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Central venous catheter (CVC) ethanol locks may reduce catheter-related bloodstream infection (CRBSI). Four children with intestinal failure on home parenteral nutrition (HPN) were selected for 70% ethanol locks because of their high rate of CRBSI. The 70% ethanol locks were instilled at a volume equal to the estimated internal volume of the CVC. Two children (aged 4 and 11 years) received 70% ethanol locks as CRBSI prophylaxis; another 2 children (aged 10 and 11 years) received 70% ethanol locks as adjunctive treatment for CRBSI. All 4 children developed either visible thrombosis in the CVC or CVC occlusion. To the authors' knowledge, this is the first report of CVC thrombosis associated with ethanol lock therapy in the pediatric HPN population. Although none of the CVCs were removed due to occlusion, these events raise serious concerns about the use of high-concentration ethanol locks.

Background and Aims: The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. Methods: Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011. Results: A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30–80) or 23.9% of the expected length for age (interquartile range 17.0%–40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2–29.2) with intestinal failure–associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients with chronic intestinal pseudo-obstruction syndrome and 2 of 4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both P < 0.001). Conclusions: Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

V ascular Ehlers-Danlos syndrome (v-EDS) is a connective tissue disease resulting from mutations in COL3A1 gene, coding for type III procollagen, a major protein in vessel walls and hollow organs (1,2). Common clinical features include translucent skin, prominent superficial veins, abnormal facial appearance, easy bruising, and arterial, intestinal, and/or uterine fragility (1,2). In children, it usually presents with sigmoid perforation or vascular rupture (3). A history of chronic constipation with megacolon starting in adolescence can precede intestinal perforation (1). Intestinal failure (IF) requiring long-term parenteral nutrition (PN) has not been reported in children with v-EDS.We report a case of IF in a child with v-EDS because of multiple high-output enterocutaneous fistulae requiring longterm PN.

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