Trauma & Vascular

Epidemiology

Incidence

Paediatric TBI accounts for hundreds of thousands of emergency department visits annually in high-income countries; severe TBI (GCS ≤8) is a minority but accounts for the bulk of mortality and long-term disability.

Age peak

Bimodal: infants/toddlers (falls, abusive head trauma) and adolescents (sport, road traffic).

Location

Brain; diffuse and focal injury patterns coexist; extra-axial collections (epidural, subdural), contusions, and diffuse axonal injury are the major patterns.

Clinical Presentation

• Initial assessment by the ATLS/APLS framework: airway with cervical-spine protection, breathing, circulation, disability (GCS, pupils), exposure.
• Mild TBI (GCS 13-15): the great majority of presentations; applies PECARN-type clinical decision rules to limit unnecessary CT.
• Moderate (GCS 9-12) and severe (GCS ≤8) TBI: ICU-level management, urgent neurosurgical assessment, prevention of secondary injury.
• Post-concussion symptoms (headache, sleep disturbance, mood change, cognitive complaints): often under-recognised in children; activity-modification and graded return are central to recovery.

Imaging

• CT is the first-line imaging in the acute setting; fast, available, identifies the surgical lesions (epidural, subdural, contusion, mass effect, midline shift).
• MRI for sub-acute imaging; superior for diffuse axonal injury (DAI), small contusions, brainstem injury, and follow-up.
• Skull radiographs have a limited role and are not a substitute for CT in suspected significant injury.
• Cervical-spine imaging is integral whenever indicated; TBI patients are spinal-injury patients until proven otherwise.

Pathology & Molecular

Histology. Diffuse axonal injury (DAI): axonal shearing at grey-white interfaces, corpus callosum, and brainstem. Focal contusion: cortical haemorrhagic injury, classically at coup and contrecoup sites.

Molecular. Secondary-injury cascades involve excitotoxicity, oxidative stress, inflammation, and apoptosis, the basis of current and trial-stage neuroprotective strategies.

Management

Surgery. Surgical management of identified lesions: evacuation of epidural and subdural haematomas with significant mass effect; elevation of depressed skull fractures meeting criteria; decompressive craniectomy in selected refractory cases.

Adjuvant therapy. Non-surgical critical-care management: stepped algorithm per BTF 3rd Edition; head elevation, normothermia, normocapnia, sedation/analgesia, osmotherapy (hypertonic saline preferred in paediatrics), CSF drainage, paralysis, and consideration of decompressive craniectomy or barbiturate coma for refractory intracranial hypertension.

Considerations. Always image and protect the cervical spine; always exclude non-accidental injury in young children with unexplained TBI; involve rehabilitation services early.

Outcomes

Outcomes correlate strongly with initial GCS, pupillary response, and CT findings. Children generally have better functional outcomes than adults with comparable injury severity, but the long-term neurocognitive impact (especially on attention, executive function, and academic progress) is substantial and often under-recognised.

Glasgow Coma Scale; adult and paediatric (verbal subscale)

TBI severity classification (by post-resuscitation GCS) and broad management implications

Epidemiology

Incidence

A minority of paediatric TBI but accounts for the great majority of mortality and severe disability.

Age peak

Bimodal; infants (often abusive head trauma) and adolescents (road traffic, sport).

Location

Brain; frequently with associated extracranial injury in road-traffic and high-energy mechanisms.

Clinical Presentation

• Initial assessment by ATLS/APLS principles with full cervical-spine protection.
• Intubation for GCS ≤8 (or deterioration); avoid hypoxia and hypotension, both independently worsen outcome.
• Urgent CT to identify surgical lesions; ICP monitor consideration once stabilised.
• Continuous physiological targets: oxygenation, CO2 control, cerebral perfusion pressure (CPP), temperature, glucose.

Imaging

• Initial CT for the surgical decision (evacuate vs medical management) and to define baseline.
• Repeat CT for clinical deterioration or scheduled re-assessment in the first 24-48 hours.
• MRI in the sub-acute period for DAI, brainstem injury, and prognostication.

Pathology & Molecular

Histology. Mixed primary injury (contusions, DAI, extra-axial collections) plus secondary injury (oedema, ischaemia, oxidative injury).

Molecular. Secondary-injury cascades (excitotoxicity, oxidative stress, inflammation) drive much of the modifiable injury.

