This is a typical scene: A little child is playing happily, something upsets her, she exhales forcefully with a brief, shrill cry -- but she doesn't take another breath. You wait, but she still doesn't breathe. She looks as if she's crying, but no sound emerges. She begins to turn blue, her face strained, and still she is not breathing. Now she is unconscious, unresponsive, limp; the sight of her lifeless body is terrifying. Now her back arches, and her blue arms and legs begin to jerk uncontrollably. Your heart is pounding, frantic...

Breath-holding spells are perhaps the most frightening of the common, benign behaviors of childhood. Desperate parents often want to splash cold water on the child's face, start mouth-to-mouth resuscitation, or even begin CPR.

Breath-holding spells (BHS) are dramatic, involuntary episodes that occur in otherwise healthy children. These episodes are often frightening to parents and worrisome to physicians. Because they can mimic serious or even life-threatening conditions, these generally benign spells require careful evaluation.

Thankfully, breath-holding spells resolve spontaneously soon after the child passes out, and unless the fall hurts the child, she will be fine afterwards. The spell usually resolves within 30 to 60 seconds, with the child catching her breath and starting to cry or scream. Sometimes children will have real seizures as part of breath-holding spells, but these brief seizures are not harmful, and there is no increased risk of the child's developing a seizure disorder.

Breath-holding spells occur in about 5% of children.

Breath holding spells are not contagious.

Childhood breath-holding conjures up an image of a stubborn toddler willfully holding his breath until he gets what he wants. The reality is quite different, however.

The typical breath-holding episode begins when a child becomes upset, is startled, or suffers a minor injury, and then begins to cry. Crying may be brief or prolonged, but typically, after a few cries, the child becomes silent and apneic in what is described as noiseless expiration. This stage quickly is followed by a dramatic change in skin color. The skin becomes cyanotic or pallid or has a mixed-color appearance.

In simple breath holding spell, the event resolves with no associated syncope or postural change. In severe breath holding spell, however, subsequent loss of consciousness and change in postural tone do occur. Usually the child falls limp, and occasionally a few myoclonic jerks may be observed. In some cases, a brief period of increased muscle tone, or opisthotonos, may be seen after or instead of limpness.

The entire episode, which lasts from several seconds to more than a minute, may end with a sudden, deep inspiration or with the return of normal breathing. Especially with severe breath holding spell, the child may be drowsy for a few moments before recovering completely and resuming normal activities.

Breath-holding spells shine a brilliant spotlight on one of the biggest challenges of parenting. Parents do not like to disappoint the little children that they love so much.

Moreover, parents don't want to get into yet another battle with the children in the short run it is always easier to give in to a tantrum than to do what they instinctively feel is best. For parents of breath-holding children, this crucial struggle of parenthood is powerfully amplified.

The first time a spell occurs, the parents should have the child examined by a doctor. Because breath-holding spells do share several features in common with seizure disorders, the two are often confused. In epileptic seizures, a child may turn blue, but it will be during or after the seizure, not before.

If the doctor confirms that the event was indeed a breath-holding spell, it is a good idea to check for anemia. Treating the anemia, if present, will often decrease the frequency of passing out.

Signs and Symptoms

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

1. Cyanotic spells.
2. Pallid spells.

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

Cyanotic spells:

They are often provoked by an upsetting situation, in anger or in frustration. The child usually cries or screams loudly and then the cry gradually becomes noiseless as child open the mouth and holds the breath in expiration for about 20 –30 sec (apnea). The child turns blue (cyanotic) and then the child may again start breathing or may proceed to lose consciousness. Sometimes, fits or seizure like activity may follow the loss of consciousness.

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

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 bradychardia is through to be responsible.

Pallid spells:

They are usually seen following a painful or fearful experience. The child turns deathly pale instead of blue or purple. These pallid spells are involuntary and unpredictable. They are brought on by a sudden startle, such as falling and striking the head. The child stops breathing, often loses consciousness within a single gasp or cry, goes limp, passes out, and rapidly drains of color. Pallid breath-holding spells also resolve spontaneously.

There is an even less common type of breath-holding spell associated with a rare genetic condition called familial dysautonomia; these involuntary spells occur in children who are already acting seriously ill.

Epidemiology

Breath holding spells are a fairly common pediatric problem. Breath-holding spells occur in about 1 in 20 children.

Simple spells occur in 27% or more of healthy children, and severe episodes may be seen in as many as 4.6%.

In most children who have breath holding spells, begin by age 12 months, although some children begin experiencing them as early as 2 months of age.

Usually, breath-holding events with cyanotic skin color change begin between a child's neonate period and 18 months of age.

For spells with pallid skin color, the age at onset is 12 to 24 months. By 24 months, almost all children who experience breath holding spells have had their first episode. Among 384 children studied by Livingston, the mean age at onset was 12 months (range, 3 months to 4 years).

By the time patients are 4 years old, about half of breath-holding cases have spontaneously resolved; by age 6, about 90% have done so; and by age 7 or 8, virtually all have resolved.

