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 isomerism) syndromes.

MATERIALS AND METHODS: A retrospective study was performed of 27 children with asplenia (21) or polysplenia (6) identified from the cardiology and radiology databases from 1988 to 1996 and in whom an upper gastrointestinal barium study had been performed. The intestinal rotation was determined by reviewing the barium meal and could be divided into four groups: (1) normal rotation, (2) incomplete rotation or nonrotation, (3) reversed rotation and (4) reversed incomplete rotation or nonrotation. Surgical correlation was obtained at laparotomy 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 reversed rotation; 14 (52 %) had reversed incomplete rotation or nonrotation.

CONCLUSION: Asplenia and polysplenia are frequently associated with intestinal malrotation, and a barium study is recommended in all of these children, as many will be at risk of midgut volvulus.