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Biomedical subjects

R Michael Siatkowski

Publications and source records attributed to R Michael Siatkowski.

At least 19 recordsLinked to original sources

Retinopathy of prematurity in infants with birth weight>or=1250 grams-incidence, severity, and screening guideline cost-analysis.

PURPOSE: To determine the incidence and severity of retinopathy of prematurity (ROP) in infants with birth weight (BW) 1250 to 1800 g, to examine the influence of systemic conditions on the development of ROP in this population, and to evaluate the cost-effectiveness of various screening guidelines. METHODS: We reviewed records from 259 consecutive infants with BW 1250 to 1800 g who were screened for ROP over a 3-year period. Extracted data included presence and severity of ROP, and the following potential risk factors (RF) for ROP development: sepsis, meningitis, necrotizing enterocolitis, intraventricular hemorrhage greater than stage I, pneumothorax, direct bilirubin>2 mg/dl, central line placement, antibiotic treatment>14 days, greater than seven red blood cell (RBC) transfusions, and mechanical ventilation>96 hours. RESULTS: The overall incidence of ROP in this population was 4.2%. Two infants had stage 3 ROP, one with plus disease. Infants with stage 3 ROP had significantly lower BW (1299 versus 1484 g, P=0.013) and gestational age (GA) (28 versus 31 weeks, P=0.002) than those with no ROP. No infant with BW>1500 g developed treatable ROP. Conditions that best predicted ROP development in the 1501 to 1800 g BW group were sepsis, ventilation >96 hours, antibiotic use >14 days, RBC transfusions greater than seven units, and central line placement (P=0.001, P=0.001, P=0.012, P=0.014 and P=0.035, respectively). All infants with BW>1500 g who developed ROP had greater than or equal to two of these RF. CONCLUSIONS: All cases of high-risk ROP would have been identified by current screening guidelines. Modified screening criteria of infants with (1) BW<or=1500 g or (2) BW 1501 to 2000 g and greater than or equal to two significant RF results in a $587.85 benefit per infant screened.

Birth Weight↗

Congenital retinal dystrophy and corneal opacity in trisomy 8 mosaicism.

Trisomy 8 mosaicism can present with a wide variety of systemic and ophthalmologic manifestations. We report a patient with an extinguished electroretinogram and diffuse retinal pigment epithelial abnormalities suggestive of a rod-cone dystrophy, which has not been previously reported in association with trisomy 8. A finding that has been described in several patients with trisomy 8 is a central corneal opacity, but there are no data regarding the natural history of this finding; the corneal opacity in our patient spontaneously improved. This case expands the clinical spectrum of this rare entity.

Chromosomes, Human, Pair 8↗

Visual field defects in deformational posterior plagiocephaly.

PURPOSE: We sought to determine whether visual field abnormalities occur in infants with deformational posterior plagiocephaly and to assess whether there is a relationship between the severity and laterality of visual field abnormalities with the severity and laterality of skull deformity. METHODS: A retrospective chart review was performed on 40 consecutive infants with deformational posterior plagiocephaly. Each was tested with standardized binocular arc perimetry in the horizontal plane. Sixteen patients also had 3-dimensional computed tomography of the skull. Hemifield asymmetry of >/=20 degrees and/or a decrease in hemifield values by >/=20 degrees from established normal patients was considered abnormal. Visual field data from study patients was plotted against previously published normative data. Graphs comparing visual field defects and laterality to cranial asymmetry also were generated. RESULTS: Thirty-five percent of infants with deformational posterior plagiocephaly had constriction of one or both hemifields by at least 20 degrees from established normal patients. Hemifield asymmetry of 20 degrees or more was found in 17.5% of infants tested. There was a significant difference in the worse hemifield values measured in each patient and the standard visual fields obtained from normative data ( P = 0.036). There was no correlation between the laterality of the visual fields to the laterality of the defects. A correlation between severity of hemifield constriction and % asymmetry on computed tomography was noted ( P = 0.209). CONCLUSIONS: Deformational posterior plagiocephaly may affect visual field development but neither the laterality nor the severity of skull deformity is predictive of the severity of visual field defects.

