Hydrocephalus Definition •Hydrocephalus – from the Greek words „hydro“ meaning water and „cephalus“ meaning head. •Hydrocephalus can be defined broadly as a disturbance of formation, flow, or absorption of cerebrospinal fluid (CSF) that leads to an increase in volume occupied by this fluid in the CNS. •This condition also could be termed a hydrodynamic disorder of CSF. •Cerebral atrophy and focal destructive lesions - are not the result of a hydrodynamic disorder. Anatomy PE-AnatBrainFig7 ventricles_comp_web-440x569 Anatomy Physiology •CSF is formed by the choroid plexus (~80%), the parenchyma (~20%) and the ependyma (negligible). •CSF 120 ml (80-150). •500 ml /24 hours. •CSF production is pressure independent under normal physiological conditions. • f15-8b_production_and_c_c Physiology •The only proven force responsible for bulk CSF absorption is that of a hydrostatic gradient. •The arachnoid villi drain CSF. •Resorption is dependent on ICP. •Accessory routes: the mucosa of the paranasal sinuses, nasal mucosa, cranial nerve root sheaths, and cervical lymph nodes. fp3-7 normal archnoid villi Physiology Circulation of CSF 14_04d afp20040915p1071-f1 Epidemiology Estimated prevalence: 1-1,5%. Incidence of congenital hydrocephalus 0,5-2,5/1000 births. about_cases 92bb1dc83f3c9c616c970af924ede5_gallery alg_baby_klaus Epidemiology Some common causes of pediatric hydrocephalus: –myelomeningocele (20%) –post-intraventricular hemorrhage of prematurity (15%) –congenital aqueductal stenosis (12%) –communicating hydrocephalus of unknown origin (10%) –neoplasm (8%) –post-infectious (7%) –post-traumatic (3%) –arachnoid cyst (5%) –dural sinus thrombosis –syndrome-related (Dandy-Walker malformation etc.) Epidemiology Causes of hydrocephalus in adults: –subarachnoid hemorrhage (SAH) (one third) –idiopathic hydrocephalus (one third) –head injury –tumors –prior posterior fossa surgery may cause hydrocephalus by blocking normal pathways of CSF flow. –congenital aqueductal stenosis causes hydrocephalus but may not be symptomatic until adulthood. –meningitis, especially bacterial. Symptoms Influenced by: –patient's age –cause –location of obstruction –luration and rapidity of onset –vary greatly from person to person Symptoms - infants •abnormal enlargement of the head •soft spot (fontanel) is tense and bulging •scalp can appear thin •bones separated in baby's head •prominent scalp veins •vomiting •drowsiness •irritability •downward deviation of baby's eyes •seizures •poor appetite. infant Symptoms – toddlers/children •abnormal enlargement of baby's head •headache •nausea, vomiting •blurred or double vision •unstable balance •irritability •sleepiness •delayed progress in walking or talking •poor coordination •change in personality •inability to concentrate •loss of sensory motor functions •seizures •poor appetite •Older children may experience difficulty in remaining awake or waking up. toddlers Symptoms – adults •headache •difficulty in remaining awake or waking up •loss of coordination or balance •bladder control problems •impaired vision and cognitive skills that may affect job performance and personal skills. headache Symptoms – older adults •loss of coordination or balance •shuffling gait •memory loss •headache •bladder control problems olderadults Imaging studies •MRI •CT •X-ray (plain, shunt-o-gram) •SPECT •PET •Ultrasonography (infants) •(Radionuclide cisternography) 12724 Imaging studies - MR • 1071_f2b Mala_T1_sag Mala_T2_sag T2W - axial T1W - sagittal T2W - sagittal Imaging studies - MR Cine MR PCA1 PCA2 Imaging studies - MR Cine MR Imaging studies - MR Prenatal MR zj40090601630013 3 Imaging studies – MR spectroscopy • Imaging studies - CT Vesely_CT Ventricular indexes Evan's (less than 0,3) DVD_tab Imaging studies – X-ray Mala1 Mala3 Bartos3 Bartos4 Imaging studies – X-ray • Bartos5 V-A shunt V-P shunt Imaging studies – X-ray • • LP shunt Imaging studies – X-ray • • Shunt-o-gram Imaging studies - ultrasonography • hydrocephalus-4a