Sandifer syndrome (SS) is characterized by unusual, paroxysmal movements and posturing of the head, neck, and trunk, such as arching of the back or torticollis (a twisted neck). These movements are a direct result of severe gastroesophageal reflux disease (GERD). The condition is not a primary neurological disorder, but rather a movement disorder secondary to intense discomfort in the digestive tract. The condition is rare, occurring in less than one percent of infants with GERD, but the alarming nature of the episodes causes significant parental distress.
Understanding the Underlying Cause of the Movements
The abnormal movements that define Sandifer syndrome are a physical manifestation of pain caused by stomach acid flowing back into the esophagus. When severe GERD or esophagitis occurs, the stomach contents irritate the delicate lining of the food pipe, causing a burning sensation. The infant’s body then involuntarily attempts to find a position that minimizes this intense internal discomfort.
This reaction is hypothesized to be a protective or compensatory mechanism, often referred to as self-splinting. By arching the back and extending or twisting the neck, the infant may unknowingly reduce the amount of acid flowing back up the esophagus or promote the clearance of acid already there. These dystonic episodes, which involve involuntary muscle spasms, are closely linked temporally to feeding and the subsequent reflux event. The physiological link between the irritated esophagus and the muscles of the head and neck is thought to relate to shared nerve pathways.
The Typical Timeline and Resolution
Sandifer syndrome is overwhelmingly self-limiting and temporary. The condition resolves once the underlying gastroesophageal reflux disease is successfully managed or naturally outgrown. This natural resolution typically occurs as the infant matures, generally taking place between 12 and 24 months of age.
The spontaneous improvement of Sandifer syndrome is directly tied to the natural development of the infant’s digestive system. Over the first two years of life, the lower esophageal sphincter, the muscle that acts as a valve between the esophagus and stomach, strengthens and functions more effectively. Additionally, the physical mechanics contributing to reflux improve as a baby spends more time upright and begins consuming a diet with more solids.
The prognosis for children with Sandifer syndrome is excellent, with no reported long-term complications once the GERD is addressed. Treatment aimed at controlling the reflux dramatically decreases the frequency and severity of the dystonic episodes, leading to their eventual disappearance. If the condition is severe enough to cause secondary complications like iron deficiency anemia, this also typically resolves after a few months of successful GERD treatment.
Managing Symptoms and Underlying Reflux
The resolution of Sandifer syndrome is accelerated by active management focused on treating the root cause, which is the gastroesophageal reflux. Initial interventions often involve simple, non-pharmacologic adjustments to feeding and positioning.
- Keeping the infant upright for at least thirty minutes after a feeding uses gravity to help keep stomach contents down and away from the sensitive esophageal lining.
- Modifying the feeding itself can also be effective, which may include thickening breast milk or formula with a small amount of rice cereal.
- If a cow’s milk protein allergy is suspected as a contributing factor to the esophageal inflammation, a switch to a hydrolyzed protein formula or the elimination of dairy from a breastfeeding parent’s diet may be necessary.
If these lifestyle and dietary changes do not sufficiently control the reflux, pharmacological treatment may be introduced to reduce the acidity of the stomach contents. Medications often used include acid suppressants, such as H2 receptor blockers or proton pump inhibitors (PPIs). These medications reduce the production of stomach acid, making any reflux episodes less irritating and painful, which in turn removes the trigger for the abnormal posturing.
Distinguishing Sandifer Syndrome from Other Conditions
The dramatic movements and posturing of Sandifer syndrome frequently cause parents to fear their child is experiencing a seizure or has a serious neurological disorder. The movements of SS are non-epileptic, meaning they are not caused by abnormal electrical activity in the brain. The child remains fully conscious during the episode.
The movements are almost always temporally associated with feeding or reflux events, and they do not occur when the child is asleep. Conversely, true seizures can happen at any time and often involve rhythmic, clonic components or an altered state of awareness. If the clinical picture is unclear, diagnostic tests are used to confirm the diagnosis and rule out other conditions.
A normal electroencephalogram (EEG) can effectively rule out epilepsy. A 24-hour pH monitoring study or an upper endoscopy can confirm the presence and severity of the underlying GERD. This careful differentiation is necessary because misdiagnosis can lead to unnecessary and potentially harmful treatment with anti-epileptic medications. The successful resolution of the movements following GERD treatment ultimately confirms the diagnosis of Sandifer syndrome.