Humans↗

Prevalence of myopia at 9 months in infants with high-risk prethreshold retinopathy of prematurity.

PURPOSE: To examine the prevalence of myopia and high myopia at 9 months corrected age in premature infants who participated in the multicenter randomized trial of Early Treatment for Retinopathy of Prematurity (ETROP). DESIGN: Randomized, controlled clinical trial. PARTICIPANTS: Four hundred one infants with birth weights of <1251 g in whom prethreshold ROP developed in one or both eyes and who were determined to have a significant risk (> or =15%) of poor structural outcomes without treatment, based on the risk management for ROP program. INTERVENTION: Infants with bilateral high-risk prethreshold ROP (n = 317) had 1 eye randomized to early treatment, and the fellow eye was managed conventionally. In asymmetric cases (n = 84), the eye with high-risk prethreshold ROP was randomized to early treatment or conventional management (control). Eyes randomized to early treatment at high-risk prethreshold ROP and eyes randomized to conventional management in which threshold ROP developed received peripheral retinal photocoagulation or cryotherapy. Conventionally managed eyes in which threshold ROP did not develop were observed. Cycloplegic retinoscopy data were obtained at 9 months corrected age from 321 eyes treated early and 307 eyes managed conventionally. MAIN OUTCOME MEASURES: Prevalence of myopic (spherical equivalent > or = 0.25 diopters [D]) and highly myopic (> or =5.00 D) eyes in each group. RESULTS: The prevalence of myopia (64.5% vs. 69.4%; P = 0.06) and high myopia (25.5% vs. 28.3%; P = 0.20) was similar between eyes treated at high-risk prethreshold and high-risk prethreshold eyes managed conventionally. Among high-risk eyes managed conventionally, the prevalence of myopia (78.2% vs. 53.3%) and high myopia (37.6% vs. 11.2%) was higher when threshold ROP developed than when regression without treatment occurred. Among eyes treated at high-risk prethreshold ROP, the prevalence of myopia (93.3% vs. 91.7% vs. 60.6%) and of high myopia (53.3% vs. 33.3% vs. 20.8%) was higher in eyes with abnormal angle of temporal retinal vessels or macular ectopia than in eyes with no retinal residua. This also held true for conventionally managed eyes. CONCLUSIONS: Early treatment at high-risk prethreshold did not place eyes at greater risk of myopia and high myopia than did conventional management of eyes with high-risk prethreshold ROP.

Cryotherapy↗

Functional visual loss in adults and children patient characteristics, management, and outcomes.

OBJECTIVE: To compare the characteristics of functional visual loss (FVL) in adults and children. DESIGN: Retrospective chart review. PARTICIPANTS: One hundred forty patients diagnosed with FVL over a 5-year period. METHODS: Medical records of these patients were reviewed and data analyzed using statistical software. OUTCOME: Demographics, underlying organic and psychiatric disease, concomitant psychosocial events, and resolution rates were studied. RESULTS: Functional visual loss, with or without functional overlay, was initially diagnosed in 140 patients and was, in retrospect, a correct diagnosis in 138. There were 56 (40.6%) children and 82 (59.4%) adults (mean age, 13.4 and 40.0 years). The gender ratio, incidence of concomitant psychosocial events, incidence of functional overlay, prevalence of migraine or facial pain, and proportion referred for counseling were similar in the 2 groups. Concomitant psychosocial events were primarily social in children and related to trauma in adults. Thirty-two (39.0%) adults had a history of psychiatric illness, versus 10 (17.9%) children (P = 0.008). Symptoms were bilateral in 65.0% of cases. Functional visual loss manifested as visual acuity (VA) loss only occurred in 26.1% of patients, FVL manifested as visual field (VF) loss only was present in 28.3% of patients, and FVL with loss of both VA and VF occurred in 45.6% of patients. There was no significant difference in children versus adults in the proportion of VA, VF, or both being affected. Functional visual loss with coexistent organic disease (functional overlay) was present in 16.7% of patients. Follow-up information was available for 26.1% of patients. Normalization of any one parameter occurred in 58.3% of patients and was more likely in children. Three patients (2.2%) originally felt to have solely functional disease were subsequently diagnosed with organic disease. CONCLUSION: Functional visual loss is most common in teenagers, is typically bilateral, and involves both VA and VF. Normal VA was proven half the time at initial consultation. At all ages, patients were predominantly female, and one fifth had migraine, facial pain, or coexistent organic pathology. Concomitant psychosocial events were mainly social in children and related to trauma in adults. Psychiatric disease was twice as likely in adults. Normalization of visual function occurred in a majority of patients. Early-onset macular dystrophies and hereditary optic neuropathies may be misdiagnosed as FVL.

