Surgical Options in Post Haemorrhagic Ventricular Dilation

Transcription

1 Surgical Options in Post Haemorrhagic Ventricular Dilation Benedetta Pettorini Consultant Paediatric Neurosurgeon Alder Hey Childrens Hospital Liverpool, UK

2 Risk Factors for IVH 1. Prematurity: Occurs most frequently in infants born <32 weeks or <1500g Incidence is 26% for infants weighing g 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 table Volpe JJ. Neurology of the Newborn, 4 th Edition, WB Saunders, Philadelphia p McCrea HJ and Ment LR Clinical Perinatolgy (35(4): 777 vii

3 Pathogenesis of Intraparenchymal Hemorrhage Terminal vein passes through germinal matrix Increased pressure from germinal matrix hemorrhage obstructs venous flow venous infarction

4 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 p 428

5 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 Clinical Perinatolgy (35(4): 777 vii

6 Grading IVH

7 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

8 Clinical features of IVH 3 clinical presentations ocatastrophic osaltatory osilent

9 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

10 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

11 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

12 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 Clinical Perinatolgy (35(4): 777 vii

13 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 Clinical Perinatolgy (35(4): 777 vii

14 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%

15 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

16 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

17 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

18 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

19 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

20 Literature review

21 Literature review

22 Literature review Not reliable!!!

23 PHH Management LP and transfontanelle (TF) tap No consensus EVD Reservoir VSGS Centre effect Permanent shunt procedure Ventricular washout +/- fibrinolysis (DRIFT Trial) ETV

24 Results 24 consultants from 14 units What is your preferred temporizing measure if any (assuming child not ready/suitable for permanent CSF diversion)?

25 Case example 25 weeks-old Referred at 2 weeks of age Normal fontanelle, HC on 2 nd centile

26 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

27 Case example Patient re-referred after 3 weeks Gestational age 29 weeks HC on 75 th centile Bulging fontanelle

28 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

29 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?

30 Case example 27 week-old Hypoventilation and apnea USS at 4 days of age Normal fontanelle, HC on 2 nd centile

31 Case example Managed locally unsafe transfer Two lumbar punctures required Patient stable, still on oxygen Bulging fontanelle, HC on 75 th centile

32 Case example Patient transferred to AH MRI done Insertion of right frontal subgaleal shunt

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34 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

35 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

36 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

37 Alder Hey experience 35 patients treated between Mean age: 25 weeks gestational age Average age at surgery: 29 weeks gestational age

38 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

39 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

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41 Thank you!