Introduction: The requirement for frequent intraventricular drug delivery in the setting of shunt dependence is challenging and can be a complicating factor for the treatment of CNS infection, neoplastic disease, and hemorrhage. This is especially relevant in the pediatric population where hematogenous malignancy requiring intrathecal drug delivery and shunt-dependent hydrocephalus are relatively more prevalent. Intrathecal chemotherapy agents can be prematurely diverted in these shunt-dependent patients. We report the use of a stop-flow programmable shunt valve to maximize delivery of intrathecal chemotherapy.

Methods: Literature review and case presentation. A 3-year-old male with acute lymphoblastic lymphoma (ALL) with CNS spread and disseminated intravascular coagulation (DIC) presented with spontaneous intracerebral and intraventricular hemorrhages. The patient then developed post-hemorrhagic hydrocephalus and eventually progressed to shunt dependence but still required frequent intrathecal chemotherapy administration. A ventriculoperitoneal shunt, equipped with a valve that allows for near cessation of CSF flow (Certas®, Codman, Raynham, MA), and a contralateral Ommaya reservoir were inserted to maximize intraventricular dissemination of chemotherapy.

Results: To the best of our knowledge, this is the first reported case of the use of a high-resistance programmable valve being used to virtually cease CSF flow through the distal shunt catheter temporarily in order to maximize intraventricular drug dissemination in a pediatric patient with CNS leukemia.

Conclusions: A high-flow programmable valve with a very high resistance setting option can be used in conjunction with an Ommaya reservoir to allow CSF diversion in shunt-dependent patients. Simultaneously it can be used to temporarily halt premature evacuation of the drug-containing CSF from the CNS in those who require intrathecal chemotherapy.

Patient Care: Introduce a technique that could facilitate in the treatment of shunt-dependent hydrocephalus with concomitant need for intrathecal drug delivery.

Learning Objectives: By the conclusion of this session, participants should be able to: 1) Describe the importance of intrathecal drug delivery in the setting of hydrocephalus, 2) Discuss, in small groups the pros and cons of a stop-flow ventriculoperitoneal shunt valve with contralateral drug delivery reservoir, and 3) Identify an effective treatment for simultaneous craniospinal neoplastic disease and shunt-dependent hydrocephalus.

References: 1. Pui CH. Recent research advances in childhood acute lymphoblastic leukemia. Journal of the Formosan Medical Association = Taiwan yi zhi. Nov 2010;109(11):777-787. 2. Bleyer WA, Poplack DG. Intraventricular versus intralumbar methotrexate for central-nervous-system leukemia: prolonged remission with the Ommaya reservoir. Medical and pediatric oncology. 1979;6(3):207-213. 3. Bleyer WA, Poplack DG, Simon RM. "Concentration x time" methotrexate via a subcutaneous reservoir: a less toxic regimen for intraventricular chemotherapy of central nervous system neoplasms. Blood. May 1978;51(5):835-842. 4. Ribeiro RC, Pui CH. The clinical and biological correlates of coagulopathy in children with acute leukemia. J Clin Oncol. Aug 1986;4(8):1212-1218. 5. Higuchi T, Toyama D, Hirota Y, et al. Disseminated intravascular coagulation complicating acute lymphoblastic leukemia: a study of childhood and adult cases. Leuk Lymphoma. Aug 2005;46(8):1169-1176. 6. Fritz RD, Forkner CE, Jr., Freireich EJ, Frei E, 3rd, Thomas LB. The association of fatal intracranial hemorrhage and blastic crisis in patients with acute leukemia. N Engl J Med. Jul 9 1959;261(2):59-64. 7. Quinones-Hinojosa A, Gulati M, Singh V, Lawton MT. Spontaneous intracerebral hemorrhage due to coagulation disorders. Neurosurg Focus. Oct 15 2003;15(4):E3. 8. Graus F, Rogers LR, Posner JB. Cerebrovascular complications in patients with cancer. Medicine (Baltimore). Jan 1985;64(1):16-35. 9. Pui CH, Thiel E. Central nervous system disease in hematologic malignancies: historical perspective and practical applications. Seminars in oncology. Aug 2009;36(4 Suppl 2):S2-S16. 10. Eklund A, Koskinen LO, Williams MA, Luciano MG, Dombrowski SM, Malm J. Hydrodynamics of the Certas programmable valve for the treatment of hydrocephalus. Fluids Barriers CNS. 2012;9(1):12. 11. Pui CH, Howard SC. Current management and challenges of malignant disease in the CNS in paediatric leukaemia. Lancet Oncol. Mar 2008;9(3):257-268. 12. Czech T, Reinprecht A, Dietrich W, Hainfellner JA, Slavc I. Reversible occlusion shunt for intraventricular chemotherapy in shunt-dependent brain tumor patients. Pediatric hematology and oncology. Jul-Aug 1997;14(4):375-380. 13. Lin N, Dunn IF, Glantz M, et al. Benefit of ventriculoperitoneal cerebrospinal fluid shunting and intrathecal chemotherapy in neoplastic meningitis: a retrospective, case-controlled study. J Neurosurg. Oct 2011;115(4):730-736. 14. Zada G, Chen TC. A novel method for administering intrathecal chemotherapy in patients with leptomeningeal metastases and shunted hydrocephalus: case report. Neurosurgery. Sep 2010;67(3 Suppl Operative):onsE306-307; discussion onsE307. 15. Lin CL, Loh JK, Kwan AL, Howng SL. Spontaneous intracerebral hemorrhage in children. Kaohsiung J Med Sci. Mar 1999;15(3):146-151. 16. Zacharia BE, Vaughan KA, Hickman ZL, et al. Predictors of long-term shunt-dependent hydrocephalus in patients with intracerebral hemorrhage requiring emergency cerebrospinal fluid diversion. Neurosurg Focus. Apr 2012;32(4):E5. 17. Nishikawa T, Ueba T, Kajiwara M, Miyamatsu N, Yamashita K. A priority treatment of the intraventricular hemorrhage (IVH) should be performed in the patients suffering intracerebral hemorrhage with large IVH. Clin Neurol Neurosurg. Jun 2009;111(5):450-453. 18. Chan KH, Mann KS. Prolonged therapeutic external ventricular drainage: a prospective study. Neurosurgery. Oct 1988;23(4):436-438.