4/30/2014

Hydrocephalus

Management of Hydrocephalus Current Concept and Controversies Renee Reynolds, MD Assistant Professor of Neurosurgery University at Buffalo Neurosurgery Women and Children’s Hospital of Buffalo Pediatric Grand Rounds

• An imbalance between the production and absorption of cerebrospinal fluid within the cranial cavities that is often but not always accompanied by expansion of the cerebral ventricles as well as increased intracranial pressure

Etiology •

•

Congenital • Malabsorption • Aqueductal Stenosis • Dandy-Walker • Arachnoid Cysts • Atresia of the foramen of Monro Acquired • Tumor • Infection • Hemorrhage • Traumatic Brain Injury

• Communicating • Obstructive

Communicating Hydrocephalus

Obstructive Hydrocephalus

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• Untreated HCP leads to pathologic changes in brain morphology, microstructure, circulation, biochemistry, metabolism, and maturation leading to progressive neurologic decline and death

Hippocrates • Thought to have first recognized hydrocephalus • Credited with puncturing the dilated cerebral ventricles • Actually only drained the subdural space!! • Unable to clearly distinguish between fluid collecting inside or outside the brain

Vesalius (1514-1564) • Gave the first clear description of internal hydrocephalus • “The water had not collected between the skull and its outer surrounding membrane, or the skin (where the doctors’ books teach that water is deposited in other cases), but in the cavity of the brain itself, and actually in the right and left ventricles of the brain. The cavity and breadth of these had so increased – and the brain itself was so distended – that they contained about nine pounds of water or three Augsburg wine-measures (so help me God)”

• Diagnosis and treatment still remained a mystery

Symptoms and Signs Infant/Young Child • • • • • • • • • • • • •

Irritability Lethargy Vomiting FTT/Poor feeding Development delay Increasing head circumferences Loss of milestones Tense anterior fontanelle Dilated scalp veins Sun-setting eyes Apneic spells Bradycardia Seizures?

Older Child/Adolescent • • • • • • •

Headache Vomiting/nausea Impaired consciousness Diplopia/6th nerve palsy Impaired upgaze Papilledema Worsening seizure control?

Galen (130-200 A.D.) • Recognized that the ventricles were in communication • Believed that the soul or “animal spirit” contained in the ventricles underwent putrefaction with waste products finding their way through the pituitary body to be discharged from the nose

Thomas Phaire • One of the first books on pediatrics • The Boke of Chyldren (1954) OF SWELLYING OF THE HEAD “Inflation or swelling of the head, cometh of a wyndye matter, gathered betwene the skynne & the flesh, & sometime between the fleshe & the bones of the sculle, the tokes whereof are manifest ynough to the sight, by the swellyng of puffing up, & pressed wt the finger, there remayneth a prynte, whiche is a singe of wynde & vicious humours, ye shalle heale it thus. Remedy First let ye nource auoyde al thinges that engendre wynd, salf or slymy humours, as beanes, peason, eeles, salmon saltfyshe, and lyke: then make a playster to the childes head, after this fashion. Take an handfull of fenell, smallache and dylle, and seeth them in water I a close vessel, afterwarde stampe the, and with a litle cummyne, and oyle of bytter almodes, make it vp, and laye it often to the chyldes head, warme, In defaulte of oyle of almons take gosegrease, addying a ltle vinegre. And it is good to bath the place with a softe coute, or a sponge in the broth of these herbes: Rue, tyme, maioram, hysope, fenell, dylle, cumyne, sal nitre, myntes, radysh rotes, rocket, or some of them, euer takying heede that there droppe no porcion of the medicines in the babes eyes, mouthe, or eares.”

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Whytt (1768) • Clearly defined internal and external hydrocephalus based on an autopsy series of 20 cases • However, thought the cause was an exhalant artery over an absorbent vein.