Management

Surgery. Evacuation of mass-effect-producing extra-axial haematoma (epidural or subdural) is urgent. Depressed skull fracture meeting criteria is elevated. Decompressive craniectomy may be undertaken for refractory raised ICP not controlled by tier-1 and tier-2 medical measures.

Adjuvant therapy. Tiered medical therapy per BTF 3rd Edition: tier 1, head-up positioning, normothermia, normocapnia (PaCO2 35-38 mm Hg), adequate sedation/analgesia, osmotherapy (hypertonic saline preferred in paediatrics; mannitol acceptable), CSF drainage via EVD; tier 2, neuromuscular blockade, mild hypocapnia (still within safe limits), barbiturate trial; tier 3; decompressive craniectomy or moderate-to-deep hypothermia (the latter not first-line and used with caution).

Considerations. Avoid prolonged or aggressive hyperventilation. Avoid prophylactic hypothermia. Glucocorticoids are not recommended.

Outcomes

Mortality from severe paediatric TBI has fallen with modern guideline-based care but remains substantial. Long-term cognitive, behavioural, and academic outcomes are determined by injury severity, secondary insults, and rehabilitation.

Stepped management of refractory ICP in paediatric severe TBI (BTF 3rd Edition, simplified)

Epidemiology

Incidence

Estimated 20-30 per 100,000 in infants under 1 year in published series; the true incidence is almost certainly higher (under-recognition is well-documented).

Age peak

Infants; peak incidence in the first 6 months of life; rare after age 4-5.

Location

Brain (subdural haematoma, parenchymal injury), retina (haemorrhages), skeletal (rib, metaphyseal, healing fractures), skin.

Clinical Presentation

• Presentation may be subtle (irritability, lethargy, vomiting, apnoea, seizures, encephalopathy) and is often disproportionate to the history offered.
• Inconsistent, changing, or absent history of trauma is itself a red flag.
• Always examine for retinal haemorrhages (dilated fundoscopy by ophthalmology), cutaneous bruising in young pre-mobile infants, and skeletal injury (skeletal survey).

Imaging

• CT acutely for surgical decision-making.
• MRI of brain and (when indicated) cervical spine for full characterisation; sub-acute and chronic blood, parenchymal injury, ligamentous injury at the cervico-medullary junction.
• Skeletal survey (radiographic) per AAP protocol, with selective bone scintigraphy if survey is inconclusive.

Pathology & Molecular

Histology. Subdural haematoma (often bilateral and of different ages), parenchymal injury, hypoxic-ischaemic injury, retinal haemorrhages.

Molecular. Not applicable; clinical diagnosis.

Management

Surgery. Evacuation of significant subdural collections producing mass effect; treatment of associated injuries; CSF diversion if hydrocephalus develops in the recovery phase.

Adjuvant therapy. Critical-care management for severe cases per BTF guidelines.

Considerations. Mandatory child-protection notification per local jurisdiction. Multidisciplinary team: paediatric neurosurgery, paediatrics, child-protection (safeguarding), ophthalmology, social work, radiology. Documentation must be meticulous, with the diagnosis stated clearly in clinical notes.

Outcomes

Substantial mortality (commonly cited 15-25%) and high rate of severe long-term disability among survivors; neurodevelopmental, motor, visual, and behavioural. Recurrence of abuse is a recognised risk if the diagnosis is missed.

Epidemiology

Incidence

Common, a frequent finding on CT performed for paediatric head injury.

Age peak

Throughout childhood; growing skull fractures particularly in infants under 3 years owing to the dynamic skull-brain relationship.

Location

Calvarial (frontal, parietal, occipital) most common; basal in higher-energy injuries.

Clinical Presentation

• Linear fracture: often clinically silent; identified on CT or as a step palpable beneath the scalp; conservative management is usual.
• Depressed fracture: palpable depression; consider operative elevation if depression > thickness of skull, associated with dural tear, parenchymal injury, cosmetic concern, or open injury.
• Basal skull fracture: signs include 'raccoon eyes' (periorbital ecchymosis), Battle's sign (mastoid ecchymosis), haemotympanum, CSF rhinorrhoea or otorrhoea, and cranial-nerve deficits (especially CN I and VII).
• Growing skull fracture: an enlarging, pulsatile scalp swelling weeks to months after a known fracture in an infant; characteristic and requires surgical repair.

