AI Article Synopsis
- - Sphenoid wing dysplasia is commonly seen in children with neurofibromatosis type 1 (NF1), which can lead to issues like proptosis and vision loss due to spheno-orbital encephalocele.
- - A 6-year-old boy experienced painless progressive vision loss and pulsatile proptosis, prompting imaging that showed an encephalocele extending into the orbit caused by a defect in the dysplastic orbital wall.
- - A custom 3D-printed implant was created and placed to address the defect, successfully preventing further brain tissue herniation, reducing proptosis, and improving the child's vision.
Article Abstract
Sphenoid wing dysplasia is a characteristic finding in children with neurofibromatosis type 1 (NF1). Some of these children develop proptosis and vision loss secondary to the spheno-orbital encephalocele. A 6-year-old boy presented to us with complaints of painless progressive uni-ocular vision loss and progressive pulsatile proptosis. Imaging revealed spheno-orbital encephalocele into the orbit through the dysplastic posterior orbital wall. 3D printed customized implant was designed and placed to fit the defect. This prevented further herniation of the temporal lobe into the orbit, leading to reduction of proptosis and improvement in vision of the child.
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