Susan Protheroe

Management of end-stage central venous access in children referred for possible small bowel transplantation.

The 3-year survival after small bowel transplantation (SBTx) has improved to between 73% and 88%. Impaired venous access for parenteral nutrition can be an indication for SBTx in children with chronic intestinal failure. Aim: To report our experience in management of children with extreme end-stage venous access. Subjects: The study consisted of 6 children (all boys), median age of assessment 27 months (range, 13-52 months), diagnosed with total intestinal aganglionosis (1), protracted diarrhea (1), and short bowel syndrome (4), of which gastroschisis (2) and malrotation with midgut volvulus (2) were the causes. All had a documented history of more than 10 central venous catheter insertions previously. All had venograms, and 1 child additionally had a magnetic resonance angiogram to evaluate venous access. Five of 6 presented with thrombosis of the superior vena cava (SVC) and/or inferior vena cava. Methods: Venous access was reestablished as follows: transhepatic venous catheters (5), direct intra-atrial catheter via midline sternotomy (4), azygous venous catheters (2), dilatation of left subclavian vein after passage of a guide wire and then placing a catheter to reach the right atrium (1), radiological recanalization of the SVC and placement of a central venous catheter in situ (1), and direct puncture of SVC stump(1). Complications included serous pleural effusion after direct intra-atrial line insertion, which resolved after chest drain insertion (1), displacement of transhepatic catheter needing repositioning (2), and SVC stent narrowing requiring repeated balloon dilatation. Outcome: Four children with permanent intestinal failure on assessment were offered SBTx, 3 of which were transplanted and were established on full enteral nutrition; the family of 1 child declined the procedure. In the remaining 2 children in whom bowel adaptation was still a possibility, attempts were made to provide adequate central venous access as feeds and drug manipulations were undertaken. One of them received liver and SBTx nearly 3 years after presenting with end-stage central venous access, because attempts to achieve independence from parenteral nutrition had failed. The other child died immediately after a transhepatic venous catheter placement, possibly from a nutritional depletion syndrome as no physical cause of death was found. Direct intra-atrial catheters in transplanted children proved to be adequate for the management of uncomplicated transplantation, although the usual infusion protocol had to be modified considerably, and the lack of access would have been critical if massive blood transfusion had been required during the transplant procedure. Conclusion: It was possible to reestablish central venous access in all cases. However, this was time consuming and difficult to assemble a skilled team consisting of one of more: surgeon, cardiologist, interventional radiologist, and transplant anesthetist. Small bowel transplantation is easier and safer with adequate central venous access, and we advocate liaison with an SBTx center at an early stage.

Feeding the child with chronic liver disease

Protein energy malnutrition leading to growth failure is an inevitable consequence of chronic liver disease in 60% of children. Malnutrition should be anticipated by serial anthropometric assessment and prevented by early intervention with nutritional support. Both morbidity and mortality postliver transplantation have been related to the degree of pretransplant malnutrition, and thus nutritional status is an important risk factor for survival postliver transplantation. As survival following pediatric liver transplantation improves, with most centers reporting 1 y survival rates of 90-95% and 5 y survival rates of 80-85%, attention has focused on achieving nutritional rehabilitation, normal psychosocial development, and normal quality of life. An understanding of the etiology of protein malnutrition in liver disease is essential when planning therapeutic strategies. Considerable research progress has been made exploring the pathophysiology of malnutrition, including long-chain fat malabsorption with essential fatty acid deficiency, abnormal energy metabolism, substrate utilization, and nitrogen metabolism in liver disease. Effective strategies are emerging and future advances include docosahexaenioc acid, branched chain amino acids, and structured lipids. The key to success is a multidisciplinary approach to nutritional intervention, including pediatric dietitian, liaison nurse, feeding psychologist, and clinician.

Isolated liver transplant in infants with short bowel syndrome: insights into outcomes and prognostic factors.

Objective:Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated liver disease (IFALD) may benefit from isolated liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx. Patients and Methods:A retrospective review of medical records from 1998 to 2005 was undertaken. Risk factors were assessed by comparing long-term survivors with those who died after iLTx. Results:Fifteen iLTx were performed in 14 infants with IFALD. All were parenteral nutrition (PN) dependent, but had tolerated enterally 54% (38–100) of energy intake before iLTx. Median residual bowel was 60 cm (30–200). Eight out of 14 had intact ileocaecal valve (ICV). Median bilirubin was 298 μmol/L (87–715) and all had portal hypertension. Eight out of 9 survivors were weaned from PN after median 15 months. In 4 out of 9 children, nontransplant surgery after iLTx facilitated intestinal adaptation. Growth velocity had improved at 3 years after iLTx (P = 0.001). Five children who died had poor enteral tolerance following iLTx (P < 0.002), which correlated with pretransplant dysmotility seen in 4 out of 5 children shown by contrast studies (P = 0.02) and increased frequency of line infections before (>6/year P < 0.04) and after (P < 0.001) iLTx. Conclusions:Isolated liver transplantation is a lifesaving option for selected children with SBS and IFALD. Revised criteria are proposed: progressive IFALD; 50 cm functional bowel in absence of ICV or 30 cm with ICV; 50% daily energy intake tolerated enterally for 4 weeks with satisfactory growth; and children with dysmotile bowel should be assessed for combined liver/bowel transplant unless the dysmotility is resolved and associated with minimal line infections.