Imaging

• CT is the workhorse; bony detail, identification of depressed fractures, and assessment of underlying brain injury.
• MRI for suspected dural tear (growing skull fracture risk), brain injury characterisation.
• Plain skull radiographs have a limited role and are not a substitute for CT in significant injury.

Pathology & Molecular

Histology. Not a histological diagnosis.

Molecular. Not applicable.

Management

Surgery. Linear isolated fracture: conservative, with imaging follow-up if growing fracture risk. Depressed fracture meeting criteria (depression > skull thickness, open injury, dural tear, mass effect, cosmetic concern in visible area): elevation, dural repair if torn, fragment replacement or cranioplasty. Growing skull fracture: surgical repair of dural defect with cranial reconstruction; early repair prevents progressive enlargement and underlying brain herniation. Basal fracture with CSF leak: most resolve with conservative management; persistent or recurrent leak warrants imaging and consideration of repair (endoscopic endonasal or transcranial depending on location).

Adjuvant therapy. Antibiotic prophylaxis for open or depressed fractures per institutional protocol; vaccination status review.

Considerations. An infant with a linear fracture and underlying dural tear is at risk of growing skull fracture; schedule follow-up imaging at 1-3 months.

Outcomes

Excellent for isolated linear fractures. Outcomes for depressed fractures depend principally on the associated brain injury. Growing skull fracture has a good outcome when recognised and repaired early.

Epidemiology

Incidence

Less common than adult cervical injury but with distinctive patterns; SCIWORA classically affects children under 8 years.

Age peak

Bimodal; young children (upper cervical, often higher-mortality injuries) and adolescents (more adult-pattern subaxial injuries).

Location

Under age 8: predominantly upper cervical (C0-C1, C1-C2). Over age 8: more adult-pattern subaxial.

Clinical Presentation

• Maintain a high index of suspicion in any child with head injury, multi-system trauma, or unexplained neurological signs.
• Cervical-spine immobilisation per ATLS/APLS protocols pending clearance.
• Atlanto-occipital dislocation: often fatal; survivors may have subtle initial findings; high mortality demands early recognition.
• SCIWORA: classical presentation is delayed onset of neurological deficit after apparent recovery; spinal-cord syndromes (central cord, complete) may occur.

Imaging

• Plain radiographs (lateral, AP, open-mouth odontoid where age permits) for initial screening, but with full awareness that paediatric pseudosubluxation (especially C2-C3) is a normal variant and not injury.
• CT for bony injury; sensitive and increasingly used in major trauma, balanced against radiation exposure.
• MRI is essential when SCIWORA is suspected; demonstrates cord oedema, contusion, and ligamentous injury invisible on bony imaging.

Pathology & Molecular

Histology. Spinal cord oedema, contusion, haemorrhage; ligamentous disruption.

Molecular. Secondary injury cascades parallel TBI.

Management

Surgery. Bony / ligamentous injury: reduction (closed or open), instrumentation and fusion as indicated. Upper cervical injuries often require occipito-cervical or atlanto-axial instrumentation; choice of construct depends on anatomy and age. SCIWORA: surgical management is generally supportive (external immobilisation) unless instability is demonstrated.

Adjuvant therapy. ICU monitoring; respiratory support for high cervical injury; early rehabilitation involvement.

Considerations. High-dose methylprednisolone for acute spinal cord injury is not currently recommended in paediatric SCI.

Outcomes

Atlanto-occipital dislocation: high mortality. SCIWORA with severe initial deficit: outcomes often poor. SCIWORA with mild initial deficit: meaningful recovery is possible but residual deficit common.

Epidemiology

Incidence

Rare overall; the leading cause of spontaneous intracerebral haemorrhage in children.

Age peak

Bimodal in children; presentation in early childhood and adolescence; many are clinically silent until adulthood.

Location

Anywhere in the cerebral hemispheres, brainstem, or cerebellum; supratentorial locations predominate.

Clinical Presentation

• Haemorrhage is the most common paediatric presentation; sudden severe headache, focal deficit, depressed consciousness.
• Seizures, headache, or focal neurological deficit without haemorrhage in a subset.
• Annual haemorrhage risk in unruptured AVMs is commonly cited as approximately 2-4% per year, but is heavily modified by lesion features and prior haemorrhage.

