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Short bowel syndrome refers to the malabsorptive state caused by loss of significant portions of the small intestine, whose clinical framework is characterized by malnutrition, diarrhea, dehydration, weight loss, and low-weight-related symptoms/signs. These clinical conditions seem to be related to the length of resection. Twenty-one years ago we reported the clinical case of an infant, who underwent a massive resection of the loops of the small intestine, of the cecum and of part of the ascending colon, due to intestinal malrotation with volvulus. The residual small intestine measured just 11 cm and consisted of the duodenum and a small part of jejunum, in the absence of the ileocecal valve, configuring the case of a <em>ultra-short bowel syndrome</em>. In this report, we update the case, reporting the patient succeeded to obtain a good weight gain and to conduct a quite normal lifestyle, despite the long-term consequences of such resection

Clinical case and short review of extreme short bowel syndrome: an update 21 years after