INTESTINAL ROTATIONAL ABNORMALITIES IN POLYSPLENIA AND ASPLENIA SYNDROMES

Objective. To review the anomalies of intestinal rotation occurring in association with asplenia (right isomerism) and polysplenia (left iso merism) syndromes. Materials and methods. A retrospective study was pe rformed of 27 children with asplenia (21) or polysplenia (6) identifie d from the cardiology and radiology databases from 1988 to 1996 and in whom an upper gastrointestinal barium study had been performed. The i ntestinal rotation was determined by reviewing the barium meal and cou ld be divided into four groups: (1) normal rotation, (2) incomplete ro tation or nonrotation, (3) reversed rotation and (4) reversed incomple te rotation or nonrotation. Surgical correlation was obtained at lapar otomy in 17 patients. Results. Of the 27 children studied, 3 (11%) had normal rotation; incomplete rotation or nonrotation occurred in 5 (19 %), and 2 in this group developed midgut volvulus; 5 (19%) had reverse d rotation; 14 (52%) had reversed incomplete rotation or nonrotation. Conclusion. Asplenia and polysplenia are frequently associated with in testinal malrotation, and a barium study is recommended in all of thes e children, as many will be at risk of midgut volvulus.