Patient R.B.

Zachary R. BarnardMGH Neurosurgery Sub-

internJuly 2012

Chief Complaint

54 year-old RHM presented to EW with progressive nausea, vomiting, headaches, and gait instability over

the past one to two years.

History of Present Illness1992: Evaluation for neck pain resulted in MRI showing

incidental T2 hyperintense, non-enhancing 2cm mass in 4th ventricle

1992: Biopsy by Dr. Peter Black was inconclusive1993: f/u MRI revealed no interval growth1993-2011: Lost to follow-up2011: Nausea/vomiting with MRI showing 4cm 4th

ventricular mass6/23/12: MGH EW- MRI showing 2.8cm AP x 4.6cm RL x

4.3cm SI mass with mildly increased obstructive hydrocephalus. Exam remarkable for R>L nystagmus. Patient declined surgery at this time.

7/7/12: MGH EW- worsening balance, HA, N/V, “left foot feels clumsy”. Decided to proceed with definitive surgical therapy by Dr. Sheth and Dr. Butler.

Pre-debulking Imaging

2011(FLAIR)

6/23/12(T1- post)

Operation

1. Right frontal stereotactic endoscopic third ventriculostomy (ETV) and external ventriculostomy drain (EVD)

2. Suboccipital craniectomy and C1 laminectomy followed by tumor biopsy and debulking by CO2 laser

ETV & EVD

• Stereotaxy was used to to determine trajectory of rigid endoscope through the cortex into the right lateral ventricle, through the right foramen of Monro, and into the 3rd ventricle– Endoscopic forceps were used to create initial

perforation in floor of 3rd ventricle followed by expansion with a Fogarty #4 balloon.

– The endoscope was advanced into the subarachnoid space and the basilar artery was visualized

• An EVD was left in the right lateral ventricle following the endoscope track.

Suboccipital craniectomy & C1 laminectomy

• Tumor was noted to be protruding midline and anterior to the vermis

• Biopys/frozen: low-grade glioma with pilocytic features

• Fiberoptic CO2 laser debulking

Post-op Imaging

6/23/12(T1- post)

7/7/12(T1- post)

Post-operative Course

• Patient currently in Neuro ICU on Decadron taper with the EVD at 20 and clamped. ICPs ranging 5-17

• Final pathology pending

“Prophylactic ETV in patients undergoing

resection of 4th ventricular tumors”

Background: History of 3rd Ventriculostomy

• 1923: W.J. Mixter performed first puncture of the floor of 3rd ventricle with a direct vision urethroscope– 4 month old, incision made in fontanelle

followed by small durotomy and insertion of scope. It was navigated down the foramen of Munro and made a 4mm puncture in floor of 3rd ventricle.

– Ten days later confirmation of patency was performed by inserting a lumbar needle and a ventricular needle and injecting indocarmine solution into the ventricular needle. After 30 secs the indocarmine solution was seen in the lumbar needle confirming connectivity

Mixter, Boston Medical and Surgical Journal,

1923

Background: Hydrocephalus with Posterior Fossa Tumors

• Pre-operative obstructive hydrocephalus in 70-80% of children with posterior fossa tumor3,5

• Persisting hydrocephalus post-resection variable at 11.5-39%3.– Due-Tonnessen et al• 69/87 patients symptomatic HCP, 41/69

symptom resolution after tumor resection (astrocytoma 83%, medulloblastoma 47%, ependymoma 54%)5

Who and When

• Who gets an ETV?• When do they get an ETV?• Who fails?• When do they fail?

Long-term Reliability of ETV

• Kadrian et al, 2005– Retrospective case

series– 203 patients,

communicating and non-communicating hydrocephalus, age 2 days to 78 yrs.

