Bilateral endoscopic craniectomies in the treatment of an infant with Apert syndrome

Case report

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Patients with Apert syndrome commonly present with ocular proptosis due to bilateral coronal craniosynostosis and midfacial hypoplasia. Severe proptosis can cause visual compromise and damage, which is most commonly treated with bilateral orbital frontal advancement. The authors present the case of a patient who was treated at 8 weeks of age with endoscope-assisted bilateral coronal craniectomies followed by treatment with a custom-made postoperative cranial orthosis. The patient underwent the procedure without any complications. Over the ensuing months, the patient's proptosis corrected, the forehead and orbital rims advanced without the need for an orbital frontal advancement and craniotomies. This approach may provide an alternative treatment modality for these patients.

Article Information

Address correspondence to: David F. Jimenez, M.D., Department of Neurosurgery, The University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, MC 7843, San Antonio, Texas 78229. email:

Please include this information when citing this paper: published online August 24, 2012; DOI: 10.3171/2012.7.PEDS11281.

© AANS, except where prohibited by US copyright law.



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    Top view of the patient before surgery, at 2 months of age, showing marked brachycephaly, shallow anterior cranial fossa, and significant orbital proptosis.

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    Lateral view of the patient showing brachycephaly, frontal bone recession, and proptosis.

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    Intraoperative photograph showing the location of the incisions, which measured approximately 1.5 cm and were located midway between the anterior fontanel and the pterion on each side.

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    Lateral 3D CT scan obtained the day after surgery. The width of the osteotomy was 6 mm and extended from the anterior fontanel down to the squamosal bone behind the coronal suture and a linear osteotomy down to squamosal suture. Although not visualized on the 3D reconstruction, the linear osteotomy could be seen on the axial slices and was visually documented at the time of surgery. The arrow indicates the osteotomy site.

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    Submental-vertex view of the patient with the cranial orthosis in place. Contact is made bitemporally while space is made in front of the frontal bones and orbits to allow for forward movement and internal advancement.

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    Top view of the patient obtained 4 months after surgery, demonstrating advancement of the forehead and significant correction of the proptosis that was present prior to surgery.

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    Left: Top view obtained at 6 months, showing persistent forehead rounding and an increased cephalic index. Right: Lateral view obtained at 6 months, showing advancement of the forehead and much less proptosis.

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    Left: Top view obtained at 9 months after surgery, showing maintained correction of forehead symmetry. Right: Lateral view obtained at 9 months postoperatively, showing an advanced forehead and normally closing eyelids. The supraorbital rims have advanced in comparison with the preoperative photograph (Fig. 2).

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    Artist's rendition. A: Preoperative top view showing brachycephaly and proptosis. B: Bilateral coronal osteotomies extending from the anterior fontanel to the squamosal sutures are done with endoscopic assistance. C: Postoperatively, the cranial molding orthosis places bilateral compression (horizontal arrows) while allowing for brain and cranial expansion in the anteroposterior direction (diagonal arrows). D: Proptosis correction, internal frontal bone advancement, and increase in cephalic index are achieved after orthotic therapy 10 months later. Printed with permission from David F. Jimenez, M.D.

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    Three-dimensional sagittal (left) and axial (right) CT scans obtained at 9 months postoperatively, demonstrating that the osteotomies increased in width by 1.8 cm and that the volume of the anterior cranial vault has increased. Additionally, there is evidence of reossification and progressive closure of the osteotomies.


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