Short-bowel syndrome is functionally defined as a state of malabsorption fo
llowing loss of small bowel. Most: cases occur in the neonatal period after
extensive resection for necrotizing enterocolitis, or due to congenital an
omalies of the gastrointestinal tract. A smaller percentage originate later
in life from surgical treatment of Crohn's disease, neoplastic disorders,
or vascular events. The physiological, morphological and functional intesti
nal gradient determines the clinical picture leading to better tolerance of
jejunal than ileal resections. The subsequent adaptation process requires
enteral feeding with a different impact of specific nutrients, and is also
influenced by a number of humoral mediators such as enteroglucagon, gastrin
, growth factors, prostaglandins and polyamines, Nutritional management sta
rts parenterally via a central venous line covering basic demands, substitu
ting current losses and restoring pre-existing deficiencies. Continuous ent
eral tube feeding is added as soon as postoperative ileus resolves, beginni
ng with an elemental diet, which is gradually increased first in concentrat
ion, then in quantity, and supplemented by small oral meals. Cycling of par
enteral nutrition is the next step. As soon as sufficient stability is reac
hed, the child should be discharged home under continued outpatient care. M
ain long-term problems comprise bacterial overgrowth, fluid and electrolyte
disequilibration, nutritional deficiencies, parenteral nutrition-related l
iver disease, and central venous line complications such as sepsis and thro
mbosis.