Surgical Options in Post Haemorrhagic Ventricular Dilation Benedetta Pettorini Consultant Paediatric Neurosurgeon Alder Hey Childrens Hospital Liverpool, UK
Risk Factors for IVH 1. Prematurity: Occurs most frequently in infants born <32 weeks or <1500g Incidence is 26% for infants weighing 501-1500g 2 The highest prevalence is in the least mature infants Mortality: ~15% 2 (worse prognosis with increasing severity) 2. Timing: Virtually all IVH in premature infants occurs in first five days 2 50, 25, and 15% on the first, second and third day, respectively 2 By the end of the first week, 90% of hemorrhages can be detected 3 3. Other Risk Factors 3 : Intrapartum asphyxia Chorioamnionitis Hypoxemia Hypercarbia Pnuemothorax/Pulmonary Hemorrhage Maternal fertility treatment 1. Vermont Oxford Network. 2002. table 1.2 2. Volpe JJ. Neurology of the Newborn, 4 th Edition, WB Saunders, Philadelphia 2001. p 428 3. McCrea HJ and Ment LR. 2008. Clinical Perinatolgy (35(4): 777 vii
Pathogenesis of Intraparenchymal Hemorrhage Terminal vein passes through germinal matrix Increased pressure from germinal matrix hemorrhage obstructs venous flow venous infarction
IVH o IVH rates have declined from 50-80% in 1980s to current rate of 10-15% 1 o Continues to be a significant problem as improved survival of extremely premature infants has resulted in more survivors with this injury 1. Volpe JJ. Neurology of the Newborn, 4 th Edition, WB Saunders, Philadelphia 2001. p 428
Preventive Strategies Many potential pharmacologic interventions have been studied, though few are currently in wide use Pharmacologic Agent Phenobarbital Indomethacin Ibuprofen Pavulon Ethamsylate Vitamin E Proposed Mechanism Stabilize blood pressure and free radical production Promotes microvasculature maturation; blunt fluctuations in BP and cerebral blood flow Improve autoregulation and cerebral blood flow Prevent asynchronous breathing in ventilated newborns; secondary BP stabilization Promote platelet adhesion, increase capillary basement membrane stability Free Radical Protection Adapted from McCrea HJ and Ment LR. 2008. Clinical Perinatolgy (35(4): 777 vii
Grading IVH
Grade That Hemorrhage!! 1. Where is the hemorrhage? 2. Name the Anatomy 3. Grade the Hemorrhage Normal Anatomy Choroid Plexus Grade II Hemorrhage Extension into lateral ventricle without dilation
Clinical features of IVH 3 clinical presentations ocatastrophic osaltatory osilent
Clinical features of IVH Catastrophic Syndrome (least common) o o o o o o o o o o o o evolution over minutes to hours Stupor or coma arrhythmias, hypoventilation and apnea Generalized seizures and Decerebrate posturing Fixed Pupils, eyes fixed to vestibular stimulation Flaccid quadriparesis falling hematocrit bulging anterior fontanelle hypotension and bradycardia temperature changes SIADH and very rarely DI Outcome generally poor because of associated large intraparenchymal bleeds
Clinical features of IVH Saltatory o more subtle o alteration in level of consciousness o change in movement (decrease) o hypotonia o minor changes in eye movements o decreased popliteal angle o outcome more favorable depends of degree of underlying IVH
Clinical features of IVH Clinically silent o symptoms may not be detected on routine exam o 50% of cases of IVH o unexplained fall in hematocrit
Sequelae of IVH 1. Posthemorrhagic Hydrocephalus Usually presents with rapid increases in head circumference Signs and symptoms may not be evident for several weeks due to compliance of neonatal brain Believed to be due to impaired CSF reabsorption following the inflammation related to blood in the CSF No proven effective interventions have been described to date McCrea HJ and Ment LR. 2008. Clinical Perinatolgy (35(4): 777 vii
Sequelae of IVH 2. Periventricular Leukomalacia (PVL) Classic white matter abnormality following IVH Attributed to profound and long-lasting decreases in CSF PVL may progress to porencepholy (Greek for hole in the brain ) Depending on severity, may lead to spastic dysplagia, visual defects, or cognitive impairment McCrea HJ and Ment LR. 2008. Clinical Perinatolgy (35(4): 777 vii
Sequelae of IVH 3. Seizures 4. Cerebral Palsy 5. Mental Retardation Risk for major neurological deficits: Grade I:5-9% Grade II: 11-15% Grade III: 30-40% Grade IV: 50-70%
Clinical Aspects of Hydrocephalus onset 1-3 weeks after IVH rapidity of progression relates to degree of IVH Head growth and signs of increased ICP follow ventricular dilation o days to weeks Posterior horn dilate earlier and greater than anterior horns
