ED Management of VP Shunt Malfunction In Infants and Children Obtain Shunt series1 and Head CT2,3 for CNS symptoms/signs4 Obtain CBC/Diff 5 & Blood Cx6 if fever or other unexplained symptoms7 Consider abdominal imaging study if unexplained abdominal symptoms/signs8 Do not pump the shunt9 Do not tap shunt even if suspect shunt infection10 Neurosurgery consult IV Abx11 for shunt infection12 Notes: 1 Shunt series=plain radiographs of shunt valve & tubing (necessary to assess continuity of system and rule out kinking of the tube) 2 Evaluate ventricular size & compare to previous study if available. Frequently children have abnormal baseline ventricular size even with a normally functioning shunt. Even with a CT unchanged from baseline, early obstruction may be present. Obstruction occurs most often in the proximal portion of the tubing during the first 2 years following shunt placement and is due to occlusion by choroid plexus, glial or ependymal tissue, or clotted blood. Distal obstruction (due to pseudocyst formation at tip, kinking, thrombosis, or occlusion of the tip with omentum) is most common for shunts in place for > 2 years. 1 Guideline for ED Management of VP Shunt Malfunction in Infants & Children 3 4 5 6 7 8 9 10 11 12 Valves are an infrequent cause of shunt malfunction. Consider head ultrasound if anterior fontanel is open. CNS symptoms/signs=headache, vomiting, altered mental status, meningismus, seizure activity, papilledema, paralysis of upward gaze, diplopia, full/expanding fontanel, increasing head circumference (see head circumference charts for boys and girls 2-18 years adapted from Nelhaus) Note: WBC count is normal in 25% of patients with documented shunt infection. CSF eosinophilia has been associated with shunt infection. Shunt infections are most frequently caused by Staphylococcus epidermidis followed by Staphylococcus aureus, gram-negative rods (especially in neonates), and Propionibacterium acnes. Unexplained symptoms include fever without source, poor feeding, behavior change. Patients with VP shunts can present with shunt-related abdominal processes even in the absence of neurologic complaints. Typical causes of abdominal pain include peritonitis due to infection of the distal tubing, intestinal perforation, or volvulus. Pumping the shunt gives little useful information and may actually suck debris or choroid plexus into the shunt. Shunt taps should be performed by neurosurgery due to risk of infecting the hardware and difficulty in interpreting CSF cell count and opening pressure. A shunt tap is performed by inserting a small gauge needle into the shunt reservoir and may show poor proximal flow from the ventricular catheter, an elevated opening pressure, or consistent improvement in symptoms after removal of CSF. Suggested IV Abx: vancomycin (60 mg/kg/day div Q6H) + cefotaxime (300 mg/kg/day div Q6H) Half of shunt infections occur in the first two weeks and 75% in the first 2 months after shunt placement. CSF should be analyzed for cell count, gram stain/cx, protein & glucose. Organisms on gram stain confirm infection. There is variability in the literature regarding the acceptable number of WBCs in the cell count. In one study, the median number of CSF WBCs was 18 cells/mm3 in non-infected patients and 79 cells/mm3 in infected patients. In another study, 47% of infected patients with a positive CSF culture had < 20 cells/mm.3 Even with normal CSF analysis, 17% of patients may have a positive culture. References: Nelhaus G. Head circumference from birth to eighteen years. Practical composite international and interracial graphs. Pediatrics 1968;41:106-114 Grosfeld J, Cooney D, Smith J, et. al. Intra-abdominal complications following ventriculoperitoneal shunt procedures. Pediatrics 1974;54:791-796 Myers MG, Schoenbaum SC. Shunt fluid aspiration. Am J Dis Child 1975;129:220222 2 Guideline for ED Management of VP Shunt Malfunction in Infants & Children Schoenbaum SC, Gardner P, Shillito J. Infections of cerebrospinal fluid shunts: Epidemiology, clinical manifestations and therapy. J Infect Dis 1975;131:543-552 Hubschmann OR, Countee RW. Acute abdomen in children with infected ventriculoperitoneal shunts. Arch Surg 1980;115:305-307 Walters BC, Hoffman HJ, Hendrick EB et. al. Cerebrospinal fluid shunt infection. Influences on initial management and subsequent outcomes. J Neurosurg 1984;60:1014-1021 Odio C, McCracken G, Nelson JD. CSF shunt infections in pediatrics—seven year experience. Am J Dis Child 1984;138:1103-1108 Yogev R. Cerebrospinal fluid shunt infections: A personal view. Pediatr Infect Dis J 1985;85:113-118 Madsen MA. Emergency department management of ventriculoperitoneal cerebrospinal fluid shunts. Ann Emerg Med 1986;15:1330-1343 Guertin SR. Cerebrospinal fluid shunts evaluation, complications and crisis management. Pediatr Clin North Am 1987;34:203-217 Coker SB. Cyclic vomiting and the slit ventricle syndrome. Pediatr Neurol 1987;3:297-299 Sainte-Rose C, Hoffman HJ, Hirsch JF. Shunt failure. Concepts Pediatr Neurosurg 1989;9:7 Vinchon M, Vallee L, Prin L et. al. Cerebro-spinal fluid eosinophilia in shunt infections. Neuropediatrics 1992;23:235-240 Piatt J. Physical examination of patients with cerebrospinal fluid shunts: Is there useful information in pumping the shunt? Pediatrics 1992;89:470-473 Kontny U, Hofling B, Gutjahr P et. al. CSF shunt infections in children. Infection 1993;21:89-92 Sood S, Kim S, Canady AI et. al. Useful components of the shunt tap test for evaluation of shunt malfunction. Child Nerv Syst 1993;9:157-162 Cantrell P, Fraser F, Carty P. The value of baseline ct head scans in the assessment of shunt complications in hydrocephalus. Pediatr Radiol 1993;23:485-486 Blount JP, Campbell JA, Haines SJ. Complications in ventricular cerebrospinal fluid shunting. Neurosurg Clin North Am 1993;4:633-656 3 Guideline for ED Management of VP Shunt Malfunction in Infants & Children Watkins L, Hayward R, Andar U et. al. The diagnosis of blocked cerebrospinal fluid shunts: A prospective study of referral to a paediatric neurosurgical unit. Child Nerv Syst 1994;10:87-90 Kast J, Duong D, Nowzari F et. al. Time-related patterns of ventricular shunt failure. Child Nerv Syst 1994;10:524-528 Ronan A, Hogg GC Klug GL. Cerebrospinal fluid shunt infections in children. Pediatr Infect Dis J 1995;14:782-786 Drake JM, Sainte-Rose C. Shunt complications. In Drake JM, Sainte-Rose C (eds): The Shunt Book. Cambridge, MA, Blackwell Scientific, 1995, pp 121-192 Piatt JHJ: Pumping the shunt revisited: A longitudinal study. Pediatr Neurosurg 1996;25:73-77 4 Guideline for ED Management of VP Shunt Malfunction in Infants & Children