Adolescent↗

Safety and efficacy of 2% pirenzepine ophthalmic gel in children with myopia: a 1-year, multicenter, double-masked, placebo-controlled parallel study.

OBJECTIVE: To evaluate the safety and efficacy of the relatively selective M(1) antagonist pirenzepine hydrochloride in slowing the progression of myopia in school-aged children. METHODS: This was a parallel-group, placebo-controlled, double-masked study in healthy children, aged 8 to 12 years, with a spherical equivalent of -0.75 to -4.00 diopters (D) and astigmatism of 1.00 D or less. Patients underwent a baseline complete eye examination and regular examinations during a 1-year period. The setting was 13 US academic clinics and private practices. Patients were randomized in a 2:1 ratio to receive 2% pirenzepine ophthalmic gel or a placebo control twice daily for 1 year. RESULTS: At study entry, the spherical equivalent was mean +/- SD -2.098 +/- 0.903 D for the pirenzepine group (n = 117) and -1.933 +/- 0.825 D for the placebo group (n = 57, P = .22). At 1 year, there was a mean increase in myopia of 0.26 D in the pirenzepine group vs 0.53 D in the placebo group (P < .001). No patients in the placebo group and 13 (11%) of 117 patients in the pirenzepine group discontinued participation in the study because of adverse effects (5 [4%] of 117 due to excessive antimuscarinic effects). CONCLUSIONS: Pirenzepine is effective and relatively safe in slowing the progression of myopia during a 1-year treatment period.

Child↗

Progressive cerebellar tonsillar herniation with recurrent divergence insufficiency esotropia.

The Chiari malformations are characterized by herniation of posterior fossa contents through the foramen magnum. Chiari I malformation is currently defined as ectopia of the cerebellar tonsils more than 5 mm below the foramen magnum. Extension of the cerebellar tonsils up to 3 mm may be found in the normal population. Although Chiari malformations are congenital, symptoms often do not manifest until the third and fourth decades of life, or even later. Patients usually present with headache, lower cranial nerve palsies, downbeat nystagmus, ataxia, or dissociated anesthesia of the trunk and extremities. Definitive diagnosis is made by magnetic resonance imaging (MRI), which shows the compressed tonsils extending through the foramen magnum into the cervical subarachnoid space. One of the rare presenting signs of Chiari I malformations is acquired esotropia with a divergence insufficiency pattern. We report such a case in which the initial neuroimaging showed tonsillar herniation, but of insufficient magnitude to meet diagnostic criteria for Chiari I malformation. When the strabismus recurred after initially successful eye muscle surgery, follow-up scan showed progressive tonsillar herniation.

Adolescent↗

Ocular rosacea in childhood.

PURPOSE: To describe the clinical characteristics and treatment response of ocular rosacea in the pediatric population. DESIGN: Retrospective case series. METHODS: The clinic charts of consecutive pediatric cases of ocular rosacea were evaluated over a 34-month period. Minimal diagnostic inclusion criteria were the presence of posterior eyelid inflammation including meibomian gland inspissation and lid margin telangiectasis, in conjunction with conjunctival injection or episcleritis. RESULTS: Six patients ranged from 3 to 12 years of age at presentation. All shared a long history of ocular irritation and photophobia. Five patients (83%) were female and had bilateral involvement. Eyelid telangiectases and meibomian gland disease were present in all cases. Three patients (50%) had sterile corneal ulcers. Only two patients (33%) had cutaneous involvement at the time of diagnosis. All patients experienced significant improvement with a combination of oral antibiotics (doxycycline or erythromycin), with or without topical antibiotics (erythromycin or bacitracin) or topical steroids (fluorometholone). CONCLUSION: Ocular rosacea in children may be misdiagnosed as viral or bacterial infections. Unlike in adults, associated cutaneous changes are uncommon. Most disease is bilateral, although involvement may be asymmetric. Response to conventional treatment is excellent, although long-term treatment may be necessary to prevent relapses.

