Paeds · neurology-neurodisability-and-neuromuscular

Hydrocephalus and shunt emergencies

Also known as Ventriculoperitoneal shunt malfunction · Shunt obstruction · Shunt infection · Slit ventricle syndrome · Raised intracranial pressure in the shunted child

Fellowship guide to paediatric hydrocephalus and shunt emergencies. Covers the Rekate functional classification of hydrocephalus by the site of cerebrospinal fluid obstruction and overproduction, the cerebrospinal fluid pathway from choroid plexus to arachnoid granulations, the age-specific presentation of raised intracranial pressure from the bulging fontanelle and sunset sign in the infant to the early-morning headache and Cushing triad in the older child, the shunt series of skull, chest, and abdomen radiographs that traces the catheter from ventricle to peritoneum, the shunt tap that measures pressure and sends cerebrospinal fluid for culture, the hyperosmolar bridge to theatre with mannitol and hypertonic saline, the distinction of shunt malfunction from overdrainage, slit ventricle syndrome, and infection, the Hydrocephalus Clinical Research Network protocol that cut shunt infection, and the endoscopic third ventriculostomy and ETV Success Score work of Warf and Kulkarni as the non-shunt alternative.

high12 referencesUpdated 16 July 202628 min readVerification in progress

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Red flags

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

Life stages

fetalneonateinfanttoddlerpreschoolschool-ageadolescent

Care settings

outpatientwarded-acutenicupicuretrievalrural-remote

Clinical exam formats

written-onlyracp-dce-long-caseracp-dce-short-casemrcpch-history-managementmrcpch-communicationrcpsc-structured-oral

Board mappings

Defines hydrocephalus as an imbalance of cerebrospinal fluid production and absorption causing progressive ventricular enlargement, and lists the communicating and non-communicating formsRecognises 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 pressureDescribes the shunt series as a set of skull, chest, and abdomen radiographs used to trace shunt continuity, and escalates urgently to neurosurgeryApplies the Rekate functional classification of hydrocephalus by site of cerebrospinal fluid obstruction or overproduction and relates each site to the imaging and surgical approachDistinguishes 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 ventriculostomyManages 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 drainageRaised 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 herniationThe components and interpretation of a shunt series and the role of CT or rapid MRI in comparing ventricular size to baselineThe shunt tap technique and the hyperosmolar bridging agents mannitol and hypertonic saline with dosesRuns the resuscitation and structured workup of a child presenting with suspected shunt malfunction within the time-critical window and refers urgently to neurosurgerySelects and interprets the shunt series, the CT or rapid MRI head, and the shunt tap CSF analysis to distinguish obstruction, overdrainage, and infectionCounsels 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-upLevel 1: Recognition that a child with a shunt and new headache, vomiting, or drowsiness has a possible shunt malfunction requiring urgent assessmentLevel 2: Application of the shunt series, CT or rapid MRI, and shunt tap to localise obstruction, overdrainage, or infection, with timely neurosurgical referralLevel 3: Coordination of the acute resuscitation, the operative revision or external ventricular drainage, the infection management, and the longitudinal neurodevelopmental surveillanceThe Rekate functional classification of hydrocephalus by site of cerebrospinal fluid obstruction and overproduction, and the common congenital and acquired causes in childhoodThe clinical presentation of raised intracranial pressure across infancy and childhood and the interpretation of the shunt series and neuroimagingThe indications for ventriculoperitoneal shunt, endoscopic third ventriculostomy, and endoscopic third ventriculostomy with choroid plexus cauterisation, and the ETV Success ScoreStructured assessment of a child with a suspected shunt malfunction including fontanelle, head circumference, sunset sign, and conscious levelDistinguishing shunt malfunction from overdrainage, infection, and non-shunt causes of raised intracranial pressureCommunication of the diagnosis, the operative and intensive care plan, and the long-term outlook to the familyHydrocephalus as an imbalance of cerebrospinal fluid dynamics presenting with macrocephaly, bulging fontanelle, vomiting, and developmental delay in infantsShunt malfunction as a neurosurgical emergency presenting with headache, vomiting, lethargy, and signs of raised intracranial pressureThe shunt series and neuroimaging to diagnose malfunction and the management with neurosurgical revision or external ventricular drainageRecognition of the child with a shunt malfunction and the signs of raised intracranial pressure across the age rangeStructured workup with the shunt series, CT or rapid MRI, and shunt tap, and timely neurosurgical referralManagement of shunt infection and the longitudinal neurodevelopmental and educational follow-upCanadian approach to paediatric hydrocephalus and shunt malfunction including the shunt series and urgent neurosurgical referralDistinguishing obstruction, overdrainage, slit ventricle syndrome, and infection and the role of the shunt tapEndoscopic third ventriculostomy with or without choroid plexus cauterisation as an alternative to shunting in selected aetiologies

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Saved locally on this device.

