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

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.

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, 118.1 +/- 14 months). Groups did not differ in age, grade, or absences. Neuropsychological tests with established sensitivity to CNS integrity compared performance over 6 cognitive domains. Emotional status also was measured. Analyses were completed with 2-tailed t tests.

Groups did not differ on tests of intellectual ability and emotional function. Language, memory and learning, and problem-solving testing results 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.

Although emotional status did not differ from that of children with CF, SBS patients showed 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.