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Craniosynostosis syndrome in Child
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Craniosynostosis syndrome in Child

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Contributors: David Sullo MD, Eric Ingerowski MD, FAAP
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Synopsis

Craniosynostosis is disruption in the growth pattern of the infant's skull when one or more cranial sutures fuse prematurely, causing deformity of the skull as it continues to grow where sutures have not yet ossified. This must be distinguished from the much more benign diagnosis of positional plagiocephaly. Age of closure ranges from 2-72 months, depending on the suture location. There are many types of craniosynostosis, each leading to a distinctive pattern of skull deformity based on the sutures involved. Syndromic craniosynostosis is characterized by multiple premature closures with other genetic anomalies, including Pfeiffer, Carpenter, Apert, Muenke, Crouzon, and Saethre-Chotzen syndromes.

Associated anomalies can include midface retrusion, hypertelorism, exorbitism, ear anomalies, hearing loss, eyelid ptosis, and syndactyly. Complications include increased intracranial pressure; impairment of brain growth due to constricted space; cranial nerve impingement; developmental, hearing, vision, speech, and cognitive deficits; psychosocial difficulties; poor weight gain; and poor feeding.

Management is multidisciplinary and often requires surgical correction.

Codes

ICD10CM:
Q75.0 – Craniosynostosis

SNOMEDCT:
57219006 – Craniosynostosis syndrome

Differential Diagnosis & Pitfalls

  • Positional plagiocephaly
  • Genetic disorders

References

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Last Updated: 10/31/2016
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Craniosynostosis syndrome in Child
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Craniosynostosis syndrome (Craniosynostosis Syndrome) : Craniosynostosis, Hypertelorism, Skull defect, Poor feeding, Impaired cognition
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