Lynn C. Richman

Learning disability, school achievement, and grade retention among Children with cleft : A two-center study

OBJECTIVE This study examined the prevalence of learning disability (LD), level of school achievement; and prevalence of grade retention by type of cleft and gender at two craniofacial centers. SETTING The setting included two university-based craniofacial centers. DESIGN/PATIENTS Participants included 84 consecutively evaluated patients from one center who were matched by cleft type, age, and gender with 84 patients evaluated at the second center. OUTCOMES The outcomes included learning disability, school achievement, and grade retention. RESULTS The results revealed that 46% of subjects with cleft had LD, 47% had deficient educational progress, and 27% had repeated a grade (excluding kindergarten) in school. Males with cleft palate only (CPO) had a significantly higher rate of LD than any other subject group. Males with CPO and females with cleft lip and palate (CLP) were more likely to repeat a grade in school than were females with CPO and males with CLP. CONCLUSIONS Children with cleft are at risk for learning disability, low school achievement, and grade retention.

Structural brain abnormalities in adult males with clefts of the lip and/or palate.

Purpose: To evaluate brain morphology of adult males with nonsyndromic clefts of the lip and/or palate (NSCLP) in comparison to a matched healthy control group.Methods: Brain structure was measured using quantitative analysis of magnetic resonance images.Results: Subjects with NSCLP had significant abnormalities in brain morphology consisting of abnormally enlarged anterior regions of the cerebrum, and decreased volumes of the posterior cerebrum and cerebellum. Overall, the most severely affected region was the left temporal lobe. Furthermore, these structural abnormalities were directly related to cognitive dysfunction.Conclusions: These findings highlight the important relationship and interplay between face and brain development.

Different Cleft Conditions, Facial Appearance, and Speech: Relationship to Psychological Variables

OBJECTIVE The purpose of this investigation was to study the adjustment and learning characteristics of children with different types of clefts. The hypotheses were that there may be different relationships among cleft variables (speech and appearance) according to the cleft types. DESIGN The study compared three cleft groups on behavior rating, anxiety scales, depression scales, and self-perception (analyses of variance) and examined the influence of facial and speech ratings on self-perception (multiple regression analyses). SETTING All patients were treated at a university hospital cleft palate clinic. PATIENTS Sixty-five children aged 8 years to 17 years were selected based on nonsyndromic cleft (unilateral cleft lip and palate [ULP], bilateral cleft lip and palate [BLP], and cleft palate only [CPO]) and no significant neurological condition or hearing loss. RESULTS The findings indicated children with CPO showed greater problems with parent- and teacher-reported depression, anxiety, and learning related to speech than children with ULP or BLP. The later two groups showed fewer problems and a greater relationship of problem to facial appearance. The children with ULP self-reported lower levels of depression than the other two groups. CONCLUSIONS Children with cleft show relatively good overall adjustment, but some problems appear related to speech and facial appearance. Subgroups may need to be studied separately.

Right ventromedial prefrontal cortex: a neuroanatomical correlate of impulse control in boys

Emerging data on the neural mechanisms of impulse control highlight brain regions involved in emotion and decision making, including the ventromedial prefrontal cortex (vmPFC), anterior cingulate cortex (ACC) and amygdala. Variation in the development of these regions may influence ones propensity for impulsivity and, by extension, ones vulnerability to disorders involving low impulse control (e.g. substance abuse). Here we test the hypothesis that lower impulse control is associated with structural differences in these regions, particularly on the right side, in 61 normal healthy boys aged 7-17. We assessed parent- and teacher-reported behavioral ratings of impulse control (motor impulsivity and non-planning behavior) in relation to vmPFC, ACC and amygdala volume, measured using structural magnetic resonance imaging and FreeSurfer. A regression analysis showed that the right vmPFC was a significant predictor of impulse control ratings. Follow-up tests showed (i) a significant correlation between low impulse control and decreased right vmPFC volume, especially the medial sector of the vmPFC and (ii) significantly lower right vmPFC volume in a subgroup of 20 impulsive boys relative to 20 non-impulsive boys. These results are consistent with the notion that right vmPFC provides a neuroanatomical correlate of the normal variance in impulse control observed in boys.

Cognitive profiles of children with insulin-dependent diabetes.

Intellectual and reading skills were evaluated and related to disease variables in 42 children with Type I (insulin-dependent) diabetes. A significant interaction revealed lower Wechsler Intelligence Scale for Children-Revised (WISC-R) Performance IQ for children with early disease onset (>7 years) and long disease duration (≤5 years). IQ scores were nevertheless in the average range. Although there was no specific pattern of visual spatial impairment, functioning on the Performance subtests was uniformly lower for this group. Slower responding to the timed tasks of the Performance scale may account for generally lower scores. Children with early onset-long duration also evidenced higher rates of reading and memory impairment. These results indicate the importance of ascertaining educational skills in diabetic children before planning diabetic treatment regimens, especially for children with disease of early onset and long duration, who may be especially vulnerable to skill deficits.

