Management of neonatal hydrocephalus

Management of neonatal hydrocephalus

Symposium: neurology Management of neonatal hydrocephalus arises in choosing the best treatment option for a ­particular aeti-­ ology and is discuss...

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Symposium: neurology

Management of neonatal hydrocephalus

arises in choosing the best treatment option for a ­particular aeti-­ ology and is discussed below. Intraventricular haemorrhage Full term IVH probably occurs more often than we think but is more common at lower birth weights.3–5 IVH does not invariably cause HC. There is controversy as to how HC follows IVH. Some have suggested that proteinaceous material blocks the arach-­ noid granulations, thus resulting in excess cerebrospinal fluid (CSF) due to reduced reabsorption.6 In some cases there must be some kind of block to CSF passage at the exit foraminae of the fourth ventricle, as this would explain why endoscopic third ventriculostomy (ETV) works in some patients with IVH (18%).7 However, the majority need to have shunts inserted and, despite many recently advocating the use of ETV,8–11 this is still consid-­ ered by most to be the treatment of first option. ETV has also been used to washout heavy protein loaded CSF and to enable shunting to be implemented somewhat earlier or even avoided.12 In heavy blood/protein loaded CSF, drainage may be needed for some time before shunting to reduce the risk of shunt blockage, although lumbar drainage has been successful in avoiding shunting in IVH (see below).13

Neil Buxton

Abstract Neonatal hydrocephalus is a complex disorder due to many different causes. This review seeks to encapsulate the management of neonatal hydrocephalus in the term neonate. The current treatments are explored and explained.

Keywords hydrocephalus; intraventricular haemorrhage; neonatal ­meningitis; neonates; shunts

Neonatal meningitis Meningitis can also lead to HC. This may be due to a heavy pro-­ tein load causing problems at the arachnoid granulations or dis-­ crete blockages of exit foraminae by debris or membranes. Hence, again, ETV has been shown to be effective in some post-­meningitic HC cases but the majority will require shunt insertion. During the acute, infective phase, and whilst there is heavy protein load in the CSF, it may be necessary to drain the HC with an external ventricular drain (EVD) in order to reduce the intra-­ cranial hypertension. Obviously, such a device can be used to drain excess CSF, but is also useful for obtaining CSF samples for serial cultures and for the administration of intraventricular anti-­ biotics (a technique restricted to specialist neurosurgical units). Draining the CSF in this way will allow it to return to its normal constituent levels, so allowing shunting to be implemented. It is generally believed that the higher the protein load of the CSF the more likely it is that the shunt will fail due to blockage by debris; this is not the case with HC secondary to tuberculous meningitis. In these circumstances, the protein load is much less important and shunting can take place earlier.14,15

Introduction Neonatal hydrocephalus (NHC) is increasingly becoming the most difficult management problem in paediatric neurosurgery but survival from the hydrocephalus has improved.1 There are many problems associated with aetiology, body weight and immaturity, including unfused sutures, relating to risks of infec-­ tion and controversies with actual treatment protocols. This review is intended to give an overview of current thoughts on the management of hydrocephalus in the term neonate. The manage-­ ment of hydrocephalus (HC) in the premature child will not be covered. Neonatal hydrocephalus occurs in approximately 1 in 1000 live births. It is secondary to full term intraventricular haem-­ orrhage (IVH), infection or congenital causes such as tumours, aqueduct stenosis, Dandy–Walker syndrome and its variants or, of course, it can be truly idiopathic. Where possible, treatment of the cause is the first priority but in many cases the treatment of the hydrocephalus takes prece-­ dence. For example, in meningitis with HC, draining an enlarged ventricular system may be necessary even before the infection has been completely cleared. Birth weight influences treatment choices as well. There is a reluctance to introduce any permanent shunt systems into a child less than 2 kg in weight because below this weight there is a substantially increased risk of shunt failure due to infection.2 Fortunately, most term babies exceed this weight.

Non-communicating hydrocephalus The terminology communicating and non-communicating hydro-­ cephalus is becoming controversial, partly because of issues with post-infectious and post-haemorrhagic HC, as briefly men-­ tioned above. It is clear that tumours, aqueduct stenosis and Dandy–Walker syndrome and its variants can have physical blocks to the passage of the CSF, and thus can lead to truly non­communicating HC. In these types of HC, seemingly in all ages, ETV is the treatment of choice.16,17 Unfortunately, ETV in the truly non-communicating hydro-­ cephalus in some younger children will still fail. There are no accepted theories for this but it may be that the pressure of CSF required to initiate CSF reabsorption via the arachnoid granu-­ lations exceeds the pressure needed to expand the cranium in those with unfused sutures; in such a situation, ETV is certain to

Treatment choices All authorities in Western countries agree that, except in the most devastating of circumstances, the HC should be treated. ­Difficulty

Neil Buxton MB ChB DMCC FRCS(Ed) FRCS(Neuro Surg) is a Consultant Paediatric Neurosurgeon, Royal Liverpool Children’s Hospital, Liverpool, UK.

