Review the clinical results of 24 years of intestinal lengthening procedures at one institution.
Retrospective review of a single center experience comparing the outcome of 2 intestinal lengthening procedures (Bianchi and serial transverse enteroplasty [STEP]) in terms of survival, total parenteral nutrition (TPN) weaning, and complications.
Sixty-four patients, including 14 adults, underwent 43 Bianchi and 34 STEP procedures between 1982 and 2007. Three patients had prior isolated liver transplants. The median (range) remnant bowel length before first lengthening was 45 (11-150) cm overall; (Bianchi=44 cm, STEP=45 cm) and 68 (20-250) cm after lengthening; (Bianchi=68 cm, STEP=65 cm). Actual survival is 91% overall (Bianchi 88%, STEP 95%) with median follow-up of 3.8 years (Bianchi=5.9 years, STEP=1.7 years). Average enteral caloric intake in pediatric patients was 15 kcal/kg before lengthening and 85 kcal/kg at 1 year after lengthening. Sixty-nine percent of patients are off TPN at most recent follow-up, including 8 who were weaned from TPN after intestinal transplantation. Liver disease (when present) was reversed in 80%. Surgical complications occurred in 10%, more commonly requiring reoperation after Bianchi than STEP. Intestinal transplantation salvage was required in 14% at a median of 2.9 years (range=8 months to 20.7 years) after lengthening.
Surgical lengthening with both Bianchi and STEP procedures results in improvement in enteral nutrition, reverses complications of TPN and avoids intestinal transplantation in the majority with few surgical complications. Intestinal transplantation can salvage most patients who later develop life-threatening complications or fail to wean TPN.
"leading to intestinal failure may include gastroschisis, necrotizing enterocolitis (NEC), malrotation with volvulus, jejunal atresia, and other more rare intestinal anomalies or enteropathies. Clinical interventions to minimize the damaging effects of PN to the immature pediatric liver include transitioning the child to enteral nutrition with a slowly increasing feed volume with the hope of adaptation, use of " hepatosparing " PN formulas, autologous gastrointestinal reconstruction, and minimization of the inflammatory effects of bacterial translocation   . Children with intestinal failure benefit from early referral to a center of excellence in intestinal rehabilitation for development of a comprehensive treatment plan . "
[Show abstract][Hide abstract] ABSTRACT: Infants with parenteral nutrition dependence may develop liver dysfunction and progress to liver failure requiring transplantation. The aspartate aminotransferase-to-platelet ratio index (APRI) has good correlation with liver fibrosis progression in adults. This study applies APRI scoring to parenteral nutrition-dependant, short-gut infants to determine hepatic fibrosis progression.
Laboratory values and biopsies were collected from initial intestinal resection (time 0) up to transplantation (end). Fibrosis scoring ranged from F0 (normal) to F4 (cirrhosis). Children were divided into 3 groups: (1) isolated intestine; and combined liver/intestine with gestational age (2) 34 weeks or greater and (3) 30 weeks or less. Liver function values over time, including calculated APRI, were analyzed as predictors of fibrosis.
Fifteen children who had 33 biopsies were included. Median APRI by fibrosis grade was F < or = 2: 1.88, F3: 3.23, and F4: 14.16 (P < .01). Median APRI at transplant by study group was (1) isolated intestine: 2.47, (2) liver/intestine 35 weeks or longer EGA: 14.16, and (3) liver/intestine 30 weeks or less EGA: 14.74 (P = .04).
Progression of APRI up to 60 days initially demonstrates similar values among study groups, but over time the score distinguishes those children with impending liver cirrhosis and differentiates fibrosis grade and study group.
Journal of Pediatric Surgery 06/2010; 45(6):1266-73. DOI:10.1016/j.jpedsurg.2010.02.098 · 1.39 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.