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Answer to Case of the Week: Feb 10-Feb 17, 2006
5 month old

 


Diagnosis: Trigonocephaly
  • Trigonocephaly results from the premature fusion of the metopic suture. The characteristic finding is the "keel-shaped" forehead with a midline frontal ridge.
  • Craniosynostosis is the premature closure (bony fusion) of one or more cranial sutures.
  • Causes include:
    - Primary - due to faulty dural development
    - Local injury or ischemia
    - Secondary due to:
    Metabolic - Rickets, hypercalcemia, hyperthyroidism, hypervitaminosis D
    Hematologic - Sickle cell, Thalassemia
    Decreased intracranial pressure (shunted hydrocephalus or deficient brain growth)
    - Craniofacial syndromes
    Apert syndrome
    Carpenter syndrome
    Crouzon syndrome
    - Bone dysplasias
    Achondroplasia
    Hurler disease
  • M>F 3:1
  • - Sagittal suture - more than half of cases
  • - Bony bridging may be partial or complete
  • - Narrowing of suture
  • - Parasutural sclerosis
  • Head shape depends upon which suture(s) are fused:
    - Sagittal suture: dolichocephaly or scaphocephaly
    - Coronal (2nd most frequent)
    Unilateral: plagiocephaly, harlequin eye
    Bilateral: brachycephaly
    - Metopic synostosis (between the frontal bones): trigonocephaly
    Associated with holoprosencephaly
    - Lambdoid synostosis (less common)
    - All sutures closed early (universal craniosynostosis): cloverleaf skull
    1/3 Thanatophoric dwarfism
  • Bilateral Coronal craniosynostosis
    - Crouzon syndrome - craniofacial dysostosis

    - Apert syndrome - acrocephalosyndactyly, developmentally delayed.
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Disclaimer: This information is intended solely for resident review of presented cases which may or may not be pathologically proven. Information is derived from a number of published sources of varying reliability and does not represent original research from the institution. It is not intended to be comprehensive and should therefore not substitute for careful review of the literature.