Search

Purpose: To summarize the prevalence and patterns of uveitis in children.Methods: Pertinent articles were reviewed.Results: Children constitute 5-10% of the patients with uveitis seen at tertiary referral centers, and girls appear to develop uveitis slightly more frequently than boys. Among all children with intraocular inflammation, anterior uveitis accounts for 30-40%, posterior uveitis accounts for 40-50%, intermediate uveitis accounts for 10-20%, and diffuse uveitis accounts for 5-10%. The most common cause of anterior uveitis is juvenile idiopathic arthritis (JIA), whereas the most frequent type of posterior uveitis is toxoplasmic retinochoroiditis. Most cases of intermediate and diffuse uveitis are bilateral, chronic, and idiopathic. The most common causes of vision loss in children with uveitis are cataract, band keratopathy, glaucoma, and cystoid macular edema. Up to one-third of the children with uveitis are left with severely impaired vision as a result of these complications.Conclusions: Uveitis is an important cause of ocular morbidity in children. Prompt diagnosis and treatment is essential to minimize the risk of long-term vision loss. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical presentation and natural history of Vogt-Koyanagi-Harada (VKH) syndrome in children, an often unrecognized cause of pediatric uveitis.Methods: We performed a clinic-based cross-sectional survey and retrospective review of pediatric patients with VHK seen in the uveitis clinic at the Aravind Eye Hospital, Madurai, India, between January 1993 and December 1995.Results: A total of 98 patients with VKH syndrome were seen in the uveitis clinic at the Aravind Eye Hospital during the three years covered by the survey, of whom three (about 3%, with an approximate incidence of 1 case/year) were children 16 years of age or younger. Initial symptoms included eye pain, redness, and blurred vision in all three patients. Headache and dysacusis accompanied the onset of visual symptoms in one patient. Each patient eventually developed areas of vitiligo and poliosis, although these signs were not present at the time of presentation in any of our patients. Signs of active uveitis included anterior chamber and vitreous inflammation, optic disc edema, exudative retinal detachments, and Dalen-Fuchs'-like nodules. All three patients developed cataracts, two developed retinal pigment epithelium atrophy, and one developed glaucoma. Each patient responded well, with good visual recovery, to topical and/or systemic corticosteroids.Conclusions: Although frequently unrecognized, VKH syndrome may affect children. As with adults, visual loss occurs most typically as the result of cataract, glaucoma, or retinal pigment epithelium atrophy. [ABSTRACT FROM AUTHOR]

We review recent studies on the central neural control of esophageal motility, emphasizing the anatomy and chemical coding of esophageal pathways in the spinal cord and medulla. Sympathetic innervation of the proximal esophagus is derived primarily from cervical and upper thoracic paravertebral ganglia, whereas that of the lower esophageal sphincter and proximal stomach is derived from the celiac ganglion. In addition to noradrenaline, many sympathetic fibers in the esophagus contain neuropeptide Y (NPY), and both noradrenaline and NPY appear to decrease blood flow and motility. Preganglionic neurons innervating the cervical and upper thoracic ganglia are located at lower cervical and upper thoracic spinal levels. The preganglionic innervation of the celiac ganglion arises from lower thoracic spinal levels. Both acetylcholine (ACh) and enkephalin (ENK) have been localized in sympathetic preganglionic neurons, and it has been suggested that ENK acts to pre-synaptically inhibit ganglionic transmission. Spinal afferents from the esophagus are few, but have been described in lower cervical and thoracic dorsal root ganglia. A significant percentage contain calcitonin gene-related peptide (CGRP) and substance P (SP). The central distribution of spinal afferents, as well as their subsequent processing within the spinal cord, have not been addressed. Medullary afferents arise from the nodose ganglion and terminate peripherally both in myenteric ganglia, where they have been postulated to act as tension receptors, and, to a lesser extent, in more superficial layers. Centrally, these afferents appear to end in a discrete part of the nucleus of the solitary tract (NTS) termed the central subnucleus. The transmitter specificity of the majority of these afferents remains unknown. The central subnucleus, in turn, sends a dense and topographically discrete projection to esophageal motor neurons in the rostral portion of the nucleus ambiguous (NA). Both somatostatin-(SS) and ENK-related peptides have been localized in this pathway. Finally, motor neurons from the rostral NA innervate striated portions of the esophagus. In addition to ACh, these esophageal motor neurons contain CGRP, galanin (GAL), N-acetylaspartylglutamate (NAAG), and brain natriuretic peptide (BNP). The physiological effect of these peptides on esophageal motility remains unclear. Medullary control of smooth muscle portions of the esophagus have not been thoroughly investigated. [ABSTRACT FROM AUTHOR]

