Multiclonal outbreak of methicillin-resistant Staphylococcus aureus infections on a collegiate football team.

An outbreak of methicillin-resistant Staphylococcus aureus (MRSA) skin and soft tissue infections (SSTIs) occurred in a college football team in August 2006. Of 109 players on the team roster, 88 (81%) were interviewed during a cohort investigation. Twenty-five cases were identified, six of which were culture-confirmed. Available culture isolates were typed by pulsed-field gel electrophoresis (PFGE), which identified two different MRSA strains associated with the outbreak. Playing positions with the most physical contact (offensive linemen, defensive linemen, and tight ends) had the greatest risk of infection [risk ratio (RR) 5.1, 95% confidence interval (CI) 2.3-11.5. Other risk factors included recent skin trauma (RR 1.9, 95% CI 0.95-3.7), use of therapeutic hydrocollator packs (RR 2.5, 95% CI 1.1-5.7), and miscellaneous training equipment use (RR 2.1, 95% CI 1.1-4.1). The outbreak was successfully controlled through team education and implementation of improved infection-control practices and hygiene policies.

A survey of vision screening policy of preschool children in the United States.

A state-by-state survey regarding preschool vision screening guidelines, policies, and procedures was conducted. Currently 34 states provide vision screening guidelines and 15 states require vision screening of at least some of their preschool-aged children. The Department of Public Health administers the programs in 26 states, the Department of Education in 13. A wide range of professional and lay personnel conduct preschool vision screenings, and nurses participate in the screening process in 22 states. Visual acuity is assessed in 30 states, eye alignment in 24 states, refractive error in eight states, and color vision in 10 states. A combination of screening tests is recommended in 24 states. Currently, 45 states do not require screening of all preschool children. Thus, although laws, guidelines, and recommendations exist in most states, many preschool-age children do not have access to vision screening programs.

Vision screening of preschool children: evaluating the past, looking toward the future.

Vision problems of preschool children are detectable with a comprehensive eye examination; however, it is estimated that only 14% of children below the age of 6 years receive an eye examination. Screening is advocated as a cost-effective alternative to identify children in need of further vision care. Thirty-four states recommend or require vision screening of preschool children. Although laws and guidelines exist, only 21% of preschool children are actually screened for vision problems. There is little agreement concerning the best screening methods, and no validated, highly effective model for screening vision of preschool children. Newer screening tests have been designed specifically for preschool populations, and can be administered by lay screeners. Many have not been validated. Several are recommended by states or organizations without convincing scientific evidence of their effectiveness. This paper summarizes current laws and guidelines for preschool vision screening in the United States, reviews advantages and disadvantages of several test procedures, and provides recommendations for developing future preschool vision screening programs.

Cephalometric analysis of families with dominantly inherited Crouzon syndrome: an aid to diagnosis in family studies.

Crouzon syndrome (CS) is an autosomal dominant condition comprising orbital proptosis, midfacial hypoplasia, premature sutural synostosis, and altered proportions of bone lengths in the hands. In families the CS trait is highly variable. Several cases of affected sibs born to unaffected parents have been explained by germinal mosaicism. We hypothesized that cephalometric and metacarpophalangeal analysis may help to classify affected and unaffected subjects within families when clinical diagnosis is difficult. Posterior-anterior and lateral cephalometric radiographs and hand films were taken of 10 CS patients and 18 unaffected relatives. Sixty-two craniofacial and 19 hand linear and angular measurements were made on each subject and standardized by conversion to z-scores using published normal standards. Ten craniofacial variables were selected for use in a stepwise forward discriminant function analysis to develop an equation which could be used to discriminate CS patients from normal subjects. A two-group discriminant function using four craniofacial variables and one hand variable correctly classified the CS patients and relatives 100% of the time. The results suggest that relatively few facial variables are needed to differentiate most cases of CS but the addition of one or more hand variables may increase the sensitivity. DNA testing is necessary to adequately demonstrate incomplete penetrance in CS, but pretesting subjects for molecular studies using these methods may improve results.

Plasticity of human motion processing mechanisms following surgery for infantile esotropia.

Monocular oscillatory-motion visual evoked potentials (VEPs) were measured in prospective and retrospective groups of infantile esotropia patients who had been aligned surgically at different ages. A nasalward-temporal response bias that is present prior to surgery was reduced below pre-surgery levels in the prospective group. Patients in the retrospective group who had been aligned before 2 yr of age showed lower levels of response asymmetry than those who were aligned after age 2. The data imply that binocular motion processing mechanisms in infantile esotropia patients are capable of some degree of recovery, and that this plasticity is restricted to a critical period of visual development.

Cephalometric evidence for a dominantly inherited predisposition to cleft lip-cleft palate in a single large kindred.

Several studies have demonstrated an association between facial shape in parents and the presence of oral clefts in their offspring. However, these observations have been of little practical value because it has been assumed that facial shape was just one predisposing component among many in a multifactorial model of inheritance. Cephalometric analysis of a large family with 5 generations of affected individuals suggests that facial shape can be used to identify presumed carriers of a major gene associated with an increased risk for oral clefts. Discriminant function analysis indicates that such at risk individuals can be recognized effectively through a combination of increased midfacial and nasal cavity widths, reduced facial height, and a flat facial profile. The ability to identify minimally affected gene carriers within families would provide critical information needed in the search for molecular markers that segregate with the genetic risk for clefting.