Anti-Bacterial Agents↗

Pediatric neuro-ophthalmology.

PURPOSE OF REVIEW: This review will update the ophthalmologist on recent developments in pediatric neuro-ophthalmology. RECENT FINDINGS: Research into the genetics of congenital strabismus syndromes has brought new insights into the development of the ocular motor system. There is also new literature on childhood ocular myasthenia gravis and childhood neurosarcoidosis. The results of three different surgical treatments for congenital nystagmus are described. Reviews on cortical visual impairment, dyslexia, Aicardi syndrome, and neuronal ceroid lipofuscinosis are presented. SUMMARY: Pediatric neuro-ophthalmology is a diverse and challenging field. As we strive to provide excellent care to these patients, we will use the results of basic science, genetic, and neurobiological research.

Child↗

A strabismus susceptibility locus on chromosome 7p.

Strabismus has been known to have a significant genetic component, but the mode of inheritance and the identity of the relevant genes have been enigmatic. This paper reports linkage analysis of nonsyndromic strabismus. The principal results of this study are: (i) the demonstrated feasibility of identifying and recruiting large families in which multiple members have (or had) strabismus; (ii) the linkage in one large family of a presumptive strabismus susceptibility locus to 7p22.1 with a multipoint logarithm of odds score of 4.51 under a model of recessive inheritance; and (iii) the failure to observe significant linkage to 7p in six other multiplex families, consistent with genetic heterogeneity among families. These findings suggest that it will be possible to localize and ultimately identify strabismus susceptibility genes by linkage analysis and mutation screening of candidate genes.

Chromosome Mapping↗

Retinopathy in patients with diabetic ophthalmoplegia.

PURPOSE: To review the clinical characteristics, prevalence, and severity of retinopathy in diabetics with cranial nerve (CN) 3, 4, and/or 6 palsies, and to determine the relationship between type and duration of diabetes mellitus (DM), presence of retinopathy, and occurrence of CN palsy. DESIGN: Retrospective, comparative cohort study. PARTICIPANTS: Chart reviews of 2229 patients with CN 3, 4, and/or 6 palsies were performed at the Bascom Palmer Eye Institute (BPEI) from January 1991 through December 1997 and at the Dean A. McGee Eye Institute (DMEI) from January 1994 through July 2001. A total of 306 patients qualified for the study group. The Wisconsin Epidemiologic Study of Diabetic Retinopathy (WESDR) was used as a control. METHODS: Demographic and clinical data were extracted to determine characteristics of patients with diabetic ophthalmoplegia. The subsets of data regarding type of DM and level of diabetic retinopathy in the study population were compared with the WESDR control data for statistical analysis. MAIN OUTCOME MEASURES: The prevalence of diabetic retinopathy in patients with diabetic ophthalmoplegia. RESULTS: Of 2229 patients at both institutions with ocular motor CN palsy, 306 (13.7%) were associated with DM. The frequency of CN involvement was 6 (50.0%), 3 (43.3%), and 4 (6.7%). There was a total of 12 patients (3.9%) with consecutive palsies and 8 patients (2.6%) with simultaneous palsies (5 unilateral and 3 bilateral). At both institutions, the prevalence of retinopathy controlling for duration of DM was lower in both insulin-dependent DM (IDDM) and non-IDDM (NIDDM) type II diabetics as compared with controls (BPEI, P = 0.009 and P = 0.005; DMEI, P = 0.004 and P = 0.29). When data from both locations were combined, the difference was even more significant (IDDM, P = 0.001 and NIDDM, P = 0.006). There were no significant differences between the two institutions in gender, type or duration of DM, age at presentation, or frequency of CN involvement. CONCLUSIONS: Diabetic ophthalmoplegia most commonly involves CN 3 and 6, with relative sparing of CN 4. Multiple cranial nerves are affected simultaneously in 2.6% of cases, and consecutive palsies occurred in 3.9% of cases. Type II diabetics with ocular motor CN palsy have significantly less diabetic retinopathy than do controls. This may imply a different pathophysiologic mechanism for these two microvascular complications of DM.

