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OCCASIONAL REVIEW

Approach to the pre- According to the European Society of Cardiology, syncope is


defined as a transient loss of consciousness due to cerebral

school child with sudden hypoperfusion. It is characterized by a rapid onset, short dura-
tion, and spontaneous complete recovery. Syncope shares clin-

loss of consciousness ical features with disorders that may be mistaken for one
another, forming the group of disorders labelled as ‘transient loss
of consciousness’ (T-LOC). T-LOC is formally defined as a real or
Pradeep Naganna apparent transient loss of consciousness, expressed through all
Niha Mariam Hussain four of the following qualifiers: abnormal motor control, loss of
responsiveness, amnesia for the period of unconsciousness, and
Nahin Hussain a short duration.

Abstract Epidemiology
There are several causes for sudden loss of consciousness in pre-
TLOC is a common symptom in all age groups, affecting up to
school children. These include Transient Loss of Consciousness (T-
50% of the population; 47% of females and 31% of males will
LOC), which encompasses a group of disorders with the following
have fainted at least once in their lives. In addition, around 1% of
characteristics: abnormal motor control, loss of responsiveness,
toddlers may have a form of vasovagal syncope, their risk
amnesia for the period of unconsciousness, and a short duration.
increasing with age and peaking at around 15 years old.
There are three main categories of T-LOC in pre-school children: syn-
The incidence of syncope requiring medical attention is 125/
cope, resulting from a sudden and reversible lack of oxygenated blood
100,000. It accounts for 3% of Emergency Department atten-
supplied to the brain, often caused by transient impairment of cardiac
dances, and 1% of all hospital admissions.
output or systemic arterial hypotension. Secondly, epileptic seizures
Although syncope is a relatively common condition with a
due to excessive and hypersynchronous cortico-neuronal electrical
good prognosis, in some cases it has the potential to become life-
activity in the brain, and thirdly, “unexplained T-LOC,” which includes
threatening. Diagnosis can be incredibly challenging, and the rate
cases not yet diagnosed fully, and those for which a pathophysiolog-
of recurrence in children who were misdiagnosed following
ical mechanism has not yet been identified, despite thorough investi-
hospital admission are high.
gations. Reflex Anoxic Seizures (RAS) are important in the differential
diagnosis of non-epileptic paroxysmal events in infants and pre-
school children. Parents and carers who witness these episodes are Classification and pathophysiology
understandably anxious, and the mainstay of management is ensuring
that the correct diagnosis is made, reassurance is given, and both the
The two main groups of TLOC are ‘TLOC due to head trauma’
patient and parents are educated about the condition. Diagnosis can
and ‘non-traumatic TLOC’. Traumatic TLOC is outside the scope
be made based off a detailed history, including an eyewitness account
of this article and will therefore not be mentioned further;
of the episode, as well as clinical examinations. This review aims to however, non-traumatic TLOC disorders may be further classi-
describe an approach to the diagnosis and management of T-LOC fied based on history and pathophysiology. Approximately 50%
in pre-school children. of paediatric syncope are non-cardiovascular, 20e30% are car-
diovascular, and a further 20e30% are of unknown aetiology.
Keywords ECG; epileptic seizures; reflex asystolic syncope; syn-
See Figure 1.
cope; transient loss of consciousness

Neurally mediated/reflex syncope


Introduction
The reflex syncope is the most common cause of syncope. It
A sudden loss of consciousness in a child can cause anxiety occurs due to an abnormal response between sympathetic and
amongst both parents and clinicians. It presents in various forms, parasympathetic reflexes; it is characterised by peripheral vaso-
such as ‘fainting,’ ‘black outs,’ ‘collapse,’ and ‘passing out.’ dilation, a fall in blood pressure and bradycardia, resulting in
global cerebral hypoperfusion.
Sudden cessation of blood pressure for even 6e8 seconds has
been known to cause a complete loss of consciousness. Systemic
Pradeep Naganna MBBS MRCPCH, Specialist Registrar Department of blood pressure is the product of cardiac output and peripheral
Paediatric Neurology, Leicester Royal Infirmary, University Hospitals
vascular resistance. Any event which results in a fall of either
of Leicester NHS Trust, Leicester, UK. Conflicts of interest: none
factor (or both) can subsequently lead to syncope.
declared.
Niha Mariam Hussain, Medical Student College of Medical and
Dental Sciences, University of Birmingham, Birmingham, UK. Vasovagal syncope
Conflicts of interest: none declared. Vasovagal syncope is the most common cause of reflex syncope
seen in day-to-day practice. It is triggered by several factors:
Nahin Hussain MBBS FRCPCH, Consultant Paediatric Neurologist
Department of Paediatric Neurology, Leicester Royal Infirmary, emotional stress, prolonged standing, standing up suddenly,
University Hospitals of Leicester NHS Trust, Leicester, UK. Conflicts tiredness, hunger or dehydration, the sight of blood, fear,
of interest: none declared. anaemia, as well as post-exercise are included.