Objective: To review the physiologic basis for normal and abnormal vagal reflexes arising from the pharynx, larynx, and esophagus, as well as the relevance of vagal reflexes to the pathogenesis of such clinically common cardiorespiratory responses as bradycardia, tachycardia, dysrhythmia, coronary angiospasm, bronchospasm, laryngospasm, prolonged apnea, and singultus (hiccups).Data Sources: Pertinent articles and reviews were identified through a MEDLINE search (April 1966 to October 1991). Older studies and others not identified in the MEDLINE search were found through a manual search of the bibliographies of the retrieved articles.Study Selection: Experimental studies in both humans and animals, as well as case series and single case reports, were selected for evaluation and citation. In instances where a similar phenomenon was described in multiple independent reports, only studies that provided a novel finding or interpretation were cited. More authoritative book chapters and peer-reviewed summaries were also cited in support of commonly accepted principles.Data Extraction and Synthesis: Most of the clinical data are derived from case reports and small case series and are therefore anecdotal; equal weight was given to all such studies. Reports of conflicting observations or interpretations were clearly identified and were cited without exception.Conclusions: Stimulation of the upper aerodigestive tract can lead to clinically significant cardiorespiratory responses. Although the prevalence of and risk factors for such responses have not been established, we suggest that a pharyngeal, a laryngeal, or an esophageal source for abnormal cardiorespiratory responses be sought whenever a detailed clinical evaluation fails to reveal a cause, particularly when there are concurrent symptoms or signs of upper aerodigestive tract disease, such as dysphagia or gastroesophageal reflux. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the efficacy of a second year of pegaptanib sodium therapy in patients with neovascular age-related macular degeneration (AMD)., Design: Two concurrent, multicenter, randomized, double-masked, sham-controlled studies (V.I.S.I.O.N. [Vascular Endothelial Growth Factor Inhibition Study in Ocular Neovascularization] trials)., Participants: Patients with all angiographic neovascular lesion compositions of AMD were enrolled. In combined analyses, 88% (1053/1190) were re-randomized at week 54, and 89% (941/1053) were assessed at week 102., Interventions: At week 54, those initially assigned to pegaptanib were re-randomized (1:1) to continue or discontinue therapy for 48 more weeks (8 injections). Those initially assigned to sham were re-randomized to continue sham, discontinue sham, or receive 1 of 3 pegaptanib doses., Main Outcome Measures: Mean change in visual acuity (VA) over time and mean change in the standardized area under the curve of VA and proportions of patients experiencing a loss of > or =15 letters from week 54 to week 102; losing <15 letters (responders) from baseline to week 102; gaining > or =0, > or =1, > or =2, and > or =3 lines of VA; and progressing to legal blindness (20/200 or worse)., Results: In combined analysis, mean VA was maintained in patients continuing with 0.3-mg pegaptanib compared with those discontinuing therapy or receiving usual care. In patients who continued pegaptanib, the proportion who lost >15 letters from baseline in the period from week 54 to week 102 was half (7%) that of patients who discontinued pegaptanib or remained on usual care (14% for each). Kaplan-Meier analysis showed that patients continuing 0.3-mg pegaptanib for a second year were less likely to lose > or =15 letters than those re-randomized to discontinue after 1 year (P<0.05). The proportion of patients gaining vision was higher for those assigned to 2 years of 0.3-mg pegaptanib than receiving usual care. Progression to legal blindness was reduced for patients continuing 0.3-mg pegaptanib for 2 years., Conclusions: Continuing visual benefit was observed in patients who were randomized to receive therapy with pegaptanib in year 2 of the V.I.S.I.O.N. trials when compared with 2 years' usual care or cessation of therapy at year 1.