Imaging

• CT acutely identifies haemorrhage; CT angiography or MR angiography confirms vascular abnormality.
• Digital subtraction angiography (DSA) is the gold standard for definition of nidus, feeders, and drainage and is essential before any intervention.
• MRI defines parenchymal relations and identifies haemorrhage products.

Pathology & Molecular

Histology. Tangled abnormal vessels with both arterial and venous features, no intervening capillary bed.

Molecular. Most sporadic; hereditary haemorrhagic telangiectasia (HHT) and capillary malformation-AVM (RASA1) are recognised genetic associations.

Management

Surgery. Microsurgical resection is the definitive treatment for accessible lesions of appropriate grade; goal is complete excision of the nidus. Endovascular embolisation is used adjunctively (pre-surgical, pre-radiosurgical) and rarely as definitive monotherapy. Stereotactic radiosurgery is appropriate for small (≤3 cm), deep, or eloquent-area lesions; obliteration occurs over 2-3 years with continued haemorrhage risk during latency.

Adjuvant therapy. Anticonvulsant management as indicated.

Considerations. Decision-making in paediatric AVM weighs the long natural-history horizon (decades of life ahead) against procedural risk; generally favours intervention more often than equivalent adult lesions.

Outcomes

Outcomes depend on Spetzler-Martin grade, presentation (ruptured vs unruptured), and modality. Low-grade lesions (SM I-II) treated microsurgically have low procedural risk and high cure rates. High-grade lesions (SM IV-V) carry significant procedural risk and are often managed conservatively or with multimodal staged approaches.

Spetzler-Martin AVM grading (Spetzler & Martin, 1986)

Epidemiology

Incidence

MRI-based prevalence approximately 0.5%; familial forms have higher prevalence in some founder populations (e.g., Hispanic).

Age peak

Any age; familial forms often present in childhood with multiple lesions.

Location

Anywhere in the brain or spinal cord; brainstem and deep locations are surgically challenging.

Clinical Presentation

• Seizures are the most common symptomatic presentation in supratentorial lesions.
• Focal neurological deficit from haemorrhage in eloquent or brainstem locations.
• Many are incidentally discovered on MRI performed for unrelated reasons.
• Annual haemorrhage risk for known lesions is commonly cited as approximately 0.5-2% per year, higher with prior haemorrhage or brainstem location.

Imaging

• MRI is the gold standard: classic 'popcorn' or 'mulberry' appearance with mixed signal on T1 and T2 due to blood products of differing ages, surrounding hypointense haemosiderin rim.
• Gradient-echo (GRE) or susceptibility-weighted imaging (SWI) is the most sensitive; reveals lesions invisible on conventional sequences and demonstrates the burden of disease in multifocal familial forms.
• Angiography is typically negative; CCMs are angiographically occult, distinguishing them from AVMs.

Pathology & Molecular

Histology. Dilated, thin-walled vascular sinusoids lined by endothelium, lacking intervening brain parenchyma. Surrounding parenchyma shows haemosiderin staining from chronic micro-haemorrhage.

Molecular. Familial forms: autosomal-dominant mutations in CCM1 (KRIT1), CCM2 (MGC4607), or CCM3 (PDCD10). Multiple lesions on imaging or family history should prompt genetic testing.

Management

Surgery. Symptomatic accessible lesions (especially seizure-generating supratentorial lesions, or those that have haemorrhaged) are candidates for microsurgical excision. Brainstem and deep lesions are managed conservatively unless there has been clinically significant haemorrhage and the lesion is surgically accessible by a safe entry zone.

Adjuvant therapy. Antiepileptic management; surveillance MRI for known untreated lesions.

Considerations. Stereotactic radiosurgery for CCM is controversial and not first-line.

Outcomes

Complete microsurgical excision is typically curative for that lesion. Seizure outcomes after excision are favourable, especially if surgery is performed early in the course.

Epidemiology

Incidence

Rare; accounts for a small proportion of paediatric vascular malformations overall but the great majority of those presenting in the neonatal period.

Age peak

Neonatal (cardiac failure), infant (hydrocephalus / macrocrania), older child (seizures, developmental delay); three classical presentation windows.

Location

Midline posterior third ventricle / quadrigeminal cistern.

