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Congenital sphenoid wing dysplasia is one of the major diagnostic criteria for neurofibromatosis type 1, and is often considered pathognomonic for the disease. Between 5% and 12% of neurofibromatosis type 1 cases have evidence of sphenoid wing dysplasia. Sequelae of this deficiency include slow expansion of the middle temporal fossa and progressive herniation of the temporal lobe into the orbital cavity, resulting in pulsatile exophthalmos. Herein, we report a patient with greater sphenoid wing agenesis and middle t