– ETV for various Dx.– Outcomes measures

1. Surgical success2. Reliability of ETV3. Reliability of revision

• Results– Overall ETV success 89%

(95% CI 84-93%)• Factors NOT associated

with surgical success: age, surgeon experience, technique, diagnosis

– Five year success rate 58-78%

• Possible reason for failure– CSF resorption issues,

inadequate fenestration, infection, increase CSF protein, increase bleeding around fenestration,

Background: Predictors of Outcome with ETVs

• Woodworth et al, 2012– Retrospective case

series– 103 patients with

obstructive hydrocephalus, mean age 45+16 years

– Either first intervention or shunt failure

– Primary endpoint was operating room for shunt

– Mean follow-up 5 yrs. (2-9 years)

– Overall 55% of patients remained symptom and surgery-free through last follow-up

Stereotaxy ImagingSteroids

Children’s Hospital Alabama ETV Success Score (ETVSS)

Validation• Naftel et al, 2011

– Retrospective Chart review

– 151 children age + 6.0 yrs. (136 > 6 months follow-up), variable diseases causing hydrocephalus

– Independent variables age, shunt Hx., EVD, neuronavigation, causes of hydrocephalus

– Outcome measures were ETV failure/complications

• Results– 6 month success 68.4%– Biggest issue at ETVSS 70

ETV vs. Shunt for Treatment of Hydrocephalus in Children

• Kulkami et al, 2010– Retrospective review of

3 trials– Adjust for treatment

selection bias (age, Dx.)– International cohort

newly diagnosed hydrocephalus, children <19 yrs.

– Data taken from one ETV trial (n=489) and two prior shunting trials (n=720)

– Outcome was failure of treatment defined as subsequent surgical procedure or death

Prophylactic ETV in Children with Posterior Fossa Tumors

• Bhatia et al, 2009• Retrospective case series• 59 children with

posterior fossa tumors, variable pathology (medulloblastoma 25, pilocytic astrocytoma 24)

• 37 prophylactic ETV (mean time to ETV 1.5 days, mean time to tumor resection 4.6 days)

• Outcome measure was redevelopment of hydrocephalus

• Results– 5/37 redeveloped

hydrocephalus after ETV over 7.5 years follow-up

– Worse hydrocephalus at presentation lead to worse outcome

– No correlation with recurrent hydrocephalus and tumor type

Prophylactic ETV vs. VPS in Pediatric Posterior Fossa

Tumors • El-Ghandour,

2o11– 53 children with

obstructive hydrocephalus due to medulloblastoma or ependymomas

– 32 ETV and 21 VPS followed 1-2 weeks later by tumor resection

– Mean follow-up 27.4 months (ETV) and 25 months (VPS)2

El-Ghandour, Childs Nerv Syst

2011

Future Research

• Physiology of CSF flow dynamics• Larger, prospective randomized

trials comparing ETV and shunts• Better adjustment for age, disease,

prior shunting, and other cofounders• Standardization of protocols for ETV

and tumor resection

Summary

• Literature is variable on how to treat different ages, diseases, and when to treat

• ETV have advantages over shunts in certain situations

• Better predictive models are necessary to make better decisions for ETVs

• Significantly more research must be done

Conclusions

• “The good physician treats the disease; the great physician treats the patient who has the disease.”