Quantitation of craniofacial anomalies in utero: fetal alcohol and Crouzon syndromes and thanatophoric dysplasia.

The study of fetal growth and development by ultrasound has been greatly facilitated in the past few years by the availability of anthropometric standards for the fetal body. Thus, the obstetrician is able to discern between normal and grossly abnormal, and even to quantitate certain fine fetal structures such as the face. This paper presents results obtained from a group of 5 patients referred to the Medical Center from private practices in Indianapolis, Indiana. Prenatal cephalometric analyses by ultrasound suggested the presence of craniofacial anomalies in all 5 cases. However, such defects were not detectable by routine ultrasonographic examination. A clinical examination after birth of each of these 5 patients suggested the following diagnoses: Fetal Alcohol Syndrome (FAS) in 2 individuals, Fetal Alcohol Effects (FAE) in one individual, Crouzon Syndrome (CS) in one patient, and Thanatophoric Dysplasia (TD) in one patient. In order to compare the craniofacial measurement values for each patient to normal standards, we developed Z-Score profiles and Pattern Variability Indexes (PVI) as described by Garn et al. [1984, 1985]. The values presented here support the idea that even mildly abnormal fetal craniofacial patterns are detectable by this relatively new application of ultrasound. At the present time, no conclusions can be made regarding the diagnostic accuracy of these patterns and profiles. However, the potential value of fetal cephalometry for documenting craniofacial dysmorphology is clearly indicated.

Developmental significance of delayed closure of the mandibular symphysis.

The unique finding of a midline suture at the mandibular symphysis in cleidocranial dysplasia is presented and discussed in regard to its relation to facial skeletal development.

X-linked (recessive) hypomaturation amelogenesis imperfecta: a prosthodontic, genetic, and histopathologic report.

A 16-year-old white girl requested esthetic restorations for her teeth. She was a manifesting heterozygote for the X-linked recessive form of amelogenesis imperfecta with hypomaturation defect. Lyonization theory states that in the somatic cells of female mammals, one of the two X chromosomes is randomly inactivated early in development. Therefore, females who are heterozygous for a given X-linked gene will be mosaic with varying proportions of cells in which only one of a particular pair of alleles is active. This mosaicism produced by lyonization ensures considerable phenotypic variability in the clinical expression of X-linked disorders. Histologic examination of the patient's extracted third molars demonstrated the expected lyonization effect. The patient received six porcelain laminate veneered crowns to restore her maxillary incisor and canine teeth. This article represents the first reported use of such restorations to alleviate the cosmetic and functional handicaps that accompany this genetic disease.

Clouston syndrome: a rare autosomal dominant trait with palmoplantar hyperkeratosis and alopecia.

A caucasian family is reported in which four males and four females in two generations have exhibited alopecia, dysplastic nails, and hyperkeratosis of palmar and plantar surfaces. This type of ectodermal dysplasia, Clouston syndrome, features normal teeth with severe hair and nail dysplasia.

Anomalous motion VEPs in infants and in infantile esotropia.

Visual evoked potentials (VEPs) were recorded monocularly in response to vertical gratings that underwent oscillatory apparent motion at a temporal frequency of 10 Hz. In normal infants 6 months or younger and in patients with a history of constant strabismus onset before 6 months of age, the oscillatory motion VEP contains a prominent first harmonic component that is temporally 180 degrees out of phase in the two eyes. This pattern is not seen in normal adults and is consistent with the presence of a nasalward/temporalward asymmetry of cortical responsiveness in infants and in patients with early onset strabismus.

Improved outcome in Pierre Robin sequence: effect of multidisciplinary evaluation and management.

Infants with complications of Pierre Robin sequence are at increased risk of airway obstruction and resultant hypoxia, cor pulmonale, failure to thrive, and cerebral impairment. In an effort to minimize such complications, patients with Pierre Robin sequence were examined prospectively by a multidisciplinary team using polysomnography and continuous oximetry. Obstructive apnea and desaturation occurred in 18 of the 21 patients studied. Four children required only home apnea monitoring, and six required only monitoring and supplemental oxygen. Seven children had lip-tongue adhesion procedures performed, and four required tracheostomy. No patients died. All patients with isolated Pierre Robin sequence had normal development at follow-up except for one child who had experienced a respiratory arrest before referral. With improvements in neonatal intensive care, testing for respiratory assessment, improved surgical and postoperative intervention and home monitoring, the morbidity and mortality for children with Pierre Robin sequence can be reduced markedly.

Bone dysplasias: the prenatal diagnostic challenge.