PAEDIATRICS AND CHILD HEALTH 18:1

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Symposium: neurology

fail and a shunt is required. Whilst seemingly very simple, shunt-­ ing is controversial too and in order to address this small number of children and their requirements an international randomized trial is looking at the efficacy of shunting versus ETV.

c­ ommon alternatives include the right atrium via a neck vein and the superior vena cava (the historical site of choice) and also the pleural space. The pleura is used as a last resort in those in whom there has been extensive abdominal surgery, peritonitis or necrotizing enterocolitis, and whose neck veins have been dam-­ aged by central lines. Pleural shunts always cause effusions and, if there is a significant CSF volume, then the effusion resulting may embarrass lung function. A balance must be struck. Simi-­ larly, in the abdomen there can be a bulging tense abdominal wall and the development of CSF hydrocoeles.

Idiopathic So-called idiopathic HC is best treated by treating the underlying anatomical precedence. If there is evidence of flow obstruction, then ETV may well work; otherwise it is likely that shunting will be required. Tumours In the presence of third or fourth ventricular tumours or tectal plate or brainstem tumours causing HC, the HC can easily be treated by ETV and in some biopsies obtained.11,18 In successful resection of a cerebellar tumour, for example, CSF flow may be restored and no longer requires diversion. ETV or shunt is often needed, however, although temporary EVD may ‘buy enough time’ for definitive tumour treatment. Arachnoid cysts in or near the third ventricle behave like tumours and can be successfully managed by ETV.19

Shunt failure This is almost inevitable in the lifetime of a patient with a shunt, with the greatest number, approximately 20%, occurring in the first year after insertion. Failure manifests itself in many ways, e.g. increased head circumference, tense fontanel, drowsiness, vomit-­ ing, squint, CSF tracking alongside the shunt tubing, signs of infec-­ tion, and banging the head with the hands or against something, which can indicate headache. In these circumstances shunt revi-­ sion is usually required. It is a neurosurgical rule that if the primary carer says that the child is ‘not right’ and that they ‘think it’s the shunt’, it is a brave and foolhardy person to ignore the warning. In some in whom a shunt subsequently fails, an ETV may well work and paediatric neurosurgeons will always assess a ‘new’ shunt failure for anatomical suitability for the procedure.9,22,23

Low birth weight If the birth weight is less than 2 kg, then shunting tends not to be ­recommended.2 This is because of concerns about the anaesthetic, neonatal care, risk of infection, operating on someone so small, etc, with infection of the shunt being the most worrisome. The HC can be ameliorated until the baby gains weight by serial lumbar punctures, serial ventricular taps, an EVD (which can be used up to 3 weeks without changing, with care) or a more permanent Ommaya reservoir (an implanted ventricular tube with an injection part).20 Intuitively there is concern about introducing a permanent or semi-permanent foreign body into the child, just as there is con-­ cern definitive shunting; however, this seems to be safe in cases of low birth weight and post-haemorrhagic hydrocephalus.21

Conclusion The numbers of children surviving to term in the West with HC are increasing as better obstetric care, earlier antenatal diagnosis and better awareness lead to more informed decisions. In the last 20 years, paediatric neurosurgery has evolved into a distinct subspecialty on a par, for example, with spinal neurosurgery. More aggressive, better targeted treatment for paediatric HC, no longer in isolation from other children’s specialists, provided by surgeons with expertise and training in the management of these difficult clinical scenarios is improving the situation for these patients. Treatment in the West has moved out of the hands of paediatric general surgeons but their historical contribution can-­ not be underestimated as without their skills and expertise there would be no paediatric neurosurgery at all. With the develop-­ ment of paediatric neurosurgical centres there is no longer an excuse to dabble in the management of such complex problems and such an approach is to be discouraged. ◆

Which shunt? The type of shunt largely depends on the individual surgeon, their experience with a particular model and their own biases. Whilst this approach is not scientifically sound, a surgeon will get ‘used’ to a particular model, understand its idiosyncrasies and become confident with its use. This is perhaps more impor-­ tant than any other consideration such as cost, ‘newness’, etc, as the surgeon uses their own experience to decide what is best for a particular patient. This is where experience counts and, dare it be said, some of the art in the science remains. Notwithstanding all of the above, most would agree that the smaller the child the less bulky the shunt and the quicker it should be to insert, with fewer components to increase surgery time (hence infection risk) and in the long run with fewer options for malfunction. Unfor-­ tunately, the answer to the problem of deranged physiology is difficult to identify when we have mechanical devices with fixed tolerances for, literally, a fluid system. Where does the distal end go? Obviously in treating HC we are inserting the upper end into the lateral ventricle. This tends to be on the right (the non-dominant hemisphere). This is con-­ nected to a one-way valve device to the distal catheter. The dis-­ tal end is placed into the peritoneal cavity by choice, although

