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Neuroradiology Case of the Week

Case 174

Veniamin Barshay, MD and Per-Lennart Westesson, MD, PhD, DDS

Clinical Presentation: Patient is a 2-month-old male with skull deformity.  There is clinical suspicion for craniosynostosis.

Radiological Findings: CT scan with 3D reconstruction shows anterior-posterior elongation and transverse narrowing of the skull.  Sagittal suture is fused with bony bridging.  Metopic, coronal, and lambdoid sutures are open.

Diagnosis: Craniosynostosis and scaphocephaly

Discussion: Craniosynostosis is a defect of the skull resulting from a premature (before the completion of the brain growth) closure of one or multiple cranial sutures. Natural growth of the brain is restricted. Instead, compensatory growth in the areas of open sutures ensues with consequent skull deformity. Single suture is most commonly affected.
      Only 2-8% of the cases are primary craniosynostosis. Secondary causes include rickets, hyperthyroidism, hypercalcemia, bone marrow hyperplasia, or inadequate brain growth (microcephaly and shunted hydrocephalus). Genetic mutation of fibroblastic growth factor receptors 2 and 3 causing syndromic craniosynostosis were identified. Multiple suture involvement suggests secondary craniosynostosis. Sagittal suture is involved in 50-58% of the cases. Other possibilities include coronal suture (20-29%), metopic (4-10%), and lambdoid (2-4%). Various skull deformities can result.

• Scaphocephaly – AP elongation and biparietal narrowing of the skull from the fusion of the sagittal suture. 

• Anterior plagiocephaly – fusion of one coronal suture.

• Brachycephaly – fusion of both coronal sutures resulting in increased biparietal and shortened AP diameter.

• Posterior plagiocephaly – can result from premature fusion of lambdoid suture or posterior molding (vast majority).

• Trigonocephaly – results from the premature closure of the metopic suture resulting in triangular pointed skull shape.

Treatment: Surgery is indicated for increased intracranial pressure and for cosmetic reasons.  Secondary craniosynostosis requires the treatment of the underlying cause.

References:

1. Sheth RD, Bermans JI: Craniosynostosis. http://www.emedicine.com/neuro/topic80.htm.
2. Barkovich AJ: Pediatric Neuroimaging, 4th ed.; Lippincott, Williams & Wilkins, 2005; pp 410-420.