Management of Hydrocephalus Conservative o acetazolamide with or with out furosemide o serial lumbar punctures o serial ventricular punctures Early serial LPs (before ventricular index crosses 97 th centile, up to 10mL per puncture) reduces the need for surgical intervention
Management of Hydrocephalus Temporary treatment VP shunt is not an option until 2 kg of weight and/or full term higher risk of complication due to immature immune system and reduced peritoneal absorption Babies with early VP shunt insertion require twice as many revisions compared to babies who had temporary shunts up to 15% of babies won t required a permanent shunt
Management of Hydrocephalus NO PERFECT SOLUTION!!! Clinical judgement able to balance: Higher risk of infection in preterm than in fullterm Potential damage due to inadequately treated raised ICP Risks of surgery Risks of permanent shunt
Management of Hydrocephalus Goal of placing a temporary shunt is to delay insertion of a permanent shunt, if needed, until the baby is older, better nutrition and immunity Surgical options o External ventricular drainage (and DRIFT trial) o Rickham reservoir o Washout o VP or subgaleal shunt o ETV
Literature review
Literature review
Literature review Not reliable!!!
PHH Management LP and transfontanelle (TF) tap No consensus EVD Reservoir VSGS Centre effect Permanent shunt procedure Ventricular washout +/- fibrinolysis (DRIFT Trial) ETV
Results 24 consultants from 14 units What is your preferred temporizing measure if any (assuming child not ready/suitable for permanent CSF diversion)?
Case example 25 weeks-old Referred at 2 weeks of age Normal fontanelle, HC on 2 nd centile
Case example 1 week after: HC on 25 th centile Patient stable Advice to tap fontanelle/lumbar puncture in case of deterioration?treatment at this point
Case example Patient re-referred after 3 weeks Gestational age 29 weeks HC on 75 th centile Bulging fontanelle
Case example Patient transferred to AH MRI done Insertion of Ommaya reservoir (left frontal, no image-guidance) Intermittent connection to EVD bag through orange needle or serial reservoirs puncture
Case example Patient sent back to local centre after 48 hours of drainage Plan to come back in ten days for further drainage Irritability, temperature and bulging fontanelle E.Coli CSF infection!!! Could we avoid it?
Case example 27 week-old Hypoventilation and apnea USS at 4 days of age Normal fontanelle, HC on 2 nd centile
Case example Managed locally unsafe transfer Two lumbar punctures required Patient stable, still on oxygen Bulging fontanelle, HC on 75 th centile
Case example Patient transferred to AH MRI done Insertion of right frontal subgaleal shunt
Case example Patient sent back locally the day after HC stable on 2 nd centile for 8 weeks No need of subgaleal collection puncture HC on 75 th centile after 8 weeks Patient was 2 weeks corrected age, 3 kg Insertion of right parietal VP shunt
Advantages Disadvantages External ventricular drainage Easy and effective Long length of stay High infection rate Difficult nursing Reservoir Easy and effective 0-50% complication rate Fluctuations of ICP Numerous taps required (up to 126 reported) Subgaleal shunt Easy and effective Continuous diversion Lower complication rate Additional surgery
DRIFT Trial Phase I trial of intraventricular tissue plasminogen activator followed by ventricular irrigation Results: fewer patients required permanent shunt insertion in comparison with historical controls A subsequent randomised trial failed to demonstrate a benefit in early results and more patients treated with the novel regimen appeared to suffer secondary IVH Waiting for DRIFT2 Whitelaw A, et al: Pediatrics, 2003 and 2007
Alder Hey experience 35 patients treated between 2009-2017 Mean age: 25 weeks gestational age Average age at surgery: 29 weeks gestational age
Alder Hey experience EVD Reservoir VSG 2 patient 12 patients 21 patients 1 infection 4 infections 2 CSF leaks 1 aseptic meningitis 100% shunt conversion 100% shunt conversion 87.5% shunt conversion 3 pts: VSG revision 3 pts: collection puncture
Summary Current therapies are unsatisfactory and prevention remains the best way to reduce the incidence and impact of IVH Center effect in determining temporization procedure Need to standardize care pathway, timing of neurosurgical consultation, frequency and modality of imaging?multicenter trial
Thank you!