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.

The spells occur sporadically, but when they do occur, it is not uncommon for there to be several spells within a single day. Once parents have witnessed one breath-holding spell, they can often predict when another one is about to happen.

Frequency

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

Spells can occur as often as several times a day or as rarely as once a year. More commonly, patients have several episodes a week, and overall, occurrence ranges from daily to monthly. The greatest frequency of events tends to be in the second year of life.

Cause

These spells are provoked by the child's not getting her own way. They can be an attempt to exert control on the circumstances around her. Breath-holding is quite rare before 6 months of age. It peaks as children enter the twos, and disappears finally by about age five.

Other Possible explanations

Because they occurred in the setting of anger, agitation, or frustration, breath holding spells were thought for many years to derive from emotional or behavior problems.

In the past, children who had them were described as stubborn, disobedient, and aggressive. In 1993, however, DiMario and Burleson3 found no significant differences between the behavior profiles of breath-holders and those of other children.

Instead, several different physiologic mechanisms have been proposed. For some children with breath holding spells, especially those with the pallid type, noxious stimuli may lead to centrally mediated cardiac inhibition through the vagus nerve. In turn, this mechanism may induce bradychardia or brief a systole and subsequent spells. This cardiac phenomenon has been demonstrated repeatedly in patients through the use of ocular compression.

In children with the cyanotic type of breath holding spells, about 25% have a positive reaction to ocular compression; in those with the pallid type, the percentage increases to 61% to 78% .Similarly, cyanotic episodes may be caused by central inhibition of respiratory movements, again mediated through the vagus nerve. In both situations, cerebral hypoxia results.

In other patients the problem may be genetic, resulting in a more generalized deregulation of the autonomic nervous system. The role of reduced central nervous system sensitivity to hypoxia and hypercapnia, as well as abnormalities in pulmonary reflexes and lung mechanics, also has been studied.

How can breath holding episodes be prevented?

If the spells are frequent or severe, preventive medications may be prescribed for some types of breath holding.

Most would expect that a breath-holding spell would be difficult. Most are surprised, however, to find that in many ways, the biggest challenge is life between spells. Parents become timid about setting limits or disappointing their children because of the very real possibility of provoking another spell. For all of us, love consists of having the courage to act in spite of our fears.

Diagnosis

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 are normal.

Differential Diagnosis

Because potentially life-threatening conditions may present similarly to breath holding spells, diagnosis of the benign condition can be difficult.

Therefore, evaluation of breath-holding episodes requires the consideration and elimination of other, more worrisome explanations.

Seizure disorders often are the first entity to exclude because breath holding spells can closely resemble them, particularly when myoclonic jerks are observed.

Likewise, in episodes with vivid color change and, in some cases, sudden loss of consciousness, cardiac problems and rhythm disturbances (including congenital QT syndrome) must be considered.

Orthostatic syncope and apnea are also differential diagnostic considerations.

Differentiation of severe Breath holding spells from generalized seizures and cardiac disturbances
Feature	Severe Breath holding spells	Generalized seizures	Cardiac disturbances
Age at onset	Often infancy	Rarely infancy	Variable
Family history of BHS	Often positive	None	None
Precipitating event	Usually present	Usually absent	Usually absent
Sleep state	Always awake	Asleep or awake	Awake, often with stressor
Pallor or cyanosis	Always; before syncope	Variable; after syncope	Variable
Myoclonic jerks	Variable; few beats	Usually	Absent
Incontinence	Uncommon	Common	Absent
BHS, breath-holding spells.

Although it is a much less likely scenario, breath holding spells may be secondary to CNS lesions and malformations, such as Arnold-Chiari malformation.

Spells may be associated with developmental disorders, such as Rett syndrome, or with Riley-Day syndrome, a familial dysautonomia. However, these uncommon entities are themselves associated with multiple other findings and can be excluded readily on the basis of history and physical examination results.

Lastly, sporadic reports have linked breath holding spells with underlying hematological abnormalities, including transient erythroblastopenia of childhood and iron-deficient states. In the latter case, treatment with iron supplements was found to significantly reduce the number of spells.

Evaluation

Thorough history taking, including a detailed description of the episode, is an essential starting point in the evaluation. In particular, close attention should be paid to the circumstances and sequence of events at the time of the spell. This information can offer important diagnostic clues.

For example, most breath holding spells are preceded by agitation and crying, in contrast to seizures, cardiac disturbances, and orthostatic syncope, which often occur with no emotional provocation. Also, without exception, cyanosis or pallor is seen in breath holding spells before other manifestations, such as syncope or postural changes; color change with seizures, if noted at all, tends to occur after loss of consciousness.

In an older child with breath-holding episodes, additional diagnostic clues from the patient's history may include urinary incontinence, which often occurs with certain types of seizures but is uncommon with benign breath holding spells. Also, caregivers report that these spells occur with the child fully awake and alert. This characteristic differentiates breath holding spells from apneic conditions, which generally are linked to sleep states.

