A quick glance at a recent study (JPGN 2014; 58: 438-42) suggests a favorable outlook for patients with ultra-short bowel syndrome (U-SBS). U-SBS has been defined as having a residual small bowel length <10 cm distal to the ligament of Treitz. A more cynical definition by a colleague years ago was that U-SBS was when patients can fart and burp at the same time.
Looking at the details: This study enrolled 11 patients into a prospective Italian database since 2000 and examined their outcomes. Inclusion criteria included U-SBS diagnosed in the neonatal period (<28 days) and necessitating home parenteral nutrition at discharge.
The demographics note that these patients were bigger at birth and less premature than typical series of patients with SBS:
- Only one of the patients had necrotizing enterocolitis as the sole underlying disease and six patients had volvulus.
- All but two had ≥50% of their colons, with five having their entire colon.
- All but one of these patients had gestational age ≥32 weeks and only two patients had documented birth weight less than 2300 gm.
The authors note that these patients currently receive SMOFlipid as outpatients and Omegaven as inpatients. All patients receive some enteral feedings. Loperamide is used selectively.
- Inpatient hospital care ranged from 23 to 104 days/year, but had improved during the last year of followup.
- With >5 years of followup, 2 of the 11 patients had died. One of these patients had severe intestinal failure associated liver disease (IFALD) despite use of Omegaven.
- One patient underwent isolated intestinal transplantation.
- No children in this series underwent a bowel-lengthening…”given the shortness of the residual small bowel, the gain of length after any procedure will not significantly improve absorption.”
Given their results, the authors note that despite recommendations for early referral for intestinal transplantation in patients with U-SBS, this may not result in a survival benefit. They note a study by Pironi et al (Gut 2011; 60: 17-25) that showed that among 80 intestinal transplant candidates, 5-year survival was greater in those who were not transplanted.
Bottomline: This small cohort shows that certain populations of U-SBS may do well clinically for a long time with medical management. Caution should be used in extrapolating these results to SBS patients with different demographics.