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Published on: December 1, 2012
Intestinal rehabilitation of infantile onset very short bowel syndrome
Mikko P Pakarinen1, Niklas Pakkasjärvi1, Laura Merras-Salmio2
1Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Finland; Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Finland.
Insights
Infantile very short bowel syndrome (VSBS) patients show comparable survival and growth to those with longer remaining bowel. However, achieving intestinal autonomy remains a significant challenge for VSBS infants.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatology
Background:
- Infantile short bowel syndrome (SBS) is a severe condition requiring long-term parenteral nutrition (PN).
- Very short bowel syndrome (VSBS) represents an extreme form with a remaining small bowel length less than 30% of predicted or requiring PN for over 3 months.
Purpose of the Study:
- To evaluate and compare the surgical treatment and outcomes of infantile VSBS versus standard SBS.
- To identify factors influencing treatment success and long-term prognosis in these pediatric populations.
Main Methods:
- Retrospective review of 42 children with infantile onset SBS.
- Comparison of surgical treatments and outcomes between VSBS (n=12, <25 cm small bowel) and SBS (n=30, >25 cm small bowel) groups.
- Analysis of intestinal length, ileocecal valve presence, colon proportion, autologous intestinal reconstruction, intestinal autonomy, PN weaning, survival, and growth parameters.
Main Results:
- VSBS patients had significantly shorter initial small bowel length (15 cm vs. 48 cm).
- While survival and growth were comparable, 5-year PN weaning probability was lower in VSBS (46%) versus SBS (92%).
- Intestinal autonomy was achieved in 2/4 VSBS patients compared to 9/11 SBS patients.
Conclusions:
- Infantile VSBS patients demonstrate comparable survival, liver function, and growth to SBS patients with longer bowel remnants.
- Regaining intestinal autonomy remains a significant challenge for infants with the shortest small intestinal segments.
- Further research into advanced surgical techniques and therapies is warranted for VSBS management.
Aim:
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).
Main 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.
Conclusion:
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.
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