advance_tech_thumb_4d8c_vocalhybrd_l Imaging studies - ultrasonography • Sophia%2029weeks s213 Fetus ultrasonography, 3D reconstruction Imaging studies – SPECT • Imaging studies – PET Schulthness-C2I2-1 Schulthness-C2I2-2 Imaging studies – SPECT, PET • • Classification •Communicating •Non-communicating (obstructive) • •Congenital •Aquire • •Pediatric •Adult • •Special types of HCP –Normal pressure hydrocephalus –Hydrocephalus ex vacuo –Others • •Acute hydrocephalus occurs over days, •subacute hydrocephalus occurs over weeks, •chronic hydrocephalus occurs over months or years. • st031053 Communicating hydrocephalus Communicating hydrocephalus occurs when the flow of CSF is blocked after it exits the ventricles. This form is called communicating because the CSF can still flow between the ventricles, which remain open. 1071_f2b Mala_T1_sag Mala_T2_sag Communicating hydrocephalus •Increased CSF production (rarely) •Decreased CSF resorption (most often) –posthemorrhagic (SAH, ICH) –postinfectious –tumors –dural sinus thromosis –idiopathic Communicating hydrocephalus Increased CSF production (plexus papiloma) CHOROID PLEXUS PAPILLOMA 1-2 CHOROID PLEXUS PAPILLOMA 3 CHOROID PLEXUS PAPILLOMA 2 choroid plexus papilloma choroid plexus papilloma Communicating hydrocephalus Decreased CSF resorption (SAH) Ct2 Ct4 Obstructive hydrocephalus Noncommunicating hydrocephalus, also called obstructive hydrocephalus is caused by blockage in the ventricular pathways in the brain through which cerebrospinal fluid flows. Aqueductal stenosis (most common) Hydrocephalus_05 Obstructive hydrocephalus Obstructed CSF circulation –within the ventricular system (lateral ventricle) postinfectious crypto 2 crypto 7 crypto 11JPG crypto 9 Obstructive hydrocephalus Obstructed CSF circulation –within the ventricular system (foramen Monroi) colloid cyst colloid cyst 2 colloid cyst 1 colloid cyst 3 Obstructive hydrocephalus Obstructed CSF circulation –within the ventricular system (3rd ventricle) suprasellar germinoma sagT1FS2 sagT1FS sag T1FS+Gd3 sag T1FS+Gd Obstructive hydrocephalus Obstructed CSF circulation –within the ventricular system (4rd ventricle) intracerebelar hemorrhage ependymoma HTNbleed_cerebellum_2 ependymomaaxialt1postgad.jpg ependymomaaxialT2.jpg Pediatric hydrocephalus Congenital hydrocephalus: incidence 0,5-2,5/1000 birth. –aqueductal stenosis –Dandy-Walker malformation –Chiari malformation –postinfectious –NTD (myeloceles, meningomyeloceles) –vein of Galen malformation –holoprosencephaly –neurofibromatosis –idiopathic –x-linked hydrocephalus (due to aqeuductal stenosis) –autosomal recesive hydrocephalus (rare) – – – Dandy-Walker malformation Rare congenital malformation. Agenesis or hypoplasia of the cerebellar vermis + cystic dilatation of the fourth ventricle + enlargement of the posterior fossa. • 336139-408059-4383DWMsono 336139-408059-5277DWM sono Dandy-Walker malformation Posterior fossa cystic malformations have been divided into: –Dandy-Walker complex •Dandy-Walker malformation •Dandy-Walker variant •mega cisterna magna –Posterior fossa arachnoid cyst. 336139-408059-5276DWM in utero Dandy-Walker malformation Agenesis or hypoplasia of the cerebellar vermis + cystic dilatation of the fourth ventricle + enlargement of the posterior fossa. 336139-408059-2370_DWMT2 336139-408059-2367_danda walker malf 336139-408059-2378_DWM s ganezeí corpus callosum Dandy-Walker variant Dandy-Walker variant consists of vermian hypoplasia and cystic dilatation of the fourth ventricle, without enlargement of the posterior fossa. • 336139-408059-2379_DandyWalker variant Mega cisterna magna Mega cisterna magna consists of an enlarged posterior fossa secondary to an enlarged cisterna magna, with a normal cerebellar vermis and fourth ventricle. • 336139-408059-2391_megacistrna magna DW – arachnoid cyst •Retrocerebellar arachnoid cysts of developmental origin are uncommon but clinically important. •True retrocerebellar arachnoid cysts displace the fourth ventricle and cerebellum anteriorly and show significant mass effect. •Because there are different surgical therapy approaches for posterior fossa arachnoid cyst and Dandy-Walker malformation, it is essential to differentiate between the 2 entities. 