Sandifer Syndrome

Updated: Apr 10, 2020
  • Author: Pegeen Eslami, MD; Chief Editor: Carmen Cuffari, MD  more...
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Practice Essentials

Sandifer syndrome is a condition that involves spasmodic torsional dystonia with arching of the back and rigid opisthotonic posturing, associated with symptomatic gastroesophageal reflux, esophagitis, or hiatal hernia. [1, 2]  Initial treatment consists of lifestyle changes, such as dietary modification and positioning. For example, positioning of the head may relieve abdominal discomfort caused by acid reflux. [3]  In patients with severe gastroesophageal disease that is unresponsive to conservative interventions and medical therapy, fundoplication may alleviate symptoms. [3] See the image below.

Diagram illustrating the Nissen fundoplication. No Diagram illustrating the Nissen fundoplication. Note how the stomach is wrapped around the esophagus (360º wrap).

Signs and symptoms of Sandifer syndrome

Posturing associated with Sandifer syndrome is often brief, temporally associated with feeding, paroxysmal, and absent during sleep. Typically, the back is arched posteriorly with hyperextension of the spine, and elbows may be flexed and held posteriorly with hyperextended hips.

Physical examination findings are usually normal in infants with Sandifer syndrome. In older children, the syndrome may be associated with mental impairment or abnormalities of the gastrointestinal (GI) tract, such as hiatal hernia.

Infants with significant gastroesophageal reflux disease (GERD) or unsuspected milk protein allergy may exhibit evidence of poor nutrition and insufficient weight gain.

See Presentation for more detail.

Diagnosis of Sandifer syndrome

Laboratory studies

A stool guaiac test may be useful to detect occult GI bleeding. If concerns exist about nutritional or metabolic status, screening tests such as basic chemistry panels, blood cell counts, or urinalysis may be helpful.

Imaging studies

Upper GI imaging (eg, fluoroscopic imaging of swallowed barium) may be useful to screen out anatomic abnormalities such as hiatal hernia.

Cranial magnetic resonance imaging may be helpful in defining the nature of neurologic deficits in children with mental impairment or in ruling out concomitant cranial anatomic abnormalities.

Other tests and procedures

Video-electroencephalography monitoring can help differentiate seizures from posturing related to reflux and can be combined with a pH probe/multichannel intraluminal impedance (MII) study to demonstrate the nature of the episodes and any correlation with findings of reflux.

Endoscopy with performance of esophageal biopsy is the most sensitive way to diagnose esophageal inflammation due to reflux; however, this invasive procedure should be reserved for patients with unusual presentations and refractory symptoms.

See Workup for more detail.

Management of Sandifer syndrome

Sandifer syndrome generally does not require treatment and typically resolves within the first 12-24 months of life. Initial interventions should be directed at the following lifestyle changes:

  • Modification of or attention to feeding habits
  • Adjustment of feeds (whether breast milk or formula)
  • Exclusionary diets
  • Positioning

Typical medications directed at GERD include acid suppressants (H2 receptor antagonists, proton pump inhibitors), buffers (antacids), and prokinetic agents. These medications are used to treat GERD in older children and adults, but data about their usefulness in infants under age 12 months are very limited.

In patients with severe, confirmed GERD that is unresponsive to medical therapy and conservative interventions and that interferes with growth and development, some evidence suggests that fundoplication may alleviate symptoms.

See Treatment and Medication for more detail.



Sandifer syndrome involves spasmodic torsional dystonia with arching of the back and rigid opisthotonic posturing, mainly affecting the neck, back, and upper extremities, associated with symptomatic gastroesophageal reflux, esophagitis, or the presence of hiatal hernia. [1, 2]

Pediatric neurologists may be the first to see patients with Sandifer syndrome because the primary care provider and the parents may believe that the spasms represent seizures. Pediatric emergency department physicians and gastroenterologists see patients with this complex with some frequency.

Although case reports of Sandifer syndrome are available, the syndrome is underrecognized. Delays in diagnosis are due to atypical presentations or cases in which the diagnosis is not part of the differential. [3, 4, 5, 6, 7]

The true pathophysiologic mechanisms of Sandifer syndrome remain unclear.



Dysfunction of the lower esophagus with resultant acidic reflux is thought to be the most common precipitating factor, with the episodes of posturing coinciding temporally with recent feedings. Although gastroesophageal reflux is common and indeed physiologic in infants, gastroesophageal reflux disease (ie, concerning symptoms or complications associated with the reflux) is much less common. [8]

Concerning symptoms may include evidence of pain (presumed to be the cause of the posturing), irritability, poor weight gain, sleep disturbance, and respiratory symptoms. Typically, empiric treatment for gastroesophageal reflux disease alleviates symptoms of Sandifer syndrome.  

Hiatal hernia may be associated with Sandifer syndrome, particularly in children older than infancy, and presumably exacerbates the gastroesophageal reflux.



International data

The incidence of Sandifer syndrome is unknown, although some evidence suggests that in clinical practice it occurs in fewer than 1% of children with gastroesophageal reflux. [3]

Race-, sex-, and age-related demographics

Race does not seem to influence incidence, and no sex predilection is recognized.

Typically, Sandifer syndrome is observed from infancy to early childhood. Peak prevalence is in individuals younger than 24 months. Children with mental impairment or spasticity may experience Sandifer syndrome into adolescence.




Morbidity consists of the discomfort associated with Sandifer syndrome. [9]  Infants may lose weight if persistent or severe gastroesophageal reflux disease (GERD) is present. Associated morbidities may also include the presence of a hiatal hernia and esophagitis.

Typically, Sandifer syndrome is not life-threatening. Many patients with the condition eventually outgrow the spasms in later childhood.


Sandifer syndrome per se is not associated with specific complications. However, misdiagnosis (eg, as a neurologic problem) may result in a significant delay in the diagnosis of associated GERD and timely intervention. [10]