Physicians need to elicit information about whether a child has apparent distress with eating or other physical activities or experiences chest pain or other associated physical symptoms. These findings suggest something other than benign breath holding spells, such as an underlying cardiopulmonary problem.

Family history is another key element in patient assessment. Previous observations have noted that perhaps 20% to 30% of children with benign breath holding spells have family members who were similarly affected during childhood.

Also noteworthy, family histories of these children often reveal an increased incidence of syncope in adult family members.

Inquiry should include family history of neurologic disorders as well as cardiac diseases--especially events that occurred early in life or were associated with sudden death.

In addition, data from more detailed analyses suggest an autosomal dominant pattern of inheritance in some cases of severe breath holding spells.

A complete physical examination should include assessment of the child's general appearance, behavior, growth, and development. The cardiovascular system requires particular attention, including careful auscultation of the heart for murmurs and rhythm irregularities.

Likewise, a thorough neurological evaluation to identify focal deficits is important. A complete blood cell count can identify hematological abnormalities, and an electrocardiogram can rule out prolonged QT syndrome.

If assessment findings are significant or if the diagnosis is still uncertain, additional evaluation may be needed. If history taking and physical examination suggest seizures or other CNS disease, electroencephalographic testing is recommended, and referral to a pediatric neurologist should be considered. If a cardiovascular problem is suspected, it would be appropriate to use a Holter monitor and consult a pediatric cardiologist.

Prognosis

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.

The only significant finding on subsequent follow-up of children with breath holding spells was a mildly increased incidence of syncope later in life, especially in childhood or adolescence. Later syncope is rare in children with the cyanotic type of BHS, whereas it occurs in 17% of those with the pallid type.

Management

The most important aspect of treatment consists of parental support and reassurance. Though these episodes are innocuous, they usually cause lot of parental fear and anxiety.

It is most important to assure parents that although breath holding spells are frightening to observe, they are benign and children will outgrow them. Also, parents should be told that evidence suggests no serious long-term effects of benign breath-holding episodes in otherwise healthy children; these patients do not have increased risk of epilepsy or other neurological problems.

Adequate education of caregivers about breath holding spells and alleviation of their anxiety about these episodes are essential. Because of fear, parents may try to prevent every conflict or minor mishap in a child's life. Such efforts are neither practical nor possible and may lead parents to overindulge their child or to forego appropriate discipline to pacify him or her. Behavior problems may ensue.

A thorough understanding of the benign nature of this problem can help parents avoid these pitfalls and improve how they deal with the episodes.

Nevertheless, when a child with breath holding spells becomes upset and cries, reasonable efforts to calm the child should be made. If an episode occurs despite these measures, observation of the child and prevention of injury are generally all that is required.

In cases in which a child loses consciousness, the child should be placed in a lateral supine position to help avoid injury and possible aspiration. If a spell occurs while eating and food occludes the oral airway, the airway obviously should be cleared. Other resuscitative efforts are not necessary. Once an episode has resolved, the child should be reassured. However, drawing excessive attention to the event or expressing extreme worry to the child should be avoided.

Parents can also be taught how to prevent a spell. Some children can be distracted from their breath holding if intervened before they becomes 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.

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.

The parents' most important job, however, is to not reinforce the breath-holding behavior. A parent reinforces this behavior by bending to the child's will or by paying more attention to her when she has these spells.

Instead, if they are certain she hasn't choked on something, place her in a safe spot (without giving in to whatever she held her breath to achieve) and ignore her behavior.

Behavior modification program may help if a child has frequent tantrums. Parents should be reassured that long term prognosis is very good. Treating the anemia, if present, will often decrease the frequency of passing out.

Medication

Although some patients receive medication for breath holding spells, drugs are not generally indicated. In severe cases with associated bradycardia or asystole or in patients with multiple daily episodes, a 0.1-mg dose of oral atropine three times daily has been found to be effective in preventing breath holding spells.

Oral theophylline, transdermal scopolamine, and pacemakers also have been used. Anticonvulsants have not proved useful in prophylaxis of benign breath holding spells. Referral to a pediatrician is recommended in all cases in which pharmacologic therapy is being considered.

Anticonvulsants have no role in breath holding spells. Atropine sulphate 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 .

Summary

Breath holding spells are benign and self-limited. However, they require careful evaluation to evaluation to exclude more serious problems. Thorough history taking, physical examination, and limited screening studies can establish the diagnosis. Consultation should be considered if the clinical situation warrants. Treatment includes parental reassurance and education about the condition. Patient care involves attempts to calm the child, but if such care proves unsuccessful, observation and protection from injury are generally all that is required during an episode. Medication is not indicated except in severe cases.

Homoeopathic Repertorial References

Respiration; HOLDING the breath

Asaf; Bell; Chin; Cic; Coff; Cupr; Dros; Mez; Mosch; Op; Puls; Ruta; Zinc

DR. SUMIT GOEL M.D. (Hom)

DR. AMITA AGARWAL BHMS

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