Jutta Koglmeier¹, Susan Hill¹, Mamoun Elawad¹, Jonathan Hind², Anil Dhawan², Neil Shah¹

¹Paediatric Gastroenterology, Great Ormond Street Hospital, London, United Kingdom; ²Paediatric Hepatology, King's College Hospital, London, United Kingdom

Background: Over the last 20 years the incidence of intestinal failure (IF) in children has constantly increased in the United Kingdom. This is due to better survival rates of extremely premature infants and the increase in conditions such as gastroschisis, but also because of better management of structural, inflammatory and motility disorders leading to the need for long term parenteral nutrition (PN). At present around 150 British children are managed on home PN. Although an effective intervention, life- threatening complications commonly arise in those with prolonged PN dependency.

A new multidisciplinary Intestinal Failure Assessment Centre (IFAC) was hence nationally commissioned. Clinical services started in April 2008. Referrals for children for possible small bowel (SBT) or bone marrow transplantation (BMT) are accepted.

Aims: We aimed to indentify all patients who were referred for IF assessment and document their outcome.

Method: Details of the children referred were obtained from the IFAC database.

Results: A total of 45 children were referred. 31/45 underwent assessment for SBT and 14/45 for BMT. 5/45 children underwent SBT, 6/45 children are currently on the waiting list for SBT. 5/45 patients underwent BMT with 7/45 children awaiting transplantation. 12/45 did not fulfill the criteria for SBT and remain on PN. 6/45 successfully weaned off PN with improved clinical management, 2/45 are off PN on complex immunosuppressive therapy.

Two families declined the SBT offer.1 child died post SBT (severe graft rejection), 1 child died post BMT (respiratory failure unrelated to BMT). No patient died on the waiting list.

Conclusion: The multidisciplinary IFAC offer children with life threatening complications treatment options in the form of SBT or BMA with the potential for good long term outcome. Improved medical management initiated in a highly specialist centre can prevent the need for transplantation in a significant proportion of patients.