Breath Holding Spells

Patient Education

What are breath-holding spells?

Breath-holding spells or breath-holding spasms are a benign, involuntary phenomenon seen in children between 6 months and 6 years of age. Either due to pain, fear, or in anger frustration the child cries and then holds the breath. For eg. child (who has severe temper tantrums) in one of his tantrums suddenly became blue and became unconscious, he is suffering from a breath-holding spell.

These spells occur in children with normal neurological exams and otherwise meeting age-appropriate developmental milestones. These episodes can cause children to lose consciousness and even precipitate a seizure.The frequency of these episodes can be decreased by distracting the child, avoiding emotional triggers, and providing psychosocial help to parents and children.

In addition, it is important to rule out certain conditions such as iron deficiency anemia and long QT syndrome when assessing a child and to follow up on any other red flags with a more detailed workup. Thankfully, the prognosis is excellent, and these spells usually resolve by age 6 and do not affect the child's subsequent neurological development.

How do children with breath-holding spells present?

Breath-holding spells occur only when the child is awake. There are 2 ways in which children with breath-holding spells present

• Cyanotic spells

• Pallid spells

Cyanotic spells are the most common variety though some children may present with both the varieties (mixed)

Etiology

Breath-holding spells were once considered attention-seeking behavior, but studies have shown that these episodes are not intentional and result from an involuntary reflex. While cyanotic and pallid are the 2 predominant types of breath-holding spells, some children can have a mixed phenotype.No preceding aura exists in either phenotype, and the spells self-resolve within 60 seconds.

Cyanotic breath-holding spells are more common, accounting for over 60% of patients.The trigger for these episodes is the anger or frustration of the child. The child typically cries briefly, becomes silent, stops breathing, and becomes cyanotic. This can result in loss of consciousness and precipitate a seizure, though the episode is self-limiting.A prolonged spell (>60 s) should prompt a workup for an alternate diagnosis.

The pallid form usually follows a painful or frightful experience.After an inciting event, the heart rate slows, the child stops breathing, loses consciousness, and becomes pale. Children may become sweaty and have body jerks or lose bladder control. Episodes are usually brief, and the child regains consciousness without any intervention.

Cyanotic spells

The entire episode may last less than one minute and the child generally regains full activity within a few minutes.

Pallid spells

Pathophysiology

The exact mechanism for cyanotic breath-holding spells is not clear. Pallid breath-holding is caused by an increased parasympathetic response, causing a reduced blood flow to the brain. This can be demonstrated by the fact that these spells can be incited by increasing the vagal tone via ocular compression.In both types of cases, the association of iron deficiency anemia indicates that if a child's oxygen-carrying capacity is reduced from anemia (including iron deficiency anemia, sideroblastic anemia, or transient erythroblastopenia of childhood), the child may be more prone to these spells and loss of consciousness.

How common are breath holding spells?

Breath-holding spells are seen in 5% of children between 6 months to 6 years of age. They are commonly seen in children between 12 and 18 months and usually disappear by 4-5 years of age.

How frequently does a child get an attack of breath-holding spells?

The frequency of breath-holding spells may vary from 1-2 times in a month to several times in a day.

Why does a child suffer from breath-holding spells?

Breath-holding spells are an involuntary phenomenon (the child does not do it deliberately). They are usually seen in children with temper tantrums and other behavioral disorders.

Do breath holding spells run in families?

Yes, a positive family history may be found in about 25% of the cases.

Why does a child with a breath-holding spell become unconscious?

It has been postulated that loss of consciousness in a cyanotic spell is due to centrally mediated inhibition of respiratory effect. In pallid spells, the vagally mediated bradycardia is through to be responsible.

How does one diagnose a case of breath holding spells?

Breath-holding spells are usually diagnosed clinically. However, seizures and syncope (due to cardiac or vasovagal stimulation) should be considered in the differential diagnosis. Seizures unlike breath-holding spells usually do not have a precipitating factor, are associated with post-ictal drowsiness and convulsive activity precedes the change in color.

Vasovagal syncope is rare in children below 12 years of age.

Usually, investigations are not necessary in a classical case of breath-holding spasm. However, an EEG and ECG may be done to rule out epilepsy and cardiac arrhythmias respectively. Inter-ictal EEG in breath-holding spasms is normal.

How does one manage a case of breath holding spells?

The most important aspect of treatment consists of parental support and reassurance. Though these episodes are innocuous, they usually cause a lot of parental fear and anxiety. Parents should be told about the involuntary nature of the attacks and cautioned against giving in to the child's wishes. They should be reassured that breath-holding spells are not dangerous and do not lead to epilepsy or brain damage. The parents should be encouraged to handle the episode in a relaxed manner. During an attack, the parent should not hold the child upright, instead should make him lie down flat to prevent head injury. Nothing should be put in the child's mouth as it could cause choking or vomiting. After the attack is over, the parents should not give in to the child's wishes.

Parents can also be taught how to prevent a spell. Some children can be distracted from their breath-holding if intervened before they become blue by distracting them or making them look at something interesting. The parents should be cautioned against running and picking up the child every time he cries to decrease an undue number of attacks.

A behavior modification program may help if a child has frequent tantrums. Parents should be reassured that long term prognosis is very good.

What pharmacologic treatment is available for breath holding spells?

Pharmacological treatment is usually not required. Anticonvulsants have no role in breath-holding spells. Atropine sulfate may be tried in children with frequent pallid spells. Though there are reports of iron therapy in breath-holding spells (as these children usually have iron deficiency anemia), its usefulness is still not completely documented.

What are the Differential Diagnosis?

Breath-holding spells are quite common; however, differential diagnoses to consider include the following:

• Congenital laryngeal stridor
• Arrhythmia
• Apnea of prematurity
• Genetic disorders
• Epilepsy
• Sepsis
• Nonaccidental trauma
• PDA-dependent congenital heart lesion

What is the prognosis of children with breath holding spells?

The prognosis is excellent. Most of the episodes resolve by the time the child is 4-5 years old. Children with pallid spells may have an increased incidence of syncope as adults.