• Confusion continued until the 19th century and included treatment of HCP with: • • • • •

Bleeding Purging Diuretics Local cephalic applications such as cold, blisters, etc. Head wrapping with plaster or rubber bandages

Early Surgical Attempts • Open Ventricular Drainage • Closed Ventricular Drainage • Ventricular • Spinal

• Third Ventriculostomy (ETV) • Choroid Plexus Cauterization

Closed Ventricular Drainage • End of the 19th century • Fluid was diverted to the subcutaneous, subdural or vascular spaces with some success • Materials utilized included • Glass tubes • Strands of cat gut • Linen – 13 died w/in 36hrs, 5 no improvement, 23 improved • Rubber • Hardened calf veins – 8/15 survived

19th Century Breakthroughs • Magendie • Described the outlet of the 4th ventricle

• Key and Retzius • Identified the correct pathway of CSF flow by dye injection techniques

• Dandy and Blackfan • Created hydrocephalus in dogs by obstruction of the foramina of Monro or cerebral aqueduct and were able to distinguish between obstructive and communicating hydrocephalus with dye studies

Open Ventricular Drainage • Ventricular puncture was reported by the Greeks with head wrapping and injection of astringents into the ventricles with disastrous results. • Practice continued into the latter part of the 19th century and included the use of indwelling setons or collared cannulas for continued drainage. • Repeated lumbar puncture was also introduced • Both ineffective with death from meningitis common.

Closed Spinal Drainage • First lumboperitoneal shunt – 1898 • Removed the arch of L5, pulled the cord aside, and drilled a hole through the body of the vertebra passing a silver wire inferiorly in the thecal sac to the peritoneum

Miculicz’s gold tube

• All 3 cases did poorly and variations were fraught with complications

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ETV Endoscopic Third Ventriculostomy

• Dandy – 1922 • Opened the floor of the 3rd ventricle from a subfrontal approach • Required division of the optic nerve • Modified the technique -lateral subtemporal approach

• Initial results - 1945 • 29 patients > 1 yo • • • •

24 cured 1 died during surgery 3 died later 1 could not be successfully performed

• 63 patients < 1 • 32 died (10 during surgery, 22 later) • 12 cured • 10 lost to follow up

Recently been revived as the long-term complications of shunts have become known.

Success in the Modern Era

Choroid Plexectomy • Lespinasse – 1910 – cauterized • Hildebrande – 1923 - resected

Despite early success ETV fell from favor as success with CSF shunts improved

• 1950’s – Successful Closed CSF drainage due to: • Persistence • Recognition that a one way valve was required • New synthetic materials – silicone • Recognized diversion to different compartments • • • • • •

Abdomen Heart Lung Gallbladder Ureter Venous sinuses

Imaging • HCP has remained a surgical condition with no known effective medical therapy

• Pneumoencephalography

• Treatment involves preventing raised intracranial pressure through diversion of cerebrospinal fluid out of the ventricles of the brain • ETV • Shunt

• Ultrasound

• Need access to the ventricle/subdural space • Can perform intraoperatively • Painful • Can be utilized the first 12-18 months • Fast, easy (bedside) • Reasonable for the lateral ventricle but deficient for the 3rd and 4th ventricles • Quality is user dependent and reproducibility not high

• CT

• Shunt represents a lifelong commitment and should be considered carefully

• • • •

Easy, fast, reliable No sedation required Not sufficient detail Radiation?!?!?!

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CISS/FIESTA • Strong T2/T1 weighted high resolution 3D images • Advantages

• MRI • Single best imaging modality for covering all imaging demands

• Demonstrates the fine anatomic details and membranes within the cisterns • Shows the location, number and extent of membranes

• Anatomic information • Level of obstruction • Shape and size of ventricles • CSF flow dynamics

A MRI Brain Brain imaging w + w/o contrast T2 A X 1/14/2014 4:27:33 4:27:33 P M MR140000799 -----

11/17/2007 6 YEA R M

LO C: 42.95 LOC: THK: 3 SP : 3 HFS

H MRI MRI Brain Brain imaging imaging w w+ + w/o w/o contrast contrast Sag 2 Sag T T2 1/14/2014 1/14/2014 4:47:06 4:47:06 PM PM MR14 000079 9 MR140 000799 -----

11/1 007 1 1/17/2 7/20 07 6Y EA YE AR R M

LO 2 LOC C:: -1.4 -1 .42 THK: 0 T HK: 3 3 SP: SP: 3.3 3 .30 HFS HFS

H MRI Brain imaging w + w/o w/o contras t 33D D FIESTA SA G G intravent cys t 1 /14 /20 14 55:20 :20 :43 PM MR1 40 000 799 MR140 -----

