History and Physical Examination
The abnormal head shape is often noticeable at birth and leads to a referral to a specialist in cranial deformity. Typically, a pediatric neurosurgeon or a craniofacial plastic surgeon is able to determine the diagnosis on physical examination before a confirmatory imaging study is performed.
Many infants referred by pediatricians to neurosurgeons and craniofacial plastic surgeons for abnormalities in head shapes do not have craniosynostosis and instead have positional (or deformational) plagiocephaly. Positional plagiocephaly most often masquerades as either lambdoid or unilateral coronal craniosynostosis.
The most common type of positional plagiocephaly is characterized by unilateral flattening in the occipital region (mimicking lambdoid craniosynostosis). The severity of the flattening can vary but often leads to a parallelogram shape of the skull as viewed from above, with anterior displacement of the ipsilateral ear, forehead, and cheek. This forehead asymmetry mimics unilateral coronal craniosynostosis.
Careful clinical evaluation usually suffices to distinguish craniosynostosis from positional plagiocephaly. Lambdoid craniosynostosis causes a parallelogram-shaped skull as viewed from behind, in which the ear ipsilateral to the flattening is inferiorly deflected and the contralateral parietal bone experiences compensatory superior growth. Lambdoid synostosis consistently presents with a mastoid bulge or bossing on the side of the fusion which is not present in children with deformational plagiocephaly.
Although both positional plagiocephaly and unilateral coronal craniosynostosis are associated with forehead asymmetry, the nature of the asymmetry is quite different. Whereas in positional plagiocephaly the forehead and orbital rim contralateral to the flattened occiput are normal, the forehead and orbital rim in unilateral coronal craniosynostosis are recessed and the contralateral frontal bone is overgrown.
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Normal anatomic suture configuration.
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Metopic craniosynostosis (trigonocephaly).
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Sagittal craniosynostosis (scaphocephaly).
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Unilateral coronal craniosynostosis (anterior plagiocephaly).
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Lambdoid craniosynostosis (posterior plagiocephaly).
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3D reconstruction of child with sagittal craniosynostosis.
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3D reconstruction of child with right unilateral coronal craniosynostosis.
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3D reconstruction of child with bilateral coronal (bicoronal) craniosynostosis.
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3D reconstruction of child with metopic craniosynostosis.
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3D reconstruction of child with left lambdoid craniosynostosis. 3D-reconstructed CT venography of same child with left lambdoid craniosynostosis demonstrating dominant right transverse sinus.
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Bilateral coronal craniosynostosis repair. 3D rendering of standard CT performed on postoperative day 1 after consolidation and then months later. Note lengthening of skull, as well as decrease in vertical height.
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Bilateral coronal craniosynostosis repair. 3D rendering of standard CT performed on postoperative day 1 after subsequent fronto-orbital advancement.
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Lambdoid craniosynostosis repair. 3D rendering of standard CT performed on postoperative day 1 following reconstruction. Bilateral parietal craniotomies were performed, leaving sagittal suture in place. Bioccipital craniotomy was accomplished, and overgrown mastoid bone was removed and then elevated with bone grafts from craniotomies. Bone from contraleral parietal bone was used for occipital reconstruction. Ipsilateral partietal bone was placed contralaterally, and bone from bioccipital craniotomy was used to reconstruct ipsilateral parietal bone.
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Preoperative view of patient with metopic craniosynostosis (supine position). Note bifrontal narrowing and posterior parietal widening giving triangular shape (trigonocephaly) to skull on top-down view.
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Preoperative CT of patient with metopic craniosynostosis. Note bifrontal narrowing and posterior parietal widening giving triangular shape (trigonocephaly) to skull on top-down view. Note fused (absent) metopic suture anteriorly at midline with other sutures patent (visible).
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Intraoperative view of released fronto-orbital bandeau (top-down) prior to remodeling. Note triangular shape with flattening laterally.
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Intraoperative view of metopic craniosynostosis skeletal deformity after scalp flap reflection.
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Completion of repair for metopic craniosynostosis with scalp closed.
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Preoperative view of sagittal craniosynostosis with bicoronal incision marked. Prone position. Note biparietal narrowing and occipital narrowing.
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Intraoperative view of sagittal craniosynostosis after scalp flap reflection. Note Raney clips on skin edges. Markings designate sites of craniotomy with exception of central marking, which identifies fused sagittal suture at midline. Central segment of bone including suture will be removed. Prone position.
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Intraoperative view of sagittal craniosynostosis after removal of bone. Parietal bones on each side have been divided into segments and widened. Occiput has been flattened, as demonstrated by surgeon's finger. Prone position.
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Sagittal craniosynostosis after repair with scalp closed. Prone position.
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Preoperative top-down view of patient with unilateral (right) coronal craniosynostosis. Note flattened right forehead with elevated orbit and contralateral left side prominence (bossing) of forehead.
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Preoperative top-down CT view of same patient with unilateral (right) coronal craniosynostosis. Note flattened right forehead with elevated orbit and contralateral left side prominence (bossing) of forehead.
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Intraoperative view of same patient with unilateral (right) coronal craniosynostosis, looking directly down onto forehead. Top of head is at bottom of picture. Two sides of forehead were exchanged, placing prominent left side on right and flattened right side on left after contouring. Right side is overcorrected in position as compared with left.
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Intraoperative top-down view of same patient with unilateral (right) coronal craniosynostosis at completion of repair. Right side is overcorrected in position as compared with left at orbital rim (upper right portion of image).
Tables
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Approach Considerations
- General Principles of Surgical Therapy
- Sagittal Craniosynostosis Repair
- Unilateral Coronal Craniosynostosis Repair
- Bilateral Coronal Craniosynostosis Repair
- Metopic Craniosynostosis Repair
- Lambdoid Craniosynostosis Repair
- Postoperative Care
- Complications
- Long-Term Monitoring
- Show All
- Media Gallery
- References
