Sandifer Syndrome in Pediatric Patients

Sandifer syndrome is a condition in which the infant presents with seizure-like episodes of twisting and writhing during feeding.

Written and verified by the dermatologist Maria del Carmen Hernandez.

Last update: 28 June, 2023

What is Sandifer syndrome?

Sandifer syndrome is a rare neurobehavioral disorder in infants that combines digestive symptoms, such as gastroesophageal reflux (GERD), with abnormal head and neck movements.

The latter are thought to be a form of compensation or protective response of the body to the irritation of the esophagus caused by reflux. Another characteristic is that it usually affects children between the ages of 18 and 24 months.

Therefore, most evident in infants are episodes of twisting and arching of the neck, twisting of the body, and lateral twisting of the head.

These movements may seem strange and worrisome, as many parents often believe that they’re seizures. However, unlike seizures, Sandifer syndrome isn’t associated with abnormal electrical activity in the brain.

The causes of Sandifer syndrome

While there’s no certainty as to the exact cause, there’s thought to be a close relationship with esophageal, gastric, and hiatal hernia problems. All of these can trigger gastroesophageal reflux disease (GERD), which is common in childhood, according to a publication in the International Journal of Molecular Sciences.

GERD often causes chest pain and throat discomfort, so it’s thought that these movements are just the child’s response to the pain or a way to relieve it. The investigative book called Sandifer Syndrome suggests that 16% of participants with paroxysmal episodes (i.e., episodes that resemble a seizure) had GERD as an underlying cause.

Furthermore, scientific evidence for its origin in the nervous system is still lacking, even if the episodes resemble a seizure.

The cardinal symptoms of the syndrome

The main symptoms of this rare syndrome are dystonia and torticollis. The latter refers to involuntary movement of the neck, while dystonia is movement and twisting caused by involuntary muscle contractions. Also, the combination of such movements often results in arching of the spine in children.

Additional symptoms of Sandifer syndrome include the following:

• Gurgling sounds
• Rumination
• Coughing
• Nodding of the head
• Sleep problems
• Constant irritability
• Recurrent pneumonia
• Poor weight gain
• Respiratory difficulty
• Slowed feeding

The episodes are characterized by movements in which the baby turns their neck, arches their spine, and throws their head backward. This movement is what resembles a seizure. They last about 3 minutes or less and may occur up to 10 times during the course of the day.

How is Sandifer syndrome diagnosed?

Some of the symptoms are difficult to differentiate from other neurological conditions, such as epilepsy. Therefore, a physician will order an electroencephalogram (EEG) to analyze the electrical activity of the brain and rule out other causes.

If the EEG is normal, a pH test through the esophagus is ordered to corroborate any signs of heartburn for 24 hours. Multichannel intraluminal impedance breath testing is also helpful in diagnosing gastroesophageal reflux, as it measures the flow of liquids, air, and solids in the esophagus.

In addition, keeping a record of feeding times and when symptoms appear can be helpful in order for the physician to detect if there’s any characteristic pattern of the syndrome.

Therapeutic options for Sandifer syndrome

Treatment of Sandifer syndrome involves reducing the symptoms of gastroesophageal reflux disease. Therefore, according to an article published in the journal Drugs in Context, making certain changes in eating habits is advised, such as the following:

• Don’t overfeed the baby.
• Keep the baby in an upright position after feeding for at least half an hour.
• If the doctor suspects sensitivity to cow’s milk protein, eliminate dairy foods from the breastfeeding mother’s diet or change the formula to a hydrolyzed one.

In the event that none of these indications are helpful, the physician may prescribe the following medication:

• H2 receptor blockers
• Antacids
• Proton pump inhibitors

The doctor should always be consulted about the possible risks that this type of medication causes in infants. In fact, these drugs don’t always reduce the symptoms of the syndrome.

Rarely, a surgical procedure called Nissen fundoplication, which wraps the upper part of the stomach around the lower esophagus, may be required. This prevents acid from ascending into the esophagus and causing pain. If a hiatal hernia is present, corrective surgery of the defect may also be advised, as the results are usually very favorable.

Sandifer syndrome: A transient condition

In conclusion, gastroesophageal reflux usually resolves on its own around 18 to 24 months of age, when the esophageal muscles finish developing. Therefore, Sandifer’s syndrome also resolves when this occurs.

While not a serious condition, it can be painful and cause digestion problems, affecting growth. Therefore, if you notice any of the above symptoms, don’t hesitate to see a doctor.