The prenatal diagnosis of bone dysplasias presents difficult challenges for the clinician involved in monitoring pregnancies. Such diagnoses highlight delicate ethical issues and may require difficult decision-making when the differential diagnosis includes a lethal bone dysplasia. Despite the rapid technological advances in ultrasonography, the ability to make prenatal diagnoses within this group of disorders is limited by the restricted ultrasonographic capability to appreciate fully the detailed fetal anatomy. However, we perceive that a significant further limitation involves the lack of a systematic protocol to guide the clinician in the ultrasonographic evaluation of a fetus suspected of having a skeletal dysplasia. In an attempt to aid the clinician who is evaluating these suspected pregnancies, we report here 8 cases and propose a model protocol for the ultrasonographic diagnostic approach to fetal skeletal problems in utero.

Facial morphometrics in the identification of gene carriers of X-linked hypohidrotic ectodermal dysplasia.

Roentgenographic measurements and morphometric analysis were employed in the investigation of contrasting patterns of craniofacial variation between normal individuals and those affected by X-linked hypohidrotic ectodermal dysplasia (HED). The research objective was to identify and describe the facial characteristics of heterozygous gene carriers who show minor expression of the disorder. In this study of 13 HED families with 16 affected males, 12 carriers, and 12 normal individuals, affected individuals had at least 3 of the following 4 clinical signs and symptoms: a) hypodontia, b) hypohidrosis, c) hypotrichosis, and d) clinically distinct facial physiognomy. By contrast, the gene carriers manifested only one or 2 or none of the 4 clinical manifestations. In a preliminary comparison of gene carriers vs. normal individuals, we have generated 2 discriminant functions (each based on 3 facial measurements taken either from the lateral or frontal cephalograms). These 2 functions correctly diagnose 100% of the gene carriers and normal HED relatives. Facial anomalies characteristic of the gene carriers were 1) abnormally narrow and short maxillary width and palatal depth dimensions; 2) very small and retrusive malar and maxillary regions; 3) markedly reduced lower facial depth, height and width dimensions; 4) small head height, prominent forehead, and high-set orbits; 5) a generalized, symmetric reduction of the whole craniofacial complex.

A morphometric analysis of the fetal craniofacies by ultrasound: fetal cephalometry.

We present here a set of 24 standardized linear measurements that describe the growth of different craniofacial structures in the normal fetus from 16 to 36 weeks of gestation. These measurements were taken from 89 pregnant women, who had from 1 to 3 ultrasonographic evaluations during the pregnancy (16, 26, and 36 weeks of gestation). All the values presented here were obtained using the technique described by Escobar et al. The mean and standard deviation was calculated for each measurement and was used to estimate the normal growth pattern of each variable. Approximate confidence intervals for the mean of each variable were constructed for use in identifying unusually low or high values. The confidence intervals are available in graphic form by request. These data will not only contribute to an understanding of fetal craniofacial growth and development in utero, but in addition, it will help to make the diagnoses of mild craniofacial anomalies that would not be detected by the routine ultrasonographic examination. We suggest that this procedure should be included if not in all routine obstetrical ultrasound evaluations, then at least in the more extensive level II obstetrical ultrasound.

A study of cephalometric features in cleft lip-cleft palate families. I: Phenotypic heterogeneity and genetic predisposition in parents of sporadic cases.

Several previous studies have indicated that unaffected parents of children with nonsyndromic cleft lip and palate show unusual craniofacial features. This study reexamines this question by applying multivariate cluster analysis to lateral cephalometric head plates from 82 individuals who are parents of sporadic cases of cleft lip with or without cleft palate (CL/P). Considerable phenotypic heterogeneity was present within the sample. Three major groupings were defined. Two of these groups showed cephalometric similarities to individuals with overt clefts, while the third showed a generalized concordance to published norms. In almost every case only one member of each parental pair showed the cleft related cephalometric phenotype, suggesting the possibility of a substantial genetic component in many cases of sporadic CL/P. However, there were several cases in which neither parent showed the phenotypic traits. Such cases may have a different etiology or a greater environmental component.

Visual assessment of the multiply handicapped patient.

The visual capabilities of the multiply handicapped and/or developmentally delayed patient are difficult to assess with methods that depend on the patient's subjective responses. Fifty-nine patients with multiple neurological handicaps and unknown visual capabilities were examined using a modified ophthalmic examination which included visual acuity measures using visual evoked potential (VEP) and preferential looking (PL) techniques. Patients ranged in age from 3 to 33 years; median age 9 years. Significant refractive error (in 73%) and strabismus (in 71%) were the most common ocular disorders. Of the 43 patients with a significant refractive error, only 16 (37%) were wearing their proper correction (ranging from -21 to +20 D). In 27 patients the uncorrected refractive error ranged from -10 to +20 D. Binocular acuities (with refractive correction) could be obtained from 56 patients (95%) using a spatial frequency sweep VEP technique, and in 41 patients (70%) using PL grating acuity cards. The VEP and PL grating acuity measures agreed to within 1 octave (a factor of 2 in minimum angle of resolution) in 27 of 41 patients. VEP acuity was 1.1 to 2.7 octaves higher in 12 patients. Grating acuity of at least 6/12 (20/40) was estimated in 12 patients. Residual vision can be measured in "difficult to examine" multiply handicapped patients with VEP and PL techniques.