PAEDIATRICS AND CHILD HEALTH xxx:xxx 1 Ó 2020 Elsevier Ltd. All rights reserved.

Please cite this article as: Naganna P et al., Approach to the pre-school child with sudden loss of consciousness, Paediatrics and Child Health,
https://doi.org/10.1016/j.paed.2020.06.003
OCCASIONAL REVIEW

Figure 1 A summary of the various causes of TLOC. PNES, psychogenic non-epileptic seizures; PPS, psychogenic pseudosyncope; VVS,
vasovagal syncope; CSS, carotid sinus syndrome; TIA, transient ischaemic attack. Adapted from Brignole et al., 2018.

Reflex asystolic syncope (RAS)/reflex anoxic seizures and consciousness. Sometimes a dramatic extensor spasm as
Reflex Asystolic Syncope (RAS) is a paroxysmal disorder which described in RAS may occur. RAS can sometimes be indistin-
typically occurs in a neurodevelopmentally intact pre-school guishable on history or video record from EAS.
child. Any unexpected stimulus, such as pain, shock or fright,
causes the heart and breathing to stop. Orthostatic syncope
Presentation of RAS may involve an extremely pale dis- Orthostatic syncope is due to an impairment of reflexes during
colouration; the child is often blue around the mouth and under standing, resulting in fall in blood pressure and/or tachycardia.
the eyes (reflecting central cyanosis). Furthermore, their eyes Accompanying symptoms include dizziness, light headedness,
may roll up, their jaw may clench, and the body would stiffen, nausea, fatigue, blackouts and palpitations.
sometimes alongside the jerking of the limbs. Episodes tend to Causes of orthostatic syncope include:
last approximately 30 seconds, after which the body relaxes and  Orthostatic hypotension due to impaired in venous return
the heart and breathing resume, leaving the person unconscious during exercise, post-prandial etc;
in a post-ictal state. One or two minutes later the person may  Volume depletion, caused by haemorrhage, profound
regain consciousness; however, the child may remain uncon- anaemia, gastroenteritis, Addison’s disease etc;
scious or sleepy for well over an hour. During recovery the  Anaphylaxis
person may be emotional and look extremely pale. RAS attacks  Septic shock
rarely occur several times per day, per week, or per month (more  Valsalva manoeuvres
common), or per year. These attacks appear to cluster.  micturition (post-micturition)
Orthostatic hypotension (OH) is defined as a decrease in
Expiratory apnoea syncope (EAS) systolic blood pressure of 20 mmHg or a decrease in diastolic
EAS is a common cause of reflex syncope in toddlers and infants. blood pressure of 10 mmHg, occurring within three minutes of
It is also known as cyanotic breath-holding spells. It typically standing up from a sitting/supine position. The incidence of
starts with the child becoming upset and crying, resulting in the syncope in children is unknown but studies estimate it is <5% in
child becoming apnoeic in expiration. This leads to a loss of tone people under the age of 50.

PAEDIATRICS AND CHILD HEALTH xxx:xxx 2 Ó 2020 Elsevier Ltd. All rights reserved.