336139-408059-2364_arachnoidální cysta zadní jámy Aneurysm of the vein of Galen • Vein_of_Galen_diagram vein_of_galen_3d_2 VEIN-OF-%20GALEN-aneurysm-1c vein_of_galen_sag_mip Aneurysm of the vein of Galen Chiari malformation Herniation of rhombencephalonic derivatives. Chiari I Caudal migration of the cerebellar tonsils through the foramen magnum, usually greater than 5 mm. Manifestation in adults. Syringomyelia (A-yes, B-no). 08-3_ACM PE-chiariFig3 Chiari II •Chiari type II malformation is less common and more severe. •Symptomatic in infancy or early childhood. •Almost invariably associated with myelomeningocele and hydrocephalus. •Its hallmark is caudal displacement of lower brainstem (medulla, pons, 4th ventricle) through the foramen magnum. •Symptoms arise from dysfunction of brainstem and lower cranial nerves. CHIARI%20II%20MALFORMATION%20a gr4-midi_chiari2 gr8-midi_chiari2 Chiari III Type III malformation refers to herniation of cerebellum into a high cervical myelomeningocele. Exceedingly rare and incompatible with life. Chiari IV Cerebellar hypoplasia gr1-midi_Chiari IV gr2-midi_chairiIV Meningomyelocele (NTD) •Protrusion of the membranes that cover the spine but some of the spinal cord itself through a defect in the bony encasement of the vertebral column. •Infants with MMC are at risk for bacterial meningitis due to the spinal defect. •Leak of cerebrospinal fluid (CSF) leak is commonly observed. •The major indication for early operative repair (within 48h of delivery) is prevention of infection. •Hydrophalus in 60%-90%. meni Meningomyelocele Posthemorrhagic HCP in preterm infants Occasionally occurs antenatally 1st day 50%, 2nd day 25%, 3rd day 15%, > 4th day 10%. Germinal matrix – subependymal, source of neural precursors, capillars – vascular end zone of arterial supply. Hematoma – rupture of fragile capillaries. Posthemorrhagic HCP in preterm infants Papile classification: • –Grade I – subependymal hemorrhage –Grade II – intraventricular hemorrhage without ventricular dilatation –grade III – intraventricular hemorrhage with ventricular dilatation –grade IV – intraventricular hemorrhage with parenchymal hemorrhage • Posthemorrhagic HCP in preterm infants 973235-976654-148 No hemorrhage Posthemorrhagic HCP in preterm infants 973235-976654-436 Grade I Posthemorrhagic HCP in preterm infants 973235-976654-476 Grade II Posthemorrhagic HCP in preterm infants 973235-976654-503 Grade III Posthemorrhagic HCP in preterm infants 973235-976654-524 Grade IV Posthemorrhagic HCP in preterm infants Neuropathological consequences: –Germinal matrix destruction –Periventricula hemorrhagic infarct 15% –Posthemorrhagic hydrocephalus 30% – Posthemorrhagic HCP in preterm infants Posthemorrhagic hydrocephalus Posthemorrhagic HCP in preterm infants •Neuropathological leukomalacia – bilateral, non-hemorrhagic ischemic white matter injury. •Pontine neuronal necrosis – 46% to 71% of infants with IVH exhibited pontine neuronal necrosis. • Posthemorrhagic HCP in preterm infants Periventricular leukomalacia Posthemorrhagic HCP in preterm infants • Benign external hydrocephalus •Infants with rapidly enlarging heads. •CT scan – widening of the subarachnoid space with mild or no ventricular dilatation •Age-related self-limited condition occuring in infants with open cranial sutures. •Usually resolves without intervention by 2-3 years of age. Multilocular hydrocephalus •Hydrocephalus arising from intraventricular septations. •Presence of multiple cysts inside the ventricles. •Complication of neonatal hydrocephalus. •Causes: preterm infant with intracranial hemorrhage grade II-III or central nervous system infection. •Requires a specific therapeutic approach. • Multilocular hydrocephalus • Multilocular hydrocephalus