Clinical Presentation

• Neonate: high-output cardiac failure, often requiring intensive support; pulmonary hypertension; failure of the usual neonatal cardiovascular transition.
• Infant: progressive hydrocephalus, macrocrania, developmental delay; cranial bruit may be audible.
• Older child: seizures, developmental delay, or, rarely, haemorrhage.

Imaging

• Antenatal ultrasound or fetal MRI may identify the lesion before birth; allows planned delivery and neonatal management in a specialist centre.
• Postnatal MRI / MRA defines the anatomy; transfontanellar Doppler ultrasound is useful in neonates.
• Digital subtraction angiography is essential for treatment planning, performed in conjunction with endovascular treatment.

Pathology & Molecular

Histology. Persistent embryonic median prosencephalic vein dilated by choroidal-type arteriovenous shunts.

Molecular. A minority of cases harbour EPHB4 mutations; most are sporadic.

Management

Surgery. Open neurosurgical treatment has a very limited role and is generally restricted to ventriculo-peritoneal shunt for residual hydrocephalus. Microsurgical resection of the malformation itself is rarely undertaken in the contemporary era.

Adjuvant therapy. Endovascular embolisation (most commonly transarterial) is the mainstay of definitive treatment, typically staged over multiple sessions in childhood and timed by the Bicêtre group framework (Lasjaunias et al., 2006) which uses the neonatal cardiac/neurological evaluation to guide timing of intervention.

Considerations. Neonatal cardiac and respiratory stabilisation may be the priority over immediate vascular intervention; the multidisciplinary team (paediatric neurosurgery, neuro-interventional radiology, neonatology, cardiology, anaesthesia) is essential.

Outcomes

Substantial improvement over historical figures with modern endovascular management in expert centres, but morbidity and mortality remain significant in neonatal presentations with severe cardiac failure or established brain injury.

Epidemiology

Incidence

Higher prevalence in East Asian populations (especially Japan and Korea); lower but significant in Western series.

Age peak

Bimodal; first decade (peaks around age 5) and fourth decade.

Location

Bilateral involvement of the distal ICA and proximal MCA/ACA with abnormal lenticulostriate and other collateral networks.

Clinical Presentation

• Children: recurrent transient ischaemic attacks and ischaemic strokes, often precipitated by crying, hyperventilation, or hot food (reduced PaCO2 → vasoconstriction in already compromised circulation).
• Headache; choreiform movements; cognitive decline; seizures.
• Haemorrhagic presentation is more typical in adults than children.

Imaging

• MRI shows established infarction, especially in watershed territories; FLAIR may reveal the 'ivy sign' (sulcal hyperintensity reflecting slow collateral flow).
• MR angiography (or CT angiography) demonstrates the characteristic distal ICA stenosis and the abnormal collateral network.
• Digital subtraction angiography remains the gold standard for full vascular characterisation and surgical planning, including the classical Suzuki staging.

Pathology & Molecular

Histology. Intimal thickening and luminal narrowing of the affected arteries without inflammatory features.

Molecular. RNF213 variants are strongly associated, particularly in East Asian populations.

Management

Surgery. Surgical revascularisation is the definitive treatment in children. Indirect revascularisation procedures predominate in paediatric practice, encephalo-duro-arterio-synangiosis (EDAS), encephalo-myo-synangiosis (EMS), encephalo-duro-arterio-myo-synangiosis (EDAMS), pial synangiosis (Boston technique), and multiple burr-hole approaches, relying on neovascularisation from the extracranial circulation into the underlying brain over weeks to months. Direct revascularisation (STA-MCA bypass) is technically challenging in young children but used in adolescents and adults in some centres.

Adjuvant therapy. Antiplatelet therapy (typically aspirin) is commonly used; perioperative attention to normocapnia, normothermia, and adequate hydration is critical.

Considerations. Children with sickle-cell disease, NF1, or prior cranial radiotherapy who develop unexplained TIAs or stroke should have vascular imaging to exclude moyamoya.

Outcomes

Surgical revascularisation substantially reduces the risk of future stroke. Cognitive outcomes depend on the extent of damage before surgery, hence the importance of early diagnosis and intervention.

Epidemiology

Incidence

Approximately 2-5 per 100,000 children per year for arterial ischaemic stroke; perinatal stroke (around the time of birth) is substantially more common at approximately 1 per 2,500-4,000 live births.

