History

Sandifer syndrome is most commonly mistaken for seizures.^{[3, 4, 6, 11]}The infant or child typically appears to have an alteration in mental status associated with the tonic posturing.

A relationship with feeding suggests the diagnosis of Sandifer syndrome, which commonly occurs after feeding.

Posturing is typically brief, temporally associated with feeding, paroxysmal, and absent during sleep. The child may have a sudden deviation of the head and neck to one side and the legs to the opposite side with a stretched-out appearance. Typically, the back is arched posteriorly with hyperextension of the spine, and elbows may be flexed and held posteriorly with hyperextended hips. Torticollis may be present.^{[3, 6, 11, 12]}

Although the intermittent stiff tonic posture and periods of crying and apparent discomfort may suggest seizures, in many cases the rhythmic clonic component, which may be present in seizures, is not described.

Various stiff, bizarre postures can be observed, with the following features noted:

Typically, the duration of the posture is 1-3 minutes.
This brief, paroxysmal pattern of posturing accounts for the fact that the movement observed in Sandifer syndrome may be mistaken for seizures.
During the posture, the infant may become very quiet or, less commonly, become very fussy. Fussiness and evident discomfort are most commonly observed as the posture abates
If a significant volume of gastroesophageal reflux is observed, even without actual vomitus, some infants and children may exhibit evidence of respiratory tract irritation as well, including cough, wheezing, and stridor, depending on the degree and volume of reflux. ^[13]

Physical Examination

In children with Sandifer syndrome without mental impairment, the physical examination findings are normal. Children with Sandifer syndrome with mental impairment may have evidence of spasticity and may receive a diagnosis of cerebral palsy.^[14]

Sandifer syndrome in infants is most commonly associated with normal physical examination findings. In older children, the syndrome may be associated with mental impairment or anatomic abnormalities of the gastrointestinal tract, such as hiatal hernia.

In infants with significant gastroesophageal reflux disease or unsuspected milk protein allergy, the physical examination may reveal evidence of poor nutrition, poor weight gain, and occult blood on fecal guaiac testing.

Differential Diagnoses

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Lehwald N, Krausch M, Franke C, Assmann B, Adam R, Knoefel WT. Sandifer syndrome--a multidisciplinary diagnostic and therapeutic challenge. Eur J Pediatr Surg. 2007 Jun. 17(3):203-6. [QxMD MEDLINE Link].
Obeid M, Mikati MA. Expanding spectrum of paroxysmal events in children: potential mimickers of epilepsy. Pediatr Neurol. 2007 Nov. 37(5):309-16. [QxMD MEDLINE Link].
Kostakis A, Manjunatha NP, Kumar A, Moreland ES. Abnormal head posture in a patient with normal ocular motility: Sandifer syndrome. J Pediatr Ophthalmol Strabismus. 2008 Jan-Feb. 45(1):57-8. [QxMD MEDLINE Link].
Kabakus N, Kurt A. Sandifer Syndrome: a continuing problem of misdiagnosis. Pediatr Int. 2006 Dec. 48(6):622-5. [QxMD MEDLINE Link].
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Shepherd RW, Wren J, Evans S, et al. Gastroesophageal reflux in children. Clinical profile, course and outcome with active therapy in 126 cases. Clin Pediatr (Phila). 1987 Feb. 26(2):55-60. [QxMD MEDLINE Link].
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Orenstein SR. Update on gastroesophageal reflux and respiratory disease in children. Can J Gastroenterol. 2000 Feb. 14(2):131-5. [QxMD MEDLINE Link].
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Media Gallery

Diagram illustrating the Nissen fundoplication. Note how the stomach is wrapped around the esophagus (360º wrap).

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Author

Pegeen Eslami, MD Associate Professor of Pediatrics, Division of Pediatric Emergency Medicine, Children's Hospital of Pittsburgh

Pegeen Eslami, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, International Pediatric Simulation Society

Disclosure: Nothing to disclose.

Coauthor(s)

Raj D Sheth, MD Chief, Division of Pediatric Neurology, Nemours Children's Clinic; Professor of Neurology, Mayo Clinic Alix School of Medicine; Professor of Pediatrics, University of Florida College of Medicine

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, Child Neurology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Sandifer Syndrome Clinical Presentation

History

Physical Examination

References

Tables

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