Intestinal Rehabilitation of Infantile Onset Very Short Bowel Syndrome

Abstract

AimThe aim of this study was to evaluate treatment and outcomes of infantile very short bowel syndrome (SBS). MethodsA retrospective review of 42 consecutive children treated for infantile onset SBS defined as remaining small bowel length less than 30% of predicted or more than 3months of parenteral nutrition (PN) was performed. Surgical treatment and outcomes were compared between very SBS (VSBS, small bowel length less than 25cm, n=12) and SBS (more than 25cm, n=30). Main resultsMedian follow-up was 5.7 years (IQR, 2.8 to 11). Absolute initial small bowel length (cm), presence of ileocecal valve (%), and proportion of remaining colon (%) was 15 (10 to 21) vs. 48 (32 to 60) (P<0.0001), 58 vs. 50 (P=0.74), and 95 (76 to 100) vs. 78 (60 to 100) (P=0.27) in VSBS and SBS, respectively. More autologous intestinal reconstruction procedures per patient were performed in SBS group (27/30 vs. 5/12; P=0.002) leading to intestinal autonomy in 2 of 4 VSBS patients in relation to 9 of 11 SBS patients (P=0.52). Cumulative 5-year probability of weaning from PN was 46% (95% CI, 16 to 77) in VSBS and 92% (95% CI, 81 to 100) in SBS (P<0.01). Five-year cumulative survival was 80% (95% CI, 54 to 100) in VSBS and 93% (95% CI, 83 to 100) in SBS (P>0.30). No patients were transplanted. At final follow-up, plasma alanine aminotransferase (29 U/L [21 to 47]), bilirubin (6.0 μmol/L [3.0 to 8.0]), height (−1.4 SD [−2.5 to 0.1]), and relative weight (−5% [−12 to −2]) were similar between the groups. ConclusionAlthough survival, well-preserved biochemical liver function, and growth in VSBS patients are comparable to their counterparts with longer remaining bowel, regaining intestinal autonomy remains challenging in children with the shortest small intestinal remnant.