10 Cholestasis and end-stage liver disease

Abstract Protein-energy malnutrition is an inevitable consequence of chronic liver disease, particularly in the developing infant. Severe malnutrition with loss of fat stores and muscle wasting affects between 60% and 80% of infants with liver disease ( Beath, 1993a ; Holt et al, 1997 ). Reduced energy intake secondary to anorexia, vomiting and fat malabsorption, in association with a disordered metabolism of carbohydrate and protein, increased energy requirements and vitamin and mineral deficiencies, contributes towards growth failure. Reversal of malnutrition is one of the key aims of liver transplantation and is achieved in the majority of long-term survivors. The aetiology of persistent growth failure posttransplantation is multifactorial and is related to pre-operative malnutrition, glucocorticoid administration, feeding problems and post-operative complications. Strategies to prevent pre- and post-transplant growth failure include early referral for liver transplantation and a multidisciplinary approach to nutritional support, which may increase survival and improve the quality of life and outcome of liver transplantation.

Social circumstances and medical complications in children with intestinal failure

Although most children discharged on home parenteral nutrition (HPN) will achieve enteral autonomy, some remain parenteral nutrition dependent; those who develop life-threatening complications may undergo small bowel transplantation (SBTx). The aim of this study was to investigate the relationship between social circumstances, compliance and complications. Subjects and methods An observational study in 2008–2012 on 64 children (34 HPN, 30 SBTx) from three units (two regional gastroenterology; one transplant). Social circumstances were assessed routinely as part of discharge planning; adherence by families to home care management was scored, and episodes of catheter-related blood stream infection and graft rejection were recorded for 2 years and related to compliance and social circumstances. Results A quarter of families had a disadvantaged parent: non-English speaking (n=11), unable to read (n=5), physical disability (n=3), mental health problems disclosed (n=10); 20% children were cared for by a lone parent. Discharge home was delayed by social factors (n=9) and need for rehousing (n=17, 27%). 17/34 (50%) of HPN and 12/30 (40%) of transplant families were assessed as fully adherent. 10 families were assessed as non-adherent, eight were subject to child protection review and care was taken over by another family member (n=3) or foster parents (n=2). The risk of catheter-related blood stream infection was increased by parental disadvantage and age <3 years (p<0.05). Poor compliance was associated with complications in HPN and SBTx recipients. Conclusions Children receiving complex home care may be socially isolated and measures to support improved compliance such as increased community support, social care involvement and respite care may improve outcomes.

Nutritional Requirements and Support in Liver Disease

The liver has a central role in energy metabolism, nutritional homeostasis and absorption of nutrients. Severe liver disease, whether acute or chronic, leads to multiorgan failure, which can have significant effects on growth and development in the long term. Malnutrition is common in infants and children with chronic liver disease (CLD). The pathophysiology of malnutrition in liver disease is complex and multifactorial and has extensive implications. Insufficient dietary intake is probably the most important factor and is correctable (Table 7.1). It is most severe in infants with chronic cholestatic liver disease, who are particularly vulnerable to the effects of malnutrition because of their high-energy and growth requirements [1].

G196(P) Residual Small Bowel Length Predicts Raised D-Lactate When Screening For Bacterial Overgrowth in Children with Intestinal Failure

Aims Small bowel bacterial overgrowth (SBBO) may cause non specific symptoms in children with intestinal failure (parenteral nutrition (PN) >28days). Rapid detection of raised serum D-lactate (DL) may be a clinically useful non invasive marker of SBBO. We present the first large cohort of DL in a tertiary referral centre, in patients with current or recent intestinal failure (IF) with new symptoms suggestive of SBBO. Methods Retrospective review over a 3 year period (01/01/2009 to 31/12/2011) of Patients with IF (0–18 years) and suspected SBBO was done. Demographics, aetiology of IF, symptoms, recent radiology and treatment were recorded. In those with short bowel syndrome, length of remaining small bowel was expressed as percentage of expected small bowel length appropriate for age (SBL) using a published formula. Raised DL was identified as >20µmol/L and recurrence as DL >20µmol/L at least 4 weeks apart and with standard treatment (rehydration, withholding or alteration of feeds, bicarbonate and/or antibiotics). Results Out of total cohort of 209, 49 patients (28 males; age range 0.16–13.07 and mean 4.76 years) were screened for DL. Aetiology for IF was bowel resection due to congenital malformation (17), necrotising enterocolitis (15), dysmotility (6) and enteropathy (11). 25/49 had raised DL and 24/49 did not have raised DL. There was no statistically significant difference in risk factors for raised DL comparing age, bowel resection, absence of ileo-caecal valve, abnormalities on barium study and use of proton pump inhibitors. SBL was significantly shorter (p = 0.001) in raised DL group (median 29.6%; range 11.4–100) than in group without (median 100%; range 19.10–100). Patients with <35% SBL, had 77% sensitivity for developing raised DL. Relationship to feed could not be analysed due to lack of accurate information on patients’ carbohydrate intake. Response to treatment was available in 12/25 and all had improvement in symptoms with fall in DL. Recurrence occurred in 48%. Conclusion Children with IF due to <35% expected SBL, when screened, have a 77% likelihood of having SBBO shown by raised DL. Screening in at risk patients allows prompt detection and treatment of SBBO. Recurrence is common necessitating prolonged antibiotic regimens.