eMedicine Specialties > Neurology > Pediatric Neurology

Craniosynostosis: Treatment & Medication

Author: Raj D Sheth, MD, Professor of Neurology, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic
Coauthor(s): Philipp R Aldana, MD, FAAP, Assistant Professor of Neurosurgery and Pediatrics, University of Florida College of Medicine - Jacksonville; Director of Clinical Services, Pediatric Neurosurgery Center, University of Florida Health Sciences Center; Bermans J Iskandar, MD, Director, Associate Professor, Departments of Neurological Surgery, Pediatrics, Children's Hospital, University of Wisconsin Hospital and Clinics
Contributor Information and Disclosures

Updated: Nov 12, 2009

Treatment

Medical Care

In the past 30 years, a better understanding of the pathophysiology and management of craniosynostosis has developed. Currently, surgery is usually cosmetic for infants with fusion of 1-2 sutures that result in a misshapen head. For infants with microcephaly (ie, secondary craniosynostosis), surgery usually is not required.

  • In patients with microcephaly, investigate the cause of microcephaly.
  • Measure head circumference longitudinally and monitor development. Ensure normal brain growth in patients with primary craniosynostosis.
  • Carefully monitor signs and symptoms of elevated intracranial pressure.
    • Examine the fundi and alert parents to report persistent vomiting or lethargy promptly.
    • Should elevated intracranial pressure be suspected, an emergent neurosurgical consult would be appropriate.
  • To preserve visual function in patients with elevated intracranial pressure, an emergent ophthalmological consult would be appropriate.
  • Address the cosmetic appearance of the skull abnormality.

Surgical Care

Surgery typically is indicated for increased intracranial pressure or for cosmetic reasons.

  • Do not operate in patients without raised intracranial pressure until considering the following:
    • If the shape of the head does not improve by age 2-4 months, then the abnormality is unlikely to resolve with age. Infants have a large head relative to body size. Deformity appears more prominent in the young infant and may be less obvious with age.
    • Handle minor deformities conservatively. As the child grows and more hair appears, the visible abnormality may decrease.
  • Consideration for cosmetic surgery is dependent on the age of presentation and on which sutures have fused prematurely.
    • Cosmetic surgery is performed in infants aged 3-6 months in the author's practice, although a variety of approaches exist among different surgeons.
    • Surgery in younger infants may result in a relatively large loss of blood volume.
    • Surgery in infants older than 8 months may be associated with a slowing of skull growth. Thus, the surgical defect may not be covered adequately by bone growth.
  • Infants with a defined syndrome causing craniosynostosis should be evaluated early for surgery.
    • Results are best when surgery is performed in infants younger than 6 months.
    • Patients with associated facial deformities may need a staged surgical approach (performed by a team of multidisciplinary specialists).
  • Positional molding
    • No surgery is indicated for posterior plagiocephaly secondary to positional molding.
    • The vast majority of infants improve with repositioning maneuvers and physical therapy for torticollis.
    • Some clinicians elect to use molding helmets for severe cases.

Consultations

  • A pediatric neurologist should evaluate the cause of the craniosynostosis and differentiate secondary from primary craniosynostosis. The head circumference should be plotted carefully and monitored from birth onward.
  • A geneticist/dysmorphologist should evaluate for associated syndromes.
  • A plastic surgeon should evaluate the infant when synostosis is associated with facial deformities.
  • A neurosurgeon should evaluate a patient with primary craniosynostosis or elevated intracranial pressure.
  • An endocrinologist should evaluate the patient when appropriate.

Activity

Following surgery, patients may require some restriction in activity under the direction of the treating neurosurgeon to avoid head injury.

More on Craniosynostosis

Overview: Craniosynostosis
Differential Diagnoses & Workup: Craniosynostosis
Treatment & Medication: Craniosynostosis
Follow-up: Craniosynostosis
Multimedia: Craniosynostosis
References
Further Reading
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References

  1. Jabs EW. Toward understanding the pathogenesis of craniosynostosis through clinical and molecular correlates. Clin Genet. Feb 1998;53(2):79-86. [Medline].

