16:00 - 17:30
Oral session
Room: Farabeuf
Chair/s:
Gabriel Gondolesi, Dominique Michel Jan
Long term results of a series of pediatric patients with short bowel syndrome treated at an Intestinal Failure, Rehabilitation and Transplant Program
María Inés Martínez, Carolina Rumbo, Santiago Rubio, Diego Ramisch, Adriana Fernández, Gabriel Eduardo Gondolesi
Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina

Introduction: Patients (pts) with short bowel syndrome (SBS) have high morbidity and mortality mainly related to their residual intestinal anatomy/function and clinical status. Better outcomes are achieved when this condition is managed at a comprehensive multidisciplinary unit.

Aim: To analyze long-term results and chances of achieving Intestinal Sufficiency (IS) of all children with SBS referred to a comprehensive Intestinal Failure, Rehabilitation and Transplant Unit from 3/2006 to 12/2018

Material and methods: This is a retrospective review of a prospectively filled database, including pediatric pts with SBS .Demographic data, diagnosis, nutritional status, parenteral nutrition (PN) support, as % of the basal metabolic rate (no protein Kcal), intestinal anatomy type (1 -terminal jejunostomy-; 2 -jejuno colonic anastomosis; 3 -jejuno-ileo colonic anastomosis; subtypes by length: A< 40 cm and B > 40 cm; treatment implemented [Groups: PN+MR (PN + Medical Rehabilitation); SR+MR (Surgical + Medical Rehabilitation) and ITx (Intestinal Transplantation)]. were analyzed on SPSS.

Results: 62 pts with SBS out of 102 pts with chronic intestinal failure admitted in our program, were analyzed. 41 were male, 25 premature, 48 with neonatal SBS. Etiology of SBS included: gastroschisis (15), intestinal atresia (11), Hirschprung (10), NEC (10), post natal volvulus (7) and perinatal volvulus (4).

Pts´ anatomy type, PN at referral, initial and last follow-up visit nutritional status, are shown in Table 1. Median (IQR) time on PN before referral 0.62 (0.25-2.2) years. Figure 1 shows treatment implemented and outcomes. In the long term 30 (48.4%) pts were able to achieve IS; 25 % of the pts of the PN+MR group, Median IQR 1.2 (1-2.4) years since PN initiation (sPNi); 69% of the SR+MR group Median (IQR) 5.3 (1.4-8.9) years sPNi; and 76% of the ITx group (Median (IQR): 7 (2.5-10.8) sPNi, (PN+MR vs SR+MR and ITx: p= 0.004). From the ITx group, 5 pts failed in the long-term, re-starting PN, 2 died. Other 2 ITx pts died with IS. The overall 1, 5 and 10-year survival sPNi: 97%, 65.4% and 61.2% respectively, independently of the treatment implemented.

Conclusions: Although IS could be achieved under each line of therapy group, SR+MR allowed higher rate of IS with long term maintenance; ITx had higher chances of achieving IS, but there is drop out in the long term. Long-term survival of pts with SBS can be achieved when they are managed under a comprehensive program.


Session:
Access to optimal care for intestinal failure
Presenter/s:
María Inés Martínez
Presentation type:
Oral only presentation
Room:
Farabeuf
Chair/s:
Gabriel Gondolesi, Dominique Michel Jan
Date:
Friday, July 5, 2019
Time:
16:00 - 17:30
Session times:
16:00 - 17:30