2012 hydrocephalus association conference complex ... · hydrocephalus! marion l. walker, md!...
TRANSCRIPT
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Complex Hydrocephalus
Marion L. Walker, MD Professor of Neurosurgery & Pediatrics
Primary Children’s Medical Center University of Utah Salt Lake City, UT
2012 Hydrocephalus Association Conference Washington, DC - June 27-July1, 2012
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Complex Hydrocephalus�
Difficult Problems Often Requiring Multiple Shunts�
! Slit Ventricle Syndrome�
! Dandy-Walker Syndrome�
! Multiloculated Hydrocephalus�
! Septal Fenestration �
! Avoiding an additional shunt �
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Complex Hydrocephalus�
Slit Ventricle Syndrome�
! Headaches�
! Varying degrees of lethargy�
! +/- nausea/vomiting �
Intermittent symptoms of shunt malfunction in a child who appears otherwise healthy�
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Complex Hydrocephalus�
Pathophysiology of� Slit Ventricle Syndrome�
! Shunt over drainage of CSF�
! Growing brain fills the intracranial space�
! a fixed skull filled with brain parenchyma, blood, meninges, vasculature and only small amounts of CSF�
! Loss of normal intracranial compensatory mechanisms�
Complex Hydrocephalus�
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! SVS is a phenomenon occurring in children.�
! Adults do not get SVS.�
Pathophysiology of� Slit Ventricle Syndrome�
Complex Hydrocephalus�
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Complex Hydrocephalus�
Treatment of the�Slit Ventricle Syndrome�
! Observation �
! Limit over drainage�
! Antimigrainous therapy�
! ICP monitoring & EVD placement �
• Shunt revision
• Subtemporal decompression
• Cranial morcellation
• Third ventriculostomy
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Complex Hydrocephalus�
Slit Ventricle Syndrome�Signs & Symptoms�
! Overdrainage�
! Positional headaches�
! Relief by lying down �
! Poor tolerance of school�
! May be asymptomatic for prolonged periods�
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! Slit ventricles are relatively common in patients shunted in childhood.�
! SVS is not as common and occurs only in patients shunted in childhood.�
! Treatment options include observation, medication, shunt revision and third ventriculostomy.�
! No specific treatment or shunt type has been shown to be the best option.�
Slit Ventricle Syndrome Conclusions
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Dandy Walker Syndrome�
! Dandy-Walker Syndrome (DWS) is determined by three findings: �
1. cystic dilatation of the fourth ventricle�
2. total or partial aplasia of the cerebellar vermis�
3. supratentorial hydrocephalus�
Complex Hydrocephalus�
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Dandy Walker Syndrome�
! Dandy-Walker Variant (DWV): �1. the floor & lateral walls of
the 4th ventricle are visible�2. the vermis is hypoplastic�
Complex Hydrocephalus�
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Complex Hydrocephalus�
Differential Diagnosis�! DWS must be differentiated from an arachnoid cyst of the posterior fossa�
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Complex Hydrocephalus�
Pathogenesis�! DWS develops at about the fourth
week of gestation �
! Associated with other anomalies: �
! partial or total agenesis of the corpus callosum�
! many genetic disorders�
! facial�
! cardiac�
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Complex Hydrocephalus�
Incidence of�Dandy Walker Syndrome�
! 1:25,000 - 35,000 births�
! Female:Male 1.3:1 �
! 80% diagnosed before 1 year of age�
! Dandy Walker Variant may be an incidental finding �
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Complex Hydrocephalus�
Incidence of Hydrocephalus�
! DWS occurs in 2-4% of cases of hydrocephalus�
! 90% of cases of DWS have enlarged ventricles and/or hydrocephalus�
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Complex Hydrocephalus�
Associated Anomalies�
! CNS anomalies occur approximately 70%�
! agenesis of the corpus callosum (40%)�
! encephaloceles (17%)�
! heterotopias�
! aqueductal stenosis�
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Complex Hydrocephalus�
Associated Anomalies�! Non-CNS anomalies include: �
! Cardiac�! ventricular septal defects�! patent ductus arteriosus�! arterial septal defects�
! External defects often associated with genetic disorders�
! trisomies 18, 21, and 13�! <2% recurrence risk �
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Complex Hydrocephalus�
