Patient R.B.
Zachary R. Barnard
MGH Neurosurgery Sub-intern
July 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 Illness
1992: 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 inconclusive
1993: f/u MRI revealed no interval growth
1993-2011: Lost to follow-up
2011: Nausea/vomiting with MRI showing 4cm 4th ventricular
mass
6/23/12: 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 noncommunicating
hydrocephalus, age 2 days
to 78 yrs.
– ETV for various Dx.
– Outcomes measures
1.
2.
3.
Surgical success
Reliability of ETV
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 5878%
• 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. (29 years)
– Overall 55% of patients
remained symptom and
surgery-free through last
follow-up
Stereotaxy
Steroids
Imaging
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
References
1.
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/s00381-0030745-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