1 1/1 7/20 07 7/2 007 6 YEA YE AR M

LO C: 33.63 .63 T HK: 0.8 0 SP: 00.40 .40 0.80 HFS R

L

8HRBRA I N NEX:1 EC: 1 SE FA FA: 90 TR: 4000 TE: 106.04 A QM: 224\320 P age: 26 of of 49

Z: 1 C: 1250 W: 2501 DFOV DFO V:21x21cm P

IM : 26 SE: 4 I M:

A A

P

P

A M RI Brain Brain imaging imaging w + w/o w/o c ontras t T2 A X 1/14/2014 4:27:33 P M MR140000799 ---

11/17/2007 6 YEA R M

LO LOC : 6.93 THK: 3 SP : 3 HFS H FS

8 HRBRA IN NEX:1

8HRBRA 8 HRBRAIIN N NEX:1 N EX:1 EC E C:: 1 1 SE SE FA FA:: 90 90 TR: T R: 300 3000 0 TE: 2 T E: 113 113.2 .22 AQ M: 192\288 QM: 192\288

Z: Z: 1 C 3 C:: 79 793 W: 6 W: 158 1586 DFO cm DFOV V:1 :19x19 9x19c m

Page: P age: 12 12 of of 2 21 1

F

IIM: 1 M: 12 12 SE: SE: 1 11

R

LL

8HRBRA IN IN NEX:1 EC : 1 SE FA FA: 90 TR: 4000 TE: 106.04 A Q M: 224\320 P age: 13 of of 49

P age: 10 3 of of 20 0

Z: 1 C : 8 167 C: W: 16 334 DFO V :1 8x1 8cm F

IM: 103 SE : 20

Z: 1 C : 1194 W: 2388 D FO V :21x21c m DFO P

I M: 13 SE: 4

A MRI MRI Brain imaging w/o contras t -T 2 T a- fast -T2 fas t as set 1/27/2014 1:52:08 PM MR140001679 MR140001679 ---

11/17/2007 11/17/2007 66 YEA Y EA R M M

E C: 1 GR FA : 6 5 T R: 4 .82 T E: 1 .57 A Q M: 25 6\3 20

Why not just shunt?

LO LOC : 58.67 T HK: 5 SP : 5.50 HFS

R R

L

8HRBRAI N 8HRBRA IN NEX:0.65 NEX:0.65 EC EC: 1 SE SE FA FA : 90 TTR: R: 616.99 TTE: E: 109.20 A AQ QM: M: 128\288 PPage: age: 18 of 31 31

Z: 1 C : 829 W: W: 1659 DFO :25x25cm DFOV V:25x25cm P

IM: I M: 18 SE: 4

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Shunt Placement

Shunt Components A) Proximal Catheter “Ventricular Catheter” B) Valve C) Distal Tubing “Peritoneal Tubing”

Build the Shunt-What Parts?

How do shunts work? Flow or Pressure Regulated

Programmable Shunts Codman Hakim Strata

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Adjusting a “Programmable Valve”

What Pressure? Based on EVD height or standard settings for ICP Infant 60-80 mm H2O Adult 100-120 mm H2O

Why the Craziness??? Seems like a simple procedure

Shunt Failure Rates Why so many shunt operations??? • 40 % of shunts fail by 2 years • 45% of children shunted at < 1yo required revision within 9 months

• 98% fail within 10 years • Average 6.8 shunt revisions in 18 years

Shunt Infection • Rates range from 5-15% • Infection leads to a minimum 2 week inpt stay on Abx • Externalization of shunt • Most infections occur in the first 3 months • Rare after 6 months • Most common pathogen – Gram + • Incidence higher in neonates and children < 1yo

Additonal Shunt Problems • Overdrainage • Subdural hematoma formation

• Repeated episodes of failure leads to • Cognitive decline • Reduced IQ

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Overall yearly cost $2.5 billion dollars

What are we doing to make it better?

