Clarivet Torres

A multidisciplinary approach to the treatment of intestinal failure

Intestinal failure is most commonly treated by the administration of total parenteral nutrition (TPN). In some patients, however, surgical therapy may increase the ability to use the intestine for nutrition and thereby decrease the complications of TPN therapy. A multidisciplinary comprehensive intestinal failure program was initiated at the University of Nebraska Medical Center in October 2000. Here we describe the surgical approaches to patients with short bowel syndrome and the subsequent impact on the need for TPN and on survival. Fifty patients (children = 30, adults = 20) underwent surgical procedures to restore intestinal continuity (n = 5), repair enterocutaneous fistulas (n = 5), resect dysmotile or strictured/obstructed bowel segments or mesenteric desmoid tumors (n = 7), stricturoplasty (n = 2), Bianchi tapering and lengthening (n = 20), serial transverse enteroplasty (n = 8), and other operations (n = 8). Of these 50 patients, three patients did not require TPN after surgical intervention and seven had remnant small bowel anatomy that precluded TPN weaning (e.g., end duodenostomy) and were listed for transplantation or continued on full TPN support. Of the 40 remaining patients, most received the majority of calories fromTPNat the time of referral, i.e., mean calories fromTPN _ 90%. Subsequent to the surgical and medical therapy, 26 (65%) have been completely weaned off TPN. In addition, 10 had substantial decreases in their TPN requirements (i.e., from 85% of calories from TPN at onset decreased to a median 35% of required calories at most recent follow-up). Four patients remained on the same amount of TPN support. Four of the seven patients listed for transplantation underwent successful transplantation. Despite the complications of short bowel syndrome, 86% (n = 43) of the patients are alive and well at a mean follow-up of 2 years. Patient deaths occurred primarily in those listed or eligible for transplantation and were related to advanced liver disease (n = 3), gastrointestinal hemorrhage (n = 1), or line sepsis (n = 1). Two other patients died, one from influenza A infection and one from unknown cause at home, months after complete discontinuation of TPN. In this series of patients with short bowel syndrome, surgical intervention led to weaning or discontinuation of TPN support in 85% of patients. An organized multidisciplinary approach to the patient with short bowel syndrome is recommended.

Role of an Intestinal Rehabilitation Program in the Treatment of Advanced Intestinal Failure

Objective: To analyze outcomes in children with intestinal failure treated by our Intestinal Rehabilitation Program (IRP) in a 4-year period. Patients and Methods: A total of 51 parenteral nutrition (PN)–dependent patients (20 male) were enrolled in the IRP. Median age was 1.7 years, with the primary diagnoses being gastroschisis, necrotizing enterocolitis, volvulus, and congenital atresia. Median small bowel intestinal length was 35 cm, with the majority of patients having only jejunum as remaining bowel anatomy. Thirty-six of the 51 patients had liver disease characterized by cirrhosis, advance bridging fibrosis, and portal and periportal fibrosis. Height, weight z score, platelet count, albumin, and bilirubin levels were measured at the beginning and end of the study. Results: Of the 51 patients, 29 had 46 different surgical intestinal repairs. Twenty-nine of the 36 patients with hyperbilirubinemia had normalized serum bilirubin with treatment. Ten patients required transplantation. Five patients died of sepsis, influenza, or complications after intestinal transplantation. Of the remaining 37 patients in the IRP, 31 were weaned from parenteral nutrition (5 with cirrhosis); 6 patients are in the process of weaning. Survival rate of the patients in the IRP was 90%. Growth has continued along the same curve, and some patients have exhibited significant catch-up. Conclusions: With an aggressive medical/surgical approach, even patients with intestinal failure and advanced liver disease can avoid transplantation. Patients in the IRP showed improved liver function and nutritional parameters with the ability to discontinue PN while maintaining growth. Early referral of these patients to specialized centers before the development of advanced liver disease is recommended.

Quality of Life after Pediatric Intestinal Transplantation: The Perception of Pediatric Recipients and Their Parents

The objective was to examine the perception of physical and psychosocial functioning of pediatric intestinal transplant recipients who are beyond the perioperative period and compare these with normal and chronically ill children.

