title: Intestinal rehabilitation of infantile onset very short bowel syndrome.
abstract: OBJECTIVE
  The aim of this study was to evaluate treatment and outcomes of infantile very short bowel syndrome (SBS).
  
  
  METHODS
  A 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 3 months of parenteral nutrition (PN) was performed. Surgical treatment and outcomes were compared between very SBS (VSBS, small bowel length less than 25 cm, n=12) and SBS (more than 25 cm, n=30).
  
  
  RESULTS
  Median 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.
  
  
  CONCLUSIONS
  Although 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.
authors: Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Finland; Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Finland. Electronic address: mikko.pakarinen@hus.fi.
authors: Mikko P
authors: MP
authors: Pakarinen
authors: Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Finland; Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Finland.
authors: Niklas
authors: N
authors: Pakkasjärvi
authors: Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Finland; Section of Pediatric Gastroenterology, Children's Hospital, University of Helsinki, Finland.
authors: Laura
authors: L
authors: Merras-Salmio
authors: Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Finland.
authors: Antti
authors: A
authors: Koivusalo
authors: Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Finland.
authors: Risto
authors: R
authors: Rintala
mesh_terms: D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D007421:Intestine, Small / Q000601:surgery*; D007422:Intestines / Q000601:surgery; D008297:Male; D010288:Parenteral Nutrition / Q000379:methods*; D011446:Prospective Studies; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000534:rehabilitation*; D013997:Time Factors; D016896:Treatment Outcome
keywords: Adaptation; Autologous intestinal reconstruction; Bowel lengthening; Neonatal