O M G

ICP Monitoring Placement Accuracy

Endoscopic Techniques EVD

BOLT

Medtronic Axiom • Magnetic tracking • Metal interferes with system • Two pieces • “Reference" sticks to the head • Pointer "stylet" goes into proximal catheter

J Neurosurg / Volume 113 / December 2010

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Antibiotic Impregnated Catheters • Clindamycin • Rifampin • Typically effective for 4-6 weeks

CURE Children’s Hospital of Uganda • Referral mission hospital for pediatric neurosurgery • Opened in January 2001 • Within the first 26 months after opening • 468 patients underwent surgery for the initial treatment of hydrocephalus • 864 operations for hydrocephalus were performed • After ETV became available it was used exclusively in a prospective manner in all ages and etiologies

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Overall Results

Initial ETV Attempt • 300 patients prior to intention to treat arm • 219 (73%) underwent successful ETV • 55 (18%) ETV was abandoned for VPS • 21 (7%) ETV was abandoned for reservoir placement

• 105 failed • 65 underwent repeat endoscopy • 40 underwent reopening of a closed ETV • 3 died post operatively • 14/37 (38%)remaining had successful 2nd ETV

• 39 underwent VPS • 1 did not return for further management • All presented within 2 months

Is CSF absorption the cause of failure in ETV among infants? • Add bilateral CPC to • Reduce the rate of CSF production • Improve the rates of success in children < 1 yo

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Endoscopic 3rd Ventriculostomy ETV

ETV: J Pediatr 2009:155:254-9

What to do if you suspect shunt malfunction? A AFF

• ABCs!!!!! • CT brain versus Rapid MRI if available • Shunt series

33/8/200 /8 /2 00 4 118 8 DA Y DAY FF

Brain

MRI MRI Brain Brain imaging imaging w/o contras contrastt TRA 0000 0 110 000 90 T RA TSE T SE 550 90 3/26 /20 044 7:10 /2 00 7 :10 :37 :3 7 A M MR04 00003 03 246 MR0 40 24 6 -----

A CT C T Head/Brain w/o w/o contrast contrast HeadSeq s ST HeadSeq 4.8 4 .8 H40 H4 0s 5/1 9/20 10 6:37:59 PM CT 1000032 329948 48 C T100 ----LO : -565 .70 LOC C: -56 5.70 THK: 44.80 .80 HFS HFS

33/8 /8/2004 /200 4 66 YEA R FF

----TTHK: HK: 5.5 0 SP: 66.1 .100 HFS

R R

• Both must be done!!!! • NSG consult

TT2W/50 2W/50 00

L

H H NE X:2 NEX:2 EEC C : 11 SE SE FA: FA : 90 90 TTR: R: 50 000 5 00 TTE E:: 10 0 1 00 ----PPage: age: 13 of 23 23

PPH H

Z: 2 C : 1 36 6 W: W: 2 454 45 4 DFO x233cm cm DFOV V:23 :2 3x2 C Compressed ompres sed 7:1 7:1 I M: 114 4884 4 SE: SE: 401 40 1

R R

L

RD: RD: 199 TTilt: ilt: -5 mA : 35 7 mA: 3 57 KVp: 20 KV p: 1120 A cq no: 6 Acq PPage: age: 14 of 0 of 330

P

Z: Z: 1 C: C : 35 W: W: 80 DFO V:19.9x1 9.9cm V :19.9 x19.9 cm C ompressed 11 :1 Compressed 1 1:1 IM: 4 SE :2 I M: 114 SE:

• Above can be misleading • Slit ventricle syndrome!!

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• Presents with 2 day history of severe headache and multiple rounds of emesis • Brain imaging – stable ventricular size.

A A MRI ontras tt MRI Brain Brain imaging w/o w/o ccontras T2 A-- SSFSE -as set T2 TTA SSFSE-ass et 4/11 /20 14 8:2 4:19 P M MR14000 6728 MR14 00067 28 -----

3 /8/2004 3/8/20 04 1 0 YEA R 10 YE AR FF

• Shunt tapped

LO C : 22.63 LOC 2 2.63 THK: THK: 5 5 SP: SP: 5.50 5.50 HFS HFS

R R

L

8 HRBRA IN 8HRBRA IN NEX:0.56 NEX:0.56 E C:: 1 EC SE SE FA : 90 FA: 90 TTR: R: 12 78.42 1278 .4 2 TTE: E: 10 9.62 109.62 A M:: 2 56\2 56 AQ QM 256 \256 PPage: age: 18 of of 31 31

• Opening pressure of 30!!!!

Z: Z: 2 2 C: C : 1310 13 10 W: W: 2621 26 21 DFO V :25x25c DFOV :25x25cm m PP

Success of Treatment

IM: 8 SE: IM : 1 18 SE : 4 4

Questions?

• How should we measure? • • • • •

Successful catheter placement No postoperative complications Relief of raised ICP Lack of shunt revisions/infection QOL measures

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