Objective: To evaluate the safety of pegaptanib sodium injection, a specific vascular endothelial growth factor (VEGF) antagonist, in the treatment of neovascular age-related macular degeneration (AMD) during 2 years of therapy., Design: Two concurrent, prospective, randomized, multicenter, double-masked, sham-controlled studies., Methods: Patients with all angiographic choroidal neovascularization lesion compositions of AMD received either intravitreous pegaptanib sodium (0.3 mg, 1 mg, 3 mg) or sham injections every 6 weeks for 54 weeks. Those initially assigned to pegaptanib were re-randomized (1:1) to continue or discontinue therapy for 48 more weeks; sham-treated patients were re-randomized (1:1:1:1:1) to continue sham, discontinue, or receive one of the pegaptanib doses., Main Outcome Measures: All reported adverse events, serious adverse events, and deaths., Participants: In year 1, 1190 subjects received at least one study treatment (0.3 mg, n = 295; 1 mg, n = 301; 3 mg, n = 296; sham, n = 298); 7545 intravitreous injections of pegaptanib were administered. In year 2, 425 subjects (0.3 mg, n = 128; 1 mg, n = 126; 3 mg, n = 120; sham, n = 51) continued the same masked treatment as in year 1 and received at least one study treatment in year 2; 2663 intravitreous injections of pegaptanib were administered in these subjects., Results: All doses of pegaptanib were well tolerated. The most common ocular adverse events were transient, mild to moderate in intensity, and attributed to the injection preparation and procedure. There was no evidence of an increase in deaths, in events associated with systemic VEGF inhibition (e.g., hypertension, thromboembolic events, serious hemorrhagic events), or in severe ocular inflammation, cataract progression, or glaucoma in pegaptanib-treated patients relative to sham-treated patients. In year 1, serious injection-related complications included endophthalmitis (12 events, 0.16%/injection), retinal detachment (RD) (6 events [4 rhegmatogenous, 2 exudative], 0.08%/injection), and traumatic cataract (5 events, 0.07%/injection). Most cases of endophthalmitis followed violations of the injection preparation protocol. In patients receiving pegaptanib for >1 year, there were no reports of endophthalmitis or traumatic cataract in year 2; RD was reported in 4 patients (all rhegmatogenous, 0.15%/injection)., Conclusion: The 2-year safety profile of pegaptanib sodium is favorable in patients with exudative AMD.

Uveitis refers to inflammation of the uveal tract, which includes the iris, ciliary body, and choroid. Common usage has expanded this definition, however, to include intraocular inflammation of any type. Hence, retinitis, retinal vasculitis, and papillitis are also grouped under uveitis, even in the absence of significant uveal tract inflammation. Uveitis can also be secondary to corneal, scleral, or orbital inflammation. Experience has shown that identifying the primary focus of intraocular inflammation is important, both for establishing a diagnosis and as a predictor of natural history and response to therapy. A number of ocular and systemic syndromes are recognized to cause anterior uveitis. Clues to the diagnosis of the more commonly encountered anterior uveitis syndromes are outlined below. It is important to pay particular attention to the severity of the anterior chamber inflammation, and to note the presence of anterior granulomatous findings since the intensity and character of the inflammation can sometimes suggest a diagnosis.

Purpose: To describe the characteristics of ischemic maculopathy in patients with human immunodeficiency virus (HIV) infection, as a means of understanding this uncommon disorder more fully., Methods: This is a multicenter, retrospective review of clinical data available for five HIV-infected patients who were given the diagnosis of ischemic maculopathy., Results: All cases had been diagnosed on the basis of fluorescein angiograms obtained after patients complained of vision loss. Four of the five patients had bilateral macular disease. Visual acuity at presentation in the nine affected eyes ranged from 20/20 to count fingers. Vision loss was gradual in both eyes of one patient and was abrupt in onset in seven eyes. Each of the seven eyes with abrupt vision loss had opacification of the superficial retina and/or intraretinal hemorrhages near the fovea. Fluorescein angiography revealed enlargement of the foveal avascular zone and mild staining of the juxtafoveal vessels in affected eyes. Six eyes had active or clinically inactive cytomegalovirus retinitis at presentation, and a seventh eye developed cytomegalovirus retinitis 2 weeks later. All patients were receiving anticytomegalovirus drugs when they developed visual symptoms. Visual acuity remained stable in five eyes, became worse in two eyes, and improved in two eyes; final visual acuity ranged from 20/25 to count fingers., Conclusions: Ischemic maculopathy may cause profound and permanent vision loss in HIV-infected individuals. Fluorescein angiography should be considered in all HIV-infected patients with unexplained loss of vision. The pathogenesis of ischemic maculopathy remains unknown.

Purpose: To describe central serous chorioretinopathy after bone marrow transplantation., Methods: The medical records of the patient were reviewed retrospectively., Results: A 46-year-old Filipino man developed multifocal central serous chorioretinopathy affecting his left eye 4 months after bone marrow transplantation for acute myelogenous leukemia. Other co-existing medical problems at the time of presentation included systemic hypertension and graft-versus-host-disease (GVHD), for which the patient was using both systemic corticosteroids and cyclosporine., Conclusion: Central serous chorioretinopathy is a rare cause of vision loss in patients after bone marrow transplantation. Previous descriptions of bone marrow transplantation-associated central serous chorioretinopathy in patients with thrombotic microangiopathy, as well as the occurrence of both systemic hypertension and the use of systemic corticosteroids and cyclosporine in our patient with bone marrow transplantation-associated central serous chorioretinopathy, support theories of choroidal vascular compromise in the pathogenesis of central serous chorioretinopathy.