Pediatric Intestinal Failure, 10 years of experience from a specialized unit

Abstract

Intestinal Failure (IF) includes the loss of functional intestinal mass and the requirement of longterm Parenteral Nutrition (PN) to achieve the development and growth in childhood.

Objective: To evaluate the experience in a specialized unit for pediatric patients with IF, describing the clinical characteristics of those admitted from November 2009 to December 2019.

Patients and Method: Retrospective and descriptive review from clinical records of 24 cases that matched the inclusion criteria. The following variables were recorded: gender, neonatal history, origin unit, patient age and anthropometric diagnosis at admission to the unit, cause of IF, hospital stay, anthropometric data and parenteral nutrition dependency at discharge. In those patients with a diagnosis of short bowel syndrome (SBS), the cause of intestinal resection and the characteristics of the intestinal remnant were identified: anatomical classification, remnant length (defining ultra-short as < 25 cm), presence of ileocecal valve, and characteristics of the colon.

Results: The median age at admission was 7.8 months. Seventeen cases were preterm. Regarding IF etiology, 10 patients presented SBS, 6 patients with Intestinal Neuromuscular Disease (INMD), 7 children with SBS associated with INMD, and 1 case of intestinal lymphangiectasia. Within the SBS etiologies found in this group, intestinal atresia (8 cases) and necrotizing enterocolitis (9 patients) were the main causes with a similar proportion. Eight patients had no ileocecal valve. According to anatomical classification, 1 case was Type I, 8 were Type II, and 8 were Type III. Related to bowel length, 3 were ultrashort, besides being Type II; in those with > 40 cm of bowel length, 7 were Type III. Overall average hospital stay was 456.4 days. Enteral autonomy was achieved in 16 patients and 8 cases required home parenteral nutrition.

Conclusions: IF requires life support, PN and prolonged hospital stay. The principal etiology of IF is SBS caused by congenital intestinal atresia and necrotizing enterocolitis. Nevertheless, the high frequency of INMD could be attributed to the local protocol analysis. Most of our patients had poor prognosis factors, however, the management by a specialized team allowed the achievement of enteral autonomy in 66.7% of cases.