Clin Nutr. 2023 Jul;42(7):1095-1103. doi: 10.1016/j.clnu.2023.05.017.Epub 2023 May 25.

Annika Mutanen ¹, Helene Engstrand Lilja ², Tomas Wester ³, Heimir Norrby ⁴, Helena Borg ⁵, Sara Persson ⁵, Kristin Bjornland ⁶, Anne Charlotte Brun ⁷, Lovisa Telborn ⁸, Pernilla Stenström ⁸, Mikko P Pakarinen ⁹

Author information

¹Department of Pediatric Surgery, The New Children's Hospital, University of Helsinki, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland. Electronic address: annika.mutanen@hus.fi.

²Department of Pediatric Surgery, University Children's Hospital, Uppsala University, Uppsala, Sweden.

³Unit of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karoliska Institutet, Stockholm, Sweden.

⁴Unit of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden.

⁵Department of Pediatric Surgery, The Queen Silvia Children's Hospital, Gothenburg, Sweden.

⁶Department of Pediatric Surgery, Oslo University Hospital and University of Oslo, Oslo, Norway.

⁷Department of Pediatric Medicine, Oslo University Hospital, Oslo, Norway.

⁸Department of Pediatric Surgery, Skane University Hospital, Lund University, Lund, Sweden.

⁹Department of Pediatric Surgery, The New Children's Hospital, University of Helsinki, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland; Department of Women's and Children's Health, Karoliska Institutet, Stockholm, Sweden.

Abstract

Background & aims: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population.

Methods: Patients with SBS-IF treated during 2010-2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels.

Results: Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21-80%). After median follow up of 4.4 years (IQR 2.5-6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies.

Conclusions: Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.