Isolated liver transplantation in infants with end‐stage liver disease due to short bowel syndrome

Infants with short bowel syndrome (SBS) and associated liver failure are often referred for combined liver/intestinal transplantation. We speculated that in some young children, nutritional autonomy would be possible with restoration of normal liver function. Features we believed to predict nutritional autonomy include history of at least 50% enteral tolerance, age less than 2 yr, and no underlying intestinal disease. This report documents our experience with liver transplantation alone in children with liver failure associated with SBS. Twenty‐three children with SBS and end‐stage liver disease, considered to have good prognostic features for eventual full enteral adaptation, underwent isolated liver transplantation. Median age was 11 months (range, 6.5 to 48 months). Median pretransplant weight was 7.4 kg (range, 5.2 to 15 kg). All had growth retardation and advanced liver disease. Bowel length ranged from 25 to 100 cm. Twenty‐three children underwent 28 isolated liver transplants. There were 14 whole livers and 14 partial grafts (five living donors). Seventeen patients are alive at a median follow‐up of 57 months (range, 6 to 121 months). Actuarial patient and graft survival rates at 1 yr are 82% and 75% and at 5 yr are 72% and 60%, respectively. Four deaths resulted from sepsis, all within 4 months of transplantation, and 1 death resulted from progressive liver failure. Two allografts developed chronic rejection; both children were successfully retransplanted with isolated livers. Of 17 surviving patients, three require supplemental intravenous support; the remaining 14 have achieved enteral autonomy, at a median of 3 months (range, 1 to 72 months) after transplantation. Linear growth is maintained and, in many, catch‐up growth is evident. Median change in z score for height is 0.57 (range, −4.47 to 2.68), and median change in z score for weight is 0.42 (range, −1.65 to 3.05). In conclusion, Isolated liver transplantation in children with liver failure as a result of SBS, who have favorable prognostic features for full enteral adaptation, is feasible with satisfactory long‐term survival. Liver Transpl 12:1062–1066, 2006.

Long-term outcomes for infants with intestinal atresia studied at Children's National Medical Center.

Objectives: Intestinal atresia is a common cause of intestinal obstruction in neonates. The predominant cause of late complications in these children is prolonged dependence on parenteral nutrition (PN). Our aims were to analyze the outcomes for patients with intestinal atresia at our institution, and to see how these changed with the implementation of an intestinal rehabilitation program (IRP). Methods: This is a retrospective cohort study. The patient population is all children with intestinal atresias (118 patients) treated at our institution from July 2000 to June 2010, 20 of whom became PN dependent. Results: Survival to hospital discharge was 95% for all patients, and 100% for those who had isolated atresia. Twenty of 118 patients (17%) were PN dependent beyond initial hospital discharge. At discharge, their median intestinal length was 22.5 cm, and they required PN for a mean of 88.5% of energy needs. Of these 20 patients, 2 died, 2 received transplants, and 2 transferred away for transplantation. The remaining 14 joined the IRP. Their mean energy requirement from PN is presently 10%, down from 87% at IRP enrollment, and 10 patients fully weaned off PN. Eleven of the 14 children had hyperbilirubinemia, with mean direct bilirubin of 7.5 mg/dL. All resolved their cholestasis during an average of 12 weeks. Conclusions: These results compare favorably with those reported in earlier periods. With programs such as the IRP, patients with short bowel secondary to intestinal atresia can show improvement in liver function and nutritional parameters, and discontinue PN, avoiding the need for transplantation.

Living and deceased donor liver transplantation for unresectable hepatoblastoma at a single center

Abstract:  Hepatoblastoma (HB) is the most common malignant liver tumor in children. The application of living donor liver transplantation (LDLT) in the management of unresectable HB may add new therapeutic opportunities. We evaluated the outcomes of patients who underwent liver transplantation for treatment of unresectable HB in the period between August 1985 and June 2003. Ten children had a diagnosis of unresectable HB. Mean age at transplantation was 5.8 yr. Eight patients were transplanted with deceased donor grafts. Two patients underwent LDLT. Pre‐transplant chemotherapy was used in 90% of cases. Post‐transplant survival ranges from 3.7 to 18.6 yr. Three patients died of recurrent disease at 4, 14 and 38 months. The two LDLT recipients were able to get pre‐transplant chemotherapy with a rapid decision towards transplantation; both are alive and well at 5.5 and 11 yr post‐transplant. Our experience supports the role of LDLT and deceased donor liver transplantation in the management of unresectable HB when waiting times can be detrimental to the patients survival.