Signs & Symptoms�
! Macrosomia (C-section rate 26%)�
! Delayed motor development �
! Cognitive dysfunction 40-70%�
! Focal motor findings very low �
! Almost no cerebellar dysfunction �
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Imaging Studies�CT Scan � MRI �
Complex Hydrocephalus�
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Complex Hydrocephalus�
Surgical Treatment �
! Cyst removal�
! failure rate >75%�
! Shunting for hydrocephalus�
! cyst?�
! ventricle?�
! both? �
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Multiloculated Hydrocephalus�Complex Hydrocephalus�
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! Some form of guidance is necessary�
! ultrasound �
! stereotaxic frame�
! frameless�
Multiloculated Hydrocephalus�
Complex Hydrocephalus�
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Complex Hydrocephalus�
! Often seen in patients with post-hemorrhagic or post-infectious hydrocephalus.�
! Often have required multiple shunts unless endoscopic techniques are successful.�
Multiloculated Hydrocephalus�
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• The most difficult cases! �
• Many technical difficulties�
• The poorest outcomes�
• 45% successful shunt avoidance�
Multiloculated Hydrocephalus�
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Frameless Stereotaxy�
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Maintain Intracranial Orientation �
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Complex Hydrocephalus�
! Use guidance techniques liberally�
! Ultrasound�
! Stereotaxis�
Maintain Intracranial Orientation �
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Frameless Stereotaxy�
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Complex Hydrocephalus�
Isolated Lateral Ventricular Hydrocephalus �
• Etiology: �• Neoplastic: e.g. hypothalamic glioma�
• Congenital: e.g. atresia of Foramen of Monroe�
• Benign lesions: choroid plexus cyst or hypertrophy �
• Post infectious or hemorrhagic scarring �
• Iatrogenic: unilateral shunt overdrainage�
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! Clinical signs of increased ICP�
! Radiographic: Ventricular asymmetry + �
! Non-communication of intraventricular contrast �
! Progressive monoventricular enlargement �
! Known mass obstructing Foramen of Monroe�
Isolated Lateral Ventricular Hydrocephalus �
Complex Hydrocephalus�
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Complex Hydrocephalus�
Treatment Outcome�! Success: �
! On last follow-up: Absence of ILVH �
! Failure: �
! Recurrence of ILVH symptoms & radiographic evidence�
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Total Successes: 26� Total Failures: 6�n=32�
Patients re-operated�1 �
Patients not re-operated�1 �
3rd septostomy�
Success�1 �
Patients not re-operated�5�
Patients re-operated�10�
2nd septostomy�
Success�8�
Failure�2�
First septostomy�
Success�17�
Failure�15�
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Complex Hydrocephalus�
Success Rates�! All patients: " 81.2%
(27/32)�
! All septostomies: 60.5% (26/43)�
! First septostomies: 53.1% (17/32)�
! Redo patients: " 90% (9/10)�
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Septostomy: Survival Curve�
Prop
ortion
fai
lure
-fre
e�
Months follow-up �
*No failures occurred after 6 months�
* �
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Drake, et al. Neurosurgery 43: 294-305, 1998�
Septostomy vs. VP Shunt �
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Complex Hydrocephalus�
• Endoscopic septostomy provided long term ILVH relief in 81% of patients
• After 6 months post-op, septostomy failures not observed
• 2 or more prior shunt procedures negatively affects outcome
• Good results possible in redo septostomies
• A reasonable alternative to CSF shunting in cases of isolated lateral ventricle hydrocephalus
Conclusions
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Complex Hydrocephalus�
Avoid That Second Shunt! �
! Communicate intraventricular cysts�
! Communicate through the septum pellucidum for isolated ventricles or unilateral hydrocephalus�
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Complex Hydrocephalus�
Complex Hydrocephalus Conclusions�
! Management decisions for difficult shunt problems are best approached in a systematic manner.�
! Successful treatment of the slit ventricle syndrome is often accomplished by conservative management.�
! Several surgical attempts may be necessary in order to avoid additional shunts in patients with loculated ventricles.