Postoperative exam – all parts communicate Adult hydrocephalus Hydrocephalus which occurs in an adult patients. –aquired, congenital or idiopathic –posthemorrhagic (SAH) –aqueductal stenosis –trauma –tumor –postinfectious –special forms •normal pressure hydrocephalus •others Posthemorrhagic hydrocephalus Adults –Subarachnoid hemorrhage (SAH) –Intraventricular hemorrhage (IVH) –Intraparenchymal hemorrhage (IPH) – • 000004_text HTNbleed_basalganglia sah1 Posthemorrhagic hydrocephalus •After SAH – 2.3 to 63.4% •STICH – 42% with IPH had IVH – 55% of IVH hydrocephalus – HCP in 23,1 % of all IPH •Hemorrhage-related hydrocephalus is transient and patients lose their shunt dependency • Posttraumatic hydrocephalus •Posttraumatic hydrocephalus is an active and progressive process of excessive cerebrospinal fluid (CSF) accumulation due to liquorodynamic disturbances following craniocerebral injury. •The incidence of PTH ranges from 0.7-86% (differences in diagnostic criteria and classification). •Risk of developing PTH: coma, increased age, decompressive craniectomy and subarachnoid hemorrhage. •Cave – brain atrophy (hydrocephalus ex-vacuo). Posttraumatic hydrocephalus • jns10132f21 Adult hydrocephalus - SHYMA •SHYMA - the syndrome of hydrocephalus in young and middle-aged adults. •Acquired communicating adult-onset hydrocephalus with mild ventricular enlargement and otherwise normal cerebral cortex and white matter. •Symptoms: headache, subtle gait disturbance, urinary frequency, visual disturbances and some level of impaired cognitive skills that can noticeably affect job performance and personal relationships. •Discrepancy between the prominence of symptoms and the subtlety of clinical signs. •The degree of symptoms and their resultant effect varies widely among patients. •Often remain undiagnosed and untreated. • gr1-midi gr2-midi gr3-midi Normal pressure hydrocephalus •Hakim S., Adams R.D. 1964, 1965* • •Normal pressure hydrocephalus - Adam's trias (gait and/or balance impairments, disturbances in cognition a control of urination) with ventricular enlargement and normal ICP (60-240 mm H20). • •Idiopathic (primary) – unknown cause (INPH) • •Secondary (SNPH) – known cause (trauma, subarachnoid hemorrhage, stroke etc.) • • • • *Adams RD, Fisher CM, Hakim S, Ojemann RG, Sweet WH: Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure: A treatable syndrome. N Engl J Med 273:117–126, 1965. • *Hakim S: Some observations on CSF pressure: Hydrocephalic syndrome in adults with “normal” CSF pressure. Bogata, Javeriana University School of Medicine, 1964 (Thesis No. 957). • *Hakim S, Adams RD: The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 2:307–327, 1965. • • INPH_guidelines Normal pressure hydrocephalus Pathophysiology: aberrations in CSF flow dynamics –transmantle pressure gradient –a water-hammer effect that enlarges the ventricles –abnormalities of ventricular wall compliance may contribute to ventricular dilatation –periventricular ischemia • Normal pressure hydrocephalus Patient and family member Adam´s trias –Gait disturbances –Cognitive impairment –Urinary inkontinence – • • • Gehstoerung2web wasserhahn-neuweb Demenzneuweb Normal pressure hydrocephalus •„10 m walk test“ –standard – time < 9 sekund, number of steps < 18 •Videoanalysis –Decreased step height –Decreased step length –Decreased cadence (speed of walking) –Increased trunk sway during walking –Widened standing base –Toes turned outward on walking –Retropulsion (spontaneous or provoked) –En bloc turning (turning requiring three or more steps for 180 degress) –Impaired walking balance, as evidenced by two or more corrections out of eight steps on tandem gait testing •EMG/MEP: –Hyperactivity of antagonists and antigravity musles –No pyramidal tract involvement – extrapyramidal subcortical dysfunction –Pyramidal tracts signs : clinically unfavourable signs – Normal pressure hydrocephalus Impairment of cognition –psychomotor slowing /increased response latency/ –decreased