Cognitive Deficits in School-Age Children with Severe Short Bowel Syndrome
S.R. Beers, PhD; J.A. Yaworski, MSN, RN; Carol Stilley, PhD, RN; L. Ewing, PhD, RN; and E.M. Barksdale Jr., MD. The Department of Psychiatry, University of Pittsburgh School of Medicine; the Intestinal Care Center, UPMC Children’s Hospital of Pittsburgh and University of Pittsburgh School of Nursing, Pittsburgh, Pa.
Purpose: Improved therapies for the management of short bowel syndrome (SBS) have resulted in the prolonged survival of many children. By early childhood, the physiological sequelae of severe SBS include delayed physical development and metabolic imbalances. However, little is known about how SBS affects brain development. Although many parents report school problems, no controlled study has evaluated the integrity of the central nervous system in SBS children. The purpose of this study was to investigate the neuropsychological status of school-aged SBS children to determine if there were characteristic cognitive impairments that might be amenable to early therapeutic intervention.
Methods: SBS children (n=8; mean age=116.9m+21m) were compared to an age-matched cystic fibrosis (CF) control group (n=8; mean age=118.1+14m). Groups did not differ in age, grade or absences. Neuropsychological tests with established sensitivity to CNS integrity compared performance over six cognitive domains. Emotional status also was measured. Analyses were completed with two-tailed t-tests.
Results: Groups did not differ on tests of intellectual ability and emotional function. Language, memory and learning, and problem-solving testing indicated no significant group differences. However, the SBS group performed more poorly on measures assessing visual-spatial ability, with p values ranging from .002 to .045. In a subset of subjects, we noted significantly slower left-handed, but not right-handed, performance on measures of finger dexterity and psychomotor speed.
Conclusions: Although emotional status did not differ from that of children with CF, SBS patients demonstrated visual-spatial deficits in the company of preserved language, attention and memory, and executive skills. The specificity and consistency of these findings suggests that right hemisphere CNS changes may occur in children with SBS. This unexpected finding, coupled with the indication of left-sided psychomotor slowing in right-handed subjects, raises the possibility that actual brain impairment, rather than developmental delay accompanying slowed physical growth, accounts for these findings. Longitudinal studies are needed to further clarify this issue. The educational significance of the results is discussed.
Published in the Journal of Pediatric Surgery, Vol. 35(6):860-865, June 2000.
Cognitive and Psychosocial Function in Children with Short Bowel Syndrome
S.R. Beers, PhD; E.M. Barksdale Jr., MD; L. Ewing, PhD, RN and J.A. Yaworski, MSN, RN. University of Pittsburgh School of Medicine and the Intestinal Care Center, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, Pa.
Short bowel syndrome (SBS) is characterized by the malabsorption of micro- and macronutrients that results in malnutrition. Principle etiologies occur during the prenatal or early neonatal period and lead to massive loss of intestinal absorptive capacity. By early childhood, physiological sequelae include the delay of physical development due to suspected growth hormone deficiency. Longer survival time, resulting from better clinical management, has led to increased interest in quality of life issues that include emotional adjustment. Many parents report school problems, but to our knowledge, the cognitive function of children with SBS has not been formally evaluated. We review the cognitive profiles of three boys (ages 6 to 7 years) with SBS. Neuropsychological testing evaluated sensory-perceptual skills, language, memory, visual-spatial ability, motor skills, and problem solving. All three children showed attention problems and marked deficits in visual-spatial and motor skills, while two showed evidence of psychological distress on a personality test. Results are discussed with respect to mechanisms of dysfunction, impact on school function and treatment. These three cases demonstrate that children with SBS are at risk for significant cognitive and emotional problems and stress the importance of early intervention.
Presented at the International Symposium on Therapeutic Outcome of Endocrine Disorders: Efficacy, Innovation and Quality of Life, Palm Beach Gardens, Fla., November 13-16, 1997.
