Hydrocephalus and shunt emergencies

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Target exams

Red flags

• A child with a shunt and new headache, vomiting, or drowsiness has a shunt malfunction until proven otherwise
• Cushing triad of hypertension, bradycardia, and irregular respirations means the brainstem is herniating now
• The sunset sign of impaired upgaze signals raised intracranial pressure pressing on the tectal plate
• A shunt reservoir that fails to refill on compression suggests proximal obstruction, but a normal refill never excludes malfunction
• Vomiting in a shunted child is gastroenteritis only after the shunt series is clear
• Slit ventricles on imaging do not exclude raised pressure; overdrainage causes small ventricles and positional headache
• Shunt infection usually presents within months of surgery and is most often coagulase-negative staphylococci
• About a third to a half of ventriculoperitoneal shunts fail in the first year after insertion

Life stages

Care settings

Clinical exam formats

Board mappings

• Defines hydrocephalus as an imbalance of cerebrospinal fluid production and absorption causing progressive ventricular enlargement, and lists the communicating and non-communicating forms
• Recognises a child with a shunt malfunction as a neurosurgical emergency and identifies headache, vomiting, lethargy, bulging fontanelle, and sunset eyes as red flags for raised intracranial pressure
• Describes the shunt series as a set of skull, chest, and abdomen radiographs used to trace shunt continuity, and escalates urgently to neurosurgery
• Applies the Rekate functional classification of hydrocephalus by site of cerebrospinal fluid obstruction or overproduction and relates each site to the imaging and surgical approach
• Distinguishes shunt malfunction, overdrainage and slit ventricle syndrome, shunt infection, and tumour-related or post-haemorrhagic hydrocephalus, and justifies the choice of shunt tap, imaging, revision, or endoscopic third ventriculostomy
• Manages the child with impending herniation from an obstructed shunt with airway and breathing control, neurosurgical referral, hyperosmolar therapy, and urgent shunt tap or external ventricular drainage
• Raised intracranial pressure signs in infants and children including bulging fontanelle, sunset sign, papilloedema, and Cushing triad, and the implication of Cushing triad for imminent brainstem herniation
• The components and interpretation of a shunt series and the role of CT or rapid MRI in comparing ventricular size to baseline
• The shunt tap technique and the hyperosmolar bridging agents mannitol and hypertonic saline with doses
• Runs the resuscitation and structured workup of a child presenting with suspected shunt malfunction within the time-critical window and refers urgently to neurosurgery
• Selects and interprets the shunt series, the CT or rapid MRI head, and the shunt tap CSF analysis to distinguish obstruction, overdrainage, and infection
• Counsels the family on the cause of the malfunction, the theatre and intensive care plan, the risk of revision and infection, and the long-term neurodevelopmental and educational follow-up
• Level 1: Recognition that a child with a shunt and new headache, vomiting, or drowsiness has a possible shunt malfunction requiring urgent assessment
• Level 2: Application of the shunt series, CT or rapid MRI, and shunt tap to localise obstruction, overdrainage, or infection, with timely neurosurgical referral
• Level 3: Coordination of the acute resuscitation, the operative revision or external ventricular drainage, the infection management, and the longitudinal neurodevelopmental surveillance
• The Rekate functional classification of hydrocephalus by site of cerebrospinal fluid obstruction and overproduction, and the common congenital and acquired causes in childhood
• The clinical presentation of raised intracranial pressure across infancy and childhood and the interpretation of the shunt series and neuroimaging
• The indications for ventriculoperitoneal shunt, endoscopic third ventriculostomy, and endoscopic third ventriculostomy with choroid plexus cauterisation, and the ETV Success Score
• Structured assessment of a child with a suspected shunt malfunction including fontanelle, head circumference, sunset sign, and conscious level
• Distinguishing shunt malfunction from overdrainage, infection, and non-shunt causes of raised intracranial pressure
• Communication of the diagnosis, the operative and intensive care plan, and the long-term outlook to the family
• Hydrocephalus as an imbalance of cerebrospinal fluid dynamics presenting with macrocephaly, bulging fontanelle, vomiting, and developmental delay in infants
• Shunt malfunction as a neurosurgical emergency presenting with headache, vomiting, lethargy, and signs of raised intracranial pressure
• The shunt series and neuroimaging to diagnose malfunction and the management with neurosurgical revision or external ventricular drainage
• Recognition of the child with a shunt malfunction and the signs of raised intracranial pressure across the age range
• Structured workup with the shunt series, CT or rapid MRI, and shunt tap, and timely neurosurgical referral
• Management of shunt infection and the longitudinal neurodevelopmental and educational follow-up
• Canadian approach to paediatric hydrocephalus and shunt malfunction including the shunt series and urgent neurosurgical referral
• Distinguishing obstruction, overdrainage, slit ventricle syndrome, and infection and the role of the shunt tap
• Endoscopic third ventriculostomy with or without choroid plexus cauterisation as an alternative to shunting in selected aetiologies