fine motor speed –decreased fine motor accuracy –difficulty diving or maintaining attention –impaired recall, espacially for recent events –executive dysfunction –behavioral or personality changes • Normal pressure hydrocephalus •Urinary incontinece –Urological examination –Episodic or persistent urinary incontinence not attributable to primary urological disordes; persistent urinary incontinence; urinary and fecal incontinence –Urinary urgency as defined by frequent perception of a pressing need to void; urinary frequency as defined by more than six voiding episodes in an average 12-hour period despite normal fluid intake; nocturia as defined by the need to urinate more than two times in an average night – • BHP Deloha Normal pressure hydrocephalus •Imaging studies •ventricular enlargement (Evan´s index > 0,3) •no macroscopic obstruction to CSF flow •at least one of the following supportive features –enlargement of the temporal horns of the lateral ventricles –callosal angle of 40 degrees or more –periventricular signal changes (no ischemic changes or demyelination) –An aqueductal or fourth ventricular flow void on MRI •Other brain imaging findings –A brain imaging study performed before onset of symptoms showing smaller ventricular size or without evidence of HCP –Cine MRI study or other technique showing increased ventricular flow rate –A SPECT-acetazolamide challenge showing decreased periventricular perfusion that is not altered by acetazolamide – Normal pressure hydrocephalus –Neurodegenerative disorders •Alzeimer's disease •Parkinson's disease •Huntingtonova choroba •Lewy body disease •Frontotemporal demetia •Corticobasal degeneration •Progressive supranuclear palsy •Multisystem atrophy •Spongiform encephalopathy –Vascular dementia •Cerebrovascular disease •Stroke •Multi-infarct state •Binswanger's disease •Cerebral autosomal dominant arteriopathy, subcorical infarcts, and leukoencephalopathy •Vertebrobasilar insufficiency –Infectious disease •Lyme •HIV •syphylis • –Urological disorders •Urinary tract infection •Bladder or prostate cancer •Benign prostatic enlargement –Miscellaneous •B12 deficiency •Collagen vascular disorders •Epilepsy •Depression •Traumatic brain injuries •Spinal stenosis •Chiari malformation •Wernicke's encephalopathy •Carcinomatous meningitis •Spinal cord tumor • • petnormalhi petadhi Differential diagnosis Normal pressure hydrocephalus •Supplemental prognostic tests •„shunt-responsive“ versus „shunt-nonresponsive“ • •„tap test“ (withdraw 40 to 50 ml, lesser volumes (25 ml or less) have low sensitivity, good sensitivity, low specifity) • •CSF outflow resistance via an infusion test (PPV of 75% to 92%) • •External lumbar drenaige (Haan, Thomeer 1988, 10 ml per hour for 3 days) (PPV of 80 to 100%) • Tap_tab ELD_tab Ro_tab Liquorguard investigation Supplemental prognostic tests •ICP monitoring –Increased frequency in B waves is indicative of lowered compliance and/or may play an important role in the pathophysiology of the ventriculomegaly nad neuronal dysfunction static ICP values versus dynamic (pulsatile) ICP values •(Radionuclide cisternography) Identifying „shunt-responosive“ patients •Correlation between clinical symptoms and CT/MRI exams. •Resistance testing (can be repeated and compared with testing before) •ELD •Tap test (single application, repeated) Management Aqueductal stenosis •Symptomps are similar to normal pressure hydrocephalus. •But treatment different – 3rd ventriculostomy. LOVA •LOVA = longstanding overt ventriculomegaly in adult •subtype of chronic hydrocephalus •develops during childhood, manifests symptoms during aduldhood •diagnostic criteria: –severe ventriculomegaly –macrocephaly measuring more than two standard deviations in head circumference –and/or neuroradiological evidence of a significantly expanded or destroyed sella turcica. •symptomps: headache and/or Adam‘s trias •caused by aqueductal stenosis •treatment is difficult in terms of their sensitive compliance of