Breath Holding Spells

Breath holding spells are involuntary, non-epileptic episodes in which a child, provoked by anger, frustration, pain or fright, holds their breath in expiration, becomes cyanotic or pale, loses consciousness, and sometimes has stiffening or brief clonic jerks. They are reflexive, not voluntary or manipulative.

• Occurs in 4 to 5 percent of healthy children, onset between 6 and 18 months (rarely before 6 months), peak 1 to 3 years, resolves by 5 to 6 years in more than 90 percent. Family history in about 20 to 35 percent.
• Types:
  • Cyanotic (about 60 to 70 percent): provoked by anger or frustration; the child cries, then holds breath in expiration with cyanosis, going limp or rigid.
  • Pallid (about 20 percent): provoked by minor injury, fall or fright; minimal crying; reflex bradycardia or asystole of 2 to 4 seconds from vagal activation causes pallor and loss of tone.
  • Mixed (about 20 percent): features of both.
• Associations: iron deficiency (with or without anaemia) in up to 20 to 50 percent of affected children, and treatment reduces spells; autonomic dysregulation.
• Prognosis: benign; no association with epilepsy, though there is a slightly higher risk of vasovagal syncope in later life.

• A clear trigger, short cry (or no cry), expiratory apnoea, colour change (blue around lips and face or pallor), loss of tone and consciousness for 10 to 60 seconds, occasionally with stiffening (opisthotonus) or a few brief jerks, then spontaneous recovery with drowsiness for a few minutes (rarely longer); no postictal confusion lasting minutes to hours.
• Events during wakefulness only; frequency varies from daily to a few per year.
• Examination between spells is normal: growth, development, pallor (anaemia), heart sounds, neurological examination.
• Red flags (consider seizures, cardiac arrhythmia, other causes): age under 6 months; spells without clear provocation; occurrence during sleep; prolonged episodes over 1 to 2 minutes; prolonged post-event drowsiness or focal weakness; developmental delay; abnormal neurological findings; exertional episodes; palpitations; family history of sudden unexplained death, long QT syndrome, or arrhythmia; ECG abnormalities; failure to thrive; stridor or respiratory disease.
• Differentials: epileptic seizure, long QT syndrome, other arrhythmias, gastro oesophageal reflux (Sandifer), laryngospasm, reflex anoxic seizures, Munchausen by proxy, aspiration or choking.

• Clinical, from a detailed history (video recording by parents is useful).
• Full blood count, ferritin (and serum iron studies) in all children with frequent spells to identify iron deficiency.
• 12 lead ECG (exclude long QT and conduction abnormalities) for pallid spells, syncope like episodes, family history of cardiac disease or sudden death, or atypical features.
• EEG and neuroimaging are not needed for typical spells.
• Further investigation (Holter, tilt, neurology review) only if atypical.

• Reassure the parents that spells are not harmful, do not cause brain damage, and resolve with age; explain that the child is not doing it deliberately.
• During a spell: stay calm, place the child on their side on a safe surface, loosen clothing, stay with them, do not put anything in the mouth, do not shake, slap or splash the child, and do not start rescue breathing unless apnoea continues beyond about 1 minute with no sign of breathing returning; call emergency services if the child does not recover within 1 to 2 minutes, appears injured, or is blue with no breathing.
• Behavioural measures: consistent, calm discipline; avoid giving in to the child's demands after a spell (this reinforces tantrums) and avoid excessive protection; distraction at the first signs of crying; normal routine.
• Treat iron deficiency: ferrous sulfate (elemental iron 3 to 6 mg/kg/day orally in 1 to 2 divided doses, with vitamin C containing juice, apart from milk) until haemoglobin normalises, then continue for 3 months to replenish stores; recheck haemoglobin and ferritin; iron treatment reduces spells in iron deficient children (even without anaemia).
• Pharmacological therapy is rarely needed: for severe pallid spells with documented asystole, atropine or pacing is a decision for paediatric cardiology; piracetam (40 to 100 mg/kg/day in 2 to 3 divided doses) has been used in specialist practice; anticonvulsants are ineffective.
• Follow up at 4 to 8 weeks to review iron response and parental coping; refer to paediatric neurology or cardiology for atypical features.