Biological and Environmental Predictors of Behavioral Sequelae in Children Born Preterm

OBJECTIVE: By using behavioral outcome measures of children who were born preterm, we evaluated differences between children who were born at term and children who were born at extremely low (ELBW; <1000 g) and very low birth weights (VLBW; 1000–1499 g) and assessed the relationship of birth weight, socioeconomic status, and cognitive ability to behavioral outcome. METHODS: We studied a total of 104 children (aged 7–16 years). Of these, 49 had a preterm birth (31 of ELBW and 18 of VLBW). The remaining 55 were healthy control subjects. Children were administered tests of cognitive ability. Parents and teachers completed behavioral assessments. Multivariate analyses of covariance assessed differences between children who were born at term and those who were born of ELBW and of VLBW on behavioral measures. Hierarchical linear regressions were used to assess relationships among biological (birth weight), environmental (socioeconomic status), intellectual, and behavioral variables. RESULTS: Children who were born at term had fewer parent reports of hyperactivity/inattention and depression/anxiety symptoms than children of ELBW and VLBW. Teacher ratings were not significant between groups. Birth weight was consistently the strongest predictor of parent ratings of behavioral outcome, and intelligence level did not seem to mediate this relationship. CONCLUSIONS: Negative behavioral sequelae of preterm birth remain significant in middle childhood and adolescence, although the contribution of multiple factors to neurobehavioral outcome is complex. Research to assess these relationships, integrated with anatomic and functional neuroimaging, is needed to advance knowledge and improve outcomes for children who are born preterm.

Long-term outcome of brain structure in premature infants: effects of liberal vs restricted red blood cell transfusions.

OBJECTIVE To assess the long-term outcome of brain structure in preterm infants, at an average age of 12 years, who received a red blood cell transfusion for anemia of prematurity. DESIGN As neonates, this cohort of infants participated in a clinical trial in which they received red blood cell transfusions based on a high pretransfusion hematocrit threshold (liberal group) or a low hematocrit threshold (restricted group). These 2 preterm groups were compared with a group of full-term healthy control children. SETTING Tertiary care hospital. PARTICIPANTS Magnetic resonance imaging scans for 44 of the original 100 subjects were obtained. INTERVENTION Liberal vs restricted transfusion. MAIN OUTCOME MEASURES Intracranial volume, total brain tissue, total cerebrospinal fluid, cerebral cortex and cerebral white matter volume, subcortical nuclei volume, and cerebellum volume. RESULTS Intracranial volume was substantially smaller in the liberal group compared with controls. Intracranial volume in the restricted group was not different from controls. Whole-cortex volume was not different in either preterm group compared with controls. Cerebral white matter was substantially reduced in both preterm groups, more so for the liberal group. The subcortical nuclei were substantially decreased in volume, equally so for both preterm groups compared with controls. When sex effects were evaluated, the girls in the liberal group had the most significant abnormalities. CONCLUSION Red blood cell transfusions affected the long-term outcome of premature infants as indicated by reduced brain volumes at 12 years of age for neonates who received transfusions using liberal guidelines.

Cognitive dysfunction in adult males with non-syndromic clefts of the lip and/or palate

Cognitive deficits have been well-documented in children with non-syndromic clefts of the lip and/or palate. However, no study to date has formally assessed cognition in adults with oral clefts. This study was designed to evaluate neuropsychological functioning in adult males with non-syndromic clefts of the lip and/or palate (n=50) compared to age and sex-matched controls. Subjects with oral clefts were found to have significantly lower full scale IQ, performance IQ and verbal IQ scores compared to their matched controls. After controlling for IQ, patients showed specific deficits in verbal fluency. Adult males with oral clefts manifest a specific pattern of cognitive deficits. As the development of the face is highly interdependent with the development of the brain, it is theorized that the etiology of these cognitive deficits is a primary problem with abnormal brain development.

Neuropsychological, behavioral, and academic sequelae of cleft: early developmental, school age, and adolescent/young adult outcomes.

This article reviews behavioral, neuropsychological, and academic outcomes of individuals with cleft across three age levels: (1) infancy/early development, (2) school age, and (3) adolescence/young adulthood. The review points out that attachment, neurocognitive functioning, academic performance/learning, and adjustment outcomes are the result of a complex interaction between biological and environmental factors and vary with developmental level, sex, and craniofacial anomaly diagnosis. The degree to which associated genetic or neurodevelopmental conditions may explain inconsistent findings is unknown and suggests the need for caution in generalizing from group data on cleft.

Increased incidence of a midline brain anomaly in patients with nonsyndromic clefts of the lip and/or palate.

Background and Purpose. Nonsyndromic clefts of the lip and palate (CLP) are developmental craniofacial abnormalities that are often associated with cognitive dysfunction. This study was designed to evaluate, in patients with CLP, the presence of a specific midline brain anomaly (enlarged cavum septi pellucidi [CSP]) that has been shown in other developmental syndromes to be related to poor cognitive function. Methods. Brain images were obtained using magnetic resonance imaging on 49 adult men with CLP and 75 healthy controls. Size of CSP was measured using consecutive coronal images. Results. The incidence of large CSP in the CLP group was 8% (4 of 49), significantly higher than that found in the control group. In 2 of these 4 subjects, the anomaly was complete nonfusion of the septal leaflets, known as a combined CSP and cavum vergae. Furthermore, there was a significant inverse relationship of IQ and CSP in CLP patients that was not present in controls. That is, in individuals with CLP, the larger the CSP, the lower the IQ. Conclusions. Adult men with CLP have an increased prevalence of enlarged CSP. Moreover, this anomaly is directly related to cognitive deficits. This study provides further evidence that the development of the face and the development of the brain are intimately related and that defects in craniofacial development are most likely associated with defects in brain development.