corneal (vertical tears in the Descemet's membrane), retinal (hemorrhages), and orbital (e.g., the optical nerve and oculomotor nerve). CONCLUSION: These injuries, sometimes severe, must benefit from early and complete examination, possibly with general anesthesia, and long-term follow-up. Furthermore, a radiographic and clinical neurological examination is recommended to prevent any cerebral complication.

[Congenital megalocornea]. / La mégalocornée congénitale.

Congenital megalocornea is a bilateral enlargement of the cornea's diameter that can be associated with trabecula and/or iris dysgenesis. This condition predisposes the patient to glaucoma. The physiopathology is poorly understood and may be related to lens induction during embryological development of the anterior of the eye. X-linked genetic transmission is found in 50% of cases, autosomal transmission in 40%, and sporadic transmission in the remaining 10%. Diagnosis is based on a thorough examination of the child's eye, often with general anesthesia. Different clinical aspects can be observed: simple megalocornea without ocular or systemic anomalies, megalocornea with ocular and/or systemic anomalies as well as multiple malformation syndromes, dermatological diseases, skeletal diseases, and genetic or chromosomic diseases. A systematic general pediatric examination is therefore necessary. Congenital megalocornea must be considered, first of all, as a differential diagnosis of primary congenital glaucoma and all of its etiologies. Treatment of associated ocular diseases, the search for an association with systemic diseases, and follow-up for the rest of the patient's life are indispensable.

[Deep sclerectomy in congenital glaucoma. Preliminary results]. / Sclérectomie profonde dans le glaucome congénital. Résultats préliminaires.

PURPOSE: Trabeculectomy is an efficient procedure for congenital glaucoma, but can lead to postoperative complications. These complications seem to be less frequent with deep sclerectomy. The aim of this study is to evaluate results of this surgical technique for congenital glaucoma. MATERIALS AND METHODS: Twelve eyes from eight patients (age 2 to 84 months) with congenital glaucoma underwent sclerectomy and were followed-up for 10 months postoperatively. Success criteria was intraocular pressure inferior to 16 mm Hg under general anaesthesia. RESULTS: No per or immediate postoperative complication was observed. For nine eyes (75%), intraocular pressure was controlled at final examination. For three eyes, postoperative intraocular pressure was elevated and one of them underwent re-operation. CONCLUSIONS: Success rate of sclerectomy for congenital glaucoma is equivalent to trabeculectomy. Absence of anterior chamber opening diminishes postoperative complications risk. Further study with longer follow-up is currently under evaluation.

[Characteristics of retinal detachment in children]. / Particularités du décollement de rétine chez l'enfant.

INTRODUCTION: Pediatric retinal detachments differ from adult retinal detachments in their etiologies, prognosis and treatment. The aims of this study were the analysis and long-term follow-up of a group of pediatric retinal detachment patients. PATIENTS AND METHODS: All the cases of juvenile retinal detachments treated in our department between 1987 and 1999 were retrospectively studied. The age at the time of diagnosis, etiology, initial topography of the detachment, treatments undertaken, follow-up, and final results were recorded. RESULTS: The study investigated 64 eyes of 53 children. The follow-up was longer than 6 months for 31 eyes, with a mean follow-up of 39.5 months. The main etiologies were traumatism, high myopia, and affections leading to an exudative retinal detachment such as retinoblastoma and Coats disease. Circular scleral buckling was often used because of the vitreous cohesiveness in children. A vitrectomy was frequently associated to treat severe proliferative vitreoretinopathy. The final visual acuity was higher than 0.05 in 32% of the cases. DISCUSSION: The etiological features, late diagnosis and frequency of proliferative vitreoretinopathy are responsible for the greater severity of retinal detachments in children. These factors associated with amblyopia lead to poor visual prognosis. CONCLUSION: Retinal detachment in children remains a severe pathology. However the prognosis seems to be improving with progress in examination techniques and surgery methods.

[Extracapsular extraction and posterior chamber implantation in diabetics. Prospective study of 198 eyes]. / Extraction extracapsulaire et implantation de chambre postérieure chez le diabétique. Etude prospective sur 198 yeux.

PURPOSE: Visual results and retinal changes in diabetic patients who underwent cataract surgery. METHODS: In a prospective study we followed up 198 eyes of diabetic patients who underwent cataract surgery for at least six months postoperatively. Pre and postoperative retinal status were assessed by clinical examination and fluorescein angiography. Per and postoperative complications and the evolution of visual acuity were noted. RESULTS: In 83.3% of eyes, visual acuity improved by two or more lines. In 59.6% of patients, visual acuity at six months was more than 6/15. The quality of visual outcome diminished with the severity of retinopathy, but in the most serious cases, lens extraction allowed fundus observation and treatment. Inflammatory complications, posterior capsule opacification and cystoid macular oedema were significantly more frequent in cases of preoperative diabetic retinopathy than in normal fundus. We observed few aggravations of the diabetic retinopathy, but underline the high frequency of cystoid macular edema in diabetic patients. CONCLUSION: Visual results are good in diabetic patients with mild retinopathy and, in severe cases, surgery allows retinal examination and treatment.