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 T 2 hyperintense, non-enhancing 2 cm mass in 4 th 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 4 cm 4 th ventricular mass 6/23/12: MGH EW- MRI showing 2. 8 cm AP x 4. 6 cm RL x 4. 3 cm 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 (T 1 - post)
Operation 1. Right frontal stereotactic endoscopic third ventriculostomy (ETV) and external ventriculostomy drain (EVD) 2. Suboccipital craniectomy and C 1 laminectomy followed by tumor biopsy and debulking by CO 2 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 3 rd ventricle – Endoscopic forceps were used to create initial perforation in floor of 3 rd 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 & C 1 laminectomy • Tumor was noted to be protruding midline and anterior to the vermis • Biopys/frozen: lowgrade glioma with pilocytic features • Fiberoptic CO 2 laser debulking
Post-op Imaging 6/23/12 (T 1 - post) 7/7/12 (T 1 - 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 4 th ventricular tumors”
Background: History of 3 rd Ventriculostomy • 1923: W. J. Mixter performed first puncture of the floor of 3 rd 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 4 mm puncture in floor of 3 rd 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 tumor 3, 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 • Results – 5/37 redeveloped • Retrospective case series hydrocephalus after ETV • 59 children with posterior over 7. 5 years follow-up fossa tumors, variable – Worse hydrocephalus at pathology presentation lead to worse outcome (medulloblastoma 25, – No correlation with pilocytic astrocytoma 24) recurrent hydrocephalus • 37 prophylactic ETV and tumor type (mean time to ETV 1. 5 days, mean time to tumor resection 4. 6 days) • Outcome measure was redevelopment of hydrocephalus
Prophylactic ETV vs. VPS in Pediatric Posterior Fossa Tumors • El-Ghandour, 2 o 11 – 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
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Acknowledgements • Neurosurgery Faculty – Dr. Sheth – Dr. Butler – Dr. Curry – Dr. Martuza • Neurosurgery Residents • Neurosurgery supporting staff
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