Age peak

Perinatal (around the time of birth) and again in early childhood.

Location

Anywhere; perinatal AIS shows a predilection for the left middle cerebral artery territory.

Clinical Presentation

• Acute focal deficit (hemiparesis, hemisensory loss, facial droop, language disturbance), often misdiagnosed initially as post-ictal Todd's paresis, migraine, or functional.
• Seizures are more common in paediatric stroke than in adult stroke and may be the dominant presentation in young children.
• Perinatal stroke: often presents with early hand preference, asymmetric motor development, or focal seizures in the neonatal period.

Imaging

• MRI with DWI is the modality of choice; sensitive for acute ischaemia and avoids ionising radiation.
• CT acutely if MRI is not rapidly available; CTA / MRA to assess vasculature.
• MRV (or CTV) for suspected cerebral sinovenous thrombosis.

Pathology & Molecular

Histology. Ischaemic infarction, with histopathology following the timeline of cytotoxic and vasogenic oedema, infarction, and gliosis.

Molecular. Underlying causes (sickle cell, prothrombotic states, congenital heart disease) drive the molecular and physiological mechanism.

Management

Surgery. Decompressive craniectomy for malignant MCA-territory infarction with mass effect (particularly in adolescents) is performed when life-threatening oedema develops, by extension from adult evidence and emerging paediatric series. Surgical revascularisation for moyamoya is covered separately. Aneurysm or vascular-malformation treatment when these are the cause.

Adjuvant therapy. Acute supportive care; aspirin or anticoagulation depending on mechanism and after exclusion of haemorrhagic transformation; sickle-cell-specific protocols including emergency exchange transfusion; intra-arterial thrombectomy in selected adolescents with large-vessel occlusion (drawing on adult evidence).

Considerations. Multidisciplinary team, paediatric neurology, neurosurgery, haematology (especially for sickle cell), and rehabilitation, is the appropriate management framework.

Outcomes

Substantial neurological morbidity is common; cognitive, motor, language, and behavioural sequelae are frequent and often under-recognised. Mortality is lower than adult stroke.

Epidemiology

Incidence

Rare; paediatric aneurysms account for a small fraction (a few per cent) of intracranial aneurysms overall.

Age peak

Throughout childhood and adolescence.

Location

Higher proportion of posterior-circulation and distal-vessel aneurysms compared with adult disease; cavernous and ICA bifurcation aneurysms also occur.

Clinical Presentation

• Subarachnoid haemorrhage (worst headache, meningism, depressed consciousness), as in adults but typically dramatic given paediatric resilience.
• Mass effect from giant aneurysms (cranial neuropathies, focal deficit, hydrocephalus).
• Infectious (mycotic) aneurysms in the setting of endocarditis or sepsis; distal middle-cerebral-artery distribution typical.

Imaging

• CT for acute haemorrhage; CTA or MRA for vascular characterisation.
• Digital subtraction angiography is the gold standard and is essential before any intervention.
• Consider screening of family members and the patient's other vascular territories when an aetiology suggests systemic vasculopathy.

Pathology & Molecular

Histology. Depends on aetiology; saccular (similar to adult), dissecting, mycotic (with inflammatory features), traumatic (pseudoaneurysm).

Molecular. Heritable connective-tissue disorders (Marfan, Ehlers-Danlos type IV, autosomal-dominant polycystic kidney disease) increase risk; familial intracranial aneurysm syndromes are recognised.

Management

Surgery. Microsurgical clipping remains the standard for many paediatric aneurysms, particularly distal lesions and where reconstruction or parent-vessel bypass may be required (paediatric vessels are smaller and may not tolerate stent-assisted constructs as readily as adult vessels).

Adjuvant therapy. Endovascular treatment (coiling, flow-diversion in selected centres) has a growing role; modality selection is centre-dependent and individualised. Antibiotic therapy for mycotic aneurysms; treatment of any underlying systemic disease.

Considerations. Lifelong vascular surveillance after treatment of one aneurysm, the de-novo aneurysm risk is higher than in adults.

Outcomes

Outcomes after treatment in modern series are generally favourable, but paediatric patients carry a lifelong risk of new or recurrent aneurysm formation and warrant long-term follow-up.