Abducens Nerve Diseases↗

Pseudotumor cerebri in idiopathic aplastic anemia.

Idiopathic intracranial hypertension, also referred to as pseudotumor cerebri (PTC), is characterized by high intracranial pressure, normal cerebral ventricles,and cerebrospinal fluid. Although papilledema is described in various anemias, only one case of PTC with idiopathic aplastic anemia has been reported in the English literature. We report a case of papilledema in an adolescent with aplastic anemia and propose its mechanism.

Acetazolamide↗

Optic neuritis in adults and children.

Most ophthalmologists will encounter optic neuritis in the course of their practice. The disease behaves very differently in children versus adults. The ONTT and CHAMPS trials have given us important data regarding prognosis and management in adults, including probability of developing MS. Optic neuritis in children, while less common, must be considered in the differential diagnosis of subacute visual loss, especially in children with bilateral impairment. Secondary causes of optic neuritis are more common in children and an investigation should be performed to assess for associated disease states. In both populations, the ophthalmologist will play a central role in the diagnosis, treatment, and management of these patients, whether it is an isolated case of optic neuritis or the initial presentation of a long-term struggle with MS. With the advent of immunomodulating therapy to minimize both the subsequent incidence and severity of MS, early diagnosis and appropriate management by the ophthalmologist has become even more critical.

Adult↗

Surgical management of skew deviation.

There are no published data on the outcomes of realignment surgery for skew deviation. A retrospective chart review disclosed 10 patients who had undergone surgical correction of skew deviation by three surgeons at a single institution between 1991 and 2002. Nine of 10 patients had satisfactory relief of diplopia with an acceptable field of single binocular vision. Vertical rectus recession or resection was the most common procedure. Four patients required more than one procedure. For nonalternating hypertropias, resection of the inferior rectus muscle or recession of the superior rectus muscle of the hypertropic eye was successful. For alternating hypertropia, resection of both inferior rectus muscles was successful. Oblique muscle surgery was not associated with good outcomes.

Adult↗

Factors associated with horizontal reoperation in infantile esotropia.

PURPOSE: Risk factors for requiring multiple surgeries in infantile esotropia remain unclear. We identified clinical and demographic factors associated with horizontal reoperation in this disease. METHODS: A retrospective chart review of patients who underwent surgery from 1994-1997 was performed. Subjects were divided into 2 groups: those requiring only one operation and those requiring 2 or more operations to achieve orthotropia +/-10 PD. RESULTS: In 149 patients, the overall horizontal reoperation rate was 34%. There were no statistically significant differences between the 2 groups with respect to mean age at first surgery, mean preoperative deviation, gender, prematurity, Medicaid coverage, parental age, family history of strabismus, or refractive error. The presence of nystagmus, oblique muscle dysfunction, dissociated vertical deviation (DVD), or a variable angle of esotropia was not associated with increased horizontal reoperation rate. There was a greater frequency of horizontal reoperation in patients with amblyopia, although not significant. Premature infants and infants with neurologic dysfunction had a lower incidence of horizontal reoperation, but also not significant. Deviations of less than 30 PD were associated with fewer horizontal reoperations (16% vs 31%, P =.047). Significantly more patients underwent horizontal reoperation when initial surgery was performed at less than or equal to 15 months of age (67% vs. 47%, P =.022). CONCLUSIONS: Several factors thought to predispose to poor sensorimotor outcome (dissociated vertical deviation, oblique muscle dysfunction, and nystagmus) were not associated with an increased incidence of horizontal reoperation. Horizontal reoperation was less frequent in patients with angles less than 30 PD. Although some studies suggest that early surgical intervention in patients with infantile esotropia affords better sensory outcome, it may be associated with a higher horizontal reoperation rate.

Child, Preschool↗