-Sir William Osler

References1. Scarff JE. Treatment of obstructive hydrocephalus by puncture of the lamina terminalis and floor of the third ventricle. Journal of neurosurgery. 1951;8(2):204-13. Epub 1951/03/01. doi: 10.3171/jns.1951.8.2.0204. PubMed PMID: 14824983.2. El-Ghandour NM. Endoscopic third ventriculostomy versus ventriculoperitoneal shunt in the treatment of obstructive hydrocephalus due to posterior fossa tumors in children. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2011;27(1):117-26. Epub 2010/08/26. doi: 10.1007/s00381-010-1263-2. PubMed PMID: 20737274.3. Di Rocco F, Juca CE, Zerah M, Sainte-Rose C. Endoscopic Third Ventriculostomy and Posterior Fossa Tumors. World neurosurgery. 2012. Epub 2012/03/03. doi: 10.1016/j.wneu.2012.02.018. PubMed PMID: 22381845.4. Bognar L, Borgulya G, Benke P, Madarassy G. Analysis of CSF shunting procedure requirement in children with posterior fossa tumors. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2003;19(5-6):332-6. Epub 2003/04/24. doi: 10.1007/s00381-003-0745-x. PubMed PMID: 12709823.5. Due-Tonnessen BJ, Helseth E. Management of hydrocephalus in children with posterior fossa tumors: role of tumor surgery. Pediatric neurosurgery. 2007;43(2):92-6. Epub 2007/03/06. doi: 10.1159/000098379. PubMed PMID: 17337918.6. Cinalli G, Spennato P, Ruggiero C, Aliberti F, Zerah M, Trischitta V, et al. Intracranial pressure monitoring and lumbar puncture after endoscopic third ventriculostomy in children. Neurosurgery. 2006;58(1):126-36; discussion -36. Epub 2005/12/31. PubMed PMID: 16385337.7. Bhatia R, Tahir M, Chandler CL. The management of hydrocephalus in children with posterior fossa tumours: the role of pre-resectional endoscopic third ventriculostomy. Pediatric neurosurgery. 2009;45(3):186-91. Epub 2009/06/06. doi: 10.1159/000222668. PubMed PMID: 19494562.8. Baldauf J, Fritsch MJ, Oertel J, Gaab MR, Schroder H. Value of endoscopic third ventriculostomy instead of shunt revision. Minimally invasive neurosurgery : MIN. 2010;53(4):159-63. Epub 2010/12/07. doi: 10.1055/s-0030-1268415. PubMed PMID: 21132606.9. El Beltagy MA, Kamal HM, Taha H, Awad M, El Khateeb N. Endoscopic third ventriculostomy before tumor surgery in children with posterior fossa tumors, CCHE experience. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2010;26(12):1699-704. Epub 2010/05/27. doi: 10.1007/s00381-010-1180-4. PubMed PMID: 20502903.10. Kulkarni AV, Drake JM, Kestle JR, Mallucci CL, Sgouros S, Constantini S. Predicting who will benefit from endoscopic third ventriculostomy compared with shunt insertion in childhood hydrocephalus using the ETV Success Score. Journal of neurosurgery Pediatrics. 2010;6(4):310-5. Epub 2010/10/05. doi: 10.3171/2010.8.PEDS103. PubMed PMID: 20887100.11. Kulkarni AV, Drake JM, Kestle JR, Mallucci CL, Sgouros S, Constantini S. Endoscopic third ventriculostomy vs cerebrospinal fluid shunt in the treatment of hydrocephalus in children: a propensity score-adjusted analysis. Neurosurgery. 2010;67(3):588-93. Epub 2010/07/22. doi: 10.1227/01.NEU.0000373199.79462.21. PubMed PMID: 20647973.12. Kulkarni AV, Hui S, Shams I, Donnelly R. Quality of life in obstructive hydrocephalus: endoscopic third ventriculostomy compared to cerebrospinal fluid shunt. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2010;26(1):75-9. Epub 2009/08/29. doi: 10.1007/s00381-009-0983-7. PubMed PMID: 19714338.13. Kulkarni AV, Shams I, Cochrane DD, McNeely PD. Quality of life after endoscopic third ventriculostomy and cerebrospinal fluid shunting: an adjusted multivariable analysis in a large cohort. Journal of neurosurgery Pediatrics. 2010;6(1):11-6. Epub 2010/07/03. doi: 10.3171/2010.3.PEDS09358. PubMed PMID: 20593981.14. Durnford AJ, Kirkham FJ, Mathad N, Sparrow OC. Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus: validation of a success score that predicts long-term outcome. Journal of neurosurgery Pediatrics. 2011;8(5):489-93. Epub 2011/11/03. doi: 10.3171/2011.8.PEDS1166. PubMed PMID: 22044375.15. Naftel RP, Reed GT, Kulkarni AV, Wellons JC. Evaluating the Children's Hospital of Alabama endoscopic third ventriculostomy experience using the Endoscopic Third Ventriculostomy Success Score: an external validation study. Journal of neurosurgery Pediatrics. 2011;8(5):494-501. Epub 2011/11/03. doi: 10.3171/2011.8.PEDS1145. PubMed PMID: 22044376.16. Woodworth GF, See A, Bettegowda C, Batra S, Jallo GI, Rigamonti D. Predictors of Surgery-Free Outcome in Adult Endoscopic Third Ventriculostomy. World neurosurgery. 2011. Epub 2011/11/29. doi: 10.1016/j.wneu.2011.09.018. PubMed PMID: 22120264.• 

Acknowledgements

• Neurosurgery Faculty– Dr. Sheth– Dr. Butler– Dr. Curry– Dr. Martuza

• Neurosurgery Residents• Neurosurgery supporting staff