Autologous gastrointestinal reconstruction surgery for short bowel syndrome: the cornerstone for intestinal rehabilitation.

PURPOSE OF REVIEW: Intestinal failure (IF) evolved from being the last recognized organ failure, to become one of the most progressive fields in terms of therapeutic alternatives and results. Short bowel syndrome (SBS) is the main cause of IF in adults and children. The use of surgery allowed patients with unfavorable anatomy type and length to be wean off parenteral nutrition. We aim to evaluate its current impact on intestinal rehabilitation. RECENT FINDINGS: Autologous gastro-intestinal reconstructive surgery (AGIRS), including bowel lengthening contributes by converting patient's anatomy to a more favorable one, improving quality of life, and modifying the natural history of the disease, allowing to recover intestinal autonomy in approximately 70% of the adults and 50% of the children's with SBS-IF. The current use of postsurgical medical rehabilitation strategies including the use of enterohormones complement the path to sufficiency, increasing the chances of success in both age group of patients. SUMMARY: The development of AGIRS has changed the outcome of SBS-IF patients, becoming the main surgical procedure prescribed in multidisciplinary units, allowing to enhance the number of patients achieving intestinal autonomy throughout rehabilitation, leaving transplantation as the last surgical alternative.

[Partial external biliary diversion: a therapeutic alternative for Alagille syndrome. First case in Argentina]. / Derivación biliar externa parcial: alternativa terapéutica en el síndrome de Alagille. Primer caso en la Argentina.

BACKGROUND: Alagille 's syndrome is the main syndromic chronic intrahepatic cholestasis characterized by hypoplasia of the intrahepatic bile ducts. It is a multisystem disorder of autosomal dominant inheritance with involvement of multiple organs. Usually it becomes apparent in the neonatal period, presenting as subclinical cases or severe degrees of the disease with the consequent development of liver cirrhosis and subsequent liver failure associated with multiple abnormalities: defects in the vertebral arches, typical facies, pulmonary stenosis, mental retardation and hypogonadism. OBJECTIVE: To present the first case of partial external biliary diversion in Argentina, showing the surgical technique and the improvement in the quality of life, as an alternative to be considered in patients with Alagille's syndrome before the development of cirrhosis. RESULTS: It has been shown that partial external biliary diversion can stop the process of liver fibrogenesis, halting the progression of the disease and avoiding the need for transplantation in some types of intrahepatic cholestasis when cirrhosis has not been established. DISCUSSION: This surgical technique can improve the quality of life and morbidity associated with hypercholesterolemia in patients with Alagille's syndrome, delaying and maybe avoiding the need for liver transplantation.