Please cite this article as: Naganna P et al., Approach to the pre-school child with sudden loss of consciousness, Paediatrics and Child Health,
https://doi.org/10.1016/j.paed.2020.06.003
OCCASIONAL REVIEW

Cardiopulmonary syncope Other causes


Cardiac syncope is relatively less common in children; none- Metabolic causes and hypoglycaemia
theless, thorough evaluation is recommended to rule out any Certain metabolic condition may present with either a loss/
other life-threatening diagnoses. decreased levels of consciousness. These may present in children
Causes may be subdivided, shown in Table 1. with development delay or failure to thrive. Additionally, meta-
bolic conditions may cause hypoglycaemia and subsequently
Non-syncopal seizures neuroglycopenia. This results in a gradual loss of cognitive
functions causing slower reaction times, blurred speech, seizures
Febrile seizure
and loss of consciousness.
A simple febrile seizure is defined by the International League
Against Epilepsy (ILAE) as a short, generalized seizure, not Benign paroxysmal vertigo of childhood (BPVC)
recurring within 24 h and not caused by an acute disease of the BPVC is a common cause of recurrent episodes of dizziness in
nervous system. It occurs in children aged 6 months-5 years, children aged 3e4 years. Episodes have a rapid onset and may
with no neurologic deficits. last several minutes; children will recover completely after the
episode. At this young age, children usually cannot express their
Epileptic seizure
symptoms, but will often panic from loss of control and may
Epilepsy is a neurological condition, characterised by recurrent
cling to an adult until the episode resolves.
epileptic seizures. Seizures are due to abnormal hyperexcitation
of the cortical neurons; they are often confused with syncope. Raised intracranial pressure (ICP) and hydrocephalus
Absence seizures may present as staring/vacant episodes, lasting Infections of the brain, including meningitis and encephalitis, can
only for a few seconds, occurring several times a day. Complex increase ICP, leading to an altered level of consciousness. Pa-
partial seizures typically last longer than absence seizures (>60 tients usually have a fever and other common signs of infection
seconds), may present with a preceding aura, and have a post- as well. Rare structural abnormalities in the brain may present
ictal phase. with hydrocephalus; these include Arnold-Chiari malformations,
congenital aqueduct stenosis and tumours.
Psychogenic causes
Psychogenic TLOC episodes may last anywhere from minutes to History and examination
hours. It can present in two forms: Psychogenic non-epileptic It is crucial to take a detailed history directly from the patient and
seizures (PNES) and Psychogenic pseudosyncope (PPS). PNES their parent/witness. Witnesses should be encouraged to clarify
are group of disorders resembling epileptic seizures associated any terms used by the clinician (especially medical terminology)
with motor component, but without the epileptic electrical dis- and should describe the event chronologically. If possible, video
charges. PPS differs from PNES due to a lack of motor move- recordings of the event should be shown.
ments. These disorders are manifestations of psychogenic The history taking must include the following:
distress.  Circumstances prior to the event
 Precipitating factors
 Where the child was, what they were doing & patient’s po-
sition at onset
 Past medical history, including drug history and
investigations
 Family history to elicit for cardiac disorders
A table outlining the different types of cardiopulmonary o Any sudden unexplained deaths
syncope o Any fitting of pacemakers or defibrillator
o Any arrythmias
Cardiac rhythm problems Cardiac structural  Family history of neurological disorder
abnormalities o Epilepsy
o Strokes
Sinus node dysfunction Valvular disease
o Arterial dissection
Brady/tachyarrhythmia Acute myocardial infarction/
o Aneurysms
ischaemia
 History suggestive of neurocutaneous disorders
Atrioventricular conduction system Obstructive cardiomyopathy
 Sleep history (if narcolepsy with cataplexy indicated)
disease
 History of any psychological or psychiatric Illness
Inherited syndromes, e.g. long QT Pericardial disease/tamponade
 Social history
syndrome
From a detailed history, syncope can be differentiated from
Implanted device malfunction, e.g. Pulmonary embolus
other forms of TLOC in 60% of cases. Studies have shown that 30
pacemaker, ICD
e40% of children diagnosed with epilepsy were wrongly diag-
Drug-induced arrhythmia Pulmonary hypertension
nosed; the children were most likely to have syncope instead.
Vascular steal syndromes
The evaluation should answer these key questions:
1. Was the event TLOC?
Table 1

PAEDIATRICS AND CHILD HEALTH xxx:xxx 3 Ó 2020 Elsevier Ltd. All rights reserved.

