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Craniosynostosis: Part One

Yvonne Gutierrez MD and Parul Bhatia, MD
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Parul and Bonnie discuss the physical exam findings associated with single suture craniosynostosis (SSCS), posterior positional plagiocephaly and posterior plagiocephaly secondary to lambdoid CS. They also talk about the two most commonly used surgical techniques used to correct SSCS and what is known about the neuro-developmental outcomes associated with SSCS.

  • Craniosynostosis is defined as the premature closure of one or more of the cranial sutures leading to restricted cranial growth perpendicular to the closed suture site.  There is compensatory growth of the cranium parallel to the affected suture.

  • There are both non-syndromic and syndromic forms of craniosynostosis.  80% of single suture craniosynostosis are non-syndromic.

  • The majority of cases are single suture craniosynostosis and physical exam reveals a palpable ridge over than affected suture(s)

    • Scaphocephaly: the most common form in which there is an increased AP skull diameter

    • Unilateral coronal craniosynostosis: second most common form; skull shape is an “anterior plagiocephaly” in which there is a flattening of the forehead, the cheek and the ear are pulled forward on the affected side. The child has a “surprised look” to the eye on the affected side.

    • Brachycephaly: does affect two sutures (bicoronal craniosynostosis) shows a wider head shape in the transverse diameter.

    • Turricephaly: most severe form in which all the sutures prematurally close

    • Trigonocephaly: named for the “triangle” shape of the skull when there is premature closure of the metopic suture

    • Lambdoid craniosynostosis:  the skull appears trapezoid in appearance and you may see a tilt of the cranial base as the part of the affected suture is tilted upwards compared to the other side; there is ipsilateral flattening (think: everything is pulled towards the affected suture)

      • This is in stark contrast to positional plagiocephaly in which you have unilateral flattening from laying on your head all the time and the ear is shifted and pushed forward - not drawn into the suture.  Therefore, there is ipsilateral bulging of the forehead

 

  • The skull should be examined in all positions, including the bird’s eye view.  The skull should be palpated by running your fingers along the suture lines and fontanelles.  This should be done in the newborn nursery and continuing through early infancy.

    • Ridging of the sutures could indicate craniosynostosis; this should be distinguished from overriding sutures with are more mobile.

  • Asking the family certain historical questions may elicit more concern for craniosynostosis.

    • These include:

      • “Was the head abnormally shaped at birth or shortly after birth?”

      • “Is the head shape getting better?”

      • “Was the birth multiple gestations?” “Was there oligohydramnios?”

        • Both have higher rates of craniosynostosis.  

  • Eyes should be evaluated for hypo- or hypertelorism and ptosis.  The ears should be evaluated for placement, size, set, rotation and the landmarks of the pinna.  Evaluating patient from the side may reveal other features associated with syndromic craniosynostosis.  The hands and feet may reveal syndactyly, polydactyly or broad digits associated with syndromic craniosynostosis.  

  • Typically, craniosynostosis is a clinical diagnosis made by the head shape and sometimes facial asymmetry.  No imaging is required in the pediatrician’s office to confirm the diagnosis.

  • X-rays are a good preliminary test but may miss lambdoid craniosynostosis.   

    • CT of the head without contrast with reconstruction is a better way of delineating the anatomy and can help with surgical planning; but given concern for excess radiation, these patients should be referred to a craniofacial clinic prior to ordering a CT.

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Home is Where the Placenta is! Full episode audio for MD edition 199:01 min - 93 MB - M4AHippo Peds RAP - June 2019 Written Summary 399 KB - PDF

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