Refined Multidisciplinary Protocol-Based Approach to Short Bowel Syndrome Improves Outcomes.
OBJECTIVE
Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol.


METHODS
This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared.


RESULTS
Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178).


CONCLUSIONS
Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.
*Pediatric Liver and Gut Research Group, Department of Pediatric Gastroenterology †Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
Laura
L
Merras-Salmio
Mikko P
MP
Pakarinen
D055499:Catheter-Related Infections / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control*; D002779:Cholestasis / Q000453:epidemiology / Q000209:etiology; D002985:Clinical Protocols; D004065:Digestive System Abnormalities / Q000150:complications; D020345:Enterocolitis, Necrotizing / Q000150:complications; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D045822:Intestinal Volvulus / Q000150:complications; D007421:Intestine, Small / Q000473:pathology* / Q000601:surgery; D017093:Liver Failure / Q000209:etiology / Q000517:prevention & control*; D008297:Male; D010288:Parenteral Nutrition / Q000009:adverse effects; D010348:Patient Care Team; D015995:Prevalence; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000209:etiology / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome