Invasive intracranial pressure monitoring is a useful adjunct in the management of severe hepatic encephalopathy associated with pediatric acute liver failure.

OBJECTIVE: Pediatric acute liver failure is often accompanied by hepatic encephalopathy, cerebral edema, and raised intracranial pressure. Elevated intracranial pressure can be managed more effectively with intracranial monitoring, but acute-liver-failure-associated coagulopathy is often considered a contraindication for invasive monitoring due to risk for intracranial bleeding. We reviewed our experience with use of early intracranial pressure monitoring in acute liver failure in children listed for liver transplantation. DESIGN AND PATIENTS: Retrospective review of all intubated pediatric acute liver failure patients with grade III and grade IV encephalopathy requiring intracranial pressure monitoring and evaluated for potential liver transplant who were identified from an institutional liver transplant patient database from 1999 to 2009.
INTERVENTIONS: None.
MEASUREMENTS AND MAIN RESULTS: A total of 14 patients were identified who met the inclusion criteria. Their ages ranged from 7 months to 20 yrs. Diagnoses of acute liver failure were infectious (three), drug-induced (seven), autoimmune hepatitis (two), and indeterminate (two). Grade III and IV encephalopathy was seen in ten (71%) and four (29%) patients, respectively. Computed tomography scans before intracranial pressure monitor placement showed cerebral edema in five (35.7%) patients. Before intracranial pressure monitor placement, fresh frozen plasma, vitamin K, and activated recombinant factor VIIa were given to all 14 patients, with significant improvement in coagulopathy (p < .04). The initial intracranial pressure ranged from 5 to 50 cm H2O; the intracranial pressure was significantly higher in patients with cerebral edema by computed tomography (p < .05). Eleven of 14 (78%) patients received hypertonic saline, and three (22%) received mannitol for elevated intracranial pressure. Eight of 14 (56%) monitored patients were managed to liver transplant, with 100% surviving neurologically intact. Four of 14 (28%) patients had spontaneous recovery without liver transplant. Two of 14 (14%) patients died due to multiple organ failure before transplant. One patient had a small 9-mm intracranial hemorrhage but survived after receiving a liver transplant. No patient developed intracranial infection.
CONCLUSIONS: In our series of patients, intracranial pressure monitoring had a low complication rate and was associated with a high survival rate despite severe hepatic encephalopathy and cerebral edema in the setting of pediatric acute liver failure. In our experience, monitoring of intracranial pressure allowed interventions to treat increased intracranial pressure and provided additional information regarding central nervous system injury before liver transplant. Further study is warranted to confirm if monitoring allows more directed intracranial pressure therapy and improves survival in pediatric acute liver failure.