brain parenchyma •surgery: ETV, shunts (cave - over-drainage). InfinOH •InfinOH = infratentorial intracisternal obstructive hydrocephalus •Subtype of communicating hydrocephalus with free communication between the ventricles and subarachnoid space but with infratentorial intracisternal obstruction •MRI – a downward bulged floor of the third ventricle and a discrepancy of sizes between the great and the prepontine cistern. •Treatment : ETV, shunt. Arrested (compensated) hydrocephalus •The condition in which the neurological status of the patient is stable in the presence of stable ventriculomegaly. •CSF formation and absorption are in balance and no CSF accumulates. •Spontaenous or surgical termination of a hydrocephalic condition with subsequent return to normal of the pressure across the cerebral mantle. •Cave – subtle deterioration. •?? Probably results from improvement of CSF circulation during growth. • Hydrocephalus treatment •Observation (asymptomatic) •Conservative treatment (acetazolamide, furosemide, osmotic agents) •Surgery –Temporary (acute HCP) •External ventricular drainage •External lumbar drainage –Permanent (chronic HCP) •Shunt •Neuroendoscopy •Others (Torkildsen drainage etc.) • •Pediatric hydrocephalus •Adult hydrocephalus exacta External ventricular drainage •allows the temporary drainage of CSF –to relieve raised intracranial pressure –to divert infected CSF –to divert bloodstained CSF following neurosurgery/haemorrhage –to divert the flow of CSF •indications: –to relieve raised intracranial pressure –to divert infected CSF –to divert bloodstained CSF following neurosurgery/haemorrhage –to divert the flow of CSF •catheter is placed into the lateral ventricle through a burr hole made in the skull, tunnelled under the skin and connects to an external drainage system – – – • • EVD_appendix1 External ventricular drainage •Spiegelberg Silverline –Silver ions – strong antiseptic effects with a broad spectrum. –Continual release of silver within 30 days in antiseptic concentration. •Bactiseal catheter (antibiotic impregnated) • • • silverline_final_01_s bactiseal2 External ventricular drainage •Special thick ELD •Indication: –hemocephalus Medtronic External lumbar drainage •Indications: similar to external lumbar drainage, but not in case of obstructive hydrocephalus. •SAH, wound complications Neuroendoscopy Endoscopes –Rigid – – –Flexible The 2.5mm x 100cm fiberscope. The 2.5mm x 100cm fiberscope. The standard endoscope (above) and Hopkins telescope design (below). The telescope housed within the Taylor operating sheath. Comparison between an exam/protection sheath and an operating sheath with an instrument channel. Comparison between 0o and 300 telescopes. 2.7mm telescope and 14.5 Fr Taylor operating sheath. Neuroendoscopy Light sources, cameras (full-HD), recording devices The new AIDA system. Cameras. Neuroendoscopy Instruments 3628419295_567224_567224_1_rdax_385x251 3628419295_564644_564644_1_rdax_385x264 3628419295_567223_567223_1_rdax_242x350 Neuroendoscopy Holders Neuroendoscopy •shuntscope •balloon catheter •introducer 3628419295_450680_450680_1_rdax_385x247 MEDIA NEURO 12105 The New Shuntscope The New Shuntscope Image 01 Endoscopic treatment of HCP •Endoscopic third ventriculostomy •Aqueductoplasty •Septum pellucidum fenestration •Fenestration of multiloculated ventricles •Foraminoplasty •Lamina terminalis fenestration Endoscopic third ventriculostomy •Indication – non-communicating HCP –LOVA: Long-standing Overt Ventriculomegaly in Adults (Oi, 2000) –InfinOH: Infratentorial Intracisternal Obstructive Hydrocephalus (Kehler) –unusual/controversial indications: •Empty sella syndrom •Posthaemorrhagic hydrocephalus •Postinfectious hydrocephalus •Chiari malformatin •Dandy-Walker malformation •Previous V-P shunt implantation • • • Endoscopic third ventriculostomy • • • mangum12 Endoscopic third ventriculostomy • • • Endoscopic third ventriculostomy 60-90% succes rate Outcome depends on etiology – the worst