PMC3791821_01
Three-month-old female patient with Saethre-Chotzen syndrome and bicoronal synostosis resulting in significant turribrachycephaly (a). The sagittal, lambdoid, and squamosal sutures were patent with a large fontanelle, characteristic of Saethre-Chotzen syndrome. Initial computed tomographic volume rendering shows bicoronal craniosynostosis (b).
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F1
Our patient presented at 3 months of age with bilateral coronal synostosis and profound turribrachycephaly (Fig 1).
Bilateral craniectomy was performed and distraction plates connected to external arms were implanted along the osteotomy. At home, the family turned the arms 1 mm per day totaling 34 mm over 1 month.
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F2
Internal distractors with 4-cm distraction arms were placed along the osteotomy sites of the parietal bones; the intended vector was posterior-inferior from the skull apex (Fig 2).
Clinical (a) and radiographic appearance (b) after 3 months of bony consolidation. Distraction hardware was subsequently removed.
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F3
She was distracted 34 mm over 4 weeks, with correction of her occipital head shape and successful osteogenesis at the distraction site (Fig 3).
Clinical (a) and radiographic appearance (b-d) 1 year after posterior vault distraction reveals pan-suture synostosis. Routine computed tomography demonstrated interval closure of the sagittal and lambdoid sutures.
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F4
One year postoperatively, a computed tomographic scan revealed fusion of all her cranial sutures:a delayed pan-suture synostosis (Fig 4).