Pediatric Craniosynostosis Differential Diagnoses

  • Author: Raj D Sheth, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: May 10, 2012
 
 

Differential Diagnoses

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Raj D Sheth, MD  Professor, 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)

Nathan Ranalli, MD  Assistant Professor of Neurosurgery and Pediatrics, Departments of Neurosurgery and Pediatrics, University of Florida College of Medicine – Jacksonville

Nathan Ranalli, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Student Association/Foundation, and Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Philipp Aldana, MD, FAAP  Assistant Professor of Neurosurgery and Pediatrics and Chief, Division of Pediatric Neurosurgery, Departments of Neurosurgery and Pediatrics, University of Florida - Jacksonville

Philipp Aldana, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Neurosurgeons, and International Society of Pediatric Neurosurgery

Disclosure: Nothing to disclose.

Bermans J Iskandar, MD  Professor, Director of Pediatric Neurosurgery, Departments of Neurological Surgery and 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.

Saswata Roy, MD  Pediatric Otolaryngologist, Director, Facial Anomalies and Cranial Base Program, Division of Pediatric Otolaryngology, Nemours Children's Clinic

Saswata Roy, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, and American Cleft Palate/Craniofacial Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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: Up To Date Royalty Section Editor

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

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: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Ian M Heger, MD, to the development and writing of this article.

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, Hobbs GR, Ortiz O, Bodensteiner JB. 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, Mason AC. Deformational plagiocephaly: diagnosis, prevention, and treatment. Clin Plast Surg. Jan 2005;32(1):53-64, viii. [Medline].

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

  7. Liu B, Yu HM, Hsu W. Craniosynostosis caused by Axin2 deficiency is mediated through distinct functions of beta-catenin in proliferation and differentiation. Dev Biol. Jan 1 2007;301(1):298-308. [Medline]. [Full Text].

  8. Scott JR, Isom CN, Gruss JS, et al. Symptom outcomes following cranial vault expansion for craniosynostosis in children older than 2 years. Plast Reconstr Surg. Jan 2009;123(1):289-97; discussion 298-9. [Medline].

  9. Shah MN, Kane AA, Peterson JD, Woo AS, Naidoo SD, Smyth MD. Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children's Hospital experience. J Neurosurg Pediatr. 2011/08;8(2):165-70.

  10. Jimenez DF, Barone CM. Multiple-suture nonsyndromic craniosynostosis: early and effective management using endoscopic techniques. J Neurosurg Pediatr. Mar 2010;5(3):223-31. [Medline].

  11. Keshavarzi S, Hayden MG, Ben-Haim S, Meltzer HS, Cohen SR, Levy ML. Variations of endoscopic and open repair of metopic craniosynostosis. J Craniofac Surg. Sep 2009;20(5):1439-44. [Medline].

  12. Anderson PJ, Netherway DJ, Abbott A, David DJ. Intracranial volume measurement of metopic craniosynostosis. J Craniofac Surg. Nov 2004;15(6):1014-6; discussion 1017-8. [Medline].

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

  14. Dadure C, Sauter M, Bringuier S, Bigorre M, Raux O, Rochette A. Intraoperative tranexamic acid reduces blood transfusion in children undergoing craniosynostosis surgery: a randomized double-blind study. Anesthesiology. Apr 2011;114(4):856-61. [Medline].

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

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

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

  18. Keshavarzi S, Hayden MG, Ben-Haim S, Meltzer HS, Cohen SR, Levy ML. Variations of endoscopic and open repair of metopic craniosynostosis. J Craniofac Surg. Sep 2009;20(5):1439-44. [Medline].

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

  20. Miraoui H, Ringe J, Haupl T, Marie PJ. Increased EFG- and PDGFalpha-receptor signaling by mutant FGF-receptor 2 contributes to osteoblast dysfunction in Apert craniosynostosis. Hum Mol Genet. May 1 2010;19(9):1678-89. [Medline].

  21. 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].

  22. Stelnicki E, Heger I, Brooks CJ, et al. Endoscopic release of unicoronal craniosynostosis. J Craniofac Surg. Jan 2009;20(1):93-7. [Medline].

 
Previous
Next
 
Diagram of a neonate's skull demonstrating the location of the sutures.
Frontal view showing a fused and ridged metopic suture on 3-dimensional CT.
CT image demonstrating features of secondary craniosynostosis with cerebral atrophy. Cerebral atrophy is not present in primary craniosynostosis.
Positional molding. Note the anterior displacement of the right occiput and of the right frontal region on the same side, which differentiate positional molding from posterior plagiocephaly craniosynostosis.
Posterior view of 3-dimensional cranial CT demonstrating early fusion of the lambdoid suture.
Isolated fusion of the metopic suture. Note that the remaining sutures are open.
Trigonocephaly. Note the triangular shape of the head.
Infant with primary craniosynostosis. The specific deformity of the skull is Kleeblattschadel or cloverleaf skull.
Skull deformities associated with single suture synostosis.
Positional plagiocephaly. Note anterior position of the ear on the side with occipital flattening.
Sagittal synostosis and the associated scaphocephaly seen on skull radiograph and 3-dimensional craniofacial CT scan.
Sagittal synostosis before and after cranial vault surgery and the associate improvement of scaphocephaly.
Unilateral coronal deformity with retrusion of the orbit and harlequin eye deformity. Note the ipsilateral deviation of the nasal radix and the contralateral deviation of the nasal tip.
Fusion of coronal sutures bilaterally. Note the increase transverse dimension and retrusion of the orbital rim.
Intraoperative view of bilateral coronal synostosis. Note the decreased anterior-posterior dimension, increased transverse width and retruded orbital rim.
Pre- and postoperative photos of metopic synostosis. Note the prominent forehead keel corrected after surgical repair.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.