Please cite this article as: Naganna P et al., Approach to the pre-school child with sudden loss of consciousness, Paediatrics and Child Health,
https://doi.org/10.1016/j.paed.2020.06.003
OCCASIONAL REVIEW

2. Is the TLOC of syncopal or non-syncopal origin?  Presence of a short PR interval, a wide QRS complex with
3. In case of suspected syncope, is there a clear aetiological a slurred onset of the QRS waveform (delta wave) and
diagnosis? secondary ST-T wave changes in Wolff-Parkinson-White
4. Is there evidence to suggest a high risk of cardiovascular syndrome.
events or death?  Voltage criteria for obstructive left or right ventricular
Table 2 outlines the differential diagnoses for sudden loss of hypertrophic cardiomyopathies (HCM) or
consciousness/awareness. cardiomyopathies.
2. Cardiac Echocardiogram: for suspected structural cardiac
Examination disease
 Examination covering vital signs of blood pressure and heart 3. Blood tests: Haemoglobin/haematocrit, blood gas for
rate anaemias
 Cardiac examination for: 4. Blood sugar: for hypoglycaemia
o murmurs 5. Blood pressure: Sitting and standing blood pressure for
o irregular pulse postural hypotension, POTS.
o carotid bruit 6. EEG: only to support if clinical suspicion of epilepsy. EEG must
 Neurological system examination for: not to be done on suspected syncope as it may show non-
o cranial or motor neurological deficits specific inter-ictal epileptiform discharges.
o sensory deficits 7. Head-up tilt tests are the gold standard for diagnosing and
 Neuro-cutaneous syndromes such as cafe au lait marks categorising neurally-mediated syncope (NMS). It is not used
 Plot growth parameters on appropriate growth charts routinely due to difficulties in conducting the test without the
 Head circumference necessary equipment. It is used in cases of diagnostic uncer-
 Signs for trauma tainty, such as in cases with frequent, or atypical, or treatment
 Fundi examination resistant TLOC.

Investigations Red flag signs and symptoms


1. ECG: A standard 12 lead ECG should be included in all cases. If Careful consideration should be given to red flag signs and
cardiac pathology is suspected, then further investigation such symptoms. If present, patients must be evaluated and managed
as continuous or exercise ECG may be necessary. as per the clinical indication, after seeking a relevant specialist’s
 Emphasis should be placed on the QT interval and T wave opinion. Red flag signs and symptoms include:
for evidence of long QT syndrome.  Little or no prodromal symptoms

A table outlining the differential diagnoses for sudden loss of consciousness/awareness


Differential diagnoses Clues from history

Neurally mediated syncope C Usually have a trigger


C Pre-syncope symptoms include dizziness,
light headedness, visual/auditory distor-
tions, nausea, flushing and sweating.
C History of visual disturbance and feeling of
becoming distant, and then losing balance
and falling/slumping on to floor.
Cardiac arrythmia C Sudden and brief TLOC with collapse and
an immediate recovery
Typical generalised epileptic seizure C Tonic, clonic, tonic-clonic movements with
or without impaired loss of consciousness
and impaired breathing
C Associated with cyanosis, incontinence,
tongue biting
C Followed by drowsiness or sleepiness
Psychogenic TLOC C Lack of witnesses
C long standing history of psycho-somatic
symptoms
C TLOC occurs in supine positions with
absence of neurological deficits.

Table 2

PAEDIATRICS AND CHILD HEALTH xxx:xxx 4 Ó 2020 Elsevier Ltd. All rights reserved.

Please cite this article as: Naganna P et al., Approach to the pre-school child with sudden loss of consciousness, Paediatrics and Child Health,
https://doi.org/10.1016/j.paed.2020.06.003
OCCASIONAL REVIEW

