Ultra-short-bowel syndrome is not an absolute indication to small-bowel transplantation in childhood

Short-bowel syndrome (SBS) either in adults or in children is considered as an indication to small-bowel transplantation (SBTx), particularly in its m ost severe form with a residual bowel length below 20 cm. Among factors likely to worsen the prognosis, more recent reports also indi cate the number of surgical interventions, early onset sepsis and early dev elopment of liver disease. We report six cases of ultra-short-bowel syndrome followed from birth to ve rify the importance of various prognostic factors. In our case series, the male sex is predominating (5:1). Intestinal resection was indicated in 3 pa tients for multiple intestinal atresias, in 2 for volvulus and in 1 for nec rotizing enterocolitis. The length of intestine remaining was invariably le ss than 20 cm and 2 patients had a pre-served ileocecal valve. In most case s, more than 50% of the colon remained. The number of abdominal operations ranged from 1 to 4. In almost all cases (5 of 6), sepsis and hepatopathy de veloped early. Our experience suggests that rather than depending on the length of intesti ne remaining or the presence of the ileocecal valve, the prognosis of patie nts with the extreme-short-bowel syndrome depends on recurrent neonatal ons et sepsis and early onset liver impairment. In addition, our case review sh ows that the extreme-short-bowel syndrome is not necessarily an indication for bowel transplantation.