results: •postinfectious hydrocephalus •posthaemorrhagic hydrocephalus •childern under 6 month Endoscopic third ventriculostomy Patients with shunt dysfunction - always check the possibility of endoscopic treatment Shunt failfure – MRI – if obstruction – ETV, if not – shunt revision Endoscopic third ventriculostomy Patency of the stoma on the floor of the third ventricle – MRI – phase contrast Present in 94% of successful cases, absent in 75% of the failures. Cave – late failure of the stoma. • Endoscopic third ventriculostomy Indication of repeated ETV: –Clinical improvement after primary ETV –Functional stoma proved on MRI –Clinical deterioration –Non-functional stomaon MRI in time of deterioration • Endoscopic third ventriculostomy Free-hand technique x holder Frame-less navigation • • clip_image004_001 clip_image012 Aqueductoplasty Indication: –Isolated 4th ventricle –Membrane in aqueduct – • Aqueductoplasty Aqueductoplasty with stenting Indication: –High risk of restenosis – • Aqueductoplasty Aqueductoplasty retrograde Indication: –Isolated 4th ventricle with slit ventricle syndrom – • Endoscopic treatment of HCP Fenestration of multiloculated ventricles Indication –postinfectious or poshaemorrhagic sepatation inside the ventricles Frame-less navigation Ultrasound perioperative control Endoscopic treatment of HCP Septum pellucidum fenestration Indication: –Unilateral hydrocephalus –„secure procedure“ in cases of unilateral ventricular laesinons Endoscopic treatment of HCP Foraminoplasty –Indication: •Obstruction of foramen Monro Endoscopic treatment of HCP Lamina terminalis fenestration: –Indication: •If 3rd ventriculostomy cannot be performed Endoscopic treatment of HCP Colloid cyst – intermitent non-communicating hydrocephalus Endoscopic treatment of HCP Complications: –Bleeding (minor, major – can cause neurological deficit) –CSF leak (CSF fistula, pseudomeningocele) –Infection (contaminated endoscope, consequence of CSF leak) –Ventricular collapse (subdural hematoma) –Injury to brain structures (neurological deficit, death) –Bradykarida, hypertension – elevation of ICP (to rapid irrigation etc.) – • Shunts •Ventriculo-peritoneal •Ventriculo-atrial •Lumbo-peritoneal •others : –Ventriculo-subgaleal –Superior sagital sinus –Ventriculo-pleural –Renal pelvis –Ventriculo-urethral –Ventriculo-vesical –Ventriculo-gastric –Bile ducts –Small intestine –Oviduct –Ventriculomastoidostomy –Bone marrow of the vertebra –Spinal epidural space – – – • ableitung_p ableitung_a ableitung_l Valves •Differential-pressure valves –static (monopressure) –adjustable •Hydrostatic valves (function of the valve depends on the change of physical parameters) –Flow regulating valves –Valves with ASD (anti-siphon device) –Gravity assisted valves – – Valves •~ 20 manufacturers •~ 130 different valves •~ 450 different pressure setting • CHHABRA_SLIT_N_SPRING_HYDROCEPHALUS_SHUNT_SYSTEM_with_LARGE_FLUSHING_RESERVOIR Chhabra shunt, low cost ~ 25 €, India Valves • Valves •Differential-pressure valves –static (monopressure) •The most common pressure ratings are: –Extra-low-pressure: 0-10 mm H2O –Low-pressure: 10-50 mm H2O –Medium-pressure: 51-100 mm H2O –High-pressure: 101-200 mm H2O – – csfflow cvlvcut Valves – – csflwpl Mini-liegoffen Valves •Differential-pressure valves –adjustable – proGAVFoto_transp Valves – – Messstift-frontal Verstellstift-frontal Valves – – Valves – – Chpv3 Chpv2 Valves • nph_strata Valves • Valves Hydrostatic valves (function of the valve depends on the change of physical parameters) –Flow regulating valves – osvII • OSV01 OSV02 OSV03 OSV04 OSV05 Valves Hydrostatic valves –valves with ASD (anti-siphon device) – on/off – siphon2 Valves Hydrostatic valves –gravity assisted valves – Introduction of a shunt •Careful planning •Patient characteristics : CSF–free of blood, infection; skin–healthy, free of infection •Appropriate ventricular access–larger ventricle, shortest path, catheter away from choroid plexus, guidance (ultrasound, image