How is pediatric craniosynostosis treated?

Answer

In the past 30 years, a better understanding of the pathophysiology and management of craniosynostosis has developed. Currently, surgery is usually for cranial deformity correction for infants with fusion of 1-2 sutures that results 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 appearance of the skull abnormality.

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Media Gallery

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.

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Author

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: AAN reviewer, ACNS Ed board, Infantile spasms consultant for: AAN; ACNS; Mackilrodt.

Coauthor(s)

Nathan J Ranalli, MD Assistant Professor of Neurosurgery and Pediatrics, Associate Program Director of Pediatric Maxillofacial and Craniofacial Surgery Fellowship, University of Florida College of Medicine-Jacksonville; Consulting Surgeon, Division of Pediatric Neurological Surgery, Lucy Gooding Pediatric Neurosurgery Center, Wolfson Children’s Hospital

Nathan J Ranalli, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Student Association/Foundation, 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 College of Medicine at Jacksonville

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

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, 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, American Cleft Palate-Craniofacial Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Additional Contributors

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: Partner received royalty from Up To Date for section editor.

Acknowledgements

Ian M Heger, MD Assistant Professor, Department of Neurosurgery, University of Florida College of Medicine; Assistant Director of Clinical Services, Pediatric Neurosurgery Center, Wolfson Children's Hospital

Ian M Heger, MD, is a member of the following medical societies: American Academy of Pediatrics, American Association of Neurological Surgeons, American College of Surgeons, American Epilepsy Society, American Society of Pediatric Neurosurgeons, Children's Oncology Group, and Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

How is pediatric craniosynostosis treated?

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