Practise this topic

Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General Pediatrics

Red flags

Life stages

fetalneonateinfanttoddlerpreschoolschool-ageadolescent

Care settings

outpatientwarded-acutenicupicuretrievalrural-remote

Clinical exam formats

written-onlyracp-dce-long-caseracp-dce-short-casemrcpch-history-managementmrcpch-communicationrcpsc-structured-oral

Board mappings

Overview & Definition

A child who turns up unwell with a ventriculoperitoneal shunt has a shunt malfunction until proven otherwise, and the job is to prove it is not, fast. Hydrocephalus is the active distension of the cerebral ventricles from an imbalance between the production and the absorption of cerebrospinal fluid, and a shunt is the tube that drains that fluid from the ventricle to the peritoneum when the body cannot move it itself. When the shunt blocks, the original hydrocephalus returns, the intracranial pressure climbs, and the brainstem is threatened. Kahle and colleagues set out the modern understanding of paediatric hydrocephalus as a disorder of cerebrospinal fluid dynamics that is surgical in its emergency and lifelong in its management. [1]

Three facts make this topic central to the paediatric exam. The diagnosis is a clock and a clinical pattern, because the child with a blocked shunt and a falling conscious level is dying. The investigation is a shunt series and a head scan, which trace the tube and the ventricles. And the management is the early involvement of neurosurgery, because the definitive treatment is operative and no child with a threatened brainstem waits for it. The shunted child is one of the commonest technology-dependent children in general paediatric practice, and every fellow must hold the assessment and the safety-net without hesitation. [3]

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References12Show ledger

[1]Kahle KT, Klinge PM, Koschnitzky JE, et al Paediatric hydrocephalus. Nat Rev Dis Primers, 2024.PMID 38755194
[2]Rekate HL The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Cerebrospinal Fluid Res, 2008.PMID 18211712
[3]Lu VM, Shimony N, Jallo GI Infant Hydrocephalus. Pediatr Rev, 2024.PMID 39085190
[4]Riva-Cambrin J, Kestle JR, Holubkov R, et al Risk factors for shunt malfunction in pediatric hydrocephalus: a multicenter prospective cohort study. J Neurosurg Pediatr, 2016.PMID 26636251
[5]Kestle JR, Riva-Cambrin J, Wellons JC 3rd, et al A standardized protocol to reduce cerebrospinal fluid shunt infection: the Hydrocephalus Clinical Research Network Quality Improvement Initiative. J Neurosurg Pediatr, 2011.PMID 21721884
[6]Konrad E, Robinson JL, Hawkes MT Cerebrospinal fluid shunt infections in children. Arch Dis Child, 2023.PMID 36450441
[7]Kulkarni AV, Riva-Cambrin J, Browd SR, et al Use of the ETV Success Score to explain the variation in reported endoscopic third ventriculostomy success rates among published case series of childhood hydrocephalus. J Neurosurg Pediatr, 2011.PMID 21284458
[8]Warf BC, Weber DS, Day EL Endoscopic third ventriculostomy with choroid plexus cauterization: predictors of long-term success and comparison with shunt placement for primary treatment of infant hydrocephalus. J Neurosurg Pediatr, 2023.PMID 37178026
[9]Simon TD, Schaffzin JK, Stevenson CB Cerebrospinal Fluid Shunt Infection: Emerging Paradigms in Pathogenesis that Affect Prevention and Treatment. J Pediatr, 2019.PMID 30528757
[10]Rekate HL Shunt-related headaches: the slit ventricle syndromes. Childs Nerv Syst, 2008.PMID 18259760
[11]Miller JP, Fulop SC, Dashti SR Rethinking the indications for the ventriculoperitoneal shunt tap. J Neurosurg Pediatr, 2008.PMID 18518692
[12]Cavalheiro S, da Costa MDS, Barbosa MM, et al Hydrocephalus in myelomeningocele. Childs Nerv Syst, 2021.PMID 34435215

Hydrocephalus and shunt emergencies

Also known as Ventriculoperitoneal shunt malfunction · Shunt obstruction · Shunt infection · Slit ventricle syndrome · Raised intracranial pressure in the shunted child

high12 referencesUpdated 16 July 202628 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[1]Kahle KT, Klinge PM, Koschnitzky JE, et al Paediatric hydrocephalus. Nat Rev Dis Primers, 2024.PMID 38755194
[2]Rekate HL The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Cerebrospinal Fluid Res, 2008.PMID 18211712
[3]Lu VM, Shimony N, Jallo GI Infant Hydrocephalus. Pediatr Rev, 2024.PMID 39085190
[4]Riva-Cambrin J, Kestle JR, Holubkov R, et al Risk factors for shunt malfunction in pediatric hydrocephalus: a multicenter prospective cohort study. J Neurosurg Pediatr, 2016.PMID 26636251
[5]Kestle JR, Riva-Cambrin J, Wellons JC 3rd, et al A standardized protocol to reduce cerebrospinal fluid shunt infection: the Hydrocephalus Clinical Research Network Quality Improvement Initiative. J Neurosurg Pediatr, 2011.PMID 21721884
[6]Konrad E, Robinson JL, Hawkes MT Cerebrospinal fluid shunt infections in children. Arch Dis Child, 2023.PMID 36450441
[7]Kulkarni AV, Riva-Cambrin J, Browd SR, et al Use of the ETV Success Score to explain the variation in reported endoscopic third ventriculostomy success rates among published case series of childhood hydrocephalus. J Neurosurg Pediatr, 2011.PMID 21284458
[8]Warf BC, Weber DS, Day EL Endoscopic third ventriculostomy with choroid plexus cauterization: predictors of long-term success and comparison with shunt placement for primary treatment of infant hydrocephalus. J Neurosurg Pediatr, 2023.PMID 37178026
[9]Simon TD, Schaffzin JK, Stevenson CB Cerebrospinal Fluid Shunt Infection: Emerging Paradigms in Pathogenesis that Affect Prevention and Treatment. J Pediatr, 2019.PMID 30528757
[10]Rekate HL Shunt-related headaches: the slit ventricle syndromes. Childs Nerv Syst, 2008.PMID 18259760
[11]Miller JP, Fulop SC, Dashti SR Rethinking the indications for the ventriculoperitoneal shunt tap. J Neurosurg Pediatr, 2008.PMID 18518692
[12]Cavalheiro S, da Costa MDS, Barbosa MM, et al Hydrocephalus in myelomeningocele. Childs Nerv Syst, 2021.PMID 34435215