Interdisciplinary Management of Pediatric Intestinal Failure: a 10-Year Review of Rehabilitation and Intestinal Transplantation
Anita Nucci*1,2, Cartland Burns5,2, Kristyn Lowery2, Tichianaa Armah4, Sharon Strohm1,2, Geoffrey Bond3, George V. Mazariegos3,2, Robert H. Squires6,2
1Clinical Nutrition, Children's Hospital of Pittsburgh, Pittsburgh, PA; 2Intestinal Care and Rehabilitation Center, Children's Hospital of Pittsburgh, Pittsburgh, PA; 3Pediatric Transplant Surgery, Thomas E. Starzl Transplantation Institute, Pittsburgh, PA; 4University of Rochester, Rochester, NY; 5Pediatric Surgery, Children's Hospital of Pittsburgh, Pittsburgh, PA; 6Pediatric Gastroenterology, Children's Hospital of Pittsburgh, Pittsburgh, PA
Many children with intestinal failure (IF) due to short bowel syndrome undergo progressive adaptation of their remaining bowel while others do not spontaneously adapt and are unable to wean from parenteral nutrition (PN). Management has focused on the provision of nutrition support, use of surgical lengthening procedures and, more recently, intestinal transplantation (ITx). The purpose of this investigation is to identify factors predictive of successful outcomes in a large population of children with IF. Registry data are maintained for all patients referred to our Intestinal Care and Rehabilitation center since 1996. Associations between age, gestational age, liver function at referral, initial bowel length and therapeutic outcomes were evaluated. 389 patients (61% male, 70% Caucasian) with a median age of 1 year were referred for intensive interdisciplinary management of IF. Outcomes for children dependent on PN is stratified by diagnosis in Table 1 (median time followed = 4 years). Factors predictive of successful weaning for children not transplanted included increased mean bowel length for patients with Gastroschisis (44 vs. 23 cm, p<0.05) and Atresia (35 vs. 20 cm, p<0.01) and lower mean total bilirubin for patients with NEC (6.1 vs. 12.7 mg/dL, p<0.05). Others were more likely to survive if referred with a lower mean total bilirubin (NEC, 7.9 vs. 12.7 mg/dL, p<0.05; Pseudo-obstruction, 2.3 vs. 16.3 mg/dL, p<0.01). 5 year survival rates for children who received an ITx ranged from 75-100% vs. 60% for those not transplanted. Moreover, patients weaned from PN by 2.5 years with or without ITx achieved 80-100% survival at 5 years vs. 33% for those not weaned. Lengthening procedures were performed on 25 patients. Of these, 8 weaned from PN without ITx, 9 weaned after ITx, 2 did not wean after ITx (1 graft failed, 1 died) and 6 failed without transplantation (3 were followed for <1 year, 3 died within 1.5 years). Aggressive medical and nutritional intervention along with early referral, intestinal lengthening procedures and ITx in children with IF dependent upon PN can result in the achievement of enteral autonomy and improved survival.
Table 1.
| Diagnosis | n | PN Weanedn (% survival) | PN Not Weanedn (% survival) | Unknown | ||
| ITx | No ITx | ITx | No ITx | |||
| Gastroschisis | 67 | 21 (95) | 5 (80) | 3 (0) | 20 (25) | 18 |
| NEC | 60 | 6 (83) | 12 (92) | 3 (0) | 17 (12) | 22 |
| Midgut volvulus | 49 | 23 (100) | 3 (100 | 2 (100) | 11 (36) | 10 |
| Atresias | 48 | 8 (88) | 9 (100) | - | 16 (31) | 15 |
| Aganglionosis | 26 | 8 (100) | 2 (100) | 1 (0) | 5 (20) | 10 |
| Pseudo-obstruction | 26 | 9 (100) | 3 (100) | 1 (100) | 6 (20) | 7 |
| Microvillus atrophy | 8 | 2 (50) | - | 2 (0) | 2 (0) | 2 |
| TOTAL | 284 | 77 (95) | 34 (94) | 12 (17) | 77 (23) | 84 |
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