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Last updated: 05 June 2024

Plagiocephaly in infants

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Flat Head SyndromePlagiocephalycraniosynostosis

Plagiocephaly in Infants

Overview

  • Definition: Plagiocephaly, often referred to as "flat head syndrome," is characterized by an asymmetrical cranial deformation. It includes positional (deformational) plagiocephaly and craniosynostosis.
  • Incidence: Positional plagiocephaly is more prevalent, particularly since the "Back to Sleep" campaign for SIDS prevention. Craniosynostosis is less common but clinically significant due to potential neurodevelopmental implications.

Positional Plagiocephaly

  • Etiology: Results from prolonged external pressure on one area of the skull, typically due to consistent positioning. This is exacerbated by factors such as:
    • Supine Sleeping Position: Despite its efficacy in SIDS prevention, it increases the risk of positional flattening.
    • Reduced Tummy Time: Limited prone positioning while awake, essential for alleviating cranial pressure and promoting cervical strength.
    • Prematurity: Increased vulnerability due to prolonged NICU stays and a softer calvarium.
    • Multiple Gestations: Intrauterine constraints leading to positional molding.
    • Torticollis: Congenital muscular torticollis causing unilateral head preference.

Craniosynostosis

  • Etiology: Arises from the premature fusion of one or more cranial sutures, leading to abnormal cranial and facial morphology.
  • Clinical Types:
    • Sagittal Synostosis: Results in scaphocephaly, characterized by a long, narrow skull.
    • Coronal Synostosis: Leads to anterior plagiocephaly or brachycephaly, depending on whether unilateral or bilateral.
    • Metopic Synostosis: Causes trigonocephaly with a prominent midline ridge and hypotelorism.

Clinical Presentation

  • Positional Plagiocephaly:
    • Occipital flattening with ipsilateral frontal bossing.
    • Misaligned cranial base and asymmetrical ear positioning.
  • Craniosynostosis:
    • Distinct cranial shape alterations depending on the suture involved.
    • Potential for increased intracranial pressure and neurodevelopmental deficits in severe cases.

Diagnosis

  • Clinical Evaluation: Physical examination for cranial asymmetry, facial symmetry, and assessment of torticollis.
  • Imaging:
    • Positional Plagiocephaly: Primarily a clinical diagnosis; imaging is reserved for atypical cases.
    • Craniosynostosis: CT scans or MRI to confirm suture fusion and evaluate intracranial structures.

Management Strategies

  • Positional Plagiocephaly:
    • Repositioning Techniques: Education on alternating head positions during sleep, increased tummy time, and minimizing prolonged supine positioning when awake.
    • Physical Therapy: Particularly for infants with concurrent torticollis to improve neck muscle balance and promote symmetrical head movement.
    • Orthotic Devices: Cranial orthoses (helmet therapy) for moderate to severe cases or those unresponsive to repositioning by 4-6 months. Helmets apply gentle, constant pressure to direct head growth and correct asymmetry.
      • Timing: Optimal use is between 4 and 12 months of age when the skull is most malleable.
      • Duration: Typically worn for 23 hours a day over a period of several months, with periodic adjustments to the helmet to accommodate growth.
  • Craniosynostosis:
    • Surgical Intervention: Cranioplasty or endoscopic suturectomy, typically performed within the first year of life to allow for optimal brain growth and cranial reshaping.
    • Post-Operative Care: May include helmet therapy to maintain skull shape post-surgery and multidisciplinary follow-up for developmental assessments.

Prognosis

  • Positional Plagiocephaly: Favorable with early and appropriate intervention, with most cases resolving significantly with conservative management.
  • Craniosynostosis: Dependent on the type and timing of surgical intervention. Early surgery generally yields good outcomes, although complex cases may require multiple procedures and long-term monitoring.

Research and Development

  • Ongoing Investigations: Focus on the genetic basis of craniosynostosis, optimizing surgical techniques, and long-term neurodevelopmental outcomes.
  • Innovative Treatments: Advancements in minimally invasive surgical techniques and non-surgical interventions are being explored.

Resources for Practitioners

  • Support Networks: Connections with craniofacial specialists, genetic counselors, and multidisciplinary teams for comprehensive care.
  • Continuing Education: Access to up-to-date guidelines and emerging research through professional organizations and journals.

Cite this: Cite this: ICNApedia contributors.Plagiocephaly in infants. ICNApedia, The Child Neurology Knowledge Environment. 23 November 2024. Available at: https://icnapedia.org/knowledgebase/articles/plagiocephaly-in-infants Accessed  23 November 2024. 

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