guidance available if needed). Introduction of a shunt •Maximum vigilance should be dedicated to prevent shunt infection: procedure carried out early in the morning at the beginning of the surgical schedule; surgical team reduced in the operating room; •Shunt passer trajectory straight or intervening incision, awareness of skull defects (i.e., post fossa craniotomy), away from central lines, tracheostomy, etc. •Good surgical technique is the best way to decrease shunt complications. VP shunt surgery DSC_9394 VP shunt surgery DSC_9399 VP shunt surgery DSC_9400 VP shunt surgery DSC_9402 VP shunt surgery DSC_9403 VP shunt surgery DSC_9406 VP shunt surgery DSC_9405 VP shunt surgery DSC_9416 VP shunt surgery DSC_9433 VP shunt surgery DSC_9438 VP shunt surgery DSC_9480 VP shunt surgery DSC_9486 VP shunt surgery DSC_9504 VP shunt surgery DSC_9516 VA shunt surgery Distal catether is placed in the atrium of the heart. Possibilities: • facial vein • puncture of the VJI • sonography guided puncture Correct placement: ECG changes – pulmonale wave Possible in local anesthesia vas LP shunt surgery •Only in case of communicating hydrocephalus •Preffered ndications: –idiopathic or secondary intracranial hypertension (pseudotumor cerebri) –slit ventricle syndrome LP shunt surgery DSCA_9681 LP shunt surgery DSCA_9682 LP shunt surgery DSCA_9687 LP shunt surgery DSCA_9691 LP shunt surgery DSCA_9692 LP shunt surgery DSCA_9693 LP shunt surgery DSCA_9694 LP shunt surgery DSCA_9696 LP shunt surgery DSCA_9698 LP shunt surgery DSCA_9699 LP shunt surgery DSCA_9700 LP shunt surgery DSCA_9701 Laparoscopy •lesser trauma to the abdominal wall and peritoneum •possibility of performing adhesiolysis and exquisite visualization of the peritoneal cavity, with in situ testing of catheter function •lower risk of intraabdominal adhesions than laparotomy •diagnosis of abdominal pain •revision surgery •primary placement Trocar Laparoscopy •Laparoscopy is safe even without VP catheter clamping and with only routine anesthetic monitoring (Al-Mufarrej et al, 2005). •Risk of retrograde failfure minimal even with intraabdominal pressure as high as 80 mm Hg (Al-Mufarrej et al, 2005). Pneumoperitoneum Laparoscopy Torkildsen shunt A ventriculocisternal shunt that diverts the cerebrospinal fluid flow from one of the lateral ventricles to the cisterna magna. Best indication is noncommunicating hydrocephalus with a lesion in and around the third and fourth ventricles that precludes standard endoscopic approaches. Barina1 Barina2 Barina3 Torkildsen Shunt complications •Undershunting •Overshunting –Slit ventricles –Intracranial hypotension –Subdural hematomas –Stenosis or occlusion of sylvian aqueduct –Craniosynostosis and microcephaly (controversial) •Infection •Seizures •Obstruction •Disconnection •Others Shunt complications Undershunting • –Verify the function of a shunt –Adjust the valve (lower pressure) • –NPH patients • Shunt complications Overshunting – Siphoning effect Shunt complications Overshunting –Slit ventricles •Most are asymptomatic •Slit ventricle syndrome – intermittent or permanent shunt occlusion (ventricles cannot expand because of subependymal occlusion) 0042006084f6 Shunt complications Overshunting –Intracranial hypotension •Relieved by recumbency •Therapy – adjust the valve (higher pressure), change the valve, ASD etc. Shunt complications Overshunting –Subdural hematomas shunt-fig1 Shunt complications Overshunting –Stenosis or occlusion of sylvian aqueduct, trapped fourth ventricle Shunt complications •Complications that may occur with any shunt: –Obstruction (proximal, valve, distal) –Disconnection, break at any point –Hardware erosion –Infection, seizures etc. •VP shunts (abdominal complications – peritonitis, ascites, hydrocele, tip migration, intestinal obstruction volvulus, intestinal strangulation etc.) •VA shunts (septicemia, shunt embolus, pulmonary hypertension, shunt nefritis etc.) •LP shunts (lumbar nerve root irritation, adhesions etc.) •