The purpose of this document is to provide regional guidelines for doctors, nurses and allied professionals in the in the clinical management of children (birth -18yrs) with acute hydrocephalus or dysfunction of ventricular peritoneal (VP), ventricular pleural and ventricular atrial (VA) shunts.This guideline identifies the pathway for management of a patient with hydrocephalus or suspected shunt blockage, but should not replace clinical assessment.
Hydrocephalus can develop slowly or quickly as a result of a disparity in the amount of cerebral spinal fluid (CSF) being produced compared to the amount being absorbed. This disparity leads to an increase in the pressure in the ventricles compared to the subarachnoid space. When the pressure in the ventricles rise relative to the subarachnoid space pressure the ventricles may become enlarged but this may not always be the case. The consequence of a build-up of pressure in the ventricles is a build-up of pressure in the intracranial compartment and it is this that leads to the variety of symptoms we see. If hydrocephalus is not treated the intracranial pressure continues to increase and in severe cases, this can result in permanent brain damage, blindness and death.
This guideline applies to all children and young people for whom a hospital admission is required. This guideline applies to all health providers in the regional District General Hospitals (DGH) in the Wessex region and within University Hospital Southampton (UHS) NHS Foundation Trust.
The purpose is to provide standardised care throughout the region and improve patient safety and clinical outcomes. The purpose is to provide standardised care throughout the region and improve patient safety and clinical outcomes.
VP shunt – Ventricular Peritoneal shunt.
VA shunt – Ventricular Atrial shunt
ICP – Intracranial Pressure
CSF – Cerebral Spinal Fluid
EVD – External Ventricular Drain
GCS – Glasgow Coma Score
ETV - Endoscopic Third Ventriculostomy
DGH – District General Hospital
UHS – University Hospital Southampton (NHS Foundation Trust)
SORT – Southampton Oxford retrieval Team
Symptoms of Hydrocephalus or a Blocked Shunt
The presentation of patients with hydrocephalus or a blocked shunt can vary, both in terms of symptoms and speed of onset. Symptoms that are typical include:
However, it is important to note that symptoms can be very subtle or unusual. In general terms, if the patient or the parent feels there is shunt dysfunction, advice should be sought from the paediatric neurosurgical centre in Southampton Children’s Hospital.
The child may complain of headache or nausea and may vomit, often in the morning. Babies may have symptoms such as increasing head size, tense bulging fontanelle, a high pitched cry, irritability, sun-setting eyes or distended scalp veins. Other symptoms that may be particular to the child could include seizures and cranial nerve palsies, such as a squint.
Children with increased intracranial pressure may demonstrate a compromise in systemic perfusion (Hazinski 2009). The child may be tachycardic or bradycardic and the skin may be mottled in appearance.
Evaluation of the level of consciousness is a vital aspect of the assessment of a child with raised intracranial pressure. The Glasgow Coma Scale (GCS) assessment tool should be used in conjunction with the recording of vital signs, pupil reactions and limb movement. In paediatric practice a modification of the original GCS has been made so that the assessment is more relevant to child development (Patterson et al 1992). Often children only have a drop in their level of consciousness in the latter stages and so any patient with a shunt who has a reduced level of consciousness should be treated as a medical emergency.
Shunt dysfunction or failed endoscopic third ventriculostomy (ETV), should be assumed until proven otherwise in a child who presents unwell and has a shunt in situ or has had a previous ETV.
Children with who have had insertion of a VP/VA/VPleural shunt and ETV have open access to the Children’s Neurosurgical Ward. Telephone advice can be gained from G2N (Children’s Neurosurgery) Ward on 023 8120 6692. Children who are deteriorating neurologically or are unstable will be directed to their nearest Emergency Department. Safe transfer can then be arranged to UHS, as indicated by the SORT or on-call neurosurgery team.
The parent and carer’s assessment of the child with a long term shunt is of great importance.
The child should be observed with frequent neurological and Glasgow coma score assessments. The presence of drowsiness, headache and vomiting are likely to be caused by shunt dysfunction.
Clinicians are encouraged to seek advice from the on-call paediatric neurosurgical consultant or the call neurosurgery registrar (023 8077 7222 Bleep 2877). If there is difficulty accessing a doctor then the children’s neurosurgery nurse specialist can be phoned directly on 07794368342 (Monday-Friday between the hours of 07:30-15:30hrs)
CT brain scan and shunt series X-rays should be obtained in a timely manner. A shunt series for a VP shunt comprises of an AP and lateral skull and neck X-ray, AP chest X-ray and AP abdominal X-ray so the whole shunt tubing can be followed throughout its course. If the child has a VA or VPleural shunt then abdominal x-ray is not required. It should be remembered that 15% of shunt blockages do not produce a change in the scan appearance (Thompson 2007). Scans should be made available for review by the neurosurgical team at UHS.
Where there is suspicion of shunt dysfunction and the child remains clinically stable and conscious then urgent transfer to G2N will be arranged as directed by the on-call neurosurgery team.
Where there is suspicion of shunt dysfunction and there is a clinical deterioration or reduced conscious level then the child may require urgent intubation and ventilation for transfer directly to neurosurgical theatres or to Paediatric Intensive Care (PICU) at UHS. In this situation there should be early discussion with SORT (Hotline number – 023 8077 5502) in conjunction with the neurosurgical team. (See Related Documents for referral & transfer forms). In critical situations it may be necessary to attempt CSF drainage (by means of a shunt ‘tap’ or direct cannulation of the ventricle) prior to transfer. This should only be attempted after discussion with the on-call paediatric neurosurgical consultant.
If the CT scan is not diagnostic of shunt dysfunction but the child remains clinically unwell then transfer to G2N Children’s Neurosurgical Ward may be appropriate after discussion with on-call neurosurgery team. ICP monitoring may be considered (Appendix A). Also continue to investigate for alternative causes. Contact G2N Ward prior to transfer – on 023 8120 6692
This guideline applies to all clinical staff employed or contracted by University Hospital Southampton (UHS) Foundation Trust and medical staff within the regional district general hospitals, who care for children, aged 0-18. Staff have a responsibility to ensure that they are aware of this guideline and its contents. They should clearly document their rationale if they have not complied with the recommendations detailed in this guideline. It is the responsibility of department managers, consultants, team leaders and education leaders to ensure staff are aware of this guideline. This guideline will be available via the PIER website.
Hazinski,M,F. (2009) Nursing Care of the Clinically ill Child. Mosby Year Book London.
Jennett B, Teasdale G (1974) Assessment of coma and impaired consciousness. Lancet 2: 81 –84
Paterson, R.J., Brown, G,W. Salaai-Scotter, M. Middaugh, D. (1992) Head injury in the conscious child. Am J Nurs Vol 92 (8): pg 22–27
Thompson, D. (2007) Clinical Guideline Shunt Blockage. Great Ormond Street Hospital for Children NHS Trust.
Document Version: 1.0
Lead Authors: Kate Bailey, Children’s Neurosurgery Nurse Specialist Ryan Waters, Consultant Neurosurgeon