Quality of Life after Pediatric Intestinal Transplantation

The objective was to examine the perception of physical and psychosocial functioning of pediatric intestinal transplant recipients who are beyond the perioperative period and compare these with normal and chronically ill children.

Excellent outcomes of children with short bowel syndrome and intestinal failure associated liver disease (IFALD) at Children’s National Health System (CNHS), Washington DC

Objective We report the outcomes of short bowel syndrome (SBS) parenteral nutrition (PN) dependent patients with IFALD enrolled in our intestinal rehabilitation program (IRP). Methods Over a 10 year period, 124 SBS PN-dependent patients were enrolled, 78 (63%) of them had IFALD, defined as conjugated bilirubin (CB) of >=2mg/dL at enrollment and/or abnormal liver biopsy (stage 2-4 fibrosis). Outcomes included death, transplant, time to be weaned off PN, time to achieve normal CB levels. Independent variables included gender, gestational age, diagnosis, and intestinal length. Platelets, albumin, CB were obtained at the start and end of the study. Results Seventy-eight patients (51 males) had IFALD; of these, at the time of enrollment in IRP, 11 (14%) had normalized CB but had abnormal liver biopsies at CNHS and 66 (85%) had elevated CB. One patient acquired elevated CB, related to severe comorbidities. Median CB was 7.3 mg/dl (2-32 mg/dL). Thirty-three patients (42%) had liver biopsies, of which 32 (97%) had fibrosis, including 17 (52%) with bridging fibrosis or cirrhosis. Fifty-five patients (70.5%) were premature (GA <36 weeks). Median intestinal length was 44 cm (28/78 patients (36 %) had <35 cm). Median age at enrollment was 3.7 months and median daily caloric requirement by PN was 100%. Of the 67 patients with elevated CB, 66 (98.5%) normalized their bilirubin with medical treatment over an average of 11.6 weeks (st.dev. 9.1), using soy bean intralipid emulsion. Fifty seven (85%) reversed their cholestasis while still receiving PN, 2 patients were transplanted (they had 4 and 10 cm of remaining bowel), 2 patients died (cardiac anomalies and Down Syndrome). Four patients moved to another state after normalizing their CB, decreasing their PN from 100 to 56%. Of the 70 remaining patients 57 were weaned off PN (81.4%) over a median time of 5 months. A subset of 8 (10%) patients with initial platelet count of < 100 x 103/mcl (mean 59) was identified. In this subgroup, mean CB was 18.4mg/dL, and mean aspartate aminotransferase (AST) to platelet ratio index (APRI) was 19.4. Yet, all but one of this group achieved normalization of CB within an average of 15.6 weeks (st.dev =12), and a significant improvement of platelet count at the end of follow-up (p-value < 0.001). Overall survival was 97.4%. Due to low mortality in our cohort, logistic regression controlling for independent variables did not find association between initial bilirubin levels and mortality. Conclusion We demonstrate superior outcomes among children with IFALD. In spite of high acuity (e.g. significant proportion of extreme short gut patients, initial high dependence on PN), we were able to quickly reduce their PN needs and improve IFALD without relying on fish oil base lipid. As one of the larger and well established IRP in the US, we demonstrate high survival rates of our patients without the need for liver/intestinal transplant. Early referral to IRP is recommended.

Quality of Life after Pediatric Intestinal Transplantation: The Perception of Pediatric Recipients and Their Parents: Quality of Life After Intestinal Transplant

The objective was to examine the perception of physical and psychosocial functioning of pediatric intestinal transplant recipients who are beyond the perioperative period and compare these with normal and chronically ill children.