 Prolonged loss of consciousness (longer than 5 min) FURTHER READING


 Exercise induced syncope Anderson JB, Willis M, Lancaster H, et al. The evaluation and man-
 Chest pain or palpitations agement of pediatric syncope. Pediatr Neurol 2016; 55: 6e15.
 History of cardiac disease https://doi.org/10.1016/j.pediatrneurol.2015.10.018.
 Familial history of cardiac conditions/arrythmias Bauersfeld U, Schmitt B. Sudden loss of consciousness in childhood
 Family history of sudden deaths, genetic conditions and [article in German]. Therapeutische Umschau. Rev Ther 1997; 54:
metabolic conditions 156e60. March 1997 PMID: 9333981.
 History or family history of migraine Brignole M, Moya A, De Lange FJ, et al. ESC Guidelines for the
 Neurological signs or symptoms diagnosis and management of syncope. 2018. Eur Heart J 2018;
o focal neurological deficits 39: 1883e948. https://doi.org/10.1093/eurheartj/ehy037. 01 June
o ataxia 2018.
o dysarthria Colman N, Nahm K, Ganzeboom KS, et al. Epidemiology of reflex
syncope. Clin Auton Res 2004; 14. https://doi.org/10.1007/
Treatment and general advice s10286-004-1003-3. October 2004.
The most common reflex or neurally mediated syncope (NMS) Driscoll DJ, Jacobsen SJ, Porter CJ, Wollan PC. Syncope in children
rarely needs medication or treatment. Upon diagnosis of NMS, a and adolescents. J Am Coll Cardiol 1997; 29: 1039e45. https://doi.
standardised approach of reassurance, general education and org/10.1016/S0735-1097(97)00020-X. April 1997.
lifestyle modification advice should be given. The patient needs Hussain N, Whitehouse WP. Transient loss of consciousness and
to be reminded that they can still lead a normal life without any syncope. Paediatric Epilepsy 2007; 2: 3e11. August 2007.
restrictions. General education and awareness for patients and Khan A, Hussain N, Whitehouse WP. Evaluation of staring episodes in
carers involves spotting and avoiding potential triggering factors children. Arch Dis Child 2012; 97: 202e7. https://doi.org/10.1136/
such as a hot environment, prolonged standing, dehydration, archdischild-2011-301111. August 2012.
fatigue and stress. Prompt recognition of prodromal symptoms Rose KM, Tyroler HA, Nardo CJ, et al. Orthostatic hypotension and the
and proactive steps should be encouraged to treat these, such as incidence of coronary heart disease: the Atherosclerosis Risk in
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Clinicians should stress the importance of taking enough org/10.1016/S0895-7061(99)00257-5. 01 June 2000.
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regularly exercising. It should be noted that caution should be students and their first-degree relatives. Eur Heart J 2006; 27:
exercised on fluid and salt intake if the patient has any under- 1965e70. https://doi.org/10.1093/eurheartj/ehl147. August 2006.
lying cardiac conditions or hypertension. Patients and carers Silverstein MD, Singer DE, Mulley AG, et al. Patients with syncope
should also be further directed towards information resources admitted to medical intensive care units. J Am Med Assoc 1982;
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Conclusions Task Force for the Diagnosis and Management of Syncope, European
Society of Cardiology (ESC), European Heart Rhythm Association
TLOC is the common problem in paediatric practice and though
(EHRA). Guidelines for the diagnosis and management of syncope
there are several causes, most cases are of neurally mediated
(version 2009). et al. Eur Heart J 2009; 30: 2631e71. https://doi.
syncope, which has excellent prognosis. The diagnosis can be
org/10.1093/eurheartj/ehp298. November 2009
made confidently via a history, physical examination, and in-
Van Dijk N, Boer KR, Colman N, et al. High diagnostic yield and ac-
vestigations such as an ECG. In most cases, extensive in-
curacy of history, physical examination, and ECG in patients with
vestigations are not necessary; however, an ECG must be
transient loss of consciousness in FAST: the Fainting Assessment
performed, and the patient must be referred for a cardiologist
study. J Cardiovasc Electrophysiol 2008; 19: 48e55. https://doi.
opinion if there are any red flags. If it has failed to be recognised
org/10.1111/j.1540-8167.2007.00984.x. January 2008.
and managed with reassurance, education and lifestyle modifi-
Wieling W, Ganzeboom KS, Saul JP. Reflex syncope in children and
cations, TLOC can have a significant impact on children and their
adolescents. Heart 2004; 90: 1094e100. https://doi.org/10.1136/
families. A
hrt.2003.022996. September 2004.

PAEDIATRICS AND CHILD HEALTH xxx:xxx 5 Ó 2020 Elsevier Ltd. All rights reserved.

Please cite this article as: Naganna P et al., Approach to the pre-school child with sudden loss of consciousness, Paediatrics and Child Health,
https://doi.org/10.1016/j.paed.2020.06.003

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