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July 1991

Short-bowel Syndrome in ChildrenQuality of Life in an Era of Improved Survival

Author Affiliations

From the Division of Pediatric Surgery, Department of Surgery, St Louis (Mo) University School of Medicine (Drs Tracy and Connors), and the Department of Pediatric Surgery, Cardinal Glennon Children's Hospital, St Louis, Mo (Dr Weber).

Arch Surg. 1991;126(7):841-846. doi:10.1001/archsurg.1991.01410310051007

• A number of disorders in childhood can result in short-bowel syndrome (small bowel length, <100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n=6), multiple intestinal atresias (n=4), extensive aganglionosis (n=2), meconium peritonitis (n=2), and midgut volvulus (n=2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.

(Arch Surg. 1991;126:841-846)

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