Un cas historique d’un nævus fronto-orbito-calvarial

We report the case of a 22-year-old female patient, with no particular previous history, who had nevus progressively increasing in volume since birth. Physical examination revealed a huge bluish-grey mass in the left fronto-temporal-parietal region, extending beyond the midline, infiltrating the palpebral region with orbital extension and blindness in the left eye (A). Another isolated nevus was found in the occipital region, which measured 5cm along its longer axis. Craniofacial CT scan with IV contrast showed nevus extension into the left intraorbital region and infiltration of the scalp opposite the nevus, without involvement of the brain parenchyma. A 3-stage surgical procedure was performed, in the first stage two expanders were placed in the healthy occipital and contralateral parietal region. In the second stage, the entire parieto-temporo-occipital component was removed, the frontal component was reduced and the scalp, which had gained enough elasticity thanks to the expanders, was reconstructed using transposition flaps (B). The third stage consisted of resection of the remaining upper fronto-palpebral component and part of the orbital component with repair by total skin graft to cover any loss of substance (C). The result obtained was extraordinary and the patient was so happy to be able to live a normal life.