In infants with intestinal failure, the only signs of infection may be a drop in platelets and neutrophils and rise in bilirubin. In the presence of intestinal failure-associated liver disease (IFALD), empiric treatment with antibiotics should be considered.
Important features from history
- Fever >38° C on two occasions or >38.5° C on a single occasion
- Metabolic acidosis
- Glucose instability
- Rise in C-reactive protein (CRP)
- Systemic malaise
- Signs of severe infection: rigors, shock, collapse
- Previous infections with the same central venous line (CVL) in situ
- Exclude other infectious aetiology: urine, respiratory infection or even meningitis in young infants need to be considered
Investigations
- Central and peripheral blood cultures (BC) (paired only useful if quantitative or semi-quantitative culture technique used)
- FBC, LFTs, CRP, urine culture
Management
Prompt treatment is important and may minimise liver damage.
- Broad-spectrum antibiotics until bacteria and sensitivities identified
- An individualised sepsis protocol should be established for each child with intestinal failure to tailor antibiotics as appropriate
If necessary, remove the CVL when the following are suspected:
- Fungal infection
- Staphylococcus aureus
- Symptoms not settling with antibiotic therapy
- Recurrence of the same organism with the same subtype within 1 month
Recurrent life-threatening infections, e.g. with collapse/PICU admission, is a potential indication for small bowel transplant.
Keys to decreased infection are:
- Management by a multidisciplinary nutrition support team
- Appropriate care bundles and training packs for patients and families
- Use of 2% chlorhexidine in isopropanol is now standard for cleaning hubs
- Use of antibacterial line locks is increasing, especially with taurolidine which prevents biofilm formation. Other agents include 70% ethanol
- Other considerations: use of single-lumen catheters dedicated to PN use; cycled enteral antibiotics to decrease bacterial overgrowth and translocation
- Rapid deterioration is a medical emergency, and shock may develop very quickly, necessitating emergency line removal
- Consider fungal infection: where antibiotics have been used frequently, then antifungal agents should be added to protocols
Thrombosis and central venous line occlusion
CVLs are the commonest cause of venous thrombosis in children. The importance of prevention cannot be underestimated, especially in those who rely on lifelong PN.
Important features from history
- Increasing stiffness of line on flushing, increased infusion pressures, leaking from the line or
- Inability to withdraw blood
- Superior vena cava (SVC) syndrome (neck and face swelling together with dilated veins)
- Pain and swelling of the arm if CVL is sited in upper limbs
Note that venous thrombosis may be asymptomatic and diagnosed by USS or MRV.
Differential diagnosis
- Blocked CVL from blood, drugs or PN within the line
- Clot or fibrin sheath in the vein
- CVL tip resting against the vein wall or compressed due to positioning or pressure from the clavicle