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References 1 Chi JH, Fullerton HJ, Gupta N. Time trends and demographics of deaths from congenital hydrocephalus in children in the United States: National Center for Health Statistics data, 1979 to 1998. J Neurosurg 2005; 103(suppl 2): 113–118. 2 Bruinsma N, Stobberingh EE, Herpers MJ, et al. Subcutaneous ventricular catheter reservoir and ventriculoperitoneal drain related infections in preterm infants and young children. Clin Microbiol Infect 2000; 6: 202–206. 3 Fanaroff AA, Stoll BJ, Wright LL, et al. Trends in neonatal morbidity and mortality for very low birth weight infants. Am J Obstet Gynecol 2007; 196: 147 e1–8.

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4 Fink S. Intraventricular haemorrhage in the term infant. Neonatal Netw 2000; 19: 13–18. 5 Prat Puig M, Campistol Plana J, Muniz Llama F, et al. Intraventricular haemorrhage in healthy newborn infants at term. An Esp Pediatr 1987; 27: 107–111. 6 Cherian S, Whitelaw A, Thoresen M, et al. The pathogenesis of neonatal posthemorrhagic hydrocephalus. Brain Pathol 2004; 14: 305–311. 7 O’Brien DF, Seghedoni A, Collins DR, et al. Is there an indication for ETV in young infants in aetiologies other than isolated aqueduct stenosis? Childs Nerv Syst 2006; 22: 1565–1572. 8 Scavarda D, Bednarak N, Litre F, et al. Acquired aqueductal stenosis in preterm infants: an indication for neuroendoscopic third ventriculostomy. Childs Nerv Syst 2003; 19: 756–759. 9 Giomin V, Cinalli G, Grotenhuis A, et al. Endoscopic third ventriculostomy in patients with CSF infection and/or hemorrhage. J Neurosurg 2002; 97: 519–524. 10 Scarrow AM, Levy EI, Pascucci L, et al. Outcome analysis of endoscopic third ventriculostomy. Childs Nerv Syst 2000; 16: 442–444. 11 Macarthur DC, Buxton N, Punt J, et al. The role of neuroendoscopy in the management of brain tumours. Br J Neurosurg 2002; 16: 465–470. 12 Kamikawa S, Inui A, Kobayashi N, et al. Intraventricular hemorrhage in neonates: endoscopic findings and treatment by the use of our newly developed Yamadori type 8 ventriculoscope. Minim Invasive Neurosurg 2001; 44: 74–78. 13 Huttner HB, Schwab B, Bardutzky J. Lumbar drainage for communicating hydrocephalus after ICH with ventricular haemorrhage. Neurocrit Care 2006; 5: 193–196. 14 Kemaloglu S, Ozkan U, Bukte M, et al. Timing of shunt surgery in childhood tuberculous meningitis with hydrocephalus. Pediatr Neurosurg 2002; 37: 194–198. 15 Palur R, Rajshekhar V, Chandy MJU, et al. Shunt surgery for hydrocephalus in tuberculous meningitis: a long term follow-up study. J Neurosurg 1991; 74: 64–69.

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16 Baldauf J, Oertal J, Gaab MR, et al. Endoscopic third ventriculostomy in children younger than 2 years of age. Child Nerv Syst 2007; 23: 623–626. 17 Koch D, Wagner W. Endoscopic third ventriculostomy in infants less than 1 year of age: which factors influence the outcome? Child Nerv Syst 2004; 20: 405–411. 18 Javadpour M, Mallucci CL. The role of neuroendoscopy in the management of tectal gliomas. Childs Nerv Syst 2004; 20: 852–857. 19 Kirollos RW, Javadpour M, May P, et al. Endoscopic treatment of suprasellar and third ventricle related arachnoid cysts. Child Nerv Syst 2001; 17: 713–718. 20 Khalil BA, Sarsam Z, Buxton N. External ventricular drains: Is there a time limit in children? Childs Nerv Syst 2005; 21: 355–357. 21 Peretta P, Ragazzi P, Carlino CF, et al. The role of Ommaya reservoir and endoscopic third ventriculostomy in the management of posthaemorrhagic hydrocephalus of prematurity. Child Nerv Syst 2007; 23: 765–771. 22 O’Brien DF, Javadpour M, Collins DR, et al. Endoscopic third ventriculostomy: An outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction. J Neurosurg 2005; 103(suppl 5): 393–400. 23 Buxton N, Macarthur D, Robertson I, et al. Neuroendoscopic third ventriculostomy for failed shunts. Surg Neurol 2003; 60: 201–203.

Practice points • Neonatal hydrocephalus has varied aetiology • Choice of method of treatment for the hydrocephalus can depend heavily on the aetiology • Early consultation with a paediatric neurosurgeon is essential

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