Breathholding spells
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Breath-Holding Spells (BHS)
Overview
Breath-holding spells are paroxysmal, involuntary episodes in infants and young children in which the child stops breathing, typically triggered by an emotional stimulus (pain, fear, frustration, anger). They are benign but alarming to parents. Onset is usually between 6 months and 2 years, with most resolving by age 5–6.
- Prevalence: ~5% of children
- Equal sex distribution (slight male predominance in some series)
- Family history positive in ~25–30%
Types
| Feature | Cyanotic BHS (Common) | Pallid BHS (Less Common) |
|---|---|---|
| Trigger | Frustration, anger, minor upset | Painful stimulus (minor injury, sudden fright) |
| Precipitant emotion | Anger / crying | Fear / sudden pain |
| Sequence | Cry → apnea → cyanosis → limpness ± LOC | Cry (brief/absent) → pallor → LOC → limpness |
| Mechanism | Cerebral hypoxia from apnea | Vagally mediated cardioinhibition (asystole) |
| % of BHS | ~60–70% | ~20–30% |
| Relation to epilepsy | Rare | Slightly higher association with syncope/vasovagal |
A mixed form exists. ~20% of children have both types.
Pathophysiology
Cyanotic BHS
- Child cries vigorously → breath held in expiration → cerebral hypoxia → loss of consciousness
- Thought to involve abnormal autonomic regulation of breathing
- Associated with iron deficiency anemia (lower hemoglobin impairs oxygen delivery)
Pallid BHS
- Painful/frightening stimulus → exaggerated vagal response → cardiac slowing or brief asystole → cerebral hypoperfusion → LOC
- Essentially a vasovagal syncope equivalent in infancy
- These children may later develop typical vasovagal syncope as adults
Clinical Sequence
Cyanotic spell (typical):
- Provocative event (frustration, pain)
- Child cries briefly, then holds breath in expiration
- Becomes cyanotic (perioral → facial)
- Goes limp ± loss of consciousness
- May have brief tonic posturing or clonic jerks (anoxic seizure — not epileptic)
- Spontaneous recovery in < 1 minute; child may be briefly post-ictal/sleepy
Pallid spell:
- Minor injury or fright
- Brief cry or no cry → sudden pallor
- Loss of consciousness ± brief stiffening/jerking
- Recovery within seconds to a minute
The entire episode is self-terminating. There is no true epileptiform activity.
Diagnosis
BHS is a clinical diagnosis based on history. No investigations are routinely required if the history is classic.
Workup to consider:
| Investigation | Indication |
|---|---|
| CBC | All children with BHS — rule out iron deficiency anemia (present in ~50%) |
| ECG | Pallid BHS — rule out prolonged QT syndrome, cardiac arrhythmia |
| EEG | Only if seizure disorder genuinely suspected; usually normal in BHS |
| Iron studies | If CBC suggests iron deficiency |
Key distinguishing feature: In BHS, the cry/emotional trigger comes before the event; in epileptic seizures, the cry (if present) is ictal and the trigger is absent or postictal.
Differential Diagnosis
- Epileptic seizures (most important) — no consistent emotional trigger; post-ictal confusion longer; EEG abnormal
- Long QT syndrome / arrhythmia — pallid spells; ECG essential
- Reflex anoxic seizures — overlap with pallid BHS
- Gastroesophageal reflux (Sandifer syndrome) — dystonic posturing
- Hypoglycemia — check glucose if atypical
- Cardiac syncope
- Intracranial pathology — extremely rare; atypical presentations only
Management
Parental Reassurance (Most Important)
- Explain the benign, self-limited nature
- Reassure that children do not die from BHS and do not develop epilepsy (as a direct result)
- Parents should not try to revive the child by mouth-to-mouth — it is unnecessary and reinforces anxiety
- Advise: lay child on side, do not restrain, keep safe from injury
Iron Supplementation
- First-line intervention even if anemia is only mild or borderline
- Elemental iron 5–6 mg/kg/day for 3–6 months
- Multiple RCTs and a Cochrane review show significant reduction in spell frequency with iron therapy — possibly by improving vagal tone or oxygen-carrying capacity
- Benefit seen even in non-anemic children with low ferritin
Behavioral Strategies
- Avoid reinforcing the behavior (e.g., giving in to every demand to prevent a spell)
- Consistent, calm parental response
- Do not punish or overprotect
Pharmacological (Rarely Needed)
| Drug | Use | Notes |
|---|---|---|
| Atropine | Pallid BHS with severe/frequent episodes | Reduces vagal cardioinhibition; rarely used |
| Piracetam | Refractory cyanotic BHS | Some evidence in older studies; not widely used in Western practice |
| Cardiac pacing | Pallid BHS with documented prolonged asystole | Exceptional cases only |
Anticonvulsants are NOT indicated and should be avoided — these are not epileptic events.
Prognosis
- ~50% resolve by age 4; ~90% by age 6–8
- Children with pallid BHS have a higher likelihood of developing vasovagal syncope in adolescence/adulthood
- No increased risk of epilepsy, intellectual disability, or developmental delay
- No mortality from BHS itself
Key Teaching Points
- Always exclude iron deficiency — treat empirically if present
- ECG is mandatory in pallid BHS to rule out long QT
- Tonic-clonic movements during a spell are anoxic, not epileptic — do not start anticonvulsants
- The trigger (cry/emotion) precedes the spell — this is the diagnostic clue
- Parental education and reassurance are the cornerstone of management
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