Cognitive deficits in school-age children with severe short bowel syndrome

Background/Purpose: Improved therapies for the management of short bowel sy ndrome (SBS) have resulted in the prolonged survival of many children. By e arly childhood, the physiological sequelae of severe SBS include delayed ph ysical development and metabolic imbalances. However, little is known about how SBS affects brain development. Although many parents report school pro blems, no controlled study has evaluated the integrity of the central nervo us system in SBS children. The purpose of this study was to investigate the neuropsychological status of school-aged SBS children to determine if ther e Were characteristic cognitive impairments that might be amenable to early therapeutic intervention. Methods: SBS children (n = 8; mean age, 116.9 +/- 21 months) were compared with an age-matched cystic fibrosis (CF) control group (n = 8; mean age, 11 8.1 +/- 14 months). Groups did not differ in age, grade, or absences. Neuro psychological tests with established sensitivity to CNS integrity compared performance over 6 cognitive domains. Emotional status also was measured. A nalyses were completed with 2-tailed t tests. Results: Groups did not differ on tests of intellectual ability and emotion al function. Language, memory and learning, and problem-solving testing res ults indicated no significant group differences. However, the SBS group per formed more poorly on measures assessing visual-spatial ability, with P val ues ranging from .002 to .045. In a subset of subjects, we noted significan tly slower left-handed, but not right-handed, performance on measures of fi nger dexterity and psychomotor speed. Conclusions: Although emotional status did not differ from that of children with CF, SBS patients showed visual-spatial deficits in the company of pre served language, attention and memory, and executive skills. The specificit y and consistency of these findings suggests that right hemisphere CNS chan ges may occur in children with SBS. This unexpected finding, coupled with t he indication of left-sided psychomotor slowing in right-handed subjects, r aises the possibility that actual brain impairment, rather than development al delay accompanying slowed physical growth, accounts for these findings. Longitudinal studies are needed to further clarify this issue. The educatio nal significance of the results is discussed. Copyright (C) 2000 by W.B. Sa unders Company.