Children are now surviving with extreme short bowel syndrome (SBS) i.e. resection of the entire jejunum and ileum which has been termed “No gut syndrome” (NGS) in adults. We aimed to describe the population characteristics and outcome in children with NGS.

A questionnaire was sent to the paediatric intestinal rehabilitation centres in the UK via the national intestinal failure (IF) network to identify patients discharged home with long-term IF (>27 days) and NGS. Our inclusion criteria were all infants/children < 18 years with a maximum jejunal remnant < 5 cm from the ligament of Treitz after major excision of jejunum, ileum (and in some cases duodenum) ± colon. Information obtained included patient demographics, outcome and school attendance. We also requested total number of cases of ultra-short bowel syndrome (U-SBS) < 10 cm from duodenojejunal flexure. A multicentre Health Research Authority approval was obtained via the integrated research application system (IRAS).

Nine of 20 (45%) IF centres managing a total of 204 patients on home parenteral nutrition (HPN) replied - 11/204 (5.3%) had U-SBS and 12/204 (5.8%) had NGS. Median age [IQR] was 7 years 6 months [5y 5m–10y 10m]. Two patients were excluded since were recently transitioned to adult services.

The aetiology of NGS was mid-gut volvulus in 7/12 (58%) cases, ischemia in 2/12 (17%), and one of each of necrotising enterocolitis (NEC) 1/12 (8%), post-resection of failed intestinal transplant 1/12 (8%) and intestinal atresia 1/12 (8%).

Eight/12 (67%) patients had duodenum alone and 4/12 (33%) < 5 cm jejunum. Eleven/12 had a colonic remnant (2/11 full colon, 6/11 from transverse and 3/11 sigmoid-rectum) which was in continuity in 7/11 cases (2 full colon, 4 transverse, 1 sigmoid-rectum).

There were no patient deaths. One/12 (8%) gained enteral autonomy and one/12 (8%) underwent intestinal transplant (for irreversible IF). The child who gained enteral autonomy was a term infant with neonatal volvulus and colon in continuity from 6 months, weaned from PN aged 4 years 1 month.

The other 10/12 (84%) patients remained stable on HPN, with median [IQR] 7 nights/week [6.75–7], over 12 hours/night [12–13], 2 lipid-free nights/week [0–4]. Seven/11 tolerated enteral nutrition - 1/7 blended diet and 6/7 oral food (3 only trophic). Four cases had mild IF associated liver disease (IFALD), 2 nephrocalcinosis and 1 hypoglycaemic episodes. Information about school attendance was obtained in 11/12 with 9 attending school (8/9 full time) and two nursery.

Advances in PN and health care have enabled long-term survival of patients with no gut syndrome. Children appear to have a reasonable quality of life - they attend school/nursery and PN is only infused overnight. It is also possible for a no gut child to gain enteral autonomy.