  2. Higginbottom MC, Jones KL, James HE. Intrauterine constraint and craniosynostosis. Neurosurgery. Jan 1980;6(1):39-44. [Medline].

  3. Sheth RD, Schaefer GB, Keller GM, et al. Size of the corpus callosum in cerebral palsy. J Neuroimaging. Jul 1996;6(3):180-3. [Medline].

  4. Schaefer GB, Sheth RD, Bodensteiner JB. Cerebral dysgenesis. An overview. Neurol Clin. Nov 1994;12(4):773-88. [Medline].

  5. Losee JE, Corde Mason A. Deformational plagiocephaly: diagnosis, prevention, and treatment. Clin Plast Surg. Jan 2005;32(1):53-64. [Medline].

  6. Robin NH. Molecular genetic advances in understanding craniosynostosis. Plast Reconstr Surg. Mar 1999;103(3):1060-70. [Medline].

  7. Anderson PJ, Netherway DJ, Abbott A, David DJ. Intracranial Volume Measurement of Metopic Craniosynostosis. J Craniofac Surg. 11 2004;15(6):1014-1016. [Medline].

  8. Cunningham ML, Heike CL. Evaluation of the infant with an abnormal skull shape. Curr Opin Pediatr. Dec 2007;19(6):645-51. [Medline].

  9. Dundulis JA, Becker DB, Govier DP, et al. Coronal ring involvement in patients treated for unilateral coronal craniosynostosis. Plast Reconstr Surg. Dec 2004;114(7):1695-703. [Medline].

  10. Elmslie FV, Reardon W. Craniofacial developmental abnormalities. Curr Opin Neurol. Apr 1998;11(2):103-8. [Medline].

  11. Fernbach SK. Craniosynostosis 1998: concepts and controversies. Pediatr Radiol. Sep 1998;28(9):722-8. [Medline].

  12. Liptak GS, Serletti JM. Pediatric approach to craniosynostosis. Pediatr Rev. Oct 1998;19(10):352; quiz 359. [Medline].

  13. Sheth RD, Mullett MD, Bodensteiner JB, Hobbs GR. Longitudinal head growth in developmentally normal preterm infants. Arch Pediatr Adolesc Med. Dec 1995;149(12):1358-61. [Medline].

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Further Reading

See Medscape's Pediatrics/Neonatal Care Nursing Resource Center.

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Keywords

craniosynostosis, abnormal head shape, ossification, primary craniosynostosis, failure of brain growth, secondary craniosynostosis, simple craniosynostosis, complex craniosynostosis, compound craniosynostosis, syndromic craniosynostosis, anterior plagiocephaly, Apert syndrome, brachycephaly, Carpenter syndrome, Chotzen syndrome, Crouzon syndrome, kleeblattschãdel, cloverleaf skull, oxycephaly, Pfeiffer syndrome, plagiocephaly, scaphocephaly, trigonocephaly

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Contributor Information and Disclosures

Author

Raj D Sheth, MD, Professor of Neurology, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic
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, and Child Neurology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Philipp R Aldana, MD, FAAP, Assistant Professor of Neurosurgery and Pediatrics, University of Florida College of Medicine - Jacksonville; Director of Clinical Services, Pediatric Neurosurgery Center, University of Florida Health Sciences Center
Philipp R Aldana, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association of Neurological Surgeons, American Society of Pediatric Neurosurgeons, Children's Oncology Group, Congress of Neurological Surgeons, and International Society of Pediatric Neurosurgery
Disclosure: Nothing to disclose.

Bermans J Iskandar, MD, Director, Associate Professor, Departments of Neurological Surgery, Pediatrics, Children's Hospital, University of Wisconsin Hospital and Clinics
Bermans J Iskandar, MD is a member of the following medical societies: Alpha Omega Alpha and American College of Surgeons
Disclosure: Nothing to disclose.

Medical Editor

James J Riviello Jr, MD, George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital
James J Riviello Jr, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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