A case for your consideration

A reasonably terrified mom brings her 17 month old boy into the ED after an apparent seizure. She states that her older daughter took a piece of candy away from the toddler, and then her son started to cry, and then “turned blue” and fell to the ground. He had not eaten any candy yet, and did not appear to be choking. Mom tried a finger sweep of his mouth, and then blew in his face several times after which he was again breathing normally, and within about 90 seconds was pointing at his sister saying “candy, me, candy.” There is no history of events such as these in the boy or his family. His vital signs and physical exam are unremarkable, including no signs of head trauma and a normal nonfocal neuro exam. He was kept NPO by triage, and is saying that he wants candy. Mom is scared and wants to know what happened.

Breath holding spells

Breath holding spells occur when a child has episodic apnea, followed by loss of consciousness (and postural tone). Approximately 5% of children will have one or more. They usually occur between 6 and 18 months and generally not after age 6 years or before 6 months. An equal proportion of males and females have them. 80-90% will have their first before age 18 months. Up to 15-25% have multiple episodes daily – and most children will have anywhere between 1 and 6 episodes per week. In 1/4 of cases another family member has had one. They can be confused with seizures or syncope, and are often proceeded by discipline or conflict. However, there is no post-ictal phase and no loss of continence. There is no increase in the risk of seizure development later in life in children that have breath-holding spells.

Cyanotic breath holding spells

Anger, frustration or pain usually precede crying, forced expiration and apnea which leads to cyanosis. The transient hypocapnea and hypoxia can lead to loss of consciousness. The cyanosis often happens much quicker than would be expected with voluntary holding of the breath. Most recover within 1-2 minutes, though some kids can sleep for 30-60 minutes. They are theorized to occur because of transient increases in intrathoracic pressure secondary to a Valsalva maneuver, which leads to decreased pulmonary venous return, and transient decreased brain perfusion. In general these are quite reproducible.

Pallid breath holding spells

Usually seen in response to a painful event to the head or upper torso, children with pallid spells turn pale (as opposed to blue) and lose consciousness with little crying preceding the event. The LOC can occur up to 30 seconds after the trauma, and occurs in the following sequence: apnea > pallor and diaphoresis > loss of muscle tone. of Episodes that last more than a few seconds may display abnormal posturing, clonus, or outright convulsions. Children are sleepy for several minutes, but are still at their baseline level of alertness relatively quickly. Rarely the bradycardia leads to brief asystole which can last 5-10 seconds – though reports of periods of no cardiac activity lasting up to 30 seconds are out there.

Diagnosis, Management & Disposition

The diagnosis of breath holding spells can be made clinically in almost all cases. This diagnosis does require that you have no concerns for significant TBI, and that the vital signs, and the cardiac, pulmonary and neurologic exams are normal. Obtaining a clear history of what occurred before the event is quite important. If you are not 100% certain of the diagnosis an EKG to rule out long QT would be an appropriate test. Some studies have indicated that children with iron deficiency anemia may be more prone to breath holding spells. Testing of a hemoglobin level is an option in the ED, but it would also be appropriate to have this done at the child’s PMD. It is theorized that anemia may increase a child’s vulnerability to hypoxia. Iron also plays a part in catecholamine metabolism and neurotransmitter function, and therefore a deficiency may affect autoregulation of neurocardiogenic function as well.