Pediatric Neurology 50 (2014) 431e432

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Visual Diagnosis

Neuroimaging Findings in Pediatric Propofol Infusion Syndrome Andrea Poretti MD *, Thangamadhan Bosemani MD, Thierry A.G.M. Huisman MD Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, Maryland

A 3-year-old girl with a large venous malformation involving tongue, lips, and masticator space underwent sclerotherapy during propofol anesthesia. After the procedure, she developed metabolic acidosis with acute renal failure, rhabdomyolysis, and transient neurological deficits including encephalopathy and weakness of arms and legs. This constellation of findings in a child with propofol anesthesia suggests propofol-related infusion syndrome (PRIS).1,2 Acute brain magnetic resonance imaging showed T2-hyperintensity and reduced diffusion on apparent diffusion coefficient maps within the supra- and infratentorial white matter (Figure); these changes had completely resolved at follow-up. Three months after the acute event, all symptoms and clinical findings completely resolved.

Muscle biopsy and fat metabolism analyses in patients with PRIS show findings similar to those of patients with inhibition of b-oxidation of long fatty acids.3 Neuroimaging findings in children with PRIS have not been previously reported. In our patient, acute brain magnetic resonance imaging showed T2-hyperintensity and reduced diffusion within the supra- and infratentorial white matter, which was completely reversible on follow-up. Neuroimaging findings matching those of our patients have been reported in patients with defects in peroxisomal b-oxidation of verylong-chain fatty acids before their degradation within the mitochondria. This observation matches with the suggested pathomechanism of PRIS. Familiarity with the neuroimaging findings of this rare complication is essential for correct diagnosis and prediction of prognosis.

Discussion

PRIS is a rare but potentially catastrophic complication of propofol use. It is clinically characterized by metabolic acidosis, rhabdomyolysis, arrhythmias, myocardial failure, renal failure, and hepatomegaly, and may lead to death.1,2 The following risk factors have been associated with PRIS: young age, critical illness, high fat intake, catecholamine or steroid use, inborn error of fatty acid oxidation, propofol doses exceeding 4-5 mg/kg per hour, and duration of use exceeding 48 hours.2

* Communications should be addressed to: Dr. Poretti; Section of Pediatric Neuroradiology; Division of Pediatric Radiology; Russell H. Morgan Department of Radiology and Radiological Science; Charlotte R. Bloomberg Children’s Center; The Johns Hopkins Hospital; Sheikh Zayed Tower; Room 4174; 1800 Orleans St.; Baltimore, MD 21287-0842. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2013.12.016

References 1. Fudickar A, Bein B. Propofol infusion syndrome: update of clinical manifestation and pathophysiology. Minerva Anesthesiol. 2009;75: 339-344. 2. Savard M, Dupré N, Turgeon AF, et al. Propofol-related infusion syndrome heralding a mitochondrial disease: case report. Neurology. 2013;81:770-771. 3. Withington DE, Decell MK, Al Ayed T. A case of propofol toxicity: further evidence for a causal mechanism. Paediatr Anaesth. 2004;14: 505-508.

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A. Poretti et al. / Pediatric Neurology 50 (2014) 431e432

FIGURE. Axial T2-weighted images (A,B) with matching apparent diffusion coefficient maps (C,D) show diffuse T2-hyperintense signal of the supra- and infratentorial white matter (arrows in A,B) with matching low apparent diffusion coefficient values representing reduced diffusion (arrows in C